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Endocrinology Flashcards

1
Q

As acidosis is corrected, what do you have to worry about in DKA rx?

A

Loss of K+ in urine, so should monitor carefully with EKG if necessary

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2
Q

When can cerebral edema most commonly happen after treatment initiation?

A

4 to 12 hours

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3
Q

What does hypokalemia look like on EKG?

A

U wave and Q-U prolongation

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4
Q

What is the Somogyi phenomenon?

A

hypoglycemia leading to rebound hyperglycemia.

So check 2am - if low then it is this

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5
Q

What is the rx to somogyi phenomenon?

A

decrease nighttime long-acting insulin

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6
Q

What is the Dawn phenomenon in DM?

A

Early AM glucose - check 2am, if still high, than increase nighttime long-acting insulin

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7
Q

What do you check on sick days for DM?

A

Check BG frequently, use rapid acting insulins,

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8
Q

Describe 2 dose insulin regiment?

A

2 shots/day with short&long acting insulin
2/3 daily in AM, 1/2 in PM
2/3 long-acting, 1/3 short-acting

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9
Q

How many hours of TV/computer use/day?

A

2 hrs

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10
Q

Obese at age 6, likelihood of obese as adults

A

25%

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11
Q

Obese at 12, likelihood of obese as adults

A

75%

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12
Q

Most common complication of obesity.

A

Low self-esteem

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13
Q

What is the glucose level and serum bicarbonate, effective serum osmolarity in hyperglycemic hyperosmolar syndrome?

A

Glucose >600mg/dL

Serum bicarbonate >15mmol/L, 320 mOsm/L

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14
Q

Rx for hyperglycemic hyperosmolar syndrome

A

aggressive hydration with 0.45% saline, insulin later

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15
Q

Tall and fat, endocrine or not

A

Obesity

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16
Q

Pattern: mildly obese, short, hypoglycemia, micropenis, bone age delay, single maxillary incisor, optic nerve hypoplasia, cleft lip/palate

A

Growth hormone deficiency - hypopituitarism

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17
Q

Pattern: floppy baby, mildly retarded, sucking problems, very obese as he grows, hypoplastic penis/scrotum, small testicles and hands/feet, large appetite

A

Prader-willi syndrome

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18
Q

Pattern: moderate obesity, round face with short neck, delayed dental eruption, aplasia, short 4th, short 4th metacarpals/metatarsals, extraskeletal calcification, variable hypocalcemia, hyperphosphatemia

A

PseudohypoPTH

Albright hereditary osteodystrophy

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19
Q

Pattern: obesity, mental deficiency, retinal dystrophy, polydactyly, syndactyly, brachydactyly, broad, short feet, abnormal kidneys, small penis/tests (hypogonadism)

A

Bardet-Biedl syndrome

A ciliopathic disorder

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20
Q

Pattern: obesity later, hypotonia, brushfield spots, clinodactyly with simian crease, endocardial cushion defect, small penis and small testicles

A

Down syndrome

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21
Q

Dopamine prolactin relationship

A

DA causes reduction or prolactin

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22
Q

DI also causes what?

A

Hypernatremia

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23
Q

If hypo or hypernatremia, what labs do you want to do?

A

urine osmolality, serum osmolality, serum and urinary sodium

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24
Q

High urine osmolality, low serum osmolality

A

SIADH

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25
Q

Low urine osmolality, high serum osmolality

A

DI

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26
Q

Treatment for SIADH

A

Fluid restrict, 1000cc/m2/day or less

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27
Q

What is level of TSH for congenital hypoTH dx?

A

TSH >25

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28
Q

Pattern: prolonged jaundice, severe growth and DD, constipation, coarse features, umbilical hernia and large anterior fontanelle

A

congenital hypoTH

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29
Q

What are endocrine problem should you be aware of in DM patients?

A

10-30% also have Hashimoto, autoimmune polyglandular syndrome II

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30
Q

Pattern: growth fall-off, mild relative weight gain, dry skin/myxedema, delayed relaxation phase of DTRs, coarse hair, mild hair loss

A

HypoTH

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31
Q

Range of TSH in hypoTH

A

> 50, 100, 200

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32
Q

Pattern: high TSH, microcytic anemia

A

HypoTH

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33
Q

What dose of levothyroxine for newborn with hypoTH?

