Section 6: Gynecology 2 Flashcards

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1
Q
  1. List the human papilloma virus (HPV) types associated with cervical cancer
  2. List the human papilloma virus (HPV) types associated with benign condyloma acuminata
A
  1. HPV 16, 18, 31, 33, and 35
  2. HPV 6 and 11

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14169-14190). Kaplan Publishing. Kindle Edition.

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2
Q

Outline the pap smear classifications

A

Indeterminate smears: – Atypical squamous cells of undetermined significance (ASCUS)

Abnormal smears:

  • Low-grade squamous intraepithelial lesion (LSIL): HPV, mild dysplasia, or CIN 1
  • High-grade squamous intraepithelial lesion (HSIL): Moderate dysplasia, severe dysplasia, CIS, CIN 2 or 3
  • Cancer: Invasive cancer

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14169-14190). Kaplan Publishing. Kindle Edition.

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3
Q

Risk factors associated with cervical neoplasia

A

Early age of intercourse

Multiple sexual partners

Cigarette smoking

Immunosuppression

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14169-14190). Kaplan Publishing. Kindle Edition

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4
Q

Screening for cervical neoplasia

  1. When is screening started?
  2. What screening is used
  3. What is the frequency of screening?

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14190-14204). Kaplan Publishing. Kindle Edition.

A
  1. Age 21, regardless of the onset of sexual activity
  2. Screening used:
    • Conventional method: 50 percent sensitivity
    • Liquid-based prep: Sensitivity is increased to 75– 80 percent
    • HPV DNA testing: Useful in management of ASCUS
  3. Frequency of screening:
    • If < 30 years old, annually for conventional Pap or every 2 years for liquid-based
    • If > 30 years old, screen every 2 to 3 years if > 3 consecutive negative Pap smears

Cervical cancer screening guidelines per the USPSTF:

  • Pap screening not recommended for women > 65 with recent normal Pap smear
  • Pap smear not recommended for women with total hysterectomy for benign disease
  • HPV testing alone is not sufficient for screening

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14190-14204). Kaplan Publishing. Kindle Edition.

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5
Q

A 35-year-old woman is referred because of a Pap smear reading of ASCUS. The patient states that her last Pap smear, done approximately 1 year ago, was negative. She has been sexually active, using combination oral contraception pills for the last 4 years. A repeat Pap smear after 3 months again reveals ASCUS. Which of the following is the next step in evaluation?

a. Endocervical curettage
b. Colposcopy and biopsy
c. HPV DNA typing
d. Repeat Pap smear in 6 months
e. Repeat Pap smear in 12 months

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14205-14224). Kaplan Publishing. Kindle Edition.

A

B. ASCUS is most commonly found in women with inflammation due to early HPV infection. Approximately 10– 15 percent of patients with ASCUS have premalignant or malignant disease. Two Pap smears revealing ASCUS must be followed up with colposcopy and biopsy

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14205-14224). Kaplan Publishing. Kindle Edition.

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6
Q

Management

  1. If the case describes a patient with atypical squamus cells of undetermined significance (ASCUS) on the Pap smear and follow-up is certain, what is the next step?
A
  1. ** Repeat the Pap smear in 3– 6 months and order HPV DNA typing**

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14205-14224). Kaplan Publishing. Kindle Edition.

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7
Q

If the case describes a patient with atypical squamus cells of undetermined significance (ASCUS) on the Pap smear and repeat pap smear result is negative, what next?

A

Carry out routine follow-up

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14205-14224). Kaplan Publishing. Kindle Edition.

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8
Q

If the case describes a patient with atypical squamus cells of undetermined significance (ASCUS) on the Pap smear and repeat pap smear result is again ASCUS, or HPV 16 and 18, what next?

A

Order colposcopy and biopsies.

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14205-14224). Kaplan Publishing. Kindle Edition.

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9
Q

If the case describes a patient with ASCUS on Pap smear and follow-up is uncertain (i.e., patient is not reliable to return for follow-up), what next?

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14205-14224). Kaplan Publishing. Kindle Edition.

A

Order colposcopy and biopsies.

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14205-14224). Kaplan Publishing. Kindle Edition.

