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Pediatrics > Endocrinology / Genitalia > Flashcards

Endocrinology / Genitalia Flashcards

1
Q

severe hypoglycemia, wandering nystagmus, and small penis size in neonate. What does he have?

A

hypopituitarism

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2
Q

Micropenis length in full term neonate

A

less than 2 to 2.5 cm

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3
Q

Causes of micropenis

A
  1. gonadotropin deficiency
  2. primary testicular dysfunction, such as with Klinefelter syndrome
  3. Partial androgen insensitivity syndrome (but usually more ambiguous genitalia)
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4
Q

Name syndrome associated with gonadotropin deficiency associated with anosmia or hyposmia, WITHOUT hypoglycemia

A

Kallmann syndrome

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5
Q

Syndrome where cryptorchidism and micropenis are common, but also Hypoglycemia due to poor intake, hypotonia, poor feeding, respiratory problems, and dysmorphic features

A

Prader-Willi

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6
Q

Adolescent girl has sudden onset nausea, vomiting, right lower abd /pelvic pain without fever and is hemodynamically stable. Can have dysuria/frequency. Top of your ddx: ___ and mode of dx: ____

A

Right ovarian twist
Pelvic ultrasound with doppler

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7
Q

What disease causes bilateral absence of the vas deference?

A

Cystic Fibrosis

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8
Q

Lab findings in hypoparathyroidism

A

HypoCa
High Phos (PTH causes phos excretion through kidney)
Normal/high Vit D 25-OH (PTH stimulates 1alpha-hydroxylase in kidney to form 1, 25 vit D
–> increase absorption of Ca in gut)

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9
Q

Lab findings in hypoparathyroidism

A

HypoCa
High Phos (PTH causes phos excretion through kidney)
Normal/high Vit D 25-OH (PTH stimulates 1alpha-hydroxylase in kidney to form 1, 25 vit D
–> increase absorption of Ca in gut)

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10
Q

In hypoparathyroidism, which type of vitamin D supplement do you need?

A

vit 1,25 D because PTH stimulates 1a-hydroxylase to turn vit D 25-OH to vit D 1,25 to absorb Ca in gut.

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11
Q

Name of syndrome with similar lab findings as hypoparathyroidism. What differentiates it?

A

Albright hereditary osteodystrophy

Distinction: short stature, obesity, rounded face, brachydactyly, mild cognitive impairment

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12
Q

Lab findings in vit D deficiency

A

Hypo Ca
low Phos (PTH is stimulated and makes kidneys excrete phos)

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13
Q

Definition of delayed puberty in girls

A

lack of breast development at age 13

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14
Q

Girl with turner syndrome has not started puberty at age 11-12, next step?

A

order gonatotropin levels (FSH/LH) –> in hypergonadotropic hypogonadism (primary ovarian failure), FSH and LH would be HIGH

Guidelines recommend starting estradiol for pubertal induction at age 11-12 years for Turner syndrome AFTER primary ovarian failure is confirmed

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15
Q

Mode of estradiol to be given in Turner syndrome

A

transdermal

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16
Q

Definition of primary amenorrhea

A

no menarche by age 15

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17
Q

“low-energy balance”

A

low ft4 with nl tsh
low bmi
low-normal fsh, lh

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18
Q

Addison Disease (primary adrenal insufficiency) lab findings

A

Decreased cortisol, aldosterone
High K, low cortisol
high ACTH (hyperpigmentation)
High renin (in response to low aldosterone)
Low sodium
Dehydration

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19
Q

Lab findings in secondary adrenal insufficiency (pituitary dysfunction)

A

low ACTH
low cortisol
NORMAL aldosterone (because RAAS is intact)
normal K
sodium can be low
No skin hyperpigmentation

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20
Q

Chronic mucocutanous candidiasis, hypoparathyroidism, primary adrenal insufficiency. Dx?

A

Autoimmune polyglandular syndrome type 1 (APS-1)

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21
Q

Tx of adrenal insufficiency

A

hydrocortisone
fludrocortisone if primary

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22
Q

First step of management in DKA

A

fluid bolus 10 ml/kg over 30-60 min to prevent cerebral edema

after fluids, THEN insulin

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23
Q

Criteria for constitutional delay

A

bone age < chronological age
Look at bone age and what his actual height is

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24
Q

Bone age in familial short stature

A

equal chronological age

25
Q

Growth hormone deficiency finding in terms of height

A

low height velocity <5cm/year

26
Q

Criteria for starting statin in children

A

ldl level persistently 160 mg/dL or higher despite dietary management.

27
Q

Causes of precocious puberty

A

adrenal gland, gonads, a human chorionic gonadotropin (hCG)–secreting tumor (in males), exogenous exposure to sex steroid, and severe hypothyroidism

28
Q

Most sensitive sign of HPA axis activation in central cause of precocious puberty

A

LH >0.3
In boys, testicular size >4ml
In girls, development of breast tissue

29
Q

What does measurement of 17-hydroxyprogesterone attempt to diagnose/rule out

A

Congenital adrenal hyperplasia due to 21-hydroxylase deficiency

30
Q

Growth pattern in growth hormone deficiency and lab to send

A

Delayed bone age
Low insulin-like growth factor-1

31
Q

In a boy not growing and has intrapartum excessive hemorrhage, what should you think about?