A

10-15 mcg/kg/day

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34
Q

What dose of levothyroxine for older pts with hypoTH?

A

100mcg/m2/day

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35
Q

If longstanding or TSH>100, what regiment of levothyroxine do you want to give - aka uptitration plan

A

Start at 1/4 to 1/3 dose and work up slowly over 3-4 weeks

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36
Q

What are three major causes of hyperTH?

A
  1. Graves autoimmune
  2. Thyroiditis with release of LT4 from gland in acute phase of Hashimoto or DeQuervain (painful, negative Ab’s)
  3. Inappropriate use of LTH for weight loss
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37
Q

Pattern: jittery, weight loss, agitation, fatigue (cannot sleep), heat intolerance-diaphoresis, proptosis/exophthalmos

A

Graves hyperTH

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38
Q

Pattern:

+TSH receptor Ab, + Anti TP, +Anti TG

A

Graves hyperTH - don’t be fooled by other antibodies

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39
Q

What is high risk side effects of methimazole?

A

agranulocytosis

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40
Q

Rx of Graves

A
  1. Radiactive idoine ablation
  2. Thyroid surgical resection
  3. Can use idoine (to slow down met of TH), prednisone, propanolol
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41
Q

Any solitary nodule needs what kind of workup?

A

Thyroid US
Uptake scan
Fine needle aspiration

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42
Q

Percentage of solitary nodule that is malignant

A

20%

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43
Q

Rapid growth, firm nodule, +FH of medullary CA, history of neck radiation

A

Thyroid cancer

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44
Q

Characteristic of thyroid nodule that is less likely to be cancer.

A

Cystic nodule, TPO/TG Ab’s

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45
Q

What initiates genital structure development?

A

Y chromosome with SRY causing gonads to develop towards testicles

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46
Q

What does maternal HCG signal in the fetus for genital structure differentiation?

A

Testes to secrete T, DHT –> external genital vrilization

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47
Q

What stabilizes the Wolffian internal structures?

A

Testosterone

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48
Q

Testes also produce AMH to do what?

A

induce female structures (Mullerian) to regress

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49
Q

At what week is genital structure formed?

A

14 weeks, shape, but not size

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50
Q

What hormone/level/problem seen with 46XY DSD

A

Subnormal T or DHT

Insensitive to T

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51
Q

What hormone/level/problem see with 46 XX DSD

A

excess maternal androgens, exogenous androgens, fetal adrenal CAH

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52
Q

Ovotestes 46 XX, 46 XY or mosiac

A

both ovary and testicle

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53
Q

Pattern: External female genitalia, internal no uterus, testicles present, no pubic/axillary hair, breast tissue at puberty, no menses

A

Complete androgen insensitivity - X-linked

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54
Q

Pattern: ambiguous genitalia, internal male, no uterus, some pubic hair

A

Partial androgen insenstivity

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55
Q

Sign of low DHT?

A

genitalia not virilized

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56
Q

At puberty for pt with 5 alpha reductase deficiency, why can you see virilization?

A

Testosterone is excessively high and can virilize

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57
Q

Pattern:

Female salt-wasting, ambiguous genitalia,

A

21-OH deficiency

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58
Q

Pattern:

Male salt-wasting, early adrenarche/puberty

A

21-OH deficiency

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59
Q

Pattern:

Teenage girl, hirsutism, menstrual irregularities

A

21-OH deficiency non-classical

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60
Q

Pattern:

Virilized female, hypertension

A

11-B hydroxylase deficiency

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61
Q

what are complications of untreated 11-B hydroxylase deficiency

A

advanced bone age and early adrenarche

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62
Q

Workup of CAH

A 
pre +/- post ACTH stim is gold standard
17 OH progesterone (21 OH deficiency)
DOC (11 beta OH deficiency)
17 OH pregnenolone (3-B hydroxysteroid)
Renin/aldosterone
DHEA, andro, and ACTH
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63
Q

What is the RX for acute salt wasting-adrenal crisis?

A

Fluids + hydrocortisone 25mg IM/IV then 100mg/m2/d divided q6 hours

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64
Q

What is chronic rx for salt wasting and virilizing?