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10
Q

Indications for:

  1. Repeat pap smear
  2. HPV DNA testing
  3. Colposcopy and ectocervical biopsy
  4. Endocervical curettage
A
  1. First ASCUS Pap
  2. First ASCUS Pap
  3. An abnormal Pap smear; Two ASCUS Pap smears
  4. All NONpregnant patients with an abnormal Pap smear

NOTES

Note on 1

  • Repeat Pap at 4- to 6-month intervals until there are 2 consecutive, negative Paps
  • If a repeat Pap is again ASCUS, refer for colposcopy

Note on 2

  • If liquid-based cytology was used on the initial Pap, use specimen for DNA testing
  • If conventional methods were used, a second Pap needs to be performed
  • Colposcopy is then performed only if HPV 16 and 18 identified

Notes on 3

  • Colposcopy and ectocervical biopsy – Colposcopy is a magnification of the cervix (10– 12 times)
  • Abnormal lesions (e.g., mosaicism, inflammatory punctation, white lesions, abnormal vessels) are biopsied and sent for histology

Notes on 4

  • All nonpregnant patients undergoing colposcopy for an abnormal Pap smear must undergo an ECC to rule out endocervical lesions

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14226-14258). Kaplan Publishing. Kindle Edition.

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11
Q

Indications for cone biopsy

A
  • Performed after colposcopy or ECC if Pap smear and biopsy findings are not consistent (suggests abnormal cells were not biopsied)
  • Abnormal ECC histology
  • An endocervical lesion
  • A biopsy showing microinvasive carcinoma of the cervix

NOTE: Deep cone biopsies can result in an incompetent cervix or cervical stenosis

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14226-14258). Kaplan Publishing. Kindle Edition.

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12
Q

What is the management of:

  • CIN 1
  • CIN 2 or 3 after ablation or excision
A

Observation and follow-up

Follow-up repeat Pap smears, colposcopy + Pap smear, or HPV DNA testing every 4 to 6 months for 2 years

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14262-14298). Kaplan Publishing. Kindle Edition

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13
Q

What is the management of the following abnormal cervical histology:

  • CIN 2 or 3
A

Ablative modalities

  • Cryotherapy
  • Laser vaporization
  • Electrofulguration

Excisional procedures

  • LEEP (loop electrosurgical excision procedure)
  • Cold-knife conization

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14262-14298). Kaplan Publishing. Kindle Edition.

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14
Q

What is the management of the following abnormal cervical histology:

  • Biopsy confirmed
  • Recurrent CIN 2 or 3
A

Hysterectomy

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14262-14298). Kaplan Publishing. Kindle Edition.

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15
Q

Invasive Cervical Cancer

The average age of diagnosis is 45 years

  1. List the diagnostic test
  2. What is the most common histology type of invasive cervical cancer
  3. List the metastatic work-up
  4. Rx?
  5. Indications for adjuvant therapy (radiation therapy and chemotherapy)
A
  1. Cervical biopsy
  2. Squamous cell carcinoma
  3. Metastatic workup:
    • Pelvic exam
    • CT scan (to look for metastatic disease)
    • Cystoscopy
    • Proctoscopy
  4. Treatment: simple or modified radical hysterectomy
  5. Adjuvant therapy:
    • Metastasis to lymph nodes
    • Tumor size > 4 cm
    • Poorly differentiated lesions
    • Positive margins
    • Local recurrence

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14262-14298). Kaplan Publishing. Kindle Edition.

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16
Q

A 25-year-old woman with a 15-week pregnancy by dates is found to have HGSIL (high-grade squamous intraepithelial lesion) on a recent Pap smear. On pelvic examination there is a gravid uterus consistent with 15 weeks’ size, and the cervix is grossly normal to visual inspection. What is the next step in management?

a. Colposcopy and biopsy
b. Cone biopsy
c. Endocervical curettage
d. Hysterectomy
e. Repeat Pap after pregnancy

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14298-14321). Kaplan Publishing. Kindle Edition.

A

A. A pregnant woman with abnormal Pap smear is managed in the same way as a nonpregnant woman with the exception of endocervical curettage, which is not performed because of increased cervical vascularity. An abnormal Pap smear is evaluated with colposcopy and biopsy. Pregnancy does not predispose to abnormal cytology and does not accelerate precancerous lesion progression into invasive carcinoma

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14298-14321). Kaplan Publishing. Kindle Edition.

