Other Endocrinology Flashcards

1
Q

Hypoglycemia definition in Infants + Kids (not neonates)

A

PG = 2.8

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2
Q

Ddx for hypoglycemia (not neonatal)

A

endocrine: ketotic hypoglycemia, GH deficiency, pan-hypopit, ACTH deficiency, addison’s, excess exogenous insulin
other: sepsis/shock, liver disease, inborn error of metabolism, ingestion (ethanol, salicylates, B-blockers)

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3
Q

Work-up for hypoglycemia (draw before treatment)

A

PG, blood gas, lytes, tot and free carnitines, serum amino acids, ammonia, urine organic acids, c-peptide, lactate

urine for glucose, ketones, reducing substrates

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4
Q

management of hypoglycemia

A

tx if PG is <3.3 with symptoms, or <2.8

bolus dextrose (5ml/kg of D10), then continue infusion to maintain PG

if no IV: glucagon IM or SC, repeat q20min

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5
Q

congenital hypothyroidism - presentation

A

asymtomatic (Newborn screen), FTT, constipation, jaundice, hypotonia, periorbital + hand + feet edema, large fontanelles

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6
Q

W/u for hypothyroidism

A

clinical suspiction or +ve screen:

TSH, T4, thyroid scan

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7
Q

treatment goals for hypothyroidism

A

prevent delayed milestones, intellectual impairment, poor growth, hearing loss

treat with levothyroxine, regular follow-ups

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8
Q

congenital adrenal hyperplasia mechanism

A

21-hydrozylase enzyme defect, build up of 17-hydroxyprogestone.

Decreased cortisol and/or aldosterone, leads to increased ACTH -> increased androgens.

Spectrum of severity. Recessive.

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9
Q

CAH presentations

A

classical CAH:

  • salt wasting: FTT, dehydration, hypoN, hypo K, poor feeding, lethargy
  • females: ambigious genitalia /w normal internal organs
  • if unidentified, salt wasting crisis at 1-2wks old
  • non-salt wasting: toddlers /w signs of puberty (male)

NCCAH / late onset
- early puberty, accelerated bone age, signs of androgen excess, or asymptomatic

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10
Q

diagnostic test for CAH

A

17-hydroxyprogresterone

or other hormones for rarer forms

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11
Q

Management of CAH

A
  • ambigious genitalia - hx + px for signs of salt wasting
  • w/u with karyotype, FISH for SRY, pelvic US for int. genitalia
  • 17 hydroxyprog, glucose, lytes, VBG, androgens, renin
  • classic CAH: hydrocortisone + fludrocortisone + NaCl
  • NCCAH but symptomatic: glucocorticoid replacement
  • adrenal crisis management: fluids, correct K, hydrocortisone (after 17prog drawn)
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12
Q

CAH complications

A
  • adrenal crisis (forget meds, stress/illness)
  • short stature
  • osteoporosis
  • infertility (sometimes)
  • precocious puberty or delated menarche
  • increased metabolic syndrome
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13
Q

Definition of Short Stature

A

<3%ile OR
crossing major lines OR
low velocity (<25%ile)

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14
Q

Ddx for short stature

A
  • neglect
  • bone dysplasia (rickets, achondroplasi)
  • chromsomal (turners, downs)
  • chronic disease: CF, CHD, malnutrition, renal disease, chronic infection
  • constitutional delay of growth + puberty
  • endocrine: thyroid, cushings, hypogonadism, hypopit, GH def – rare)
  • familial short stature
  • GI malabsorption (celiac, crohns)
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15
Q

Conditions with short stature but normal growth veolicty (parallel to 3rd%ile but below it)

A

CDPG

  • Fx of delay but normal mid-parental height
  • will be normal height
  • bone age + puberty delayed
  • possible short term androgens for boys - caution, will close growth plates

Familial short stature

  • FHx short stature
  • no tx indicated
  • achieve mid-parental height

Syndromic (turner, achrondroplasia)
- follow %ile specific to condition /w normal velocity

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16
Q

Work-up for pathologic short stature

A

velocity <6cm/year

  • growth chart, vitals
  • arm span to lower segment ratio (increased in achondro, hypoT)
  • visual fields/fundi (pit)
  • tanner staging
  • nutrition, dysmorphism, chronic disease
  • mid parental height
  • bone age
  • TSH, T4, GH stim test
  • chronic illness: lytes, cr, BUN, LFT, Ca, IgA, ESR, urinalysis
  • celiac, CF work-up
  • karyotype in females, and males if dysmorphic
17
Q

Management of short stature

A
  • treat underlying cause
  • no tx if non-pathological
  • GH tx requirements: GH deficient on 2 stimulation tests, ht <3%ile or velocity <3ile, delayed BA, or turners/noonans/renal failure
  • consider: testosterone or estrogen in pubertal delay / CDGP - won’t increase final height but will decrease delay, GnRH agonists (to delay puberty + growth plate fusion)
18
Q

Normal Puberty in Girls

A

onset age 8 - 13

breast -> pubarche -> growth -> menarche

19
Q

Normal Puberty in boys

A

onset age 9-14

testes -> pubarche -> growth spurt

20
Q

Central Causes of Precocious puberty

A

hypergonadotropic hypergonadism

  • idiopathic
  • obesity
  • CNS lesions - tumor, encephalitis, trauma etc
  • primary hypothyroidism
21
Q

Peripheral Causes of Precocious Puberty

A

Hypogonadotropic hypergonadism

  • adrenal: CAH, adrenal neoplasm (contrasexual), cushings
  • ovarial disorders: cysts, granulosa cell tumor (E), theca and leydig tumors (T), germ cell tumors (gondotropin/hCG)
  • endogenous steroids
  • McCune-Albright syndrome
  • hypothydroidism
  • aromatase excess syndrome
22
Q

Evaluation of Precocious Puberty

A
  • abdo, skin, neuro exam
  • tanner staging
  • growth velocity
  • bone age
  • BMI
  • LH + FSH
  • estradiol, testosterone
  • DHEA-S + androstenedione
  • 17-hydroxyprogesterone
  • TSH, T4
  • prolactin

consider:
- pelvic US
- MRI head - central cause
- b-hCG
- GnRH or leuprolide stim test
- ACTH stim test (adrenal insuf)

23
Q

Treatment of Central Precocious puberty

A
  • GnRH agonists (leupron)
24
Q

Treatment of Peripheral precocious puberty

A
  • tx underlying pathology

- spironolactone, tamixofen, anastrozole, letrozole (aromatase inhib), or ketoconazole

25
Q

central causes of delayed puberty

A
  • idiopathic (CDGP)
  • systemic illness
  • exercise, anorexia, malnutrition
  • CNS lesions
  • endo: hypoT, low GH, hypopit, high PRL, high cortisol, DM
  • genetic: kallman’s syndrome
26
Q

peripheral causes of delayed puberty

A
  • gonadal failure (high LH + FSH)
  • genetic: turners, kleinfelters, noonans
  • gonads: infection, trauma, test torsion, rads/chemo
  • gonadal dysgenesis, cryptorchidism
  • hormonal: androgen insensitivity, 5-alpha-reductase deficiency
27
Q

delayed puberty w/u

A
  • neuro exam
  • growth velocity
  • tanner staging (>3cc - pubertal testes)
  • bone age
  • CBC, lytes, renal + liver function, ESR, CRP, UA
  • FSH, LH, testosterone, estradiol
  • TSH, T4
  • IGF1, IGF1BP-3

consider:

  • IBD panel, celiac panel
  • PRL
  • karyotype
  • head MRI
  • pelvic US
  • GnRH stim test