A

hydrocortisone 15-25 mg/m2/day, fludocort (mineralcortidoid)
Follow up 17OHP, andro, renin levels q3 months

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65
Q

What are causes of Addison’s disease (primary adrenal insufficiency)?

A
  1. Autoimmune destruction APS1 (AIRE) adrenal and mucocutaneous candidiasis APS2 (Schmidt) adrenal, DM I, Hashimoto
  2. Infection (TB)
  3. Adrenal hemorrhage post-infection (WFS)
  4. Adrenal hypoplasia congenita
  5. Adrenoleukodystrophy
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66
Q

Pattern: Salt-losing crisis in neonatal period, 21 OH is not deficient, no virilzation in females, normal male genital development

A

Adrenal hypoplasia congenita

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67
Q

What is genetic cause of adrenal hypoplasia congenita

A

NROB1 (DAX-1)

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68
Q

How do you treat adrenal hypoplasia congenita?

A

Same treatment as in 21 OH, but lower doses of HC are needed than in CAH

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69
Q

Pattern:

Normal neuro development, then loss of milestones, clumsiness, adrenal insufficiency, grumpy and irritable

A

Adrenoleukodystrophy

ABCD1 gene x-linked

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70
Q

Pattern:

Hypotension, hyponatremia, hyperkalemia, acidosis, weight loss, vomiting, hyperpigamenation

A

Acute severe adrenal insufficiency

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71
Q

Causes of Cushing symptom

A
  1. Exogenous steroids
  2. Cushing’s disease (ACTH production)
  3. Adrenal tumor
  4. Ectopic expression
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72
Q

Pattern: growth failure, truncal obesity, acne/hirsutism, hypertension, purple striae, weakened bones

A

Cushing syndrome

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73
Q

How to test for Cushing’s syndrome

A

Overnight dexamethasone suppression, 1mg at 11pm, check cortisol level at 8am, if no suppression, dx made
24-hour urine for free cortisol

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74
Q

Pattern:

Headache, HTN, palpitations, sweating

A

Pheochromocytoma - caused by excess catecholamines

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75
Q

Dx of pheochromocytoma

A

Urinary catecholamines, urinary metanephrines, MRI of adrenals, MIBG scan

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76
Q

Glucose level to dx hypoG

A
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77
Q

Pattern: new born, low glucose, big tongue, umbilical hernia

A

Beckwith Wiedemann syndrome

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78
Q

Pattern: newborn, low glucose, just got UA catheter

A

malposition of UA catheter too high up supplying excess glucose to pancreas so reactive response

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79
Q

4 hormones that raise blood glucose - how many has to be deficient to cause hypoglycemia

A

epinephrine, glucagon, cortisol, growth hormone

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80
Q

Pattern: Large for gestation age baby, with seizures, hypoG, pre and post prandial, needs lots of glucose

A

Hyperinsulinism

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81
Q

Workup in baby with hypoglycemia

A

paired insulin/glucose, UA for ketones

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82
Q

Pattern: hypoG at random times, no ketones

A

Hyperinsulinsim or FA oxidation

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83
Q

Pattern: hypoG and midline defects or nystagmus

A

Pituitary (GH/Cortisol)

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84
Q

Pattern: hypoG after overnight fast,

A

GH/cort or FA oxidation defect

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85
Q

PTH’s net effect

A

increase Ca, decrease P

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86
Q

Vit D’s net effect

A

increase absorption of Ca and P

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87
Q

Vit D’s effect on PTH

A

Feed back inhibition on PTH

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88
Q

PTH increases Ca by 3 mechanisms

A
  1. increase bone resorption
  2. increase Ca resorption from kidney
  3. increase Vit D (25 to 1,25 dihyd) to increase gut absorption
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89
Q

Calcitonin net effect

A

decreases Ca blocks osteoclastic resorption.

it is secreted by C-cells

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90
Q

Hypocalcemia s/sx

A

Tetany, seizures, chvostek (tap on cheek, 7th nerve), trousseau (bp cuff), irritability

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91
Q

Hyper/Hypo Ca workup

A

1) iPTH w/ paired calcium
2) 25, hydroxy & 1,25 dihydroxy
3) Mg (co-factor for PTH secretion) & P
4) Urinary Ca and Creatinine