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17
Q

Management of Abnormal Cervical Histology During Pregnancy:

  • CIN/ dysplasia
A
  • Pap smear and colposcopy every 3 months during pregnancy
  • Repeat Pap and colposcopy 6– 8 weeks postpartum. Any persistent lesions are then definitively treated postpartum

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14307-14340). Kaplan Publishing. Kindle Edition.

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18
Q

Management of Abnormal Cervical Histology During Pregnancy:

  • Microinvasive cervical cancer
A
  • Cone biopsy to ensure no frank invasion
  • Deliver vaginally, reevaluate and treat 2 months postpartum

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14307-14340). Kaplan Publishing. Kindle Edition.

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19
Q

Management of Abnormal Cervical Histology During Pregnancy:

  1. Invasive cancer diagnosed before 24 weeks
  2. Invasive cancer diagnosed after 24 weeks
A
  1. Definitive treatment (radical hysterectomy or radiation therapy)
  2. Conservative management up to 32– 33 weeks – Cesarean delivery and begin definite treatment

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14307-14340). Kaplan Publishing. Kindle Edition.

20
Q

Prevention of Cervical Dysplasia

  1. Type of vaccine
  2. Who receives it?
  3. What subtypes of HPV does it prevent againts
  4. True or False: Testing for HPV before vaccination can be given
  5. True or False: Sexually active women can receive the vaccine
  6. True or False: Women with previous abnormal cervical cytology or genital warts can receive the vaccine, but it may be less effective
  7. True or False: It can be given to patients with previous CIN, but benefits are limited
  8. True or False: The vaccine is recommended for pregnant, lactating, or immunosuppressed women
  9. True or False: Females who have been vaccinated for HPV need not follow Pap smear recommendations
  10. True or False: Gardasil is also indicated for males age 9– 26 for the prevention of genital warts caused by HPV.
A
  1. Quadrivalent HPV recombinant vaccine (Gardasil)
  2. All females 8– 26 years of age
  3. 4 HPV types: (6, 11, 16, 18) that cause 70 percent of cervical cancer and 90 percent of genital warts
  4. False. Testing not needed
  5. True
  6. True
  7. True
  8. False. Not recommended
  9. False. They must still follow Pap smear recommendations
  10. True

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14341-14358). Kaplan Publishing. Kindle Edition.

21
Q

List the differential diagnosis for a woman with pelvic pain

A
  • Cervicitis
  • Acute salpingo-oophoritis
  • Chronic PID
  • Tuboovarian abscess

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14341-14358). Kaplan Publishing. Kindle Edition.

22
Q

List the initial workup for pelvic pain

A
  • Pelvic exam
  • Cervical culture
  • Laboratory: ESR (sedimentation rate), WBC (include blood culture if fever is present)
  • Sonogram

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14341-14358). Kaplan Publishing. Kindle Edition.

23
Q

Rx for cervicitis

A

A single dose of oral cefixime and azithromycin

Always treat cervicitis to cover both chlamydia and gonorrhea

Antibiotics that treat gonorrhea:

  • Ceftriaxone IM
  • Cefixime PO

Antibiotics that treat chlamydia:

  • Azithromycin PO
  • Doxycycline PO

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14359-14382). Kaplan Publishing. Kindle Edition.

24
Q

What is (1) diagnosis, (2) Outpatient Rx and (3) Inpatient Rx based on the following presentation:

  • Cervical motion tenderness on exam
  • Lower pelvic pain after menstruation
  • Positive cervical cultures
  • WBC and ESR are elevated
  • Pelvic abscess ruled out on sonogram
A
  1. Acute Salpingo-oophoritis
  2. Outpatient: Two oral antibiotics (e.g., ofloxacin and metronidazole)
  3. Inpatient: Three intravenous antibiotics
    • IV cefoxitin or cefotetan and
    • IV doxycycline or clindamycin and
    • gentamicin

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14359-14382). Kaplan Publishing. Kindle Edition.

25
Q

Pelvic pain

  1. When are outpatient antibiotics the answer?
  2. When are inpatient antibiotics the answer?
A
  1. Outpatient:
    • All cases of cervicitis
    • Acute salpingo-oophoritis when there is no systemic infection or pelvic abscess
  2. Inpatient antibiotics:
    • Acute salpingo-oophoritis in a nulligravida or adolescent patient, previous outpatient treatment failure, intrauterine device (IUD) in place, presence of fever, or pelvic abscess
    • All cases of tuboovarian abscess

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14399-14422). Kaplan Publishing. Kindle Edition.

