CREOG Topics Review

The following topical reviews were prepared by the residents and attendings at the MAHEC Residency Program and are distributed under a Creative Commons Attribution Share-Alike 2.5 License. Most of these topics are also excellent for medical students.


Liability Reporting

Allison Eaton, Clay Smallwood

National Practitioner Data Bank

  • Contains information on adverse licensure actions, clinical privileges actions, and professional society membership actions taken against physicians and dentists (including residents)
  • Reports payments made on behalf of a physician in a settlement and even refunds resulting from a written demand for payment; does not include payments made by individual MDs on their own behalf
  • No report is filed if the MD is dismissed from the suit before settlement or judgement
  • Hospitals must check with the NPDB when hiring and q2 years; NOT required to check residents or fellows unless they moonlight or are hired after training
  • NO PUBLIC ACCESS
  • If an MD agrees to settle a suit, they should review the report (which has a 2000 word narrative) to make sure it states the settlement was for financial reasons, not medical merit (if that is true)

Healthcare Integrity and Protection Data Bank

  • Created to deter fraud and abuse in health insurance and health care delivery; augments traditional forms of review; should alert and assist federal and state agencies in conducting independant investigations
  • NO PUBLIC ACCESS
References

Physician reporting requirements and profiling. In: ACOG Committee on Professional Liability. Professional liability and risk management. Washington (DC): American College of Obstetricians and Gynecologists, 2002:101-8

Hubler JR, Weaver J. Health insurance and professional liability insurance. In: Sanbar SS, Firestone MH, Buckner F, Gibofsky A, LeBlang TR, Snyder JW, et al, eds. Legal medicine. 6th ed. St. Louis (MO):Mosby, 2004:179-86


Bartholin Gland Cyst

Wood, Sigmon

Bartholin Gland Anatomy

Mucus-secreting glands in the vulvar vestibule.
Orifices at five/seven o'clock position just outside hymenal ring.

Cyst
Abscess
Usually small (1-3cm)
May exceed 6cm diameter
Non-tender
Typically extremely painful
Require treatment only if symptomatic unless patient over 40 (gland biopsy/ excision recommended to r/o carcinoma)
Require I & D and broad-spectrum antibiotics
Non-infectious/ caused by obstruction of duct orifice and subsequent dilation
N. gonorrhoeae is classic organism, but most abscesses with mixed flora.

Treatment (when needed)

I&D with Word Catheter Placement
  • Make incision just outside hymenal ring in anterior/posterior direction to prevent vulvar scarring
  • Typically an abscess will have multiple septations which need to be broken down
  • Word catheter – Balloon-tipped device placed into cavity and inflated to promote epithelialization (2-4 weeks). End tucked into vagina to minimized discomfort. Can be done in an outpatient setting, has replaced marsupialization as first-line treatment.

Marsupialization
  • Excision of 1-2 cm oval portion of the roof of cyst behind hymenal ring. Duct wall then everted and sutured to mucosa. Often used after failure of placement of Word catheter.
  • 10-15% of cysts recur.
  • Rarely needed as Word catheter is less traumatic with same results.

Excision of gland and duct
  • Definitive procedure for both cysts and abscesses, usually considered after other methods have failed because must be done in OR with risks including bleeding, hematoma, cellulitis, and dyspareunia.
References

Noller KL. Surgery for benign vulvar, vaginal, and urethal diseases. In: Gershenson DM, DeCherney AH, Curry SL, Brubaker L. Operative gynecology. 2nd ed. Philadelphia (PA): WB Saunders, 2002:223-36.

Horowitz IR, Buscema J, Majmudar B. Surgery for benign vulvar, vaginal, and urethral diseases. In: Rock JA, Jones HW III, eds. Te Linde's operative gynecology. 9th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2003:865-91.



Twinning

Summer Gilmer, MD, Carol Coulson, MD

Prevelance

-twin deliveries between 1980-1997 increased 52%, triplets and higher increased 404%
-frequency of monozygotic 1/250 worldwide, independent of race, heredity, age and parity
-dizygotic is variable because dependent on race, heredity, age, parity, and especially ART

Mechanism for MZ twinning

-may result from delay in timing of normal developmental events- For example, OCPs decrease tubal motility and ART may cause minor trauma to blastocyst thus increasing monozygotic twinning

-Dizygotic- maturation and fertilization of 2 ova by 2 sperm during a single ovulatory cycle
-Monozygotic- arises from single fertilized ovum that divides into 2 similar structures

-membrane status for monozygotic twinning depends on when division occurs:
-within 72 hours- 2 embryos, 2 amnions, 2 chorions ->diamniotic, dichorionic, monozygotic
may have 2 distinct placentas or single fused placenta, accounts for 20-30% of MZ twins
-4-8 days- inner cell mass is formed, chorion cells are differentiated, but amnion cells are not
2 embryos, 2 amnions, 1 chorion ->diamniotic, monochorionic, monozygotic, accounts for majority of MZ twins (70-80%)
-8-12 days- amnion already established- 2 embryos, 1 amnion, 1 chorion -> monoamnionitic, monochorionic,
monozygotic, accounts for 1% of MZ twins
-after 12 days- after embryonic disk formed, cleavage incomplete -> conjoined twins
      • the more layers shared, the higher the chance of complications including IUFD

Special Circumstances

chimerism- individual whose cells originated from more than 1 fertilized ovum
mosaicism- 2 or more cell lines of different chromosomal composition that arise from same zygote as the consequence of nondisjunction during meiotic division

superfetation- an interval as long or longer than an ovulatory cycle occurs between fertilizations- requires ovulation during the course of an established pregnancy-- is unproven in humans
superfecundation- fertilization of 2 ova within short period of time but not at same coitus, not necessarily by sperm from same male

Determining zygosity

ultrasound- presence of 2 separate placental sites, thick dividing membrane 2 mm or greater, twin peak sign ->dichorionicity, thus most likely (but not unequivocally) dizygotic
fetuses of opposite genders-> almost always dizygotic

Vascular Communications

-nearly 100% of monochorionic placentas have vascular anastomoses- artery to artery is most common

1- acardiac twin (twin reverse-arterial-perfusion)- TRAP sequence- is a complication of mono/mono twins- rare, 1/35,000
-normal, formed donor twin has features of heart failure, recepient twin is without a heart and missing other structures (upper body disrupted or missing)
-caused by artery to artery shunt, often accompanied by vein to vein shunt
-without tx, the donor twin dies in 50-75% of cases

2- Twin-Twin Transfusion syndrome- with mono/mono twins, blood is transfused from donor twin to recepient. Donor becomes
anemic and growth restricted while recepient becomes polycythemic and may develop circulatory overload->hydrops

-pathophysiology hypotheses:
-single deep arteriovenous villous anastomoses as a cause- it's hypothesized that multiple superficial vascular communications are more protective because they allow bidirectional and balanced blood flow. Occurs when a single cotyledon is fed by an artery from the donor and then drained by a vein from the recipient and this one way transfer of blood is not balanced by additional vein to artery, AA, or VV communications
-velamentous cord insertions contribute to unequal blood volumes since the membranous cord can be easily compressed, restricting blood flow to one fetus

-diagnosis: antenatal criteria
a- same sex
b- monochorionicity with placental vascular communications
c- weight difference >20%
d- oligohydramnios in smaller twin
e- polyhydramnios in larger twin
f- hemoglobin difference >5 g/dl

-fetal brain damage- the death of 1 twin causes acute hypotension in the other twin- transfusion from high pressure vessels of
living twin to low resistance vessels of dead twin leads to hemodynamic changes and ischemic brain damage in the remaining twin.

-therapy:
a- serial amnioreduction
b- fetoscopic laser occlusion of placental vessels
c- selective termination

Other Complications of Twinning

a- vasa previa- velamentous insertion with a transcervical position or more rarely, position in the dividing membrane of the second twin. velamentous insertion occurs when membranous vessels originating from the cord radiate toward the placental surface and are not protected by Wharton's jelly.
b- placenta previa
c- pre-eclampsia
d- preterm labor and delivery
e- hyperemesis gravidarum
f- postpartum hemorrhage
g- spontaneous abortion
h- malformations
i- low birth weight
j- IUFD

Antepartum Surveillance

a- ultrasound- serial sonography starting in 3rd trimester looking at growth, AFI
b- NST's
c- doppler- used to evaluate vascular resistance for growth restricted fetuses

References

Benirschke K. Multiple gestation. In:Creasy RK, Resnik R, Iams JD, eds. Maternal-fetal medicine: principles and practice. 5th ed. Philadelphia (PA): WB Saunders, 2004: 55-67.

Multifetal pregnancy. In:Cunningham FG, Gant NF, Leveno KJ, Gilstrap LC III, Hauth JC, Wenstrom KD. Williams obstetrics. 21st ed. New York (NY): McGraw- Hill Medical, 2001:765-810.



Dermatitis

Goodwin, Buys

Scaling Disorders

Atopic dermatitis (Eczema)
• pruritic, exudative or lichenified eruption on face, neck, upper trunk, wrists, and hands and in the antecubital and popliteal folds
• personal or family history of allergic manifestations, e.g., asthma, allergic rhinitis, atopic dermatitis
• Tendency to recur
Treatment
Mild soaps, pat skin dry and apply emollient quickly.
Appropriate corticosteroids according to severity of dermatitis applied 2-4 x daily
Lichen Simplex Chronicus (circumscribed neurodermatitis)
• chronic itching, self-perpetuating scratch-itch cycle
• lichenified lesions with exaggerated skin lines overlying a thickened, well-circumscribed scaly plaque
• predilection for nape of neck, wrists, external surfaces of forearms, lower legs, popliteal and antecubital areas
Treatment
High potent topical corticosteroids (clobetasol, halobetasol or diflorasone twice daily for several weeks)
Psoriasis
• silvery scales on bright red, well-demarcated plaques, usually on the knees, elbows and scalp
• nail findings including pitting and onycholysis (separation of the nail plate from the bed)
• mild itching (usually)
• may be associated with psoriatic arthritis
• histopathology is not often useful and can be confusing
Treatment
High potency topical corticosteroids twice daily for 2-3 weeks and then use in a pulse fashion 3-4x q week
For generalized disease, UVB light exposure 3x qweek
Pityriasis Rosea
• oval, fawn-colored, scale eruption following cleavage lines of trunk
• herald patch precedes eruption by 1-2 weeks
• occasional pruritus
• 50% more common in females, mostly in young adults in Spring or Fall
Treatment
Often requires none.
Usually acute self-limiting illness that disappears in 6 weeks
Seborrheic Dermatitis and Dandruff
• dry scales and underlying erythema
• scalp, central face, presternal, interscapular areas, umbilicus, and body folds
Treatment
Scalp – shampoos with tar, zinc pyrithione or selenium
Face – mild soaps, mild corticosteroid used intermittently and not near eyes
Eyelid margins – cleaning lid with undiluted Johnson and Johnson’s Baby Shampoo daily with a cotton swab

Fungal Infections of the Skin

Tinea Corporis or Tinea Circinata (body ringworm)
• ring shaped lesions with an advancing scaly border and central clearing or scaly patches with a distinct border
• on exposed skin surfaces or the trunk
• microscopic examination of scrapings or culture confirms the diagnosis
• Trichophyton rubrum is the most common pathogen
Treatment
Micronazole 2% cream, clotrimazole 1% solution, cream or lotion , terbinafine 1% cream
Continue for 1-2 weeks, usually steroid/antifungal not needed
Tinea cruris (jock itch)
• marked itching in intertriginous areas, usually sparing the scrotum
• peripherally spreading, sharply demarcated, centrally clearing erythematous lesions
• may have associated tinea infection of feet or toenails
• laboratory examination with microscope or culture confirms diagnosis
Treatment
Drying power (miconazole nitrate) dusted in area
Underwear should be loose fitting
Same as tinea corporis
Tinea Manuum and Tinea Pedis (Dermatophytosis, Tinea of Palms and Soles, “Athlete’s Foot”)
• most often presenting with asymptomatic scaling
• may progress to fissuring or maceration in toe web spaces
• itching, burning, and stinging of interdigital webs, palms, and soles seen occasionally: deep vesicles in inflammatory cases
• the fungus is shown in skin scrapings examined microscopically or by culture of scrapings
Treatment
Good personal hygiene
Open toed sandals, rubber sandals in community showers
Drying between the toes after showering is essential
Macerated stage
Aluminum subacetate solution soaks for 20 mins twice daily
Imidazoles or ciclopirox – broad spectrum antifungal creams
Dry and scaly stage
Agents used in tinea corporis
Tinea Versicolor (Pityriasis Versicolor)
• pale macules with fine scales that will not tan, or hyperpigmented macules
• velvety, tan, pink, whitish or brown macules that scale with scraping
• central upper trunk the most frequent site
• yeast and short hyphae observed on microscopic examination of scales
• Malassezia furfur usually causative organism
Treatment
Selenium sulfide lotion applied and left on for 5-15 mins daily x 7 days, then weekly for a month then monthly for maintenance

Miscellaneous

Photodermatitis
• painful or pruritic erythema, edema, or vesiculation on sun-exposed surfaces: the face, neck, hands, and “v” of the chest
• Inner upper eyelids spared, as is the area under the chin
• photosensitivity – a tendency for the individual to sunburn more easily than usual or a photoallergy which is a true immunologic reaction that often presents with popular or vesicular lesions
Treatment
Sunscreen with SPF 30-50
Soothing and cooling wet dressings
Impetigo
• contagious and autoinoculable infection of skin caused by staphylococci or streptococci
• vesiculopustular type with thick golden-crusted lesions (Staphylococcus aureus or Group A Beta hemolytic streptococcus
• bullous type associated with phage groupie S aureus
Treatment
For limited disease, mupirocin ointment 3x daily for 10 days may be effective
For larger areas, systemic antibiotics necessary
Dicloxacillin or cephalexin, 250 mg 4 x daily
Allergic Contact Dermatitis
• erythema and edema, with pruritis, often followed by vesicles and bullae in an area of contact with a suspected agent
• later, weeping , crusting, or secondary infection
• a history of previous reaction to suspected contactant
• patch test with agent positive
Treatment
Systemic corticosteroids usually needed since topicals don’t seem to penetrate weeping lesions. Prednisone 60, 40 or 20mg, one for 4-7 days without a taper is required. Medrol dosepak is inappropriate.


Diabetes Classification

Courtney Hodshon, MD, Hytham Imseis, MD

NIH/ADA Classification

  • Type 1 -- insulin deficiency secondary to destruction of pancreatic islet cells
  • Type 2 -- varying degrees of insulin resistance and deficiency
  • Gestational (type 3 in some sources)

Priscilla White Classification

Class
Age of Onset
Duration
Complications
A
any

no vascular disease
B
>20 or
<10
no vascular disease
C
10-19 or
10-19
no vascular disease
D
<10 or
>20
benign retinopathy only
F


"neFropathy" (proteinuria>500mg/24 hours)
H


arteriosclerotic Heart disease (MI, etc)
R


proliferative Retinopathy of vitreous hemorrhage
T


renal Transplanation

Gestational Diabetes Classification

Diagnosis
  • One Step Method
    • 2 hour 75gram GTT (common outside US but recognized as an alternative for particularly high risk populations)
      • 2 abnormal values (fasting 95, 1 hour 180, 2 hour 155)
  • Two Step Method
    • 1 hour 50 gram GTT
      • abnormal >135
      • >200 = diabetes and no 3hour gtt
    • 3 hour 100 gram GTT -- 2 abnormal values
      • two commonly used criteria
      • ACOG, ADA recommend Carpenter and Coustan (because most labs check plasma glucose levels which are 10-15% lower than whole blood glucose which was used in '64)


O'Sullivan and Mahan (1964)
Carpenter and Coustan (1982)
Fasting
105
95
1 hour
190
180
2 hour
165
155
3 hour
145
140
>
Classification
  • Usually split into pregestational (overt) or gestational
  • Most diabetics diagnosed during pregnancy are White's Class A
  • Further divided into diet controlled (A1) or medically controlled (A2)

References

Diabetes. In: Cunningham FG, Gant NF, Leveno KJ, Gilstrap LC III, Hauth JC, Wenstrom KD. Williams obstetrics. 21st ed. New York (NY): McGraw-Hill, 2001: 1359-81.

Moore TR. Diabetes in pregnancy. In: Creasy RK, Resnik R, Iams JD, eds. Maternal-fetal medicine: principles and practice. 5th ed. Philadelphia (PA): WB Saunders, 2004:1023-62 .


