Pediatric Male/Female Reproductive System Flashcards

1
Q

All sexually active women age [] should be screened for Chlamydia trachomatis and Neisseria gonorrhoeae.

e.g. nucleic acid amplification testing

A

All sexually active women age >24 should be screened for Chlamydia trachomatis and Neisseria gonorrhae.

e.g. nucleic acid amplification testing

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2
Q

Congenital hydrocele occurs due to incomplete obliteration of the […] in infants.

A

Congenital hydrocele occurs due to incomplete obliteration of the processus vaginalis in infants.

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3
Q

Do adolescents seeking pregnancy prevention options (e.g. OCPs, emergency contraception) need parental consent?

A

No

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4
Q

Fetal macrosomia is associated with increased risk for shoulder […].

A

Fetal macrosomia is associated with increased risk for shoulder dystocia.

potential complications of shoulder dystocia include clavicle fracture, brachial nerve palsies, and perinatal asphyxia

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5
Q

In […] syndrome a person is genotypically XY but has female external genitalia with male internal genitalia.

A

In androgen insensitivity syndrome a person is genotypically XY but has female external genitalia with male internal genitalia.

i.e. the presence of the lower vagina, clitoris, and labia with cryptorchid testes

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6
Q

In androgen insensitivity syndrome a person is genotypically XY but has […] external genitalia with […] internal genitalia.

A

In androgen insensitivity syndrome a person is genotypically XY but has female external genitalia with male internal genitalia.

i.e. the presence of the lower vagina, clitoris, and labia with cryptorchid testes

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7
Q

In 5α-reductase deficiency, a person is genotypically XY but has ambiguous […] genitalia with normal male […] genitalia.

A

In 5α-reductase deficiency, a person is genotypically XY but has ambiguous external genitalia with normal male internal genitalia.

due to lack of dihydrotestosterone, which is necessary for external genitalia development – at puberty, elevated levels of testosterone cause external genitalia growth

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8
Q

In […] deficiency, a person is genotypically XY but has ambiguous external genitalia with normal male internal genitalia.

A

In 5α-reductase deficiency, a person is genotypically XY but has ambiguous external genitalia with normal male internal genitalia.

due to lack of dihydrotestosterone, which is necessary for external genitalia development – at puberty, elevated levels of testosterone cause external genitalia growth

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9
Q

Kallmann syndrome presents as infertility in males and amenorrhea in females; […] may be present in both sexes.

A

Kallmann syndrome presents as infertility in males and amenorrhea in females; anosmia may be present in both sexes.

due to failure of GnRH migration (thus decreased FSH, LH, and testosterone) and olfactory bulb formation

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10
Q

Klinefelter syndrome is characterized by a […],[…] karyotype.

A

Klinefelter syndrome is characterized by a 47,XXY karyotype.

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11
Q

Maternal hyperglycemia is associated with macrosomia, secondary to fetal […].

A

Maternal hyperglycemia is associated with macrosomia, secondary to fetal hyperinsulinemia.

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12
Q

Patients with Turner syndrome typically have […] levels of estrogen.

A

Patients with Turner syndrome typically have low levels of estrogen.

due to ovarian dysgenesis; results in high levels of FSH/LH due to lack of negative feedback

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13
Q

Primary amenorrhea is defined as the absence of menarche by age […].

A

Primary amenorrhea is defined as the absence of menarche by age 15.

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14
Q

Size and tenderness of a fibroadenoma of the breast typically increases prior to […].

A

Size and tenderness of a fibroadenoma of the breast typically increases prior to menstruation.

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15
Q

Small for gestational age (SGA) infants have a birth weight that is below the […] percentile for gestational age.

A

Small for gestational age (SGA) infants have a birth weight that is below the 10th percentile for gestational age.

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16
Q

What hematologic complication may be seen in a newborn with low birth weight and intrauterine growth restriction?

A

Polycythemia

due to increased erythropoietin secretion in response to fetal hypoxia; other complications include hypoglycemia, hypothermia, and hypocalcemia

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17
Q

What is the first-line treatment for primary dysmenorrhea?

A

NSAIDs and/or OCPs

treatment preference depends if the patient is sexually active (OCPs) or sexually inactive (NSAIDs)

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18
Q

What is the likely diagnosis in a macrosomic newborn with crepitus over the clavicle and asymmetric Moro reflex?

A

Clavicular fracture (secondary to shoulder dystocia)

diagnosis is confirmed with X-ray

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19
Q

What is the likely diagnosis in a patient with pelvic cramping during the first few days of menses with a normal physical examination?

A

Primary dysmenorrhea

due to uterine contractions triggered by prostaglandin release from sloughing endometrium

20
Q

What is the likely diagnosis in a young girl that presents with breast development and a white, odorless vaginal discharge with an ovarian mass on ultrasound?

A

Granulosa cell tumor

white, odorless vaginal discharge is indicative of estrogen stimulation

21
Q

What is the likely diagnosis in an adolescent female that presents with amenorrhea with a non-palpable uterus and normal-size ovaries on ultrasound?

A

Mullerian agenesis (Mayer-Rokitanksy-Kuster-Hauser syndrome)

primary amenorrhea due to lack of uterine development; still have normal ovaries with normal axillary and pubic hair

22
Q

What is the likely diagnosis in an adolescent patient with a unilateral, mobile, well-circumscribed mass in the outer quadrant of the breast that becomes tender prior to menstruation?