26
Q

A 21-year-old female presents to the emergency department with lower abdominal pelvic pain starting 1 day ago. Symptoms began after her menstrual period completed. She is sexually active but using no contraception. Speculum examination reveals mucopurulent cervical discharge. Bimanual pelvic examination shows bilateral adnexal tenderness and cervical motion tenderness. She is afebrile. Her qualitative urinary β-hCG test is negative. Complete blood cell count (CBC) shows a WBC of 14,000. ESR is elevated. What is the most appropriate management?

a. Outpatient PO cefixime and azithromycin
b. Inpatient IV cefotetan, clindamycin, and gentamicin
c. Inpatient IV clindamycin and gentamicin
d. Exploratory laparotomy
e. Percutaneous drainage

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14399-14422). Kaplan Publishing. Kindle Edition.

A

B. This patient has acute salpingo-oophoritis. Her nulligravid status is an indication for inpatient IV antibiotic triple-therapy

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14399-14422). Kaplan Publishing. Kindle Edition.

27
Q
  • Recurrent, crampy lower abdominal pain
  • Nausea
  • Vomiting
  • Diarrhea
  • Occur during menstruation
  • Symptoms begin 2– 5 years after onset of menstruation (ovulatory cycles)
  1. Diagnosis
  2. Pathophysiology
  3. Firts line Rx
  4. Second line Rx
A
  1. Primary dysmenorrhea
  2. Excessive endometrial prostaglandin F2, which causes uterine contractions and acts on gastrointestinal smooth muscle
  3. NSAIDs
  4. Combination oral contraceptives

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14422-14444). Kaplan Publishing. Kindle Edition.

28
Q

It classically presents in women over 30 with

  • Dysmenorrhea
  • Dyspareunia
  • Dyschezia (painful bowel movements) and
  • Infertility
  1. Diagnosis
  2. Most common site
  3. Second most sommon site
  4. Definitive diagnosis
  5. First line Rx
  6. Second line Rx
  7. Other Rxs
A
  1. Endometriosis
  2. Ovary - (endometriomas or chocolate cyst)
  3. Cul-de-sac, causing uterosacral li
  4. Laparoscopy
  5. Continuous oral progesterone or oral contraceptive pill (OCP)
  6. Testosterone derivatives (Danocrine or danazol) or GnRH analogs (Lupron or leuprolide)
  7. Other Rxs:
    • Laparoscopic lysis adhesions: Laser vaporization of lesions can improve fertility
    • TAH and BSO can be done for severe symptoms when fertility is not desired

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14422-14444). Kaplan Publishing. Kindle Edition.

29
Q

Causes of elevated CA-125

A
  • Ovarian cancer
  • Cirrhosis
  • Endometriosis
  • Peritonitis
  • Pancreatitis

Not all elevations of CA-125 are due to ovarian cancer

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14444-14462). Kaplan Publishing. Kindle Edition.

30
Q

Premenarchal Vaginal Bleeding

This is bleeding that occurs before menarche. The average age at menarche is 12

  1. Causes
  2. List the diagnostic workups
  3. Diagnosis when workup is negative
A
  1. Causes are as follows:
    • Foreign body (most common)
    • Sarcoma botryoides (cancer of vagina or cervix suggested by a grapelike mass arising from the vaginal lining or cervix)
    • Tumor of the pituitary adrenal gland or ovary
    • Sexual abuse
  2. Diagnostic tests:
    • Perform pelvic exam under sedation
    • Order CT or MRI of pituitary, abdomen, and pelvis to look for estrogen-producing tumors
  3. Idiopathic precorcious puberty

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14444-14462). Kaplan Publishing. Kindle Edition.

31
Q

A 31-year-old woman complains of 6 months of metromenorrhagia. The patient states that she started menstruating at age 13 and that she has had regular menses until the past 6 months. The pelvic examination, including a Pap smear, is normal. She has no other significant personal or family history. What is the next step in management?

a. Obtain ß-hCG
b. Obtain LH, FSH levels
c. Perform a pelvic ultrasound
d. Recommend oral contraceptive pill
e. Recommend progestin-only pill

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14462-14503). Kaplan Publishing. Kindle Edition.