A

Sheehan syndrome (necrosis of pituitary gland) or pituitary hemorrhage

32
Q

how does acquired growth hormone deficiency present?

A

Slowing of linear growth at 6-12 mo, delayed bone age
midline defects
small penis, cryptorchidism (if gonadotropin deficiency is present)

33
Q

Most common cause of congenital hypothyroidism

A

thyroid dysgenesis (lack of or incomplete development of thyroid gland and etopic thyroid tissue)

34
Q

What happens with infant when mother has Graves disease?

A

maternal receptor-blocking antibodies can suppress infant thyroid gland, causing transient hypothyroidism

35
Q

If infant’s newborn screen shows TSH >40, next step?

A

start levothyroxine 10-15 microgram/kg/day by age 2 wk immediately without waiting for confirmatory test result

36
Q

How should levothyroxine be given?

A

on finger with small amount of breastmilk or formula or water

NOT through bottle (has affinity to plastic)

37
Q

Male infant born with hypospadias, small penis, testes, 46,XY karyotype, high LH, testosterone and DHEA, dx?

A

partial androgen insensitivity syndrome (PAIS)

38
Q

What is the process of normal sex differentiation?

A

Bipotential internal and external structures
SRY sex determining region) and other Y chromosome parts –> testes
Leydig cells secrete testosterone (testes)
Sertoli cells secrete anti-Mullerian hormone (AMH)
Testosterone STABILIZES wolffian ducts –> epididymis, vas deferens, seminal vesicles, ejaculatory ducts.
AMH –> regresion of Mullerian structures (uterus, fallopian tubes, cervix, upper vagina)
Testosterone —(5alpha-reductase)–> DHT
DHT virilizes external genitalia –> growth of phallus, fusion of labioscrotal folds, igration of urethra to normal position on penis

39
Q

Next step in a person with type 2 DM, hyperglycemia, polyuria, no acidosis

A

insulin

NOT fluids

40
Q

Signs and symptoms of hypercalcemia due to immobilization

A

wheelchair bound, pathologic fracture
appropriate PTH and PTHRH (low) in setting of hypercalcemia

41
Q

Serology for Graves Disease

A

+ TSH receptor antibody

42
Q

physical exam of congenital adrenal hyperplasia and karyotype

A

There are mullerian internal organs (uterus), 46,XX. But there is a penis, +/- fused labioscrotal fold without gonads within.

Excess 17a-hydroxyprogesterone is converted to DHEA –> testosterone (virilized female)

In 46,XY, they appear normal, however with salt wasting (hypoaldosteronism, low cortisol, high testosterone)

43
Q

Management of phimosis

A

If not pathologic, do nothing, just keep penis clean
If pathologic, can do topical steroids
Paraphimosis: emergency

44
Q

When should you be concerned of ovarian masses

A

cysts <6 cm

ones that do not self-resolve after 2-3 menstrual cycles

complicated cysts

45
Q

Labial adhesion that is asymptomatic in <5 yo, what’s the management

A

observation
It is self limiting

If symptomatic, trial of topical estrogen cream

46
Q

What age should cryptorchidism be corrected by?

A

12 mo

47
Q

What is the normal penis size of a term newborn

A

2-2.5 cm

48
Q

Definition of gynecomastia

A

> 0.5 cm diameter of fibroglandular mass

Benign: SMR 3-4; aromatization of androgen to estrogen

49
Q

Length rules of thumb

A

Increases 50% by 1 yo
Doubles by 4 yo
Triples by 13 yo
After 2 yo, velocity is 5 cm/year until puberty

50
Q

Weight growth rules of thumb

A

Doubles by 4 mo
Triples by 12 mo
Quadruples by 24 mo
After 2 yo, velocity is 5 lb/year until adolescence

51
Q

Pain referred to jaw, thyroid tenderness, fever, usually after URI

A

subacute thyroiditis

Tx: NSAIDs. May develop transient hypothyroidism

52
Q

Cause of neonatal thyrotoxicosis

A

Transplacental delivery of TSI antibodies from mother with Graves disease

Self limiting
Maternal Ab will degrade by 6 mo
May need methimazole or BB

53
Q

Albright’s hereditary osteodystrophy

A

Resistance to TSH, LH/FSH, PTH

Causes hypocalcemia (pseudohypoparathyroidism)– causes bracydactyly
Heterotopic intramembranous subcutaneous calcifications

54
Q

Rachitic rosary finding, dx?

A

Rickets
Enlargement of costochondral junction along anterolateral chest

55
Q

Rickets (cause, lab findings)

A

Vit D deficiency
High PTH, low phos, low Ca (cannot compensate for low Ca)

56
Q

Mutation in FGF23 signaling

A

hypophosphatemic rickets
Inability to renally absorb phos

57
Q

Hypophosphatasia gene and lab finding

A

ALPL
low ALP

58
Q
A
59
Q

When can you start GnRH therapy for gender affirming transition?

A

Smr 2

Pediatrics (26 decks)

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