A

Fibroadenoma

fibroadenoma is the most common cause of breast mass in an adolescent

23
Q

What is the likely underlying etiology of painless, irregular, heavy menses in an adolescent girl that started menstruation one year ago?

A

Immaturity of the hypothalamic-pituitary-ovarian axis

results in anovulatory cycles, which causes abnormal uterine bleeding

24
Q

What is the most common cause of vaginal bleeding in the neonatal period?

A

Maternal withdrawal of estrogen

typically manifests as a white vaginal discharge mixed with blood within the first 2 weeks of life

25
Q

What is the most effective emergency contraceptive?

A

Copper IUD

copper IUD > ulipristal pill > levonorgestrel pill (plan B) > OCPs

26
Q

What is the next step in management for a macrosomic newborn with a clavicular fracture secondary to shoulder dystocia?

A

Reassurance

neonatal clavicular fractures usually heal spontaneously within 7 - 10 days

27
Q

What is the next step in management for a newborn male with a cystic testicular mass that is transilluminated with light?

A

Reassurance and observation

most cases spontaneously resolve by 12 months of age; those that persist should be surgically removed to decrease the risk of inguinal hernia

28
Q

What is the next step in management for a newborn that presents with bilaterally enlarged mammary glands and whitish vaginal discharge?

A

Reassurance

breast hypertrophy and leukorrhea are due to transplacental maternal estrogen exposure

29
Q

What is the next step in management for a young girl that presents with a foul-smelling vaginal discharge with a whitish foreign body inside the vaginal introitus on physical exam?

A

irrigation with warmed fluid or removal with a calcium alginate swab

sedation and general anesthesia may be required if these techniques are unsuccessful or if the foreign body is large

30
Q

What is the next step in management for an adolescent female that presents with nausea/vomiting and RLQ abdominal pain with an unknown last menstrual period?

[…]

A

Urine pregnancy testing (e.g. hCG)

transvaginal ultrasound may help distinguish between different etiologies after pregnancy testing

31
Q

What is the next step in management for an adolescent girl that presents with primary amenorrhea and absent breast development with a uterus present on ultrasound?

A

Measure serum FSH level

helps distinguish between central (low FSH) and peripheral (high FSH) causes; absent breast development indicates a lack of estrogen, thus estrogen levels do not need to be measured

32
Q

What is the next step in management for an adolescent girl with primary amenorrhea with a uterus present on ultrasound and high FSH levels?

A

Karyotyping

high FSH is indicative of a peripheral cause, such as hypogonadotropic amenorrhea (e.g. Turner’s syndrome)

33
Q

What is the next step in management for an adolescent girl with primary amenorrhea with a uterus present on ultrasound and low FSH levels?

A

Brain MRI

low FSH is indicative of a central cause, such as hypothalamic or pituitary abnormalities

34
Q

What is the next step in management for an adolescent that presents with a tender breast mass a few days prior to menstruation?

A

Follow-up after menses

if the breast mass decreases in size and/or tenderness, it is likely a fibroadenoma and the patient can be reassured

35
Q

What is the preferred imaging modality for the initial evaluation of a patient with primary amenorrhea?

A

Pelvic ultrasound

useful for evaluating internal female anatomy (e.g. uterus, ovaries), which helps distinguish underlying etiologies

36
Q

What is the recommended treatment for a hemodynamically stable adolescent with heavy vaginal bleeding?

A

High-dose oral contraceptives or IV estrogen

estrogen stabilizes the endometrium and stops menstrual bleeding, while progestin stabilizes the lining and prevents further proliferation; dilation and curettage and/or RBC transfusion may be needed in hemodynamically unstable patients

37
Q

What is the underlying cause of hand and foot swelling in a newborn patient with Turner syndrome?

A

Abnormal development of the lymphatic system (congenital lymphedema)

38
Q

What kidney abnormality is associated with Turner syndrome?

A

Horseshoe kidney

39
Q

What musculoskeletal pathology is associated with Turner syndrome?

A

Osteoporosis

estrogen deficiency leads to increased bone resorption (estrogen typically inhibits osteoclast-mediated bone resorption)

40
Q

When should patients with androgen insensitivity syndrome have bilateral gonadectomy?

A

After puberty

gonad-stimulated puberty helps attain adult height (the testes still produce testosterone); cryptorchid gonads have a small risk of developing into a dysgerminoma or gonadoblastoma after puberty and thus should be removed

41
Q

Which sex chromosome disorder is associated with gynecomastia, female hair distribution, tall/long extremities and eunuchoid body shape in a male?

A

Klinefelter syndrome (47,XXY)

42
Q

Which sex chromosome disorder is associated with short stature, primary amenorrhea, and absent thelarche in a female?

A

Turner syndrome

other characteristic features include webbed neck, shield chest, cystic hygroma, horseshoe kidney, bicuspid aortic valve, and streak ovary

43
Q

[…] is the failure of the testicle to descend into the scrotal sac.

A

Cryptorchidism is the failure of the testicle to descend into the scrotal sac.

if not descended by 6 months, orchiopexy is performed before 1 year of age to prevent testicular torsion, improve fertility, and decrease risk of testicular malignancy

44
Q

Kallmann syndrome is characterized by a defective migration of […] cells and defective formation of the olfactory bulb.

A

Kallmann syndrome is characterized by a defective migration of GnRH cells and defective formation of the olfactory bulb.

results in delayed/absent puberty and anosmia with a normal karyotype

45
Q

[…] syndrome is characterized by a defective migration of GnRH cells and defective formation of the olfactory bulb.

A

Kallman Syndrome