A

A. Irregular bleeding in reproductive age should always be evaluated first for pregnancy. If pregnancy is ruled out, workup for anatomical causes of bleeding or anovulation can be started

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14462-14503). Kaplan Publishing. Kindle Edition.

32
Q

Primary Amenorrhea

  1. Define it
  2. Outline the workup
  3. What does presence of breasts indicate?
A
  1. Absence of menses at age 14 without secondary sexual development or at age 16 with secondary sexual development
  2. Diagnostic Testing
    • Physical exam (breasts present or absent?)
    • Ultrasound (uterus present or absent?)
    • Karyotype
    • Testosterone
    • FSH
  3. Adequate estrogen production

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14462-14503). Kaplan Publishing. Kindle Edition.

33
Q

Differential diagnosis of amenorrhea with breasts and uterus present

A
  • Imperforate hymen
  • Vaginal septum
  • Anorexia nervosa
  • Excessive exercise
  • Pregnancy before first menses

Workup as secondary amenorrhea

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14462-14503). Kaplan Publishing. Kindle Edition.

34
Q

Differential diagnosis of amenorrhea with breasts present but uterus is absent

What is the workup?

A

Müllerian agenesis

  • XX karyotype
  • Normal testosterone for female

Complete androgen insensitivity (testicular feminization)

  • XY karyotype
  • Normal testosterone for male

Workup: Order testosterone levels and karyotype

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14462-14503). Kaplan Publishing. Kindle Edition.

35
Q

Differential diagnosis of primary amenorrhea with breasts absent but uterus is present

What is the workup?

A

Gonadal dysgenesis (Turner’s syndrome)

  • X0 karyotype
  • FSH elevated

Hypothalamic–pituitary failure

  • XX karyotype
  • FSH low

Workup: Order FSH level and karyotype

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14496-14539). Kaplan Publishing. Kindle Edition.

36
Q

Differential diagnosis of primary amenorrhea with breasts and uterus absent

What is the workup?

A

RARE. Not clinically relevant

37
Q

A 17-year-old girl is brought to the clinic by her mother concerned because her daughter has never had a menstrual period. She reports that her daughter has good grades, studies hard but seems stressed out most of the time which is why she believed her period was delayed. On examination she seems to be well-nourished, with adult breast development and pubic hair present. Pelvic examination reveals a foreshortened vagina. No uterus is seen on ultrasound. What is the most appropriate advice?

a. CT scan of the brain is indicated to evaluate a pituitary tumor
b. Estrogen and progesterone supplementation is indicated
c. In vitro fertilization is an option for future fertility
d. Surgical removal of intra-abdominal testes is recommended
e. Vaginal reconstruction may be performed

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14496-14539). Kaplan Publishing. Kindle Edition.

A

E. This patient has Müllerian agenesis resulting in an absence of uterus, cervix and upper vagina. Ovaries are intact and normal levels of estrogen are present. Vaginal reconstruction may be performed to elongate the vagina for satisfactory sexual intercourse

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14496-14539). Kaplan Publishing. Kindle Edition.

38
Q

Management of:

  1. Müllerian agenesis
  2. Complete androgen insensitivity (testicular feminization)
  3. Gonadal dysgenesis (Turner’s syndrome)
  4. Hypothalamic– pituitary failure
A
  1. Surgical elongation of the vagina for satisfactory sexual intercourse and counseling about infertility
  2. Removal of testes before age 20 because of increased risk of testicular cancer. Estrogen replacement will then be needed
  3. Estrogen and progesterone replacement for development of secondary sexual characteristics
  4. Estrogen and progesterone replacement for development of secondary sexual characteristics

Müllerian duct derivatives (fallopian tubes, uterus, cervix, and upper vagina) are absent in Müllerian agenesis

There are no pubic or axillary hair in Androgen insensitivity (Testicular feminization)

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14496-14539). Kaplan Publishing. Kindle Edition.

39
Q

Criteria for diagnosis of secondary amenorrhea

A
  • Regular menses are replaced by an absence of menses for 3 months
  • Irregular menses are replaced by an absence of menses for 6 months.