Urogenital Tract Infections

Liliana Belskus, MD, Allen Van Dyke, MD

Infections of the vulva

The three most common infections of the vulva are genital herpes, condyloma acuminatum and molluscum contagiosum.
Acute bacterial cystitis
· 50% of all women experience at least one UTI in the life time
· The most common cause of acute bacterial cystitis is ascending infection from introitus and distal urethra, most frequently involved with E. coli (80%) staph Saprophyticus 5-15%.
· Treatment of choice for uncomplicated UTI is three days of Bactrim or one of the quinolones such as Cipro. Resistant to Bactrim is about 20%
· Complicated antibiotic resistant UTIs are caused by E. Coli, Enterococcus faecalis, P Mirabilis, Staph Aureus, Klebsiella, Pseudomonas, Enterobacter and Serratia, currently the drug of choice is quinilones for at least 7-14 days.
Bartholin’s gland Infection
· Most common cause of gland enlargement is duct obstruction which is caused by non specific inflammation or trauma. 2% of adult women develop enlargement of both Bartholin’s glands
· Unilateral or bilateral infection in most cases is not caused by STDs
· Most women with Bartholin’s duct cysts are asymptomatic. Size may vary from 1 to 8cm.
· Abscess of a Bartholin’s gland develop over 1-4 days with vulvar pain dyspaerunia and pain during walking.
· Without treatment most abscesses rupture by the third or four day.
· The classic surgical treatment is to marsupialize the duct.
Pediculosis Pubis and Scabies
· Lice in the pubic hair are the most contagious of all sexually transmitted diseases, over 90% of sexual partners are infected following a single exposure.
· Scabies has no predilection for hairy areas, with hands, wrists, breast, vulva and buttocks most commonly involved. Characterized by the borrow as a twisted line on the skin surface, with small vesicle at the end.
· Treatment for both pediculosis and scabies is permethrim (Nix crème 1% for lice and 5% for scabies), lindane (Kwell), or pyrethrins. Lindane is not recommended for pregnant or lactating women.
Molluscum Contagiosum
· Asymptomatic, caused by the poxvirus, does not grow in mucous membranes, transmitted by close contact. Often coexists with condyloma acumulata.
· The characteristic skin lesion is umbilicated papules,
· In adults presents primarily as 1 to 20 solitary lesions randomly distributed over the vulvar skin while on children presents over the entire body.
· Diagnosis is usually made by simple inspection. Microscopic diagnosis can be made from the waxy material from the inside of the nodule. Intracytoplasmic molluscum bodies with Wright’s or Giemsa stain confirms the diagnosis.
· Self limiting infection, however individual papules can be treated to prevent transmission or autoinoculation. The nodules are evacuated and excised with sharp dermal curet and the base treated with Monsel’s solution.
Condyloma accuminatum
· Macroscopically recognized in 30% of infected women and as subcliniucal infection in 70%.
· Types include cauliflower shape, smooth papular, keratotic and flat.
· Caused by HPV. It is an STD, transmitted by skin-skin contact. Grows in moist areas.
· Vaginal condylomata is identified in 1 of 3 women with vulvar disease.
· Management depends on location, size, extend and pregnancy status. No present treatment eliminates subclinical infection from surrounding epithelium. Tx. choices include chemical e.g. podofilox, cautery and immunologic therapy.
Genital herpes
· Currently and epidemic, 80% of infected individuals are unaware of their infection.
· Primary symptoms include severe vulvar pain, tenderness and inguinal adenopathy. Subclinical primary herpes is common.
Granuloma Inguinale or donovanosis
· Chronic, subcutaneous, ulcerative bacterial infection of the vulva.
· Require oral broad spectrum antibiotics for treatment.
Chanchroid
· Acute, ulcerative disease of the vulva. Caused by Haemophilus ducrey. Most common in males than females. The soft chancre is always painful and tender
Syphilis
· Dark-field microscopy is use to detect the spirochete Treponema Pallidum. VDRL and RPR have 1% false positive rate. FTA-ABS and MHA-TP are used when there is a positive nonspecific positive result.
· The primary ulcer is red, round, raised edges, non-purulent, clean base with yellow-green exudates.
· Concurrent HIV should be considered in women with syphilis.
· Parenteral penicillin G is the treatment of choice. Serology is checked at 6 and 12 months post treatment

Infections of the vagina

Bacterial vaginosis causes 50% of cases, candidiasis causes 25% and trichomonas causes 25%.
Bacterial vaginosis
· Diagnosed by the presence of 3 out of 4 of the following: homogeneous vag discharge, pH equal or > than 4.5, positive KOH amine like odor and clue cells greater than 20% in number of epithelial cells. Tx. Flagyl.
Trichomonas
· Second most common nonviral STD after Chlamydia. After a single exposure 75% of contacts will become infected.
Candidiasis
· 75-80% caused by C albicans, 5-20% caused by C. glabrata or C, tropicalis.
· Recurrent candidiasis or candidiasis caused by C. glabrata or C. tropicalis require at least 10-14 days treatment with topical or oral azole. Both C. tropicalis and C. glabrata maybe treated with topical gentian violet.

Infections of the Cervix

Chlamydia
· most commonly found in the columnar cells of the endocervix. In some populations gonococcal and chamydial infections is found in 20 to 40% of chamydial infections.
· Many women with STDs of the cervix are asymptomatic.
N. Gonorrhea
· The standard diagnosis is culture. Gram stain is positive in only 50% of positive cultures. A second consecutive endocervical cotton swab increases detection by 7-10%.
· Test of cure in asymptomatic women is no longer recommended by the CDC
· In women with previous hysterectomy, culturing the urethra gives highest yield.

References

Henson TH, Skinner RA Jr. Dermatologic problems. In: Ling FW, Duff P, eds. Obstetrics and gynecology: principles for practice. New York (NY): McGraw-Hill Medical, 2001:707-50.

Droegemueller W. Infections of the lower genital tract. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:641-705.


Seizure Disorder

Hingson-Gates, Harkness

Prevalence: 1% of general population. Most frequent major neurologic complication of pregnancy.

Types: Acquired (15%)--resulting from trauma, infxn, space-occupying lesions, metabolic d/o, etc.
Idiopathic (85%) -- tonic-clonic, partial complex with or without generalization, myoclonic, focal, absence.

Facts: Patients with frequent seizures before conception tend to have frequent seizures during pregnancy.
Sleep deprivation is a catalyst for seizures.
Birth weight of infants born to women with epilepsy is less than those born to normal women. Increased risk for IUGR in fetuses exposed to anticonvulsant meds.
Questionable increased risk of stillbirth and preeclampsia in women with seizure d/o.
Major pregnancy related risks to women with epilepsy are an increase in seizure freq and a risk of congenital malformation (7% vs 3% in general population).

Goal of treatment: prevention of seizures. Using multiple drugs increases risk of poor pregnancy outcome. Use lowest dose and single agent if possible.

Anticonvulsant effects: Predominant malformations are CV and cleft lip/palate. Neural tube defects are more common with carbamazepine and valproate.
Fetal hydantoin syndrome -- growth/performance delays, craniofacial abnormalities, limb anomalies (hypoplasia of nails and distal phalanges).
Need to give 4 mg of folic acid before conception and early in pregnancy b/c anticonvulsants interfere with folic acid metabolism.
Because neonatal hemorrhage (b/c of a decrease in vit K dependent clotting factors) has been seen in infants exposed to anticonvulsants in utero, infants should be given 1 mg of vit K IM at birth.

Care during pregnancy: Follow anticonvulsant levels (controversial), Quad screen to detect NTD, targeted U/S to look for congenital malformations, fetal echo (20-22 wks), vaginal delivery is route of choice.

Postpartum: all of major anticonvulsants cross into breast milk; these medications are not a contraindication to breastfeeding although need to watch baby for symptoms. OCP with 50 micrograms of estrogen.

References

Gabbe Obstetrics. Neurologic disorders. Chapter 37. pp 1231-1243.

Williams Obstetrics. 21st ed. Neurologic and Psychiatric disorders. pp 1406-1410.


Endometriosis

Meredith Mitchell, MD, James Smallwood, MD

Endometriosis is a condition caused by implantation of endometrial tissue outside of the uterine cavity. These implants are most often found inside the peritoneal cavity, but can be found on the cervix and even in the chest cavity. It is found in 3-10% of the general public. These rates are much higher in women with dysmenorrhea (40-60%) and women with infertility (20-40%). Symptoms include dysmenorrhea, chronic pelvic pain, dyspareunia, constipation/diarrhea, dyschezia, urinary pain, frequency and urgency, and hemoptysis. The gold standard for diagnosis is direct visualization of endometrial lesions and pathologic confirmation.

The most popular theory for the cause of endometriosis is retrograde menstruation with implantation of endometrial cells in ectopic locations. Cyclic changes to this tissue can cause bleeding and inflammation leading to the symptoms associated with endometriosis. This theory is not the complete explanation, however, beecause it is estimated that > 90% of women have retrograde menstruation and not all of these women have endometriosis. It is felt that the development of endometriosis is likely estrogen dependent. For example, endometrial tissue in women with endometriois expresses more aromatase P-450 than women without endometriosis. This aromatase causes higher local production of estrogen which supports the growth of the implant.

Medical treatment theories have been based on this theory of retograde menstruation and that therapies that would decrease normal endometrium, will also decrease endometriosis. Most of these therapies seek to suppress menstruation, both decreasing new seeding with retrograde mentruation and supressing ectopic implants of endometrium.

1) Danazol--this is an oral medication that inhibits the midcycle LH surge and so produces and anovulatory state. It also inhibits many steroid enzymes and so increases free testosterone levels. Low estrogen and high androgens inhibit new implants and suppress old implants. side effects include weight gian, fluid retension, decreased breast size, acne, hirsutism, atrophic vaginitis,and emotional liability. If taken by a pregnant woman it can cause in utero female pseudohermaphroditism.

2) Progesterones--oral and parenteral medroxyprogesterone, norethindrone acetate, and megestrol acetate are all used to treat endometriosis. They produce decidualization and attenuation of endometrium. In high doses they also induce ammenorrhea. Side effects include depression, breakthrough bleeding, weight gain, fluid retention, and lipid abnormalities. They can also cause bone loss, although recovery after stopping treatment is fairly rapid.

3) Combined Estrogen and Progesterone Contraceptives--these can be used continuously or cyclicly and produce endometrial decidualization and ammenorrhea. Side effects are the same as with any OCP's, but continuous pills often have breakthrough bleeding

4) Gonadotropin-Releasing Hormone Agonists--these are modified forms of GnRH that bind GnRH receptors on the pituitary and stop the release of LH and FSH. LH and FSH require pulsitile GnRH in order to be released normally--if continuous GnRH binds the pituitary an initial "flare" of LH and FSH is released followed by very low levels. Without LH and FSH the ovary does not produce estrogen. This hypoestrogentic state leads to ammenorrhea and loss of estrogenic support to the endometriosis. It also causes side effects which include hot flashes, vaginal dryness, decreased libido, irritability, fatigue, and bone mineral loss. Bone loss can be significant--as much as 1% per month. It does recover when treatment is stopped, but slowly and not always fully.

Add-back Therapy
In order to decrease both bone loss and hypoestrogenic symptoms "add back" therapy strategies have been developed. Therapy with bisphosphonates(alendronate 10 mg daily or cyclic etidronate 400mg daily for 2 weeks every 2 months), progestins(norethindrone 2.5-10mg daily) and SERM's(raloxifene 60 mg daily) have been used to try and decrease bone loss. However, lose dose combined estrogen-progestin add-back(0.625mg of conjucated estrogen and 2.5 mg of medroxyprogesterone acetate) helps with hypoestrogentic side effects and bone loss. Progesterone must be added because estrogen alone(estrdiol 1 mg daily) was associated with recurrent pain in a recent clinical trial. Studies have shown that pain is not increased when add back therapy is started with the first dose of GnRH agonist. Bone density should be measured in patients even when add back therapy is used.

References

Endometriosis. In: Speroff L, Fritz MA. Clinical gynecologic Endocrinology and Infertility. 7th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2004:1103-33.

Hesla JS, Rock JA. Endometriosis. In: Rock JA, Jones HW III, eds. Te Linde's Operative Gynecology. 9th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2003:595-638.


Anxiety Disorders

Reta Graham, Citron

Background
Psychiatric disorders are very common in PCP offices: 1 in 4 patients seen have active diagnosable psychiatric disease
  • Most common = depression, generalized anxiety disorder, somatoform disorders, substance abuse and personality disorders
  • Less than 25% of patients with these diagnoses are recognized and treated by their PCP

Anxiety disorders
Symptoms = nervousness, sleeplessness, hypochondriasis and somatic complaints
  • Chronic generalized anxiety (GAD) = most common; starts at 20-35 yo
  • Episodic panic-like anxiety = sudden onset of intense fear, arousal, and/or respiratory distress without provocation (can be confused with systemic medical illnesses such as angina or epilepsy); starts before 25 yo
  • Related mental disorders with anxious features: phobias and PTSD
Prevalence = 30% in women and 19% in men
Pathophysiology
  • Familial: twin studies with more apparent shared risk in panic disorder than generalized anxiety
  • Overactivity of noradrenergic systems projecting from locus caeruleus into forebrain regions
Diagnosis and clinical manifestations of anxiety and panic
  • DSM IV criteria emphasize the presence of unrealistic/excessive worry and apprehension about 2 or more life circumstances for >6 months or longer during which the patient is bothered most days by these concerns
  • Symptoms = motor tension (trembling, shakiness, muscle tension, restlessness), autonomic hyperactivity (SOB, palpitations, sweating, dizziness, N/D), vigilance and scanning (feeling “on edge,” exaggerated startle, difficulty concentrating or falling asleep)
  • Panic attacks =
    • sudden onset of intense apprehension, fear or sense of impending doom, spontaneous, may overlap with GAD
    • 4 attacks within 4 wk period or > 1 attack(s) followed by at least 1 month of persistent fear about having another attack
Treatment
Panic disorder·
  • Pharmacotherapy: 50-70% efficacy (all of the following classes are comparable)
  • · Four classes of medicines:
    • SSRI’s
    • All of those available in the US are indicated for treatment of panic disorder
    • Doses should be at the high end of the dose range
    • TCA’s, benzos (alprazolam is the most studied for panic) and MAOI’s
GAD
  • antidepressants much less efficacious; benzos offer short-term relief
  • Habituation and addiction are common, so psychopharmacologic intervention should be for defined time period (1-4 weeks)
  • If situational anxiety, try therapeutic measures first = cognitive behavior phychotherapy, reassurance, education, relaxation exercises, hypnosis, environmental alterations
  • Non-benzo med: scheduled benadryl (25 mg TID), buspirone (5 mg BID)

Benzodiazepines
  • side effects = disinhibition, ataxia, dysarthria, nystagmus, paradoxical agitation, anxiety, psychosis, confusion, mood lability
  • Additive with other sedatives and/or alcohol
  • Overdose = respiratory depression, hypotension, shock, coma, death
  • Withdrawal sx
  • somaticsomatic (tremor, nausea), psychologipsychologic (anxiety, irritability), perceptualperceptual (paranoia, confusion)
  • Can be dangerous/fatal if chronic excessive dosage stopped abruptly: seizures, psychosis, delirium, autonomic dysfunction
References

Eisendrath SJ, Lichtmacher JE. Psychiatric disorders. In: Tierney LM Jr, McPhee SJ, Papadakis MA, eds.

Current medical diagnosis & treatment. 43rd ed. New York (NY): Lange Medical Books/McGraw-Hill Medical, 2004:1001-61.

Schiffer RB. Psychiatric disorders in medical practice. In: Goldman L, Ausiello D, eds. Cecil textbook of medicine. 22nd ed. Vol. 2. Philadelphia (PA): WB Saunders, 2004:2212-22.



Labor Abnormalities

Allison Eaton, Ursula Harkness

First Stage
Friedman's labor curves were made without experimental verification
Protraction Disorder-dilation and descent occurs slower than normal (based on 95%)
Nulliparous 1.2cm dilation/hr. with 1.0cm/hr descent
Multiparous 1.5cm dilation/hr. with 2.0cm/hr descent
Arrest Disorders-per Friedman, cessation of dilation or descent in active phase for >2 hours; more modern guidelines support a minimum of 4 hours augmented labor before proceeding to c/s and 6 hours if adequate mvu's are not achieved. These newer guidelines showed 92% vaginal delivery rate with no serious adverse effects.

Second Stage
That delivery should be accomplished after 2 hours of pushing was based on a misinterpretation of data from 1952. More recent data shows that the duration of the second stage bears no relationship to perinatal outcome (if there is no fetal distress and there is some progress of descent or rotation). The Mueller-Hillis maneuver is helpful in predicting vaginal delivery when performed in the late first of second stage of labor. Pressure is applied to the fundus with one hand, and the other hand is in the vagina to detect fetal descent. If the head descends 1cm or more with fundal pressure the prognosis for vaginal delivery is good.
Descent In nulliparous patients, descent may not occur until the onset of the second stage, whereas descent usually begins with engagement in multiparous women. Descent occurs due to: 1. Pressure of amniotic fluid, 2. Pressure of the fundus upon the breech with contractions, 3. Bearing down efforts with the abdominal muscles, 4. Extension and straightening of the fetal body.

Shoulder Dystocia
50% of patients have no risk factors and 40-50% of cases occur in infants whose weight is <4000g. 10-20% will have a brachial plexus injury, but 80-90% will resolve completely with early PT or sometimes neurosurgery. There is an inverse relationship between the incidence of brachial plexus injuries and the experience of the OB. Prevention of shoulder dystocia by prophylactic induction not effective. Primary C/S prevents shoulder dystocia in only a small proportion of patients.