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92
Q

Where is the problem in hypoPTH, pseudoPTH

A

1) Secretion/production

2) bad receptor

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93
Q

Labs seen in hypoPTH

A

low Ca, high PO4, low PTH

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94
Q

4 disorders that can causes hypoPTH newborns/infants

A

1) DiGeorge/velocardiofacial
2) CHARGE assocation
3) Autoimmune polyglandular syndrome I/II
4) Familial hypoPTH (AD)

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95
Q

Pattern: cardiac/palatal defects, broad nose, long fingers, T-cell dysfunction (infection), deletion of 22q11 region

A

DiGeorge/Velocardiofacial

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96
Q

Pattern:

low Ca, high PO4, high PTH (>300)

A

pseudoPTH

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97
Q

What disorder is associated with pseudoPTH

A

Albright hereditary osteodystrophy

98
Q

Pattern: Short stature, shortened 4th/5th digit, DD, subcutaneous calcification

A

Alright hereditary osteodystrophy

99
Q

Pattern: low Ca, low P, high Alk Phos

A

Vit D deficiency

100
Q

Major causes of Vit D deficiency

A

Decreased intake, absorption (ADEK, CF pt), production (excessive use of sunscreen, dark skinned children at risk)

101
Q

How much VitD needed in first 2 months?

A

400 IU/day

102
Q

How much to replete VitD deficiency?

A

1000 IU/day

103
Q

What is calcitriol

A

activated in Vitamin D - 1,25-dihydroxycholecalciferol or 1,25-dihydroxyvitamin D3

104
Q

Pattern: normal Ca, low P and high urine P, high Alk Phos, bowing of legs, widening cupping and fraying of distal metaphyses

A

Hypophosphatemic rickets

105
Q

Cause of hypophosphatemic rickets

A

Genetic - X-linked (not sure what the mutation is)

106
Q

Rx of hypophosphatemic rickets

A

Calcitriol and P

107
Q

Pattern: polyuria, nephrolithiasis, bone pain, depression, ulcer/nausea

A

hypercalcemia

108
Q

Pattern/cause:

Low iPTH, hi Ca, low Phos,

A

Check PTHrP, most likely high. Secreted by tumor.

109
Q

Pattern/cause:

Low PTH, hi Ca, hi Phos

A

Hypervitaminosis D

110
Q

Pattern:

Normal PTH, hi Ca

A

Benign familial hypercalcemia fro CASR gene mutation, receptor is insensitive to Ca

111
Q

What genetic disorder should you think of when you see hypercalcemia?

A

Williams Syndrome

112
Q

Pattern/cause:

High PTH, elevated Ca, low phos

A

HyperPTH-parathyroid adenoma

113
Q

Rx for hypercalcemia

A
  1. Hydration
  2. furosemide (short term), 3. Glucocorticoids ( short term - incr urinary output), 4. Calcitonin (short term - tachyphylaxis - loses efficacy)
  3. Bisphosphonates (holds calcium/phosphorus in bones, more long term)
114
Q

Babies double weight by when and triple by when?

A

2x by 4 months

3x by a year

115
Q

Growth velocity during 1st, 2nd, and 3rd year.

A

1st year - 24cm/yr
2nd year - 12cm/yr
3rd year - 8cm/yr
(year x GV=24)

116
Q

What is growth velocity in boys and girls at puberty peak?

A

Puberty peak 10cm/yr (boy)

8cm/hr (girl)

117
Q

Pattern:

Short, delayed bone age, normal labs, following own curve

A

Constitutional delay of growth AKA late bloomers

118
Q

Pattern:

Short, normal labs, family is short

A

Familial short or tall stature

119
Q

Formula for determining potential height of child

A

If boy - average parents, add 5 inches (13cm from to moms)

If girl - average parents (subtract 5 inches from dad) +/- 4 inches

120
Q

What should you think of if pt is short

A

Growth hormone deficiency

121
Q

What genetic mutations associated with growth hormone deficiencies?

A

HESX, PROPR1, POUF1, GHR (Laron)

122
Q

Pattern:

Recurrent low blood sugar in infancy, growth slows after 6 months, growth velocity falls from curve

A

Growth hormone deficiency

123
Q

How do you diagnose GH deficiencies?