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14540-14570). Kaplan Publishing. Kindle Edition.

40
Q

Outline the steps in the workup of secondary amenorrhea in a tabular form

A

Steps in the workup of secondary amenorrhea

41
Q

Rx of premenstrual syndrome

A
  • SSRIs
  • Low dose of vitamin B6 (pyridoxine) may improve symptoms
42
Q

List the workups for secondary amenorrhea

A
  • Pregnancy Test (ß-hCG)
  • Thyrotropin (TSH) (rule out hypothyroidism)
  • Prolactin (rule out elevation)
  • Progesterone Challenge Test (PCT)
  • Estrogen–Progesterone Challenge Test (EPCT)

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14568-14612). Kaplan Publishing. Kindle Edition.

43
Q

Why does hypothyroidism lead to amenorrhea?

Rx of amenorrhea in hypothyroidism

A

In primary hypothyroidism there is an elevated TRH (thyrotropin releasing hormone) level which leads to ↑ prolactin

Rx: Thyroid replacement

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14540-14570). Kaplan Publishing. Kindle Edition.

44
Q

Appropriate steps to take when there is hyperprolactinemia (elevated prolactin) in the workup of secondary amenorrhea

A
  1. ** Review medications**: Antipsychotics and antidepressants have antidopamine side effect → ↑ prolactin
  2. CT or MRI of head to rule out pituitary tumor
    • Tumor < 1 cm: give bromocriptine (dopamine agonist)
    • Tumor > 1 cm: treat surgically
  3. If the cause of elevated prolactin is idiopathic, treat with bromocriptine

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14568-14612). Kaplan Publishing. Kindle Edition.

45
Q
  1. What does a positive progesterone challenge test (PCT) indicate?
  2. What is the Rx of (1)?
  3. What does a negative PCT indicate?
A
  1. Any withdrawal bleeding is diagnostic of anovulation
  2. Treatment: Cyclic progesterone to prevent endometrial hyperplasia. Clomiphene ovulation induction is done if pregnancy is desired
  3. Inadequate estrogen or outflow tract obstruction

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14568-14612). Kaplan Publishing. Kindle Edition.

46
Q

Estrogen–Progesterone Challenge Test (EPCT)

  1. How is this done?
  2. What does a poitive EPCT indicate?
  3. What is the next line of action after (2)?
  4. Interpret the results from (3) and Rx accordingly
  5. What does a negative EPCT indicate?
  6. What is the Rx for (5)?
A
  1. 3 weeks of oral estrogen followed by 1 week of progesterone
  2. Positive EPCT: Any withdrawal bleeding is diagnostic of inadequate estrogen
  3. Get an FSH level
  4. ↑ FSH is ovarian failure. Y chromosome mosaicism may be the cause if patient is < 25 years. Order a karyotype for confirmation
    • FSH is hypothalamic– pituitary insufficiency Order a brain CT/ MRI to rule out a tumor. Give estrogen-replacement therapy to prevent osteoporosis and cyclic progestins to prevent endometrial hyperplasia
  5. An outflow tract obstruction or endometrial scarring (e.g., Asherman syndrome)
  6. Order a hysterosalpingogram to identify the lesion. Management: Adhesion lysis followed by estrogen stimulation of the endometrium. An inflatable stent prevents re-adhesion of the uterine walls

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14568-14612). Kaplan Publishing. Kindle Edition.

47
Q
  1. Diagnosis: Amenorrhea + Positive Estrogen-Progesterone Challenge test + ↑ FSH
  2. Test to order for (1)
  3. Diagnosis: Amenorrhea + Positive Estrogen-Progesterone Challenge test + ↓ FSH
  4. Test to order for (3)
  5. Rx for (3)
A
  1. Ovarian failure. Y chromosome mosaicism may be the cause if patient is < 25 years
  2. Order a karyotype for confirmation
  3. Hypothalamic– pituitary insufficiency
  4. Order a brain CT/ MRI to rule out a tumor
  5. Give estrogen-replacement therapy to prevent osteoporosis and cyclic progestins to prevent endometrial hyperplasia

Fischer, Conrad (2012-09-22). Master the Boards: USMLE Step 3 (Kaplan Medical Usmle Master the Boards Step 3) (Kindle Locations 14568-14612). Kaplan Publishing. Kindle Edition.