References

Mechanisms of normal labor. In: Cunningham FG, Gant NF, Leveno KJ, Gilstrap LC III, Hauth JC, Wenstrom KD. Williams obstetrics. 21st ed. New York (NY): McGraw-Hill, 2001:291-308.

Bowes WA Jr, Thorp JM Jr. Clinical aspects of normal and abnormal labor. In: Creasy RK, Resnik R, Iams JD, eds. Maternal-fetal medicine: principles and practice. 5th ed. Philadelphia (PA): WB Saunders, 2004:671-706.



Cholestasis

Courtney Hodshon, MD, Allen Van Dyke, MD

Intrahepatic Cholestasis of Pregnacy

What is it?
  • Pruritis and jaundice usually developing in the third trimester
  • likely due to abnormal metabolism of bile acids and progesterone
  • Clinical Findings
    • 3rd trimerster, progressively worsening nighttime itching then moving to the day as well
    • clincal jaundice (75% of cases), mild and constant until delivery
  • Laboratory Findings
    • alk phos 5-10x elevation (specifically hepatic origin vs placental)
    • increased serum and urinary sulfated progesterone
    • mild increase bili (rarely >5mg/dl)
    • variable ast/alt
    • increased chol/triglyceride (above normal increase in pregnancy)
    • increased serum bile acids
    • decreased prothrombin production/ CHO metaboism abn.
    • liver biopsy showing certilobular dilated biel canaliculi with plugging and abundant bile pigments
Who gets it?
  • 1/1000-10000 pregnancies. Most common liver condition unique to pregnancy
  • most common in Chile (5% singletons, 21%twins) and Sweden (1-2%)
  • more rare in US, especially those of African descent
  • high recurrence in subsequent pregnancies
Why do we care?
  • Maternal issues
    • mostly just symptomatic treatment to reduce pruritus
  • Fetal issues
    • increased preterm birth (44%) and perinatal mortality (3.5%)
    • increased meconium stained fluid (45%)
    • "fetal distress" (22%)
    • severity of disease does not correlate with outcome
    • antenatal testing (nst, ustrasound, estriol levels) have not shown any beneifit
Can we treat it?
  • Medications
  • antihistamines have limited efficacy
  • choelstyramine to sequester bile acids inthe gut (hard to swallow, delayed effect-2 weeks, malabsorption of fat soluable vitamins
  • phenobarbitol induce hepatic microsomal enzymes (side effects, 1+ week to work)
  • dexamethasone suppresses fetal-placental estrogen synthesis
  • guar gum augments fetal elimination of bile acids
  • s-adenyl-methionine and ursodeoxycholic acid -- probably most effective
  • Delivery cures IHCP -- Induce at term or +FLM?
References

Landon MB. Diseases of the liver, biliary system, and pancreas. In: Creasy RK, Resnik R, Iams JD, eds. Maternal-fetal medicine: principles and practice. 5th ed. Philadelphia (PA): WB Saunders, 2004:1127-46.

Samuels P. Hepatic disease. In: Gabbe SG, Niebyl JR, Simpson JL, eds. Obstetrics: normal and problem pregnancies. 4th ed. New York (NY): Churchill Livingstone, 2002:1216-23.



Puberty

Reta Graham, Currens

Precocious Puberty


Puberty
  • process of biologic change and physical development after which sexual reproduction becomes possible
  • accelerated linear skeletal growth and development of secondary sexual characteristics
    • breast development (thelarche)
    • axillary/pubic hair (adrenarche/pubarche)
    • maximal growth velocity
    • menarche (may occur before adrenarche in 10% of normal females)
Precocious puberty
  • appearance of any signs of secondary sexual maturation at an early specific age more than 2.5 standard deviations below the mean
  • Caucasian girls < 7 years and African American girls < 6 years
  • Rare (1 in 10,000 in US)
1. Complete: central (gonadotropin-dependent) and peripheral (gonadotropin-independent)
2. Incomplete: premature thelarche and premature adrenarche

Complete precocious puberty
  • Central (90% of those with complete will have this form) = cyclic release on gonadotropins; fertility is possible
    • Idiopathic (70%) = progression of endocrine events of normal puberty occur early; diagnosis of exclusion
    • Organic brain disease = congenital defects, tumors, cysts, postcranial irradiation, postinfective lesions, hypothalamic hamartoma, neurofibromatosis, post-traumatic brain injury
  • Peripheral = no cyclic function of reproductive axis, no follicular maturation/ovulation, fertility not possible
    • ovarian tumors = most common cause, produce estrogen (granulosa cell accounts for 60% )
    • adrenal tumors: estrogen secreting, very rare
    • iatrogenic: estrogen cream, OCP’s
    • primary hypothyroidism
    • McCune-Albright syndrome: triad of precocious puberty, multiple areas of fibrous dysplasia of bone and café-au-lait spots
    • ovarian cysts: estrogen-secreting
  • Primary hypothyroidism
    • Negative feedback of thyroxin on hypothalamus and pituitary is not present
    • TSH secretion increased by pituitary
    • Concomitant increase in gonadotropins
    • Increased gonadotropins produce clinical signs of precocious puberty and may stimulate ovarian cysts
    • Short stature, bone age is retarded: only etiology of precocious puberty for which bone age is significantly less than chronologic age
    • Treatment with thyroid replacement causes child to become euthyroid and ovarian cysts regress
    • Syndrom usually observed in girls aged 6-8 years
Incomplete precocious puberty
  • Only one pubertal change is clinically apparent without evidence of systemic estrogen effect
  • No estrogen effect = bone age seen on x-ray does not exceed chronologic age, no superficial cells desquamated from vaginal mucosa
  • Remaining pubertal events occur at normal age
  • Premature thelarche = isolated early breast development
    • Benign
    • No therapy required
  • Premature adrenarche = isolated early development of axillary hair or pubic hair
    • Result of functional increase in adrenal androgen production
    • Association with organic brain disease
Diagnosis
  • History and physical exam: Tanner stage, height (review growth chart to determine age of onset of increase in growth velocity)
  • Primary emphasis = rule out life-threatening neoplasms of ovary/adrenal/central nervous system
  • Secondary emphasis = delineate speed of maturation process (crucial in decisions regarding therapy)
  • Parents’ concern = social stigma of child being physically different from peers, diminished ultimate height caused by premature closure of epiphyseal growth centers
  • Estradiol, prolactin, FSH, LH, GnRH stimulation test, thyroid function tests, x-ray (bone age), TVUS, head imaging
Treatment
  • Depends upon specific etiology
  • Goals of therapy
    • reduce gonadotropin secretion
    • reduce/counteract peripheral actions of sex steroids
    • decrease growth rate to normal
    • slow skeletal maturation to allow development of maximal adult height
  • Incomplete forms usually self-limiting
  • Definite treatment if menarche < 8 years old, progressive thelarche and pubarche, and bone age 2 years > chronologic age
  • Primary hypothyroidism = thyroid replacement
  • Remove iatrogenic sources of estrogen
  • Surgically remove ovarian and adrenal tumors
  • McCune-Albright syndrome = testolactone (aromatase inhibitor)
    • Decreases estrogen in peripheral circulation, ovarian volume and frequency of menses
    • Little effect on regression of breast development or pubic hair growth
  • GnRH dependent precocious puberty
    • Long acting GnRH agonists (deslorelin, leuprolide acetate, buserelin)
    • Initiate as soon as possible after dx to achieve maximal adult height (most effective in 4-6 year olds)
    • Continuous chronic administration until median age of puberty (effects reversible when med discontinued after normal height achieved)
      • Initial rise then decrease in pituitary release of gonadotropins
      • Decreased estrogen levels
      • Regression of ovarian volume and uterus size
      • Halt/regression of breast development
      • Menstruation ceases
      • Decreased rates of linear growth and skeletal maturation
  • Both child and family may need counseling
    • Child will have psychosocial/behavioral maturation of children her age, not age reflected by physical appearance
    • Sex education/help in anticipating and confronting various social experiences
References

Brenner PF. Precocious puberty. In: Mishell DR Jr, Goodwin TM, Brenner PF, eds. Management of common problems in obstetrics and gynecology. 4th ed. Malden (MA): Blackwell, 2002:341-4. 

Droegemueller W. Pediatric gynecology. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:269-93. 




Oncology Basic Science and Chemotherapy

Liliana Belskus, MD, Andrea Currens, MD

Radiation Therapy

Inverse Square law: The energy dose of radiation per unit area decreases proportionately to the square of the distance from the site source.
1/d^2
example: dose of radiation 2 cm fom a point source is 1/4 the value of the dose at 1 cm.

Radiation Biology
· Photons, gamma or x-rays, dislodge orbital electrons from the tissue which secondarily produce free hydroxyl radicals.
· Free radicals cause DNA damage. Oxygen is required for this reaction; therefore photon radiation therapy is reduced in hypoxic areas.
· A given dose of radiation kills a constant fraction of irradiated cells.
· Cells are most sensitive during mitosis (M phase). Large tumors have higher proportion of cell in the resting phase (G0)
· Radiation dose delivered to the tumor is affected by the energy of the source, the depth of the tumor beneath the skin and the size of the field undergoing irradiation.

Radiation Sources
· Brachytherapy: the radioactive source is placed within and existent body cavity (such as Fletcher-Suit applicators-also known as tandem and ovoids)
· Teletherapy is from and external source usually at a distance 5 to 10 times greater than the depth of the tumor

Tissue Tolerance and Radiation Complications
· Radiation acutely affect tissue undergoing rapid cell division e.g. skin, mucosa of gut, bladder and vagina. Urethral stricture is 1% at 5 yrs and 2.5% at 20 yrs.
· Systemic reactions include decreased in WBC, n/v, and diarrhea.
· Late reactions include tissue necrosis, fibrosis, fistula formations, ulcerations and bleeding. These may occur 6 months to 2 years after treatment.

Chemotherapy

· Many chemotherapy agents have actions similar to radiation therapy affecting primarily rapidly dividing cells.
· The proportion of cells involved in the proliferation of the tumor is known as the growth fraction (GF)
· Smaller tumors grow faster than larger tumors and metastases grow faster than primary tumors.

Principles of Chemotherapy
· Larger tumors are more likely to be resistant to a single cytotoxic agent. Smaller tumors with high growth fraction and short doubling time are most sensitive to treatment.
· Cytotoxic agents kill cancer cell by first order kinetics, meaning at a given dose a constant fraction of tumor cells are killed.
· The net effect is given by the proportion of cell being killed as well as the speed at which tumor cells replicate.
· A large dose at an increased frequency would be the most effective treatment but this is limited by the tolerance of normal tissues.
· Multi-therapy treatments are given because of the fact of encountering different cell lines within a tumor and also to prevent emergence of resistant cells. Spontaneous mutations are the basis for drug resistance

Approaches to Treatment
· Dosage is calculated according to body surface area
· Most gynecologic chemo protocols are given in cycles with 3-4weeks intervals.
· Dosages are adjusted according to myelosuppression and hepatic metabolism or renal excretion. Low serum albumin leads to increased free circulating chemo drugs and increased toxicity.
· Adjunctive therapy describes primary treatment for advance cancer. Also given after cancer has been controlled by alternative therapy.
· Primary or neoadjunctive therapy refers to the use of initial chemotherapy to improve the overall therapeutic result.

Chemotherapeutics Used in Gynecologic Cancer
· Alkylating Agents: directly interact with DNA interfering with its function. Cytoxan used in ovarian cancer, associated with hemorrhagic cystitis
· Antitumor Antibiotics: derived from bacteria or fungus. e.g.
Bleomycin; associated with severe pulmonary toxicity.
Doxorubicin; associated with severe cardiomyopathy.
Dactinomycin
· Antimetabolites: purines or pyrimidines analoges
Methotrexate; effective treatment for trophoblastic diseases, excreted by kidneys needs dose adjustment with decrease renal function.
5FU, Cytarabine
· Antimicrotubular (antimitotic) drugs: plant alkaloids isolated from plan extracts.
Vincristine; associated with severe neurotoxicity
Taxol; widely use for ovarian cancer, can cause severe neutropenia and neurotoxicicity.
Vinblastine, Etoposide
· Topoisomerase inhibitors. Interfere with DNA transcription and replication. Topotecan; used for cisplatin resistant ovarian cancer.
· Other compounds: Cisplatin; interferes with DNA synthesis, widely used for ovarian epithelial and germ cell tumors, associated with peripheral neurotoxicity, nephrotoxicity and myelossupression. Carboplatin is not nephrotoxic.

References

Herbst AL. Principles of radiation and chemotherapy in gynecologic cancer. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:825-42.

Basic principles of chemotherapy. In: Di Saia PJ, Creasman WT. Clinical gynecologic oncology. 6th ed. St. Louis (MO): Mosby, 2002:501-20.



Medical Coverage

Jorgensen, Harkness

Medicare does not cover most preventative services. However, it will pay for specific screening services provided at the same time as preventative care, like during an annual exam. Below is a list of services covered by medicare.
  • Screening pelvic exam
  • Pap smear
  • Osteoporosis screening
  • Mammogram
  • Hemoccult screening

How frequent Medicare covers for such services depends on the patient, and whether they are considered high risk. What defines high risk?

For pelvic and breast exams:
If a woman is of childbearing age, she is considered high risk if...
  • Cervical or vaginal cancer is present or was present
  • Abnormalities were found in the preceding 3 years.

A woman of any age is considered high risk for cervical or vaginal cancer is any of these factors apply..
  • Onset of sexual activity younger than 16yrs of age
  • Five or more sexual partners in a lifetime
  • History of sexually transmitted disease
  • Fewer than three negative pap tests within the last 7 yrs.
  • No pap tests within the last 7 yrs.
  • DES exposure

SERVICE
COVERAGE
1. Breast and pelvic exam
Q 2 yrs or annually if high risk
2. Pap Smear
Q 2 yrs or annually if high risk
3. Hemoccult
Annually if 50 yrs of age or older
4. Mammogram
Annually if 40 yrs of age or older
5. Bone Mass density tests
Q 2 yrs, must meet criteria
6. Initial Physical Exam
Once within first 6 months of coverage
7. Cadiovascular screening (lipid profile)
Every five years
8. Diabetes
Twice yearly if at risk or diagnosed with pre-diabetes
9. Tobacco Cessation
Twice yearly


References

Medicare screening benefits. In: American College of Obstetricians and Gynecologists. Frequently asked questions in obsteric and gynecologic coding. Washington (DC): ACOG, 2003:69-74.

Preventive medicine services. In: American College of Obstetricians and Gynecologists. The essential guide to coding in obstetrics and gynecology. Washington (DC): ACOG, 2004:81-92.



Conjoined Twins

Courtney Hodshon, MD, Ursula Harkness, MD

"Normal" Embryonic Development

  • 1 Sperm + 1 Egg = 1 baby
Day Contents Events
0
egg and sperm
fertilization

blastomeres

3
morula
chorion completed
6-8
blastocyst
implantation, amnion completed
12
implanted zygote
embryonic disc completed
266
baby
delivery

"Abnormal" Embryonic Development (Twins)

Dizygotic Twins
  • 2 sperm + 2 eggs = 2 Babies
Monozygotic Twins
  • 1 Sperm + 1 Egg = 2 Babies
  • Dichorionic / Diamniotic Twins
  • Division of fertilized embryo before chorion completed (before day 3)
  • Monochorionic / Diamniotic Twins
    • Division after inner cell mass formed, after chorion formed but before amnion completed (between day 4-8)
  • Monochorionic / Monoamniotic Twins
    • division after amnion formed (between days 8-12)
  • Conjoined Twins
    • Division (incomplete) after embryonic disc formed

Conjoined Twins

  • Sometimes referred to by the un-pc name of "Siamese" after Chang and Eng Bunker who were conjoined twins paraded around the world by PT Barnum
  • Common shared body sites
    • Anterior (thoracopagus) -- most common
    • Posterior (pyopagus)
    • Cephalic (craniopagus)
    • Caudal (ischiopagus)
  • Most spontaneously abort
  • Frequency not clear (maybe 1:60000 live births? 2-8/10000 twins?)
  • Higher rate of other abnormalities
References

Multifetal pregnancy. In: Cunningham FG, Gant NF, Leveno KJ, Gilstrap LC III, Hauth JCJ, Wenstrom KD. Williams Obstetrics. 21st ed. New York (NY): McGraw-Hill, 2001:765-810.

Placenta and fetal membranes. In: Moore KL, Persaud NN. The Developing Human: Clinically Oriented Embryology. 7th ed. Philadelphia (PA): WB Sanders, 2005:119-55.


Hirsutism

Jorgensen, Currens

Body hair can be classified as vellus or terminal. Vellus hairs are fine and unpigmented, like those that cover a child's face. Terminal hairs, pigmented and coarser, may be sex hormone dependent (such as the chin or abdomen in men) or sex independent (such as eyebrows and eyelashes). Androgens convert vellus hair to terminal hair in sex hormone-dependent areas.