A

IGF-1, IGF-BP3, bone age of hand/wrist (not specific), GH stimulation tests?, Pituitary MRI

124
Q

Side effects of GH

A

pseudotumor cerebri, scoliosis, SCFE, thyroid and cortisol deficiences

125
Q

6 common syndromic short stature

A
  1. Trisomy 21
  2. Turner Syndrome
  3. Noonan syndrome
  4. Prader-Willi syndrome
  5. Pseudo-hypoPTH (Albrights hereditary osteodystrophy)
  6. Russell-Silver syndrome
126
Q

Pattern:
Short stature, low hairline, wide spaced nipples, poor breast development, elbow deformity, shortened metacarpal, webbed neck

A

Turner

127
Q

Pattern:
Short stature, low hairline, shield chest, right sided cardiac (pulmonary stenosis), no renal defects, usually fertile, boy or girl

A

Noonan syndrome

128
Q

Difference between Turner and Noonan

A 
Cardiac problem:
Turner - Coart/bicusp AV; Noonan - pulmonary stenosis
Renal
Turner - yes; Noonan - no
Fertility
Turner - infertile; Noonan - fertile
129
Q

3 common symptoms b/w Turner vs Noonan

A

Short stature, low hairline, shield chest

130
Q

Pattern:

Short stature, DD, obesity/hyperphagia, hypogonadotropic, hypogonadism (small phallus/testicles, pubertal delay)

A

Prader-Willi syndrome

131
Q

Pattern:

Small at birth, limb assymetry, triangular head, cryptoorchidism, no catch-up growth

A

Russell-Silver

132
Q

7 common causes of tall stature

A
  1. Klinefelter syndrome (47XXY)
  2. marfan syndrome
  3. Homocystinuria
  4. HyperTH (end height not different, just accelerated growth)
  5. GH excess (gigantism)
  6. Precocious puberty (only temporary)
  7. Soto syndrome (cerebral gigantism)
133
Q

Pattern: short stature, triangular facies, low hairline, high arched palate, hypoplastic nipples and increased carrying angle

A

Turner - missing X

134
Q

What heart and renal conditions is associated with Turner syndrome?

A

Coarctation of aorta

Horseshoe kidney

135
Q

What test do you do when you are suspicious for Turner?

A

Karyotyping, LH/FSH (they should be high because of ovarian failure)

136
Q

What thyroid problem is associated with Turner syndrome?

A

Antibody positive chronic lymphocytic thyroiditis and hypoTH

137
Q

What is the initial screen for hypoTH?

A

TSH, then free thyroxine and antithyroid antibodies (thyroperoxidase, antimicrosomal or anithyroglobulin)

138
Q

Presence of Y chromosome in Turners results in what?

A

Androgenization, gonadal malignancy often requiring ppx gonadectomy

139
Q

What is the height and weight growth like for Cushing syndrome?

A

Weight is normal to high, attenuation of height growth

140
Q

What are the two thyroxine binding protein? Which is seen in excess in 1 to 8000 individuals? What blood measurement does this problem make inaccurate?

A

TBG - can be seen in excess
TBPA
TT4 is useless

141
Q

T3 is made in what two ways?

A

15% by thyroid gland, rest from deiodination from T4

142
Q

What conditions affect deiodination of T3?

A

Fed state is proportional to

143
Q

Can hypoTH affect bone?

A

Yes, it can delay bone age

144
Q

What is the most common cause of acquired primary hypoTH?

A

Chronic lymphocytic thyroiditis (Hashimoto’s)

145
Q

What antibodies must be measured to evaluate for lymphocytic thyroiditis?

A

Again thyroperoxidase enzyme and thyroglobulin

146
Q

If you are still suspicious a child has hypoTH but negative for antithyroid antibodies, what additional testing should you do?

A

TH US and then technetium or radioactive iodine imaging

147
Q

Describe in DETAIL 6 features of hyperTH

A
  1. Weight loss and increased appetite
  2. Decreased strength, muscle weakness, loss of thenar and hypothenar eminence muscle mass, decreased sports performance.
  3. Moist skin, thinning hair, skin darkening
  4. Poor sleep, nervousness, anxiety, irritability, pruritus, nocturia, hyperactivity, tremors
  5. Decreased school performance
  6. Decreased menses in girls
148
Q

In addition to high free T4, what other blood value is high in hyperTH?

A

T3 and TSH IgG in Graves

149
Q

How can you test whether hyperTH is due to subacute thyroiditis or Graves disease?

A

Radioactive iodine
Subacute thyroiditis - low uptake
Graves disease - high uptake

150
Q

What is the major life-threatening complication of rx for DKA?

A

Cerebral edema

151
Q

Outline the major steps to Rx of DKA?

A
  1. Hydrate but sparingly
  2. Correcting K and insulin
  3. Correcting acidosis
152
Q

How should fluid replacement be done in DKA?

A

Calculated steady replacement of 0.45% or 0.9% with glucose and K as needed

153
Q

How should K replacement be done in DKA?

A

As soon as child starts urinating, then start K repletion

154
Q

What caution should be taken when correcting peripheral acidosis?

A

Bicarbonate dissociates in bicarbonate ion and CO2 so can increase intracellular and CNS acidosis to increase risk of cerebral edema

155
Q

What happens with sodium in DKA?

A

High losses in urine

156
Q

What thyroid problem is seen in 10% of type 1 diabetics?

A

Chronic lymphocytic thyroiditis - Hashimoto thyroiditis (autoantibodies - like the ones that cause the diabetes)

157
Q

What can pt with Hashimoto thyroiditis eventually develop?

A

Interestingly HypoTH or Graves disease - ? burns out or acquire additional antibodies

158
Q

What gene is associated with polylglandular autoimmune syndrome type 1?

A

AIRE

159
Q

What autoimmune disorder is a part of polyglandular autoimmune syndromes?

A

Type 1 diabetes, candidiasis, hypoPTH, Addison, pernicious anemia, vitiligo

160
Q

What autoimmune problems are associated with polyglandular autoimmune syndrome type 2?

A

Autoimmune thyroid disease, diabetes, adrenal insufficiency

161
Q

What is the most likely outcome in baby with congenital hypoTH who does not receive treatment within the first few days of life?

A

Cognitive deficits

162
Q

What are the signs of growth hormone excess?

A

Widened spaces between teeth, large hands and feet, increased soft-tissue thickening

163
Q

What disorder is upper to lower segment ratio useful in clinical diagnosis?

A

Marfan syndrome

164
Q

What test should be ordered to work up male precocious puberty?

A

Serum T, 17-hydroxyprosterone, dehydroepiandosterone, LH/FSH and HCG (which mimics LH to activate Leydig cells to produce T)

165
Q

What tumors makes HCG?

A

Germ cell in CNS, mediastinum, liver (hepatoblastomas)

166
Q

Adrenoleukodystrophy is caused by what gene mutation and what builds up as a result?

A

ABCD1, defective oxidation of LCFA in peroxisomes. VLCFA accumulate in myelin, adrenal glands and elsewhere

167
Q

What is the typical pattern on growth chart for familial short stature?

A

In first 2 years, reach certain growth percentile and follow the curve

168
Q

What age is bone age radiograph useful for predicting adult height?

A

6 or 7 years

169
Q

What signs can be associated with early menarche?

A

Thelarche a couple of years before

170
Q

What are signs of gonadal puberty in girl?

A

Menarche and thelarche (breast bud)

171
Q

What are signs of adrenal puberty (adrenarche or pubarche)?

A

Adult body odor, pubic hair, acne

172
Q

What is Addison’s disease?

A

Primary adrenal insufficiency

173
Q

What happens to sodium and potassium in Addison disease?

A

Low serum sodium (cannot hold it without mineralcorticoid), high K (cannot excrete K)

174
Q

What happens to cortisol and ACTH levels in Addison?

A

Low cortisol, high ACTH

175
Q

Addison is a deficiency in what?

A

Aldosterone

176
Q

What are complications of aldosterone deficiency?

A

Dehydration, break down of muscle tissue, hyponatremia, hyperkalemia, elevated BUN and acidosis

177
Q

What are manifestation of ACTH deficiency?

A

Cortisol deficiency –> weight loss, nausea, inability to maintain blood pressure, low IV volume leads to release of vasopressin

178
Q

Why isn’t there hyperkalemia in ACTH deficiency?

A

Aldosterone is still released

179
Q

Hyperpigmentation accompanies which adrenal axis problem?