Definition
Male type body hair distribution, usually an androgen-dependent process:
  • Face: Mustache, beard, sideburns, lambchops
  • Body: Chest, circumareolar, linea alba, inner thighs
Etiology
Hirsutism can be divided into two groups:
  1. Androgen Dependent Causes: due to overproduction by the ovaries or adrenals.
  2. Androgen Independent Causes (Hypertrichosis): due to drugs (i.e. - cyclosporine, glucocorticoids, minoxidil) or starvation (i.e. - anorexia).
Facts
  • Hair does not grow continuously, but rather, in a cyclical fashion.
  • Anagen - The growing phase
  • Catagen - Rapid involution phase.
  • Telogen - Quiescent phase.
  • Androgens, particularly testosterone, intiate growth. Approx. 50% of testosterone is derived from peripheral conversion of androstenedione, whereas the adrenal gland and ovary contribute about 25% respectively. In hirsute women, however, only 25% of circulating testosterone arises from peripheral conversion, and most often, their beard is secondary to persistent anovulation and excessive androgen production by the ovaries.
  • Dihydrotestosterone (DHT) is the major androgen working at the hair follicle & sebaceous gland level, hence acne.
  • Remember: Testosterone is converted to DHT by the enzyme 5 alpha reductase.
  • Estrogens retard growth rate leading to finer, less pigmented, slower growing hair.
  • Pregnancy can increase the synchrony of hair growth, with periods of growth or shedding.
  • Hairy women make for warm winters.
Differential
  • PCOS
  • Adrenalcortical tumors
  • Ovarian tumors (arrhenoblastomas, hilar cell, krukenberg)
  • Pituitary adenomas
  • Cushing's Syndrome
  • Congenital adrenal hyperplasia (21 or 11 hydroxylase deficiency)
  • Exogenous androgens - anabolic steroids, danazol.
Workup
  • Labs: Circulating levels of testosterone, 17-OHP (to rule out adrenal hyperplasia), TSH.
  • With PCOS, screen for dyslipidemia and insulin resistance. Check 2 hour glucose intolerance test, fasting lipid panel, and prolactin.
Treatment for Hirsutism
  • With anovulation - low dose (less than 50 micrograms of estrogen) OCPs. Response is slow, at least 6 months. Pathophysiology - OCPs decrease LH which decreases testosterone production and OCPs also increase SHBG which increases testosterone binding capacity. Both of which lead to decreased free testosterone. If this fails, then add an antiandrogen, like spironolactone or finasteride.
  • With anovulation and PCOS in women desiring pregnancy - Metformin, clomid.

References

Clinical Gynecology and Endocrinology. 7th Edition. Pages 499-530. 

ACOG Practice Bulletin. Number 41. Dec 2002. Polycystic Ovarian Syndrome. 



Opiate Withdrawal

Meredith Mitchell, MD, Hytham Imseis, MD

Opiate Dependence


Fetal Risks (with many confounders in studies):
  • IUGR*
  • IUFD
  • Meconium stained amniotic fluid
  • decreased head circumference
  • Decreased Apgar scores
  • Preterm delivery
  • PPROM
  • Chorioamnionitis
  • SAB
  • Preeclampsia
  • Neonatal abstinence syndrome
  • Neonatal abstinence syndrome *
*Most common

No structural defects have been noted as a result of opiate use.

Maternal Risks
  • Subacute bacterial endocarditis
  • Hepatitis
  • HIV
  • Other STD's from high risk behavior
Methadone is an opiate introduced in 1965 to treat opiate dependence and decrease the risks of illegal and IV opiate use and this has been confirmed in 4 prospective randomized trials. It is an oral medication that decreases craving for opiates as well as blocking withdrawal symptoms.

Symptoms of opiate withdrawal include: vomiting, diarrhea, abdominal cramps, lacrimation, rhinorrhea, sweating, yawning, hypertension, hyperventilation, tachycardia, flushing, muscle spasms, chills, flushing, and restless sleep.

Methadone use in pregnancy is considered superior to heroin use for several reasons:
  1. Risk of IUFD is increased in heroin use, but not with methadone
  2. Although both drugs have been shown to cause IUGR, the relative risk of IUGR with methadone is 1.3 (CI crosses 1) and the relative risk of IUGR with heroin use is 4.61 (CI 2.78-7.65)
  3. Steady doses of methdone prevent fetal withdrawal , effects of which are hypoxia, bradycardia, HTN , and IUFD
  4. Decreased risk of exposure to sexually transmitted and needle exposure diseases due to using or seeking drugs
However, methadone does have serious risks. Most importantly it can cause neonatal abstinance syndrome at higher rates than heroin. Neonatal abstinance syndrome occurs in 50-95% of all infants that were exposed to opiates in utero. Symptoms can be significant and include: sleep disturbence, hyperactivity, tremors, shrill cry, convulsions, fever, hypoglycemia, mottling, sweating, vasomotor instability, nasal stuffiness, tachypnea, vomiting, diarrhea, excessive sucking and poor feeding.

Treatment includes supportive care in a safe warm environment and treatment with drugs such as phenobarbital, diazepam, and chlorpromazine. Most data show a correlation between amount of methadone use and severity of neonatal withdrawal, although, one study does refute this. However, because of this some clinicians advocate weening down or off methadone in pregnacy, although it is very common in pregnancy to need to increase doses to stop maternal and fetal withdrawal. If methadone is weened it should be done by decreasing 2 mg per day as an inpatient and 5-10 mg per week as an outpatient. Data on long term effects of methadone on children is also controversial. At least one study has shown an increase in behavioral and school problems; others have shown developmental delay.

References

Best G, Woods JR Jr. Licit and illicit drugs: tobacco, alcohol, and opiods. In: Yankowitz J, Niebyl JR, eds. Drug therapy in pregnancy. 3rd ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2001:149-64.

Andres RL. Effects of therapeutic, diagnostic and environmental agents and exposure to social and illicit drugs. In: Creasy RK, Resnik R, Iams JD, eds. Maternal-fetal medicine: principles and practice. 5th ed. Philadelphia (PA): WB Saunders, 2004:281-314.



Amenorrhea

Allison Eaton, Kiran Sigmon

Amenorrhea Associated with a Lack of Secondary Sexual Characteristics

Most clinically relevant way to approach is on basis of gonadotropic status, because delayed puberty due to enzyme deficiencies (with associated physical exam findings, ie absent uterus) are rare. Examples include XY individuals that are phenotypically female (absent uterus) and associated enzyme deficiency such as 17-alpha Hydroxylase deficiency (with hypertions and hypokalemia), 17,20-Desmolase deficiency, and 5-alpha reductase deficiency (have testes, no breasts as in AI, experience virilization at puberty at times). Note that XX individuals with 17-alpha hydroxylase deficiency will also have primary amenorrhea but have a uterus.

Hypergonadotropic hypogonadism
-gonadal failure leading to lack of negative estrogen feedback elevates FSH. 30% of patients with gonadal failure have a genetic abnormality (most commonly Turner's). If patient has amenorrhea and elevated FSH, check a karyotype. Other causes include partial deletions of X chromosome, toxins/chemotherapeutic agents, galactosemia. Patients with mosaicism (ie 45,X/46,XX) will spontaneous menstruate 20% of the time, and patients with pure gonadal dysgenesis (nl chromosomes, but streak gonads) may develop some secondary sexual characteristics and occasional bleeding.

If FSH is elevated, but the karyotype is normal, then 17-alpha hydroxylase deficiency in an XX individual must be considered and confirmed with ACTH stimulation test. The correct time to administer this test is in the morning.

Hypogonadotropic hypogonadism
-inadequate amounts of GnRH or gonadotropins are present, and the most common cause is physiologic or constitutional delay of puberty due to delayed reactivation of the GnRH pulse generator. Levels are GnRH are normal in terms of physiologic development, but not chronologic age. Kallmann's Syndrome, with insufficient pulsatile secretion of GnRH, is the second most common cause. Other causes are central nervous system tumors and hypothalamic/pituitary dysfunction. FSH will be low or normal in these patients.
Physiologic delay is diagnosis of exclusion and will have delayed bone age, no lesion on CT/MRI, and normal LH response to GnRH stimulation for their age.

References

Normal and abnormal sexual development. In: Speroff L, Fritz MA. Clinical gynecologic endocrinology and infertility. 7th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2004:319-59.

Schillings WJ, McClamrock H. Amenorrhea. In: Berek JS, Hillard PJA, Adashi EY, eds. Novak's gynecology. 13th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2002:843-69.



Gene Deletions

Wood, Buys

Alpha Thalassemia and Gene Deletion

Alpha-Globin Genes
Syndrome
Hematocrit
MCV
4
Normal
Normal

3
Silent Carrier
Normal

2
Thalassemia Minor
28-40%
60-75
1
Hemoglobin H disease
22-32%
60-70
0
Hydrops Fetalis




  • The thalassemias are hereditary disorders characterized by reduction in the synthesis of globin chains (alpha or beta).
  • Normal adult hemoglobin is primarily hemoglobin A- formed from a tetramer of 2 alpha chains and 2 beta chains- α2β2. Two copies of the α-globin gene are located on chromosome 16 and there is no substitute for the α-globin in the formation of hemoglobin.
  • Alpha thalassemia is due primarily to GENE DELETION causing reduced α-globin chain synthesis. The phenotype is dependent on the number of genes deleted.
  • The table above summarizes the number of gene deletions and resulting syndrome. The absence of all 4 genes is not compatible with life.
References

Kilpatrick SJ, Laros RK Jr. Maternal hematologic disorders. In: Creasy RK, Resnik R, Iams JD, eds. Maternalfetal medicine. 5th ed. Philadelphia (PA): WB Saunders, 2004:975-1004.

Linker C. Blood. In: Tierney LM, McPhee SJ, Papadakis MA, eds. Current medical diagnosis & treatment. 43rd ed. New York (NY): Lange Medical/McGraw-Hill.



Microhematuria

Wood, Sigmon

Microhematuria
  • Defined as 2 or more red cells per high-power field on microscopic examination
  • Often transient- possible causes include vigorous exercise, sexual intercourse, mild trauma, menstrual bleeding
  • Start with routine history and physical, r/o infectious cause or other obvious source
  • US Preventive Services Task Force does not recommend routine screening of urine for microscopic hematuria
Recommendations for further evaluation of asymptomatic microhematuria (There is no consensus on how to evaluate, so these are the recommendations of the particular authors of these resources.)
  • Repeat urinalysis a few days after first to confirm microscopic hematuria
  • If negative, no evaluation needed (unless risk factors for bladder cancer- smoking, toxin exposure)
  • If positive and patient has proteinuria or renal insufficiency, refer to nephrology
  • If positive without proteinuria or renal insufficiency, perform microscopic examination to look for red cell casts or acanthocyturia which might suggest glomerular disease. If positive, refer to primary care physician to follow for development of proteinuria or renal insufficiency. If no evidence of glomerular disease then perform helical CT urography with and without contrast to evaluate for occult stone disease or mass. (The study by Lang et al. found that CT diagnosed lesions responsible for microhematuria in 43% of patient with negative urological surveillance exams and recommends this first rather than studies like U/S or IVP)
  • If all negative consider cytology in those >age 40 ( am void x 3 days looking for malignant cells)
  • If cytology positive then perform cystoscopy.
    If cytology negative, consider cystoscopy if patients >50 or with risk factors for bladder cancer.
  • If all work up is negative then yearly follow-up with office urine evaluation.
References

Cohen RA, Brown RS. Microscopic hematuria. N Engl J Med 2003;348:2330-8.

Lang EK, Thomas R, Davis R, Myers L, Sabel A, Macchia R, et al. Multiphasic helical computerized tomography for the assessment of microscopic hematuria: a prospective study. J Urology 2004;171:237-43.



Anaphylaxis

Goodwin, Van Dyke

Pathophysiology

Immediate hypersensitivity
• Initiated by binding of antigen to IgE attached to surface of mast cells
• There is a subsequent release of agents that mediate vascular leak of
protein, smooth muscle contraction and mobilization of inflammatory cells.
• The relationship of anaphylaxis to prior sensitization is unclear

Antigenic substances include proteins and smaller solutes that combine with proteins

• Stings of bees, yellow jackets, wasps and hornets
• penicillin is the most common drug related cause (crossreaction with cephalosporins is about 10%)
• local anesthetics
• foods, such as eggs, seafood, nuts beans, chocolate
• blood products

Symptoms
• Within seconds to minutes pt may have both upper and lower airway obstruction
• Laryngeal edema may be experienced as a lump in the throat, hoarseness, or stridor
• Bronchial obstruction is associated with feelings of tightness in the chest or audible wheezing
• Pruritic, raised and erythematous urticaria with serpiginous border and blanched centers can appear in a local or diffuse distribution over skin. Rarely do they last more than 48 hours.
• Hypotension and syncope can also occur
• Angioedema of larynx and epiglottis may result in asphyxiation
• At autopsy one may find mucosal swelling, intense bronchospasm and bronchial edema with secondary emphysematous over distention of the lungs
• Vascular congestion and eosinophilic infiltration are also present
Angioedema and urticarial manifestations have been attributed to release of endogenous histamine
Leukotrienes may be the cause of marked bronchiolar constriction

Treatment
• Epinephrine 0.2-0.5 cc of 1:1000 solution, repeated twice at 20-30 minute intervals prn
• Aerosolized bronchodilation and theophylline plus oxygen may be needed if hypoxia occurs.
• For Profound shock, volume expanders like NS and 5 cc of 1:10,000 epinephrine IV q 5 minutes prn. If no response 2 -50 micrograms/kg of dopamine is indicated.

References

Richard M Effros, Jack Kaufman. Part Two: Disease State Management by Organ System, Chapter 72: Asthma and Other Allergic Disorders.

K. Frank Austen. XII: Disorders of the Immune System, Connective Tissue and Joints: Section 2: Disorders of Immune-Mediated Injury. 



Cardiovascular Disease

Karie LaFleur, MD, Allen Van Dyke, MD

Age-Related Cardiovascular Changes

-Decreased arterial compliance and increased systolic BP lead to LVH which result in a hypotensive response to tachycardia, volume depletion and decreased atrial contraction.
-Decreased B-adrenergic responsiveness leads to decreased cardiac output and heart rate in response to stress.
-Decreased baroreceptor sensitivity and decreased SA node automaticity lead to an impaired BP response to standing or volume depletion.

Disease Presentation

Often atypical especially if patient is older than 75. Onset of a new disease leads to symptoms associated with a different organ system, particularly one compromised by preexisting disease. For example, most older patients with hyperthyroidism present with atrial fibrillation, confusion, depression, syncope and weakness.

Geriatric Medicine and Cardiovascular Disease

-Alcohol Use: 1-2 oz daily can decrease CVD risk by increasing HDL, however, consumption beyond a moderate level can adversely effect on every organ system.
-Illicit Drugs: Crack, cocaine and methamphetamines lead to chest pain and MI at any age but cause more dramatic effects on the elderly population to include earlier median age of death.
-Iatrogenic Drug Reactions: Commonly used CV drugs such as digitalis, procainamide and quinidine have prolonged half lives and narrow therapeutic windows. Toxicity in older populations commonly occurs at normal doses.
-Tobacco Use: Largest single preventable cause of illness and premature death in the US. Providers should discuss cessation at every visit.
-Obesity: Associated with overall increase in cardiovascular risk secondary to relationship with HTN, dyslipidemia and type 2 DM.
-Physical Activity: 30 minutes of exercise daily reduces risk of CVD.
-Hypertension: Regular screening, lifestyle modifications and antihypertensive medications will reduce risk of CVD.
-Lipid evaluations: Yearly screening for hyperlipidemia with lifestyle modifications and medication to bring lipids into preferred range based on other comorbidities.
-Diabetes: Diabetics have more severe atherosclerosis, 2-3 times more MIs and twice as many strokes as nondiabetics of the same age. For women, diabetes nullifies the 5-10 year protection form CVD that is conferred by their gender. Risk reduction for CVD targets lifestyle modifications to reduce obesity and increase physical activity, medications to improve glucose control if diet is not enough.
-Aspirin therapy with 81mg daily reduces risk of CVD by 28 percent.

References

Resnick NM. Geriatric medicine. In: Braunwald E, Fauci AS, Kaspar DL, Hauser SL, Longo DL, Jameson JL, eds. Harrison's principles of internal medicine. 15th ed. Vol. 1. New York (NY): McGraw-Hill Medical, 2001:36-46.

Phelan EA, Paniagua MA, Hazzard WR. Preventive gerontology: strategies for optimizing health across the life span. In: Hazzard WR, Blass JP, Halter JB, Ouslander JG, Tinetti ME, eds. Principles of geriatric medicine and gerontology. 5th ed. New York (NY): McGraw-Hill Professional, 2003:85-92.



Preterm Labor and Cervical Length

Jorgensen, Imseis

Preterm Labor defined: Regular contractions that occur before 37 wks that are associated with cervical change. Interestingly, in 80% of women with presumed labor, preterm delivery will not occur.

Risk Factors
  • Prior preterm delivery: prior preterm birth between 16 - 36 wks carries a 3- to 4-fold increase risk of recurrent preterm birth. Magnitude of risk increases as gestational age of the previous preterm births decrease.
  • Multiple gestation: Twin pregancies are 5 to 6 fold more likely to deliver preterm compared to singletons. Higher order multiples have even higher risk.
  • Low prepregnancy Weight: BMI < 19.8 has been associated with preterm delivery, especially in white women.
  • Vaginal bleeding in second or third trimester: Relative risk of 1.6 to 15.
  • Race: Higher risk in African-Americans than Caucasians.
  • Age < 18 or > than 40 yrs.
  • Cigarette smoking.

Cervical Length
According to one study,a length of 18 mm (or less) had the optimal positive predictive value for the diagnosis of preterm labor, and 30 mm the optimal negative predicative value for the diagnosis of preterm labor in symptomatic women.
  • Cervical length < 20 mm and persistent contractions = preterm labor.
  • Cervical length 20 - 30 mm and persistent contractions = probable labor.
  • Cervical length > 30 mm = preterm labor unlikely regardless of contraction frequency.

Fetal Fibronectin
An extracellular matrix protein that attaches fetal membranes to the underlying uterine decidua. The presence of fibronectin after 22 wks is a marker of the disruption of the decidual-chorionic interface. Has high negative predictive value. Obtain sample from posterior fornix or external os, avoiding areas of bleeding.

Corticosteroid Use
Antenatal administration of steroids for preterm labor significantly reduces the incidence and severity of neonatal respiratory distress syndrome, neonatal mortality, and reduction of intraventricular hemorrhage and necrotizing enterocolitis. Consider administration if gestation age between 24 - 34 wks (up to 32 wks if PPROM).

Tocolytics
Avoid combination of agents, due to increased incidence of side effects.
  • Magnesium sulfate: Mechanism of action is unkown, but theory is competitive inhibition with calcium ion. Cleared renally, check creatinine, esp. in diabetics. Contraindicated in myasthenia gravis. Side effects:
    • Maternal: flushing, nausea, vomiting, headache, muscle weakness, diplopia, SOB, chest pain, pulmonary edema.
    • Fetal: Mag crosses the placenta. Lethargy, hypotonia, respiratory depression. Bony anomalies in infants exposed > 7days.
  • Indomethacin: Mechanism of action is inhibition of prostaglandins. Fetal side effects more worrisome than maternal, such as constriction of of the ductus arteriosus, oligo, and neonatal pulmonary hypertension. Dose - 50 mg loading dose PO or PR, then 25-50mg Q 6 hrs. Short term intervention - two to four days typically.
  • Beta - Mimetics: Mechanism of action is stimulation of beta receptors and uterine relaxation. Structurally related to epinephrine. Side effects: Maternal and fetal tachycardia, long list. Dose: Terbutaline - 0.25mg subcutaneously Q 20 min to 3hrs, hold if pulse > 120.
  • Calcium channel blockers - Nifedipine: Inhibit intracellular calcium entry and decrease contraction of smooth muscle. Side effects: hypotension. Dose: 30 mg loading dose then 10 - 20mg PO Q 4-6hrs. Do not use sublingual route.
Contraindications to Tocolytics
  • Severe preeclampsia
  • Significant hypertension
  • Placental abruption
  • Chorioamnionitis
  • Lethal congenital or chromosomal abnormalities
  • Advanced cervical dilation
  • Placental insufficiency or fetal compromise (IUGR, substance abuse, fetal distress)

References

SG, Niebyl JR, Simpson JL. Obstetrics: normal and problem pregnancies. 4th ed. New York (NY): Churchill Livingstone, 2002:755-826

Management of preterm labor. Practice Bulletin 43. American College of Obstetricians and Gynecologists. Obstet Gynecol 2003;101:1039-47



Dysmenorrhea

Summer Gilmer, MD, Elizabeth Buys, MD

Definition

Severe painful crampingin the lower abdomen often accompanied by sweating, tachycardia, headaches, nausea, vomiting, all occurring just before or during menses.
Primary dysmenorrhea- no obvious pathology
Secondary dysmenorrhea- associated with pelvic pathology that causes pain during menses

Incidence

Wide range of 3-90%, best estimate of prevalance of primary dysmenorrhea is 75%. ocp use and previous vaginal
deliveries associated with less dysmenorrhea. more common in women who smoke. severity is related directly to duration and amount of menstrual flow.

Pathogenesis

Related to prostaglandin F-2-alpha. there is an elevated level in the secretory endometrium. most of the release of prostaglandins during menstration occurs during the first 48 hours, which coincides with the greatest intensity of symptoms. nsaids inhibit prostaglandin synthetase and alleviate symptoms of dysmenorrhea.

Secondary Dysmenorrhea

1- cervical stenosis- may impede mentrual flow, causing an increase in intrauterine pressure at time of menses. there also
may be retrograde menstrual flow through the fallopian tubes
2- endometriosis- frequently associated with dyspareunia and infertility
- study from Japan found that prostaglandin in endometriosis implants was signifantly higher than in normal endometrium,
myometrium, fibroids and normal ovarian tissue.
3- pelvic inflammation- secondary to gonorrhea, chlamydia, or other infectious agents- may cause adhesion with healing.
4- pelvic congestion syndrome- results from engorgement of pelvic vasculature. pain is burning or throbbing in nature, worse
at night, and worse after standing. diagnosed by physical exam (vasocongestion with evidence of some uterine enlargement and tenderness), and by laparascopy (demonstrates congestion of the uterus and engorgement or varicosities of the broad ligament and pelvic side wall veins.
5- related to functional bowel disease

Treatment

-OCP's- decreased prostaglandin synthesis associated with the atrophic decidualized endometrium
-NSAIDs- propionic acid derivatives (ibuprofen, naproxen) and fenamates (meclofenamate, mefenamic acid, flufenamic acid)
-Prostaglandin synthetase inhibitors
-Laparascopy to rule out secondary dysmenorrhea

References

Menstrual disorders. In:Speroff L, Fritz MA. Clinical gynecologic endocrinology and infertility. 7th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2004:557-73.

Stenchever MA. Primary and secondary dysmenorrhea and premenstrual syndrome. In:Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:1065-78.



Penis Abnormalities

Hingson-Gates, Smallwood

Types

Hypospadias
-Congenital abnormality where the urethral opening is on the ventral surface of penile shaft.
-Results from incomplete development of the anterior urethra.
-1 in 250 male newborns.
-Most common congenital anomaly of the penis.
-Usually an isolated anomaly.
-On exam, an incomplete formation of the prepuce with characteristic excess dorsal hood is seen.
-Avoid circumcision.
-Surgery before 2 yo.

Penile torsion/curvature
Misalignment of penile skin in relationship to shaft and glans of penis.

Ventral curvature is known as chordee and is usually associated with hypospadias. Most rotations are less than 90 degrees and do not result in voiding or erectile dysfunction.

Phimosis/Paraphimosis
Phimosis is the inablility to retract the prepuce. Treat with corticosteroid cream.
Paraphimosis occurs when the foreskin is retracted behind the coronal sulcus and the prepuce cannot be pulled back over the glans. Prompt reduction with sedation or local anesthesia or rarely circumcision is required.

Microphallus
Normally formed penis that is at least 2.5 standard deviations below the mean in size.

Evaluation includes endocrine studies,karyotype, and MRI of the pituitary and hypothalamus.

Priapism 
Painful unremitting erection. Sickle cell disease is the most common cause with treatment directed to the sickle cell component and prompt corporal irrigation with alpha agrenergic agonasts. Surgical shunting is needed within 24-48 hours if priapism unresponsive to medical treatment. Regardless, impotence is not uncommon.

Meatal Stenosis
Acquired condition after neonatal circumcision. Hydronephrosis is rare. Treatment is ventral meatoplasty or meatotomy.


References

Anomalies of the Penis and Urethra. Chapter 536. pp 1812-1816.

Kidney and Urinary Tract. Chapter 21. pp 1739-1740.



DES Exposure

Eric Helms, Kiran Sigmon

DES Exposure

- DES is an orally active estrogen
- In the 1940’s it was used in patient’s with a history of pregnancy loss
- DES exposure in utero is a risk factor for:
  1. clear cell adenocarcinoma of the vagina and cervix
  2. non-malignant like vaginal adenosis, cervical ectropion, structural cervical abnormalities (cervical collar, “cockscomb cervix”), change in the shape of the endometrial cavity
Exam
- Routine exam along with careful inspection and palpation of the vagina and cervix. Cytologic samples may be taken from the vagina, ectocervix, and endocervix. Possibly colposcopy.
- Colpo is without value in detecting clear cell adenocarcinoma (no unique patterns)
- Transformation zone usu. Extends into the vagina
- Double the incidence of CIN 2-3. Higher with earlier in utero exposure

Clear Cell CA of the vagina and cervix
- 60% are assoc. with DES exposure
- ~1/1000 lifetime risk
- Age of Dx b/t 7 and 48
- Clear Cell CA in DES exposed individuals has a better prognosis than in DES unexposed persons

References

Herbst AL. Adult sequelae of fetal exposure to diethylstilbestrol (DES). In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:399-412.

Teratology. ACOG Educational Bulletin 236. American College of Obstetricians and Gynecologists. Washington (DC): ACOG, 1997:1-7.



HIV Drugs

Meredith Mitchell, MD, Carol Coulson, MD

There are risks and benefits to using antiretroviral drugs in pregnancy. Because the benefits outweigh the risks, there is an even lower threshold for starting meds in pregnant women than in non-pregnant women. Normally, HAART (highly active antiretroviral therapy) is started for a CD4 count < 350 or viral load > 30, 000 copies. However, therapy in pregnant women is recommended for a viral load > 1000 copies (the level above which ACOG recommends elective C Section). Decreased rates of vertical transmission have been demonstrated with antiretroviral therapy even when viral loads are undetectable. Essentially, there is not an HIV RNA threshold below which the absence of transmission can be guaranteed. Studies are currently being done to determine if HAART is needed or if monotherapy with AZT is sufficient. Many forms of HAART are used, although the most effective combinations are believed to be two nucleoside reverse transcriptase inhibitors and either a protease inhibitor or a non-nucleoside reverse transcriptase inhibitor. The benefits to antiretroviral drugs in pregnancy are simple but important—decreased vertical transmission and a healthier mother.

The risks vary with each class of drug:

1) Nucleoside reverse transcriptase inhibitors (NRTIs)- These are the first class of drugs used to treat HIV, thus provider experience is widespread. They are generally well-tolerated and not teratogenic. ZDV belongs to this class of meds. Fetus—No teratogenic effects identified. Possible link with infant mitochondrial dysfunction and febrile seizures. Mother—Can cause mitochondrial dysfunction because of inhibition of mitochondrial gamma DNA polymerase. Dideoxycytidine, dideoxyinosine, stavudine and amivudine are the most likely NRTI’s to cause this complication, with the combination of stavudine and dideoxyinosine being the most dangerous. Problems associated with mitochondrial toxicity include neuropathy, myopathy, cardiomyopathy, pancreatitis, and lactic acidosis. Some women have died from lactic acidosis and liver failure. This usually occurs late in pregnancy and can be very similar to acute fatty liver of pregnancy (which is thought to be caused by mitochondrial fatty oxidation disorders in the fetus--isn't biochemistry cool!). Metabolic changes in late pregnancy are thought to predispose pregnant women to this rare side effect. Stavudine and dideoxyinosine should not be used together in pregnancy. LFTs should be monitored and any symptoms of hepatic dysfunction should be evaluated carefully. If LFT’s are rising and no other explanation exists, stop ALL antiretrovirals.

2) Non-nucleoside reverse transcriptase inhibitors (NNRTIs)- This is a newer class of drug and not as much information exists on potential problems with exposure during pregnancy. Some studies have shown that they may be teratogenic, especially efavirenz. A study of 40 cynomolgus monkeys demonstrated increased rates of micro/anopthalmia, anencephaly, and cleft palate. No increase in birth defects was noted in rats or rabbits. In the Antiviral Pregnancy Registry, only 24 cases of efavirenz use in pregnancy are recorded and none of these infants had birth defects. One child with a myelomeningocle and maternal efavirenz use has been reported in the literature. If efavirenz is required for a maternal indication, it should be used, but probably not in the first trimester.

3) Protease inhibitors (PIs)- No teratogenic effects have been reported; however, indinivir may predispose to maternal nephrolithiasis and hyperbilirubinemia. In general, PI’s have been linked to altered glucose metabolism, but this is not thought to worsen in pregnancy.

Summary of Recommendations

1) For viral load > 1000 copies, start HAART once the first trimester is completed

2) If a women is on HAART before conception and is stable with a low viral load, consider stopping meds in the first trimester

3) Avoid stavudine and dideoxyinosine together in pregnancy

4) Avoid efavirenz in the first trimester

References
Efavirenz. In: Briggs GG, Freeman RK, Yaffe SJ, eds. Drugs in pregnancy and lactation. 6th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2002:462-4.

Minkoff HL. Human immunodeficiency virus. In: Creasy RK, Resnik, R, Iams JD, eds. Maternal-fetal medicine. 5th ed. Philadelphia (PA): WB Saunders, 2004:803-13.


Diabetic Retinopathy


DM is the leading cause of blindness in persons 20-74.

Diabetic Retinopathy is the pathologic proliferation of retinal blood vessels as a result of retinal nonperfusion.

DR progresses in a stepwise fashion. The stages, in order are:

Mild nonproliferative diabetic retinopathy(NPDR)- characterized by microaneurysms, blot hemorrhage, “hard” exudates, “cotton-wool spots”. Visual acuity is unaffected.

Moderate NPDR- intraretinal microvascular abnormalities. 30% progression to high risk PDR over 5 yr.

Severe NPDR- progressive retinal capillary loss and ischemia. 60% risk of progression.

Proliferative diabetic retinopathy- neovascularization. In high risk PDR invasion of the vitreous causes contractures, fibrosis, and eventual retinal detachment.

Management
-tight glycemic control
-control of associated risk factors for retinal damage (htn, hyperlipidemia, nephropathy, careful f/u during pregnancy where accelerated retinal pathology may occur)
-photocoagulation- aimed at slowing progression. Visual acuity is difficult to recover.
  • because of the risk of acceleration of retinopathy during pregnancy, all diabetics should have an eye exam in the 1st trimester.
References

Folkman J. Angiogenesis. In: Braunwald E, Fauci AS, Kaspar DL, Hauser SL, Longo DL, Jameson JL, eds. Harrison's principles of internal medicine. 15th ed. New York (NY): McGraw-Hill Medical, 2001:517-30


Sherwin RS. Diabetes mellitus. In: Goldman L, Ausiello D, eds. Cecil textbook of medicine. 22nd ed. Philadelphia (PA): WB Saunders, 2004:1424-52



Infertility/Semen

Liliana Belskus MD, Kiran Sigmon MD

Definitions
· Infertility: Inability of a couple to conceive after one year of regular unprotected intercourse. Infertility is the decrease in the ability to conceive.
· Fecundability: probability of conceiving within one year.
· Sterility: intrinsic inability to achieve pregnancy.

Incidence
· In 1995, 10% of all US couples with women in the reproductive age were infertile.
· Increased to 14 to 28% in women 30 to 44 yo.
· Sterility affects 1-2% of all couples.
· 50% of fertile couples attempting to conceive will conceive within 3 months of unprotected intercourse, 75% within 6 months and 90% within one year.

Causes of Infertility
· 10-15% due to anovulation
· 30-40% pelvic factors such as adhesions, endometritis, infection, tubal occlusion
· 30-40% male factor including oligozoospermia, high semen viscosity, low sperm motility and low semen volume
· 10-15% cervical factor, abnormal sperm-cervical mucus penetration.
The primary diagnostic tests for infertility are documentation of anovulation, semen analysis and hysterosalpingogram.

Semen Analysis
· Sample should be collected after at least three days of abstained ejaculation.
· Sample is kept at body temperature and analyzed within one to two hour of collection.
· Parameters to evaluate include volume, viscosity, sperm density, morphology and motility.
· Sperm motility is evaluated in terms of percentage motility and quality of movement, rapidity of motility and amount of progressive movement.
· If an abnormality is found the analysis should be repeated on two or three occasions at least one month apart.
About 75% of all men that achieve conception will have at least one abnormal characteristic and 25% have at least two abnormalities.

Normal semen values
pH: 7.2-7.8
Volume: equal or > 2.0 ml
Total sperm count: equal or > 40 millions/ml
Sperm motility: equal or > 50% with progressive movement
Sperm morphology: equal or > 50%
WBC: < 10 million/ml

Causes of Semen abnormalities
· Abnormal count
- Azoospermia: Absence of sperm in semen, seen in Klinefelter’s syndrome, Sertoli-cell-only syndrome, varicocele
- Oligospermia: fewer than 20 million sperm per millimeter of semen e.g. endocrinopaties, varicoceole or other anatomic abnormalities
· Abnormal volume
- No ejaculate: maybe caused by obstruction of ejaculatory ducts, retrograde ejaculation, hypogonadism
- Low volume: obstruction of ducts, absence of seminal vesicles and vas deferens, partial retrograde ejaculation.
- High volume: unknown reasons
· Abnormal motility: immunologic reasons, infections, defect in sperm anatomy and metabolism.
· Abnormal Viscosity: Etiology unknown.
· Morphology: Abnormalities cause by varicoceles, stress, infection, unknown factors.