A

Primary adrenal insufficiency (high ACTH)

180
Q

Pattern: firm, enlarged thyroid

A

Hashimoto, antibody against thyroperoxidase and thyroglobulin

181
Q

What is random glucose level that indicates DM?

A

> = 200mg/dL

182
Q

HgbA1c level for DM dx

A

> =6.5%

183
Q

Fasting plasma glucose level for DM dx

A

> = 126mg/dL

184
Q

what are marker antibodies for DM Type 1

A

Anti-islet, anti-insulin, Anti-GAD65, Anti-ZnT8A

185
Q

How much B-cell mass must be destroyed before symptoms of diabetes occur?

A

80-90%

186
Q

What is the honeymoon period in DM type 1?

A

With insulin, remaining live cells light up and start producing insulin, because they too need insulin to function - but then they are attacked

187
Q

Insulin’s 4 roles

A
  1. Increases glucose uptake, along with amino acids and lipids
  2. Decreased glucagon production and glycogenolysis
  3. Enhances glycogen, protein and fat synthesis
  4. Inhibits fat breakdown (lipolysis) and ketogenesis
188
Q

What four hormones increase glucose?

A
  1. Growth hormone
  2. Glucagon
  3. Epinephrine
  4. Cortisol
189
Q

What causes the lactic acidosis in DKA?

A

Hypertonic dehydration

190
Q

What pH and HCO3 classifies as mild, moderate, severe DKA?

A

Mild

191
Q

How to get true sodium level in setting of hyperglycemia?

A

Corrected Na+ = measured Na + 0.016 (measured glucose - 100)

192
Q

For what three reasons do you give insulin in treatment of DKA?

A
  1. Turns off ketogenesis
  2. Minimizes osmotic diuresis
  3. Enhances tissue substrate utilization
193
Q

Does bicarbonate and phosphate affect recovery rate in DKA?

A

No

194
Q

What is harm of excessive chloride in DKA Rx?

A

Prolong acidosis

195
Q

When Na+ is low in obtunded DKA pt, what do you need to think of?

A

Cerebral edema secondary to SIADH

196
Q

Pattern: HA, decrease in LOC, decreased HR, increased BP 4 to 12 hours after rx initation

A

Cerebral edema

197
Q

Pattern:

Small at birth, limb assymetry, triangular head, cryptorchidism, no catch up growh

A

Russell-silver - Will use GH for them although not deficient

198
Q

Adrenarche

A

Aka pubarche - development of pubic hair and acne secondary to adrenal androgens

199
Q

Thelarche

A

Development of breast tissue

200
Q

Gonadarche

A

Enlargement of testicles or ovaries

201
Q

LH acts on what

A

gonad to produce T and ovarian androgens

202
Q

FSH acts on what

A

Gonads to produce estrogens and inhibin

203
Q

Tanner 2 female

A

Areola - labial hair

204
Q

Tanner 3 female

A

Mound and smooth contour - hair creeps to mound

205
Q

Tanner 4 female

A

Nipple starts protruding - fuller hair

206
Q

Tanner 5 female

A

Full contour - triangular

207
Q

Progression of female development

A 
Thelarche - 8 to 13
Adrenarch/pubarch 9-14 
Growth spurt 
Menarche - 2 yrs after start of puberty average 12.4 yr
T-A-G-me
208
Q

Tanner 2 male

A

testicle enlarge, dropping of testicle

209
Q

Testicle enlargement occurs to what volume

A

to 4cc

210
Q

Progression of male development

A 
Testicular enlargement 9-14
Pubic hair follows 6mo-1yr later
Penile enlargement
Growth spurt Tanner III-IV
Axillary hair - then facial hair
T-H-P-G-A
211
Q

Age of puberty that qualifies as precocious

A

8 for girls

9 for boys

212
Q

Precocious puberty workup

A

LH, FSH, estradiol/testosterone, bone age (often advanced 2-3 years)

213
Q

How do you interpret leuprolide (GNRh) stimulation test

A

Rise in LH –> PP
No rise - benighn premature thelarche
Suppression - peripheral puberty

214
Q

Pattern:

Early development, low LH&FSH, high T or E2

A

Peripheral precocious puberty

215
Q

What is cause of peripheral precocious puberty in boys only?