Options for Management of male infertility
-Consider referral to Urology for examination. If varicocele is present and corrected, it may prevent worsening of semen analysis.
· Intrauterine insemination (IUI); use to treat oligospermia, abnormalities of semen volume, viscosity. Intrauterine insemination of sperm after preparation of semen by “washing” and centrifugation (the swim-up technique) together with ovulation stimulation has a pregnancy rate of 10% per cycle.

· Intracytoplasmic sperm injection (ICSI) It is use in combination with IVF after several cycles of IUI have failed, if there are marked abnormalities in sperm, if there is less than one million motile sperm count after separation or if donor sperm is being used. Fertilization rate of oocytes injected with sperm from a sample with several abnormalities is about 50%. Pregnancy rate per embryo transferred is 35%.

· Testicular sperm extraction (TESE) use in combination with ICSI and IVF has 50% fertilization rate

· Microsurgical epididymal serum aspiration (MESA) use in combination with ICSI and IVF

References

Eskandari N, Cadieux M. Infertility. In: DeCherney AH, Nathan L, eds. Current obstetrics & gynecology diagnosis & treatment. 9th ed. New York (NY): Lange Medical Books/ McGraw-Hill, 2003:979-90

Mishell DR Jr. Infertility. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:1169-216



Adult Respiratory Distress

Reta Graham, Ursula Harkness

Pulmonary complications

  • most frequent post op morbidity following gyn operative procedures
  • directly cause 25% of deaths in women who die during first 7 post op days
  • include bronchospasm, atelectasis, pneumonia, exacerbation of underlying lung disease, respiratory failure
  • atelectasis makes up 90% of total pulm post op complications

Atelectasis

  • Severity ranges from lack of expansion of group of terminal bronchioles/alveoli to complete collapse of lung
  • Failure to maintain patency of small airways/alveoli
  • Most common cause of post op fever
  • Usually resolves spontaneously by post op day 3-5

Pathophysiology

  • Immediately post op decrease in functional residual capacity (FRC) and lung compliance-->increase in work of breathing
  • Progressive atelectasis (small airways remain closed due to mucus plugs/bronchospasm and gas distally to obstruction is absorbed causing further collapse)
  • Decrease in O2 saturation and in PO2--->VQ mismatch
  • Reduced ventilation/perfusion ratios--->gas trapping/atelectasis/vascular shunting

Risk factors

  • Nonpulmonary: Supine position, obesity, breathing at low lung volumes (shallow chest wall breathing) due to incisional pain, narcotics, immobility
  • Pulmonary: interstitial edema, loss of surfactant, airway obstruction (inflammation, secretions)

Physical Exam

  • Triad of fever, tachypnea, tachycardia w/i first 72 hrs post op
  • Decreased breath sounds, inspiratory rales at bases
  • Productive cough, leukocytosis
  • CXR may have patchy infiltrate with diaphragm elevation

Treatment

If unresolved with conservative measures in appropriate time period, institute chest PT, intermittent positive pressure breathing, aerosol therapy or intermittent continuous positive airway pressure by mask

Prevention is key: PRE OP
  • Predict post op pulmonary/cardiac problems
  • Pre-op H & P is most important part of pulmonary eval (smoking, recent URI, productive/chronic cough, dyspnea, wheezing, EXERCISE TOLERANCE, med hx if known disease)
    • Smoking or asthma increase incidence of periop resp morbidity 4-fold
    • STOP SMOKING preop (8 weeks ideally, more likely 2-4 wks)
    • Antibiotics/delay surgery if resp infxn present
  • Pulmonary work-up indicated if hx/physical suggests restrictive/obstructive disease (chronic productive sputum, asthma, COPD) >65 yo, > 20 pack-year smoking hx, morbid obesity
    • CXR, ABG, PFT's
    • Exercise tolerance is crude test of pulmonary fcn
Think about suppression of hypothalamic/pituitary axis function if daily low dose oral/inhaled corticosteroids during past 6-9 mos.
Patient should receive IV hydrocortisone periop for potential adrenal insufficiency.


POST OP
  • Encouragement of uneven ventilation (i.e. “Ma’am, get your butt out of bed”) by walking, taking deep breaths, coughing, turning from side to side, remaining semierect rather than supine
  • Early mobilization/ambulation as effective as chest PT to prevent pulm complications
  • Pain control, bedside incentive spirometry

Pneumonia

  • Commonly associated with atelectasis post-operatively
  • Bacterial infections in collapsed areas of lungs (inhaled mucus/bacteria of mouth)
  • Predisposing factors
    • COPD
    • Smoking
    • Alcohol abuse
    • Obesity
    • Advanced age
    • NG tubes
    • Long operations
    • GN bacterial infections
    • Post op peritonitis
    • Debilitating illnesses
  • Signs/symptoms = fever, cough, dyspnea, tachypnea, purulent sputum, coarse rales over infected area
  • Higher temp/more toxic appearing than in atelectasis
  • CXR ~60% sensitivity for diagnoses
  • Management similar to that of atelectasis, BUT add IV antibiotics (based on G stain/sputum cx)

Acute Respiratory Distress Syndrome

  • Complex devastating clinical problem following acute lung injury
  • Need multidisciplinary team to manage
  • Recent clinical changes in vent settings using lower tidal vol have decreased mortality by 22%

Indications for Mechanical Ventilaton 

  • Acute increase PaCO2 to >50 mm Hg with a decrease pH to <7.30
  • RR >35 breaths/minute for prolonged period
  • Acute hypoxemia (PaO2<60 mm Hg or SaO2<90%)

References
Critical care and trauma. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:1159-80.

Luce JM. Ventilator management in the intensive care unit. In: Goldman L, Ausiello D, eds. Cecil textbook of medicine. 22nd ed. Vol. 1. Philadelphia (PA): Saunders, 2004:602-8.


Hypocalcemia

Summer Gilmer, MD, James Smallwood, MD

Definition
Serum calcium less than 7 mg/dl, is one of the major metabolic derangements in infants of diabetic mothers.

Incidence
Reported in up to 50% of insulin-dependent diabetics during the first 3 days of life. more recent studies report an incidence of
5% or less with better managed diabetics.

Proposed etiologies
- decreased parathyroid levels result in decreased calcium levels and increased phosphorus levels.
- hypoparathyroidism thought to be related to prematurity, birth asphyxia or suppressed parathyroid function secondary to hypercalemia in utero.
- hypersecretion of calcitonin

References
Moore TR. Diabetes in pregnancy. In:Creasy RK, Resnik R, Iams JD, eds. Maternal-fetal medicine: principles and practice. 5th ed. Philadelphia (PA):WB Saunders, 2004:1023-62.

Diabetes. In: Cunningham FG, Gant NF, Leveno KJ, Gilstrap LC III, Hauth JC, Wenstrom KD. Williams obstetrics. 21st ed. New York (NY): McGraw-Hill, 2001:1359-83.


Insurance

Goodwin, Imseis

Types of malpractice liability insurance

Claims Made vs Occurrence

Occurrence
  • Occurrence policy covers an insured for incidents that occur while the policy is in effect, regardless of when the incident is reported to the insurer.
  • The date the claim occurred determines the applicable policy period.
  • Most policies are for one year and they can have different material terms and coverage limits each year.
  • It is important to keep these policies on file for when they are needed.
  • NO TAIL COVERAGE NEEDED (see below)

Claims Made
  • Claims made policy covers an insured for incidents that both occur and are reported to the insurer while the policy is in force.
  • This can be burdensome since claims can be made during one policy but not reported until another is in effect.
  • If this happens, the coverage may not be adequate.
  • Retroactive date is a date before which the policy will not cover, even if the claim is made during the policy period… (earlier retroactive date usually = higher premium)
  • TAIL COVERAGE IS NEEDED to provide coverage when changing providers, retiring etc for future claims made during earlier coverage periods.
At all times, changes or new coverage needs need to be requested by the physician in writing.

References

Risk Management handbook for health care organizations. Roverta Carroll, ed – 4th ed. Chapter 22: Insurance: Basic principles and coverage’s. Jossey-Bass Inc. 2004.

Law for physicians: An overview of medical legal issues. Chapter 4: Claim protection: Selecting the right malpractice liability insurance.



Hereditary Familial Nonpolyposis Colon Cancer (HNPCC)

LaDene King, Currens

Women in families with the syndrome of HNPCC are also at elevated risk for endometrial and ovarian cancers. HNPCC is an autosomal dominant tendency to develop colon & other cancers. It causes usually right-sided colon cancer at young ages. Adenomatous polyps are seen, but florid polyposis is rare. Among women in these families, endometrial cancer is the 2nd most frequent site of cancer (lifetime risk is 40-60%). The responsible genes are for repair of mismatched DNA (HMSH2, HMLH1, HMSH6). Mutations in HMSH2 & HMSH6 are more often implicated in endometrial cancers.

The inability to provide effective DNA mismatch repair during replication leads to the accumulation of genetically altered material in the genome. If these mutations occur in the oncogenes or tumor suppressor genes, aberrant cell growth will result.

Tumors from HNPCC individuals demonstrate microsatellite instability (a feature of tumors that are genetically unstable, associated with error-prone DNA replication during cell division). The mutations may be germline (inherited) or somatic (restricted to tumor tissue only). If the mutation is germline, genetic counseling is indicated.

Individuals with inherited mutations are also at risk for ovarian, gastric, urologic tract, and small bowel cancers. The International Collaborative Group on HNPCC recommends annual endometrial ultrasound surveillance.
Preventive hysterectomy may be warranted.

References

Narod SA. Clinical genetics of gynecologic cancer. In: Hoskins WJ, Perez CA, Young RC, Barakat RR, Markman M, Randall ME, eds. Principles and practice of gynecologic oncology. 4th ed. Philadelphia Q261 (PA): Lippincott Williams & Wilkins, 2005:33-8.

Herbst AL, Waggoner SE. Immunology and molecular oncology in gynecologic cancer. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:843-56.



Galactorrhea

Liliana Belskus MD, James Smallwood MD

- Galactorrhea and amenorrhea are the predominant symptoms of prolactinomas.
Oral contraceptives (Oc) mask these symptoms.

- Amenorrheic women should not receive OCs until the diagnosis of these symptoms is established.

- If galactorrhea occurs while on OCs,
  1. Oc’s should be discontinued
  2. Serum prolactin level should be measured 2 weeks later
  3. If prolactin found to be elevated, further diagnostic studies are indicated.
- Prolactin secreating macroadenoma but not microadenoma is a contraindication for Oc use.

- Oc don’t cause enlargement of prolactin secreting pituitary microadenomas or worsen functional prolactinomas.

- Data from three studies indicated that the incidence of pituitary adenomas among users of OCs is not higher than among matched controls.

References

Mishell DR Jr. Oral contraceptives: indications, contraindications, formulations, monitoring. In: Mishell DR Jr, Goodwin TM, Brenner PF, eds. Management of common problems in obstetrics and gynecology. 4th ed. Malden (MA): Blackwell, 2002:445-9.


Mishell DR Jr. Family planning. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:295-358.



SLE

Tori O'Daniel, Carol Coulson

Definition

Multisystemic autoimmune disease of unknown etiology in which tissues and cells are damaged by autoantibodies and immune complexes. It can be chronic or of relapsing and remitting form; it has serious musculoskeletal, renal and cardiovascular effects.
Classification of Lupus: must have atleast 4 of 11 criteria serially or simultaneously:
Malar Rash
Discoid Rash
Photosensitivity
Oral ulcers
Arthritis
Serositis-pleuritis or pericarditis
Renal disorder- persistent proteinuria or cellular casts
Neurologic disorder- seizures or psychosis
Hematologic disorder- hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia
Immunologic disorder- anti DNA, anti-Sm, anticardiolipinanribodies, lupus anticoagulant, or false positive serologic test for syphilis
Antinuclear antibody

Screening tests
Antinuclear antibodies(ANA), but not specific for lupus.
Prevelance
Almost 90% of cases are in women, prevelance in child-bearing aged women is ~1-500.
Relative Risk of disese is increased threefold if HLA-DR2 or DR3 genes are found.

Medical Complications of Pregnancy with Lupus
  • Factors that determine effects of lupus on pregnancy include disease state at beginning of pregnancy, age and parity, co-existence of other medical or obstetrical disorders and antiphospholipid antibodies. The rate of flares is lower if the disease is under good control for at least 6 months prior to conception, If SLE nephritis is present, better outcomes result with Serum creatinine no more than 1.5mg/dL, proteinuria less than 3g/24hr. and BP well-controlled
*Lupus flares during pregnancy range from 15-60%, and may occur during any trimester, including postpartum. Flares are difficult to detect with biochemical tests, and they can occur rapidly.
  • Mild flares affecting skin and joints don't have as much of an adverse effect on maternal-fetal outcomes as flares affecting the kidney or heart. .
Complications during the pregnancy and postpartum period
  • Pre-Eclampsia is common especially in women with nephropathy and can be very difficult to differentiate from a lupus flare. However, clinical features of Pre-E run a much more acute course, remit after delivery, and are not associated with the other SLE-features. In addition, complement levels decrease during a lupus flare but are often unchanged in preeclampsia
Treatment (corticosteriods, immunosuppressants)
Arthralgia and serositis are managed with NSAIDS, including ASA. Therapeutic doses shouldn't be continued after 24weeks due to premature closure of FDA. Low dose ASA (60-80 mg) can be used throughout pregnancy to manage antiphospholipid antibodies.
Antimalarials inhibit platelet aggregation and reduce serum lipids helping with inflammation, skin disease, alopecia etc. Hydroxychloroquine (plaquenil) seems to be safe for the fetus.
Life threatening manifestations (nephritis etc.) are managed with corticosteroids ie. prednisone (1-2mg/kg/day, but tapered to 10-15mg/day after disease is controlled).

BE AWARE: GDM or IDDM can develop with this treatment.

Most authorities recommend continuation of immunosuppressive therapy during pregnancy in women with nephritis to avoid end-stage renal disease; it's unclear if dosage should be changed. Azathioprine may be used during pregnancy but not during lactation.

Risks to the fetus
Pregnancy loss is increased especially with Antiphospholipid syndrome, lupus anticoagulant and beta 2 glycoprotein antibodies.

Screen for anti-SS-A (Ro) and SS-B (La) antibodies, and if found search for NEONATAL LUPUS SYNDROME: cardiac dysfunction ie. Congential Heart Block and arrhythmias, cardiomyopathy, cutaneous lupus lesions, hepatobiliary disease and thrombocytopenia. This is caused by the transplacental transfer of the maternal ABs (anti-SSA/Ro). No definite treatment is available prenatally to prevent this disease; however, some studies suggest the use of maternal dexamethasone therapy to help prevent and/or treat CHB. The hematologic abnormalities are usually transient because the maternal IgG antibodies don't persist for long in the neonate after delivery.

IUGR and PPROM are increased as a result of HTN and renal compromise. Decidual vasculopathy with placental infarction and decreased perfusion are commonly seen.

Postpartum
Maintain close clinical surveillance of pt. postpartum because flares can occur after delivery.

Avoid oral contraceptives with synthetic estrogens in women with active lupus nephritis or high levels of Antiphospholipid antibodies.

References

Hankins GDV, Suarez VR. Rheumatologic and connective tissue disorders. In: Creasy RK, Resnik R, Iams JD, eds. Maternal-fetal medicine: principles and practice. 5th ed. Philadelphia (PA): WB Saunders, 2004:1147-64.

Connective-tissue disorders. In: Cunningham FG, Gant NF, Leveno KJ, Gilstrap LC III, Hauth JC, Wenstrom.



Delayed Puberty

Nathan Mullins, MD, Beth Buys, MD

Background
  • 1st sign of puberty is breast budding or Thelarche, mean age 10.8 yrs
    • requires exposure to estrogen
  • Menarche is the latest sign of puberty, mean age 12.9 yrs
  • It is considered primary amenorrhea if no menarche by 16.5 yrs
Puberty Physiology
  • prior to puberty, LH and FSH levels are low b/c CNS-hypothalamic axis is very sensitive to negative feedback effects of low levels of estrogen
  • at a critical body weight or body composition the CNS-hypothalamic axis becomes less sensitive this negative feedback from estrogen
  • GnRH secretion is increased leading to elevations of LH and FSH and onset of menses
Delayed Puberty
  • it is believed that body composition is determinant of onset of puberty and menstruation
  • ratio of fat to total body weight and lean body weight is important
  • it is known that obese individuals have earlier onset of menarche than nonobese women
  • malnutrition, as with anorexia nervosa or starvation, is known to delay onset of puberty
  • decreased body fat in individuals who perform strenuous exercise has been shown to delay puberty
Reference

Mishell DR Jr. Primary and secondary amenorrhea. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:1099-123.

Shilling SWJ, McClamrock H. Amenorrhea. In: Berek JS, Hillard PJA, Adashi EY, eds. Novak's Gynecology. 13th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2002:843-69.



Toxic Shock Syndrome

Helms, Currens

Pathogenesis
TSS is associated with a Staph Aureus exotoxin. This is usually TSST-1. Enterotoxin B is the second most common. TSS is a Staphylococcal “intoxication.”

For illness to occur the patient must be colonized/infected with a toxigenic strain of S. aureus and lack antibody to the toxin.

Clinical Findings
- related to menses in 50% of cases
- fever, myalgias, rash, vomiting, diarrhea, multiorgan dysfunction, and desquamation
- change tampons q 4-8 hr to prevent
- It is rare to have TSS in an individual with antibodies to TSST-1

References

Parsonnet J, Deresiewicz RL. Staphylococcal infections. In: Braunwald E, Fauci AS, Kaspar DL, Hauser SL, Longo DL, Jameson JL, eds. Harrison's principles of internal medicine. 15th ed. Vol. 1. New York (NY): McGraw-Hill Medical, 2001:889-900.

Hillard PJA. Menstruation in young girls: a clinical perspective. Obstet Gynecol 2002;99:655-62.



Anemia

Karie LaFleur, MD, Ursula Harkness, MD

Definition

Less than 11 g/dL first and third trimester, less than 10.5 second trimester (CDC 1990). Less than 12 g/dL in a nonpregnant woman.

Normal pregnancy
Modest fall in Hgb due to relatively greater expansion of plasma volume compared to increase in Hgb mass and red cell volume

Effects on Pregnancy
Implicated as contributory in up to 40% of maternal deaths in third-world countries. Some evidence that women with anemia at 12 weeks of gestation have 1.7 fold risk for preterm birth.

Types of Anemia

Iron-deficiency anemia
One of most common causes of anemia, ~8 million women of childbearing age in US are iron deficient. Poor nutrition overall plays a role, as does the increased need for iron during pregnancy that is not compensated for in the average diet. Expansion of blood volume in the second trimester without normal expansion of Hgb mass. Newborn infant of a severely anemic woman does not suffer iron deficiency because the amount of iron transferred to the fetus is the same.
Dx:erythrocyte hypochromia and microcytosis, although it is less prominent in pregnant women, and low serum ferritin
Tx: Oral iron supplementation, continue 3 months after the anemia is corrected to rebuild iron stores

Anemia from Acute Blood Loss
Once hypovolemia has been overcome the residual anemia should be treated with iron. If Hgb >7 g/dL, condition stable, no likelihood further hemorrhage, afebrile, asymptomatic -> iron tx for 3 mos

Anemia of Chronic Disease
During pregnancy common causes are chronic renal disease, IBD, SLE, malignancies, and RA. Acutely, severe pyelonephritis can cause overt anemia.
Tx: recombinant erythropoietin will increase red cell mass but can cause HTN

Megaloblastic Anemia
Characteristic blood and bone marrow abnormalities caused by impaired DNA synthesis. Rare in US during pregnancy but present in other parts of the world. There are 2 types:
Folic Acid Deficiency- Usually in women who don't consume green leafy vegetables or animal protein. Will develop nausea, vomiting and anorexia during pregnancy. Ethanol ingestion can be the cause or contribute to the progress. Folic acid requirement in pregnancy is 400 ug/day. Fetus extracts folate from mother and will NOT be anemic even if mother is severely anemic.

Dx: low plasma folic acid, hypersegmented neutrophils, macrocytic erythrocytes, nucleated RBCs, megaloblastic erythropoiesis in the bone marrow, eventually thrombocytopenia and leukopenia occur

Tx: 1mg/day of folic acid, nutritious diet and iron supplementation

Prevention: diet and folic acid supp, additional folic acid in situations of inflammatory bowel dx, sickle-cell dx, anti-seizures medications, multifetal pregnancy, prior NTD
Vitamin B12 Deficiency - extremely rare, usually only in cases partial or complete gastrectomy, give 1000mg of cyanocobalamin IM monthly (total gastrectomy)

Autoimmune Hemolytic Anemia
Caused by "cold-active antibodies" or "warm-active antibodies". Will have marked acceleration of hemolysis during pregnancy. IgM antibodies do not cross placenta so fetal red cells not effected. However, IgG antibodies do cross and a maternal D isoimmunization may result with hemolytic disease in the fetus.
Dx: spherocytosis, reticulocytosis, pos direct and indirect Coombs test and antiglobulin tests.
Tx: Prednisone 1 mg/kg per day

Drug-Induced Hemolytic Anemia
Generally mild and resolves when drug is removed. Caused by antidrug antibodies or anti erythrocyte antibodies. Severity of sx depend on degree of hemolysis. Consider glucose-6-phosphate dehydrogenase deficiency in AA women.
Dx: Spherocytosis, reticulocytosis, positive direct antibody test
Tx: withdraw offending drug

Paroxysmal Nocturnal Hemoglobinuria
Hemopoietic stem cell disorder that can be the result of X-linked gene mutation of PIG-A. This causes defective anchor proteins of erythrocytes and granulocytes that makes them susceptible to destruction by complement. Hemolysis initiated by stress like transfusion, infection or surgery. Hemoglobinuria develops at irregular intervals. Complications: venous thrombosis including Budd-Chiari syndrome, renal abnml, htn, maternal death and severe complications related to hemolysis and hemorrhage.

Other acquired anemias include HELLP syndrome.

Hereditary Spherocytosis
Membrane protein deficiencies caused by mutations of spectrin, ankyrin or protein 4.2. Results in various degrees of anemia and jaundice from hemolysis. Women do well in pregnancy. Folic acid supp recommended. Infants may or may not have elevated bilirubin.
Dx: spherocytes on smear, reticulocytosis, and increased osmotic fragility, enlarges spleen

Red Cell Enzyme Deficiencies
Glucose-6-phosphate dehydrogenase deficiency is most common (X-linked). Anemia is episodic and stabilizes with withdrawal of the drug or treatment of infection.
Pyruvate kinase deficiency is autosomal recessive disorder. Hydrops fetalis can occur in homozygously affected fetuses.

Aplastic and Hypoplastic Anemia
Functional defect in the number of committed marrow stem cells. 1/3 of cases the anemia is stimulated by drug, chemical, hereditary condition, etc. 2/3 of cases no cause can be determined. With severe dx, 1 year survival rate is 20%. 1/3 of women with aplastic anemia in pregnancy improved with termination of the pregnancy, possible induction of erythroid hypoplasia in pregnancy. Hemorrhage and infection are the 2 greatest risks to pregnant women with aplastic anemia

Dx: anemia, thrombocytopenia, leukopenia, hypocellular bone marrow

Tx: antithymocyte globulin, cyclosporine, possibly corticosteroids and androgenic steroids (female fetus likely to become masculinized), bone marrow transplantation in severe disease

References

Hematological disorders. In: Cunningham FG, Gant NF, Leveno KJ, Gilstrap LC III, Hauth JC, Wenstrom KD, eds. Williams obstetrics. 21st ed. New York (NY): McGraw-Hill Medical, 2001:1307-38.

Kilpatrick SJ, Laros RK Jr. Maternal hematologic disorders. In: Creasy RK, Resnik R, Iams JD, eds. Maternalfetal medicine. 5th ed. Philadelphia (PA): WB Saunders, 2004:975-1004.



Osteoporosis

LaDene King, Sigmon

Risk Factors for developing post-menopausal osteoporosis:
Non-modifiable: white or Asian race, family history of osteoporosis, dementia, poor general health. Genetics is single largest factor influencing a woman’s max bone mass.
Modifiable: low dietary calcium intake, low vitamin D intake, high caffeine intake, high alcohol intake, cigarette smoking, chronic corticosteroid use, low body weight (<127 lbs), early menopause (<45yo), premenopausal amenorrhea (>1yr), sedentary lifestyle, fall risks.

How does estrogen prevent bone loss? Postmenopausal estrogen deficiency leads to increased bone resorption; however, rate of bone formation is unchanged.
Postmenopausal serum: calcium & phosphorus slightly increased; PTH & active Vitamin D decreased; calcium absorption decreased; calcitonin levels lowered.
PTH increases serum calcium in 3 ways: 1. bone resorption, 2. renal tubular resorption of calcium in kidney, and 3. production of 1-alpha-hydroxylase (changes vitamin D from inactive to active), which increases calcium absorption from gut.

Maybe sex steroids (including estrogen, androgens, and progestins) block the action of PTH on bone.
Osteoblasts have estrogen receptors.
Estrogen increases calcitonin levels.

Cortical vs Trabecular bone: Cortical, outer shell, 75% of total bone mass. Trabecular, spongy interlacing network of struts, 25% of total bone mass, most of volume in bone. Trabecular bone is most likely to show early bone loss, first to show response to therapy.

How do we find osteoporosis? Dual-energy x-ray absorptiometry (DEXA) scans of lumbar spine or hip.

Osteopenia
T score of -1 to -2.5.
Osteoporosis
T score less than or equal to -2.5.

National Osteoporosis Foundation recommends: Bone Mass Density on >=65yo, postmenopausal women <65yo with one risk factor, postmenopausal women w/ fractures, women taking postmenopausal estrogen for prolonged periods. If no new risk factors, screen no sooner than 2 years.

Other testing methods: Peripheral bone densitometry - may be helpful as screening tool but not to replace DXA; scores do not correlate with DXA T-scores. Quantitative ultrasonography – needs further clinical experience & research. Peripheral quantitative CT – expensive, more radiation; data doesn’t correspond well with fracture. Biochemical markers – cannot diagnose osteoporosis, predict bone density, or predict fracture risk.

When do you start pharmacologic therapy? Tscore <-2 without risk factors, T score
<-1.5 with risk factor(s).

Estrogen therapy reduces the amount of postmenopausal bone loss as well as the incidence of fracture in the hip and spine. The greatest benefit of estrogen replacement therapy occurred when it was initiated early in menopause & diminished if initiated >5yrs after menopause. Minimal dose: 0.625mg conjugated equine estrogen or estrone sulfate or 0.5 mg of micronized estradiol. Bone loss can be prevented with 0.3mg of conjugated equine estrogen or esterified estrogen if woman also ingests 1500 mg of calcium daily. Transdermal 0.05 estradiol daily prevents bone loss.

Progestins may reduce rate of bone reabsorption by decreasing urinary calcium excretion.
May act synergistically with estrogen.

Dietary calcium supplementation without estrogen may decrease rate of decreasing bone density postmenopausally. Retards, but does not prevent bone loss (like estrogen can). Ingesting the RDA (1200-1500 mg/day) during adolescence leads to greater peak adult bone mass. Exercise in premenopausal years increases bone density.

1994 NIH consensus panel: postmenopausal women 50-65 years old should take in 1000mg ca daily (on estrogen) or 1500 mg (not on estrogen). Preferable to get calcium in diet. Also need adequate vitamin D (400-800IU/day). Foods rich in ca: milk, cheese, yogurt, ca-fortified orange juice, sardines w/ bones, pink canned salmon.

For women with contraindication for estrogen replacement who are losing bone or already have osteoporosis, use bisphosphonates to prevent bone resorption. Alendronate 5mg/day prevention, 10mg/day treatment. Risedronate 5mg/day prevention and/or treatment. To avoid esophageal irritation, ingest with 6-8oz water, 30 min before any other food or drink. Remain upright 30min after drug ingestion, and until food has been ingested to enhance absorption. Other treatment options: Nasal calcitonin 200 micrograms daily; Raloxifene (a SERM) 60mg daily; daily subcutaneous PTH (if other treatments fail).

References

Mishell DR Jr. Menopause. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:1217-58.

Osteoporosis. ACOG Practice Bulletin 50. American College of Obstetricians and Gynecologists. Obstet Gynecol 2004;103:203-16.



Patient Safety

Tori O'Daniel, Shelley Galvin

Quality Health Care: improve the care while controlling costs.

Reason to Improve patient safety:
44,000-98,000 preventable deaths may be due to medical error.

PAST ATTEMPTS: Risk Management: used retrospective analyses and addressed significant adverse events, but it focused specifically on legal issues and individual error. It would sanction a person for substandard care or procedures.

New Attempts: Patient Safety Initiatives: these analyses are used mostly by groups like JCAHO. They are prospective, interdisciplinary, and focus on health care processes in addition to outcomes. They recognize that most adverse outcomes are because of system deficiencies and not individual error thus system changes are necessary to prevent future problems. Examination of the entire process of care can be done using one technique: "Root-Cause Analysis."

Root-Cause Analysis: helps identify what, how and why something happened, thus preventing recurrence.

Definition of Root Causes

1. Specific underlying causes,

2. Resonably identifiable causes,

3. Causes that management has control to fix,

4. Causes for which effective recommendations for preventing recurrences can be generated.

Four Major Steps

1. Data collection;

2. Casual factor charting. - sequence diagram with logic tests that describes the events leading up to an occurrence, plus conditions surrounding the event;

3. Root Cause identification. - use a root cause map, this structures the reasoning process; helps investigators answer questions about why particular causal factors exist or occurred;

4. Recommendations generated and implemented.

Five principles to safer health systems

1. Organizational leadership must commit to safety by providing necessary personnel & financial resources.

2. The system design should recognize human limitations.

3. Working & training in teams improves safety & accountability.

4. Training should antcipate the unexpected.

5. The organizaiton should foster a learning environment for continual improvement of patient safety.

References

Gambone JC, Reiter RC, Gluck PA. Quality assessment, performance improvement, and patient safety. In: Berek JS, Hillard PJA, Adashi EY, eds. Novak's gynecology. 13th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2002:33-48.

Rooney JJ, Vanden Heuvel LN. Root cause analysis for beginners. Knoxville (TN): ABSG Consulting Inc.



Palpable Breast Mass

Nathan Mullins, MD, James C Smallwood, MD

Breast masses during pregnancy

  • 70-80% benign, but evaluation should not be deferred
  • most common problems secondary to milk stasis
    • duct obstruction – leads to ductal dilation, mass is painful
    • galactocele
      • occurs when an obstructed duct ruptures with subsequent cyst formation
      • obstruction relieved with heat, massage and continued milk evacuation
    • mastitis/abscess
      • bacteria introduced from infant suckling can proliferate in stagnant milk which acts as a great environment for growth
      • patient typically presents with fever, malaise, localized erythema, edema and pain

Benign Neoplasms

Lactating Adenoma
  • unique to the puerperium
  • present as enlarging, nontender, well-circumscribed masses that are firm to palpation
  • on U/S they appear solid, with well defined borders
  • treatment and diagnosis by excision
Fibroadenoma
  • presents as solitary, rubbery, firm well-circumscribed mass
  • hormone-sensitive tumors, so may grow during pregnancy
  • FNA or core biopsy to confirm diagnosis
Breast Hypertrophy
  • also known as gigantomastia, it is an uncommom and pathologic increase in breast size during pregnancy
  • breasts may enlarge to a point causing pressure necrosis of the overlying skin
  • usually self-limiting and resolves postpartum , but can recur in subsequent pregnancy
Breast Cancer
  • most common malignancy diagnosed during pregnancy
  • incidence of pregnancy-associated breast cancer is 1-7/10,000
  • typically presents as a self-detected painless mass -ultrasound and FNA are most useful initial studies
  • may also have other classic signs of malignancy; skin dimpling or retraction, axillary adenopathy and bloody nipple discharge
  • Excisional biopsy is the gold standard for diagnosis if FNA or core biopsy do not confirm clinical suspicion
  • Treatment options include modified radical mastectomy,lumpectomy,cytotoxic drugs and radiation therapy
Reference

Berman ML, Di Saia PJ, Tewari KS. Pelvic malignancies, gestational trophoblastic neoplasia, and nonpelvic malignancies. In: Creasy RK, Resnik R, Iams JD, eds. Maternal-fetal medicine. 5th ed. Philadelphia (PA): WB Saunders, 2004:1213-42.

Breast disease in pregnancy and lactation. In: Bennett BB, Steinbach BG, Hardt NS, Haigh LS, eds. Breast disease for clinicians. New York (NY): McGraw-Hill Medical, 2001:93-101.



Ovarian Hyperstimulation Syndrome (OHSS)

Karie LaFleur, MD, Allen Van Dyke, MD

Definition

OHSS is a medical condition that is completely iatrogenic and unique to the treatment of infertility. It is the enlargement of the ovaries to a diameter of more than 6 cm as a result of stimulation of multiple follicles. This leads to a local and systemic increase in capillary permeability and third-space fluid accumulation. This condition can cause life-threatening complications.

Incidence

The reported incidence of OHSS after controlled ovarian hyperstimulation (COH) varies from 4.2% to 33%.