A

Constitutively active LH receptor in familial male gonadotropin-independent precocious puberty

216
Q

Pattern:

Cafe au lait, fibrous dysplasia, prec pub

A

McCune Albright Syndrome - G protein abnormalitiy

217
Q

Rx of central precocious puberty

A

Lueprolide, histrellin, GnRH agonists constant will agent, decreased LH/FSH

218
Q

Rx of peripheral puberty

A

Remove offending agent, block peripheral effects of sex hormones (androgen receptor blockers; estrogen receptor blockers)

219
Q

Delay puberty workup

A

LH,, FSH, estradiol, testosterone, TSH, chemistry panel, ESR, bone age

220
Q

Age when puberty is considered delayed

A

13 in girls, 14 in boys

221
Q

Pattern:

Normal height pre-adolescence, no growth spurt, may have adrenarche, anosmia

A

Kallman syndrome

hypogonadotropic hypogonadism

222
Q

Two disorders associated with panhypopit

A

septo-optic dysplasia, empty sella syndrome

223
Q

2 genetic syndromes associated with delayed puberty

A

Prader Willi syndrome

Leptin deficiency

224
Q

Hypergonadotropic hypogonadism 5 causes

A
  1. Mumps orchitis
  2. Turner syndrome
  3. Klinefelter
  4. Functional ovarian failure
  5. Trauma, chemo, gonadal removal
225
Q

Pattern:

Tall stature, eunuchoid proportions, gynecomastia, stalled puberty, testicles noted to be small during puberty

A

Klinefelter - start developing and then stall

226
Q

What is the most common cause of childhood hypoglycemia?

A

ketotic hypoglycemia

227
Q

What is the cause of ketotic hypoglycemia?

A

Imbalance between glucose utilization and production through hepatic, renal glycogenolysis and gluconeogenesis

228
Q

Pattern: fasting hypoglycemia in child less than 8yo

A

Ketotic hypoglycemia

229
Q

To determine if hyperinsulinism is the cause of low blood glucose, what should you measure and when and what results to anticipate?

A

At time of hypoglycemia, measure insulin and c-peptide. C-peptide is secreted with insulin so if low when insulin is high, more likely to be excess amt of exogenous insulin.

230
Q

What is best first test for suspected ketotic hypoglyemia?

A

urine dipstick for ketones

231
Q

Pattern: seizures, hypotonia growth delay, signs of rickets (prominent forehead, rachitic rosary, enlarged wrists and ankles).

A

Rickets: vit D deficiency

232
Q

Pattern: slowed mentation, stupor, constipation, polyuria, renal calculi, extreme thirst

A

Hypercalcemia

233
Q

Pattern: neuromuscular irritability like hypocal, nausea, loss of appetite

A

hypomagnesemia

234
Q

Pattern: muscle weakness and changes in mental status, no neuromuscular irritability

A

Hypophosphatemia

235
Q

What are some causes of hypercalcemia?

A

hyperparathyroidism, hypervitaminosis A (stimulates osteoclast activity), hypervitaminosis D, immoblization (osteoclast activation and relative bone resorption)

236
Q

Rx for familial hypophosphatemic rickets?

A

Oral neutral phosphate salts with calcium (b/c phosphate takes Ca+ into bone)

237
Q

Pattern: 2 year old with unilateral breast tissue

A

Premature thelarche caused by release of FSH and LH stimulating ovarian estrogen production and will regress by 2 years of age

238
Q

Pattern: 3yo with lump that moves with swallowing and centrally located

A

Thyroglossal cyst –> surgical consult

239
Q

What is the kinetics of ultrashort-acting insulins

A

Action begins about 10 to 15 minute after administration, peaks at 1 hr and lasts no longer than 3 to 4 hours

240
Q

What are rules for sick days for type 1 diabetic kid?

A
  1. Check blood glucose every 2 hours
  2. check urine or blood ketones every 2 to 4 hours
  3. Provide small amounts of normal short-acting insulin every 2 to 3 hours as long as his blood glucose is >100mg/gL and he can consume sugar-containing liquids to stay hydrated
241
Q

Causes of polyuria

A

DM, renal disease, DI, hyperTH, hyperCal, hypomag

242
Q

Pattern: 11yo boy, nocturnal enuresis, no glycosuria, serum glucose normal, no UTI

A

hypercalcemia

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