Classification

Mild: mild abdominal distension and soreness, nausea and vomiting
Moderate: presence of abdominal ascites in addition to above sx
Severe: tense ascites, hydrothorax, SOB, hemoconcentration, hypercoaguulability or complications such as renal failure, thromboembolism, and ARDS

Mechanism

Associated with increasing numbers of stimulated follicles, PCOS and high levels of serum estradiol levels. Main cause of OHSS is increased production of vasoactive substances such as prorenin, renin, angiotensin-converting enzyme, AT I, AT II, and angiotensinogen by the hyperstimulated ovaries. Hyperestrogenemia, especially in the presence of hemoconcentration, has an impact on the thromboembolic risks present. Cytokines, histamine and prostaglandins also play a role. Vascular endothelial growth factor (VEGF) levels in the serum correlate with OHSS severity.

Hyperstimulation of the ovaries occurs most commonly after treatment with hMG (human menopausal gonadotropin) and FSH for infertility. Rarely does treatment with clomiphene citrate cause OHSS. A late-onset subset of OHSS is the result of multiple gestation and the endogenous hCG released.

Evaluation

Sx: abdominal discomfort, pain, bloating, early satiety, increased abdominal girth, N/V, CP, SOB, weight gain, decreased UOP
PE: Complete physical exam with exception of pelvic exam - enlarged ovaries are fragile!
Lab tests - CBC,CMP, PT, PTT. serum hCG
Other testing: CXR and O2 sats if indicated. TVUS to measure size of ovaries and document ascites.

Management

Most mild and moderate forms can be managed as outpatients. Whether inpatient or outpatient meticulous fluid balance monitoring is key. All patients with OHSS should be given heparin for prevention of thromboembolism. Paracentesis may be necessary for large amounts of ascites.

Prevention

If estradiol levels are >than 3,000 pg/ml before COH, OHSS prevention is attempted by a technique called "coasting". This involves withholding gonadotropins until estradiol decreases. Prezygote cryopreservation and in vitro maturation of oocytes has shown a decreased incidence of OHSS in women who have risk factors such as PCOS.

References

Yao MWM, Schust DJ. Infertility. In: Berek JS, Hillard PJA, Adashi EY, eds. Novak's gynecology. 13th ed. Philadelphia (PA): Lippincott Williams & Wilkins, 2002:973-1066.

Mishell DR Jr. Infertility. In: Stenchever MA, Droegemueller W, Herbst AL, Mishell DR Jr, eds. Comprehensive gynecology. 4th ed. St. Louis (MO): Mosby, 2001:1169-215.



Skin Disorders

LaDene King, Van Dyke

Papulosquamous Diseases

Psoriasis 

Multifactorial w/ genetic influence. Increased epidermal skin turnover. Immune regulation is heavy influence. Usually gradual onset, though infection may precipitate more explosive onset.

Lesions – erythematous papules & plaques surmounted by thick, silvery micaceous scales, not easily removed. Symmetric distribution, elbows & knees, trunk, penis, scalp, intergluteal cleft, umbilicus. Pitted or oil-stained nails. Psoriatic arthritis in <1/3 of patients.

Diagnosis primarily clinical, but biopsy can substantiate the diagnosis.

Differential includes tinea & onycholysis, seborrheic dermatitis, secondary syphilis, cutaneous T-cell lymphoma, Reiter’s syndrome, pityriasis rubra pilaris, pityriasis lichenoides et varioliformis acuta.

Nonpharmacologic therapy: avoidance of excessive alcohol, sun exposure, soaking baths followed by emollient use, stress reduction. Topical treatments: corticosteroids, tar, calcipotriol (a vitamin D derivative), topical retinoids, ultraviolet light therapy. Systemic therapies: methotrexate, systemic retinoids, cyclosporine.


Pityriasis Rosea 

Self-limited, young adults. Possibly viral (more common in winter, often after URI). Oval or round, tan-, ink-, or salmon-colored, scaling papules & plaques over trunk, neck, upper arms, and thighs. Eruption preceded by several days to weeks by a herald patch, commonly misdiagnosed as tinea corporis. Oval patches have unusual fine, white scale near border, forming a collarette. Lesions follow skin cleavage lines, leading to Christmas tree pattern.

Differential includes drug eruption, secondary syphilis.

Treatment: maybe nothing. Topical corticosteroids & antihistamines relieve itching, decrease erythema. UV light may clear it.


Lichen Planus 

Chronic, pruritic, wide range of clinical manifestations. An immune response to antigenic stimuli. Associated with hepatitis C.

Lesions – flat-topped papules, polygonal, with lilac or purple hue. Subtle, fine, white reticulated lines (Wickham’s striae) surmounting shiny flat tops of papules (resembling lichen), more apparent under hand lens. Typical distribution: symmetric papules on ankles, flexural wrists, mouth, genitalia. Mucous membranes commonly involved.

Differential includes discoid lupus erythematosus, drug-induced lichen planus-like eruption, graft versus host, candidiasis and/or mouth cancer.

Treatment: Topical steroids for localized patches. Oral corticosteroids for widespread disease. Intralesional steroid injections for erosive oral lichen planus. Refer to dermatology for widespread or erosive painful oral disease.


Seborrheic dermatitis 

Inflammatory scaling reaction in seborrheic areas of skin (scalp, ears, head, chest). (Mild form in the scalp is dandruff.) Stress may exacerbate. Often one of the earliest cutaneous signs of HIV infection. May or may not be pruritic. Is common & often severe in patiens with chronic neurologic conditions (Parkinson’s, CVA, spinal cord injuries).

Differential includes psoriasis, Reiter’s disease, atopic dermatitis.

Treat with shampoos containing tar, sulfur, salicylic acid, ketoconazole, selenium. Hydrocortisone cream on ears & face.


Pityriasis Rubra Pilaris (PRP) 
Rare, of unknown cause, some familial cases. Clinical findings: widespread orange-red plaques that can involve entire skin surface area, with small islands of totally normal skin (“island sparing”), thickening of palms & soles into waxy keratotic shell, follicular accentuation. Treat with short courses of high-dose vitamin A (up to 300,000 IU/day), but disease is frequently resistant to all therapies.

Secondary Syphilis 

Multiple, raised papules or small plaques with some scale. Can closely resemble pityriasis rosea. Clues that it’s syphilis: palms & soles involved; lesions in inguinal, gluteal, axillary, or mucosal areas; scalp involvement with patchy alopecia; supportive sexual history. Serology & skin biopsy for diagnosis.


References

Shaw JC, Parker F. Psoriasis and other papulosquamous diseases. In: Noble J, ed. Textbook of primary care medicine. 3rd ed. St. Louis (MO): Mosby, 2001:761-4.

McCall CO, Lawley TJ. Eczema, psoriasis, cutaneous infections, acne, and other common skin disorders. In: Kasper DL, Braunwald E, Fauci AS, Hauser SL, Longo DL, Jameson JL, eds. Harrison's principles of internal medicine. 16th ed. New York (NY): McGraw-Hill, 2005:288-95.



Improved Process

Tori O'Daniel, Elizabeth Buys

Definition of Managed Care

System for financing and delivering a specific set of health care services for a defined population through a specific network that is held accountable for delivering quality, accessible and affordable health care with the highest patient and provider satisfaction possible.

Evolution Of Managed Care

Origin: managed care began in the late 19th century, not fully developed until the late 1930's. Health Maintenance Organization Act of 1972 (HMO) distinguished itself from traditional "fee for service" health care.

The original HMO had 5 characteristics:

1. the organization assumed contract responsibility for providing health care services.

2. A defined population was enrolled into the plan

3. Subscribers and providers were voluntary

4. HMO received fixed payment from the subscriber regardless of their service.

5. Physician was employed directly by the HMO.

Different variations of HMO- 

1. PPO preferred provider organization-providers agreed to offer health services for discounted prices, and individuals wwould have greater health benefits and lower costs if they chose PPO providers. -BENEFIT:payments weren't limited, so neither the insurers nor provider were placed at financial risk for care provided. 

2. IPA independent practice associations. HMO contracted certain physician or groups to accept fixed payments for their services, but the physician could also continue their fee for service as well.

WHAT TO EXPECT IN THE 21ST CENTURY HEALTH CARE:

1. Growing emphasis on measuring and improving quality of care- organizations have developed to ensure quality health care. JCAHO( joint commission on Accreditation of Health Care Org.,1951), and NCQA(National committee for quality assurance). These organizations have developed multiple programs that evaluate member rights, medical records, quality health programs . One big example: HEDIS; health plan employer data and information set. it's a set of standardized performance measures ie. prenatal care, immunizations etc. to compare different health plans with eachother.

2. The patient as educated consumer- health care plans are defining their policies to patients about how to file complaints or concerns with their plan. They are conducting member satisfaction surveys and surveys about individual physicians. Patients also now receive "Report Cards" about their plan in comparsion to other plans in the country. They can make an educated choice about their plan and their physician.

3. Increased emphasis on phsician performance measurement- analyses are being done to compare one physician to the next based on specific goals ie. quality of care, number of children up to date on immunizations in their practice etc. The problem currently is that there isn't a standardized way to conduct this.

4. Evolution in reimbursement strategies to control cost and improve outcomes- new ways to reimburse doctors are evolving due to problems associated with current strategies:

Fee for Service : doctors provide more service to increase reimbursement

Capitation (one payment per memeber per month) : doctors underutilize services since they have don't get paid more for more services provided.

Salary: doctors may have decreased productivity. They get paid the same regardless of how many people they care for.

5. Evolution in managing health care utilization: Many programs are developing to avoid unnecessary tests, surgeries and long hospital stays. 24-hour nurse call lines, wellness programs, social workers..

6. Continued health system integration and disintegration: Networks are continually forming to include all the health care providers into one group or package. You can follow a patient care better and waste less time. Nonetheless, if the large network fails or goes under, then all the individual groups fail.

7. The new age of primary care, prevention and wellness- managed care places emphasis on preventative medicine to avoid future costs. For example they make you pay less for Pap smears, and annual exams, They utilize the primary care doctors

Community Medicine: effective skills on how to practice primary medicine in the 21st Century in light of "MANAGED CARE"

-Measuring and improving clinical care: identify the desired outcome of care, define guidelines that must be followed to attain the outcome, measure and provide feedback, and use the feedback for change in your practice.

-Get to know your patient population, identify and prioritize your community's health problems. Take the time to listen to your patient. In some cases you may involve the community in the design and implementation of a preventive measure. You can measure their satisfaction by allowing them to fill out surveys.

-Run an effective office practice and maximize net income for services - use computers to compile patient records, delegate tasks that others can do, use preprinted forms, know how to code for your services, monitor your practices financial status and negotiate a good contract with insurers.

Practicing ethically and legally

Make informed decisions about practice options such a integrating with a larger network.

Maintaining life balance- remember to make time for your family and your own life.

References

Rivo ML, Johnson GR. Managed health care: practicing effectively in the 21st century. In: Rakel RE, ed. Textbook of family practice. 6th ed. Philadelphia (PA): WB Saunders, 2002:1603-21.

Leshan L. Community health. In: Noble J, ed. Textbook of primary care medicine. 3rd ed. St. Louis.



Blood Gas Findings

Nathan Mullins, MD, Beth Buys, MD

Acid-Base Overview


Normal systemic arterial pH = 7.35 – 7.45
pH dependent on [HCO3-], which is regulated by the kidneys, and PACO2 which is regulated by the lungs
Disturbances in [HCO3-] = Metabolic disorder
Disturbances in [PACO2] = Respiratory disorder
An acid-base disorder may be either Metabolic vs. Respiratory vs. Mixed

Evaluation of pH

  1. Look at the pH, > 7.45 =Acidosis, < 7.35=Alkalosis
  2. Determine primary disturbance by looking at the PACO2
    1. Normal PACO2 = 40 mmHg
    2. PACO2 is elevated with Respiratory Acidosis and is decreased with Respiratory Alkalosis.
    3. If the change in pH is not consistent with the change in PACO2 then the primary disturbance is a Metabolic Acidosis/Alkalosis.
    4. Ex. If the pH =7.53 and the PACO2=35, then the primary disturbance is a Respiratory Alkalosis
  3. Determine the degree of compensation to distinguish a simple acid-base disorder (i.e. metabolic acidosis) from a mixed acid-base disorder.
    1. Metabolic acidosis: PACO2 = 1.5(HCO3-) + 8 ± 2
i. so, if measured PACO2 > calculated = Mixed metabolic acidosis and respiratory acidosis
ii. if measured PACO2 < calculated = Mixed metabolic acidosis and respiratory alkalosis
    1. Metabolic alkalosis: D PACO2 = 0.6 x D HCO3-
    2. Respiratory acidosis:
i. acute = 1 mEq/L HCO3- per 10 mmHg CO2
ii. chronic = 3.5 HCO3- per 10 mmHg CO2
    1. Respiratory alkalosis:
i. acute = 2 HCO3- per 10 mmHg CO2
ii. chronic = 5 HCO3- per 10 mmHg CO2

Reference

Dubois TD. Acidosis and alkalosis. In: Braunwald E, Fauci AS, Kaspar DL, Hauser SL, Longo DL, Jameson JL, eds. Harrison's principles of internal medicine. 15th ed. New York (NY): McGraw-Hill Medical, 2001:283-91

Interdisciplinary medicine. In: Ferri FF. Practical guide to the care of the medical patient. 6th ed. St. Louis (MO): Mosby, 2004:833-41



Pregnancy Loss & Cervical Insufficiency

Jorgensen, Coulson

Cervical insufficiency: The inability of the uterine cervix to retain pregnancy in the absence of contractions or labor.

Theoretical Etiologies:
  • Surgical trauma - LEEP, CKC, overdilation of the cervix during termination
  • Congenital mullerian anomalies
  • Deficiencies in cervical collagen and elastin.
  • Exposure to DES
Warning signs of cervical insufficiency
  • History of 2 or more second trimester losses (excluding those resulting from preterm labor or abruption.
  • History of losing each successive pregnancy at a progressively earlier gestational age.
  • History of painless cervical dilation up to 4 - 6 cm.
  • History of cervical trauma (as mentioned above)
Evaluation: Serial ultrasounds should be considered in a patient with risk factors, initiated between 16 and 20 wks. Ultrasounds done prior to 16 wks should be repeated because of the inability to adequately distinguish the cervix from the lower uterine segment. Scans can also be done to follow the cervix after cerclage placement.

Cerclage: Standard placement at 13 - 16wks. Elective placement in patients with 3 or more unexplained midtrimester losses or preterm deliveries. Rule out fetal anomalies prior to placement. Antibiotics with cerclage is debatable. Complication rates increase depending on the urgency of placement, where roughly a third of patients will experience ROM or chorio if a cerclage is done emergently. Much lower risks if done electively.

Congenital abnormalities of the mullerian ducts can contribute to recurrent pregnancy loss. Since the ovaries are of a different embryological origin than the mullerian structures, patients with mullerian anomalies typically have normal ovaries and ovarian function.

Incidence of Mullerian Defects
Percentages
1. Fertile and infertile women
3 - 4%
2. Women with recurrent miscarriages
5 - 10%
3. Women with late miscarriages and preterm deliveries
  • 25%

Distribution of Specific Anomalies
Percentages
1. Septate Uterus
35%
2. Bicornuate Uterus
26%
3. Arcuate Uterus "Heart shaped uterine cavity"
18%
4. Unicornuate Uterus
10%
5. Uterus Didelphys
8%

Unicornuate Uterus: Failure of the development in one mullerian duct. Occasional rudimentary horn present. Aprox 40% of patients with a unicornuate uterus will have a urinary tract anomaly (usually the kidney).

Uterus Didelphus:
Lack of fusion of the two mullerian ducts resulting in duplication of corpus and cervix. Many patients have no reproductive difficulties. Often ipsilateral renal agenesis.

Bicornuate Uterus:
Partial lack of fusion of the two mullerian ducts, resulting in a single cervix and varying degrees of separation in the two uterine horns. If history of pregnancy complications, consider metroplasty.

Septate Uterus: Partial lack of resorption of the midline septum between the two mullerian ducts resulting in slight midline septum (arcuate) or a significant midline division. Treatment with hysteroscopy led to miscarriage rates of 10%, down from 90% prior to surgery.

Exposure to DES in utero: Similar outcomes observed in two studies when managed with cerclage versus conservatively. A woman with DES exposure should undergo cerclage placement for the same clinical indications as any other patient.

References

ACOG Practice Bulletin. "Cervical Insufficiency" Number 48, November 2003.

Gynecological Endocrinology and Infertility. 7th Edition. Pages 113-44.


Fibroma

Hingson-Gates, Sigmon

Prevalence: 10-20% of all women.
Most often in young women (age 20-35).

Facts: Fibroadenomas are the most common benign tumors of the breast.
Firm, painless, rubbery, freely movable mass averaging 1-3 cm in diameter.
Usually solitary.
Don't change with the menstrual cycle.
Slow growing (usually).
Transformation into cancer is rare.
Consist of mixture of proliferating epithelial and supporting fibrous tissues.

Treatment: Surgical excision is indicated if mass causes pain or grows rapidly or if fine-needle aspiration is inconclusive.
Evaluate by examination and imaging. Then biopsy or excise. Using U/S and fine-needle biopsy, fibroadenomas can be distinguished from cysts.

References

Novak's Gynecology, 13th ed. Benign Breast Disease. pp 543-568.

Obstetrics and Gynecology. Disorders of the Breast. pp 417-431.