Endocrinology Flashcards

1
Q

thyroid hormone starts production at

A

12 weeks? 18-20 weeks?

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q
A
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

11 beta hydroxylase

A

2nd MCC CAH. Middle Eastern descent. Inability to convert deoxycorticosterone to aldosterone
Inability to convert 11 deoxycortisol to cortisol
Excess of 17 OHP –> increased serum androgen production
No salt wasting (deoxycorticosterone present)
Tx. glucocorticoid replacement, genital reconstruction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

MCC congenital heart disease of IDM

A

TGA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

MCC congenital hypothyroidism

A

thyroid dysgenesis (75%) - partial or complete absence of thyroid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is pseudohypoparathyroidism

A

defects in peripheral PTH receptors, hypocalcemia in setting of elevated PTH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Features of Vitamin-D dependent rickets

A

pathologic fractures, rachitic rosary, moth-eaten metaphyses

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Hypoparathyroidism causes

A

glandular hypoplasia, neonatal glandular suppression as result of maternal hyperparathyroidism, autoimmune parathyroid its, mutations in Ca receptor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Parathyroid hormone actions

A

stimulates activity of renal 1-alpha-hydroxylase –> increases active form of Vitamin D and indirectly increases intestinal Ca and phosphorus absorption. Increases renal Ca absorption and decreases renal phosphorus absorption

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

IDM causes what electrolyte disturbances

A

neonatal hypocalcemia, hypoglycemia, hypomagnesemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Only ____% of neonates born to women with Grave’s will be clinically affected

A

1%

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

There is a TSH _____ after birth with markedly _____ TsH concentration compared to older infants

A

surge, elevated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

T4 is highest in first ____ of life. T3 tends to rise after first ____ and increases during first _____. rT3 _____ postnatally

A

week
week, month
decreases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

IDM mechanism for surfactant deficiency

A

increased fetal insulin inhibitory action on fibroblast-pneumocyte factor (acts on type 2 alveolar cells to produce surfactant)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Thyroid gland embryogenesis is complete by _____ weeks of gestation. Begins to secrete hormone at ____ weeks

A

10-12 weeks. 12 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Fetal TSH receptors don’t become responsive to TSH and TRaBs until _____ weeks

A

20 weeks

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Progressive increase in T4 and thyroxine-binding globulin in fetus, as well as increase in free T4 between _____ weeks of gestation

A

18-36

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

5-alpha reductase deficiency

A

AR; limits conversion of testosterone to DHT.
Sx: ambiguous genitalia, appropriately differentiated Wolffian structures, absent Mullerian-derived structures, small phalus, urogenital sinus with perineal hypospadias, blind vaginal pouch
Later in life: progressive virilization with decreased facial hair and small prostates “Testicles at Twelve”
Females: normal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Aromatase deficiency

A

Prevents conversion of testosterone to estradiol (androstenedione is not ultimately converted to estrone). Affected females have Mullein duct structures and absent Wolffian duct structures, evident by ambiguous genitalia or cliteromegaly. Multicystic ovaries, tall strature, virilization at puberty, delayed bone age

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Name the CAH enzymatic disorders

A

3-beta-hydroxysteroid dehydrogenase deficiency, 17-alpha hydroxyls, 21-hydroxylase, 11-beta hydroxylase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Hyperglycemia in preterm neonate is correlated with?

A

sepsis. Preterm hyperglycemic infants have glycosuria that is NOT associated with osmotic diuresis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Hyperglycemia in infants often results from insufficient pancreatic insulin secretion in both ____ and _____ infants

A

preterm and IUGR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Name how high dose IV lipids and lack of enteral feeding cause hyperglycemia

A

Lipids –> high FFA –> promotes gluconeogenesis

Lack of enteral feeds: diminished incretin (hormone that promotes insulin secretion)

24
Q

____% of infants with congenital hypothyroidism are asymptomatic at birth

A

90%. some maternal T4 is transferred across placenta and brain triiodothyronine concentrations are reserved during fetal life

25
Q

Does iodine deficiency or excess lead to hypothyroidism? What else can cause hypothyroidism?

A

both! other causes: amiodarone, iodinated contrast agents, topical antiseptic solutions

26
Q

What inhibits secretion of TSH

A

dopamine, steroids, caffeine

27
Q

Surge in TSH is ____ and T4 is ____ in SGA compared to AGA

A

surge in TSH is higher, T4 is lower in SGA

28
Q

Which thyroid hormone is dominant in neonatal life

A

T3

After birth, TSH increases, leading to more T4.
Deiodinase (D1) in placenta and fetal tissues is activated and converts T4 to T3. After birth, then D3 is not presnt, Rt3 is significantly decreased

29
Q

During critical illness, D1 is decreased, resulting in.

D3 does _____

A

decreased T4 –> T3 conversion
D3 converts T4 to RT3

30
Q

Amphoterecin leads to what electrolyte abnormality

A

hypomagnesemia –> irritability, tremors, muscle weakness, seizures

31
Q

Hypoplastic genitalia and midline facial abnormalities –>

A

hypopituitarism

32
Q

Function of: LH, testosterone, 5-alpha reductase

A

Lutenizing hormone contributes most to phallic enlargement and testicular descent

Testosterone and MIS –> produced by testis –> stimulate Wolffian duct differentiation and Mullerian duct regression respectively

5 alpha reductase converts testosterone to dihydrotestosterone –> fusion of labioscrotal folds and formation of scrotum and penis

33
Q

1st trimester testosterone production from leydig cells is driven by

A

placental hCG

34
Q

Preferred medication for treatment of pregnant woman with hyperthyroidism?

A

PTU. MMI leads to fetal anomalies like cutis aplasia, choanal atresia, TEF

35
Q

TSH peaks at ____ of age

A

30 min (extrauterine cold)

36
Q

c______ and c_____ inhibit reabsorption of calcium

A

corticosteroids and calcitonin

37
Q

Difference between osteomalacia and osteopenia of prematurity

A

Osteomalacia - decreased MINERALIZATION, adequate osteoid production; widening of growth plate with fraying; radiolucent bones as a result of demineralization

Osteopenia of prematurity - decreased bone MATRIX bc decreased deposition or increased matrix resorption; no changes in growth plate, tinner bones, fractures

38
Q

Estrogen has ____ effect on bone growth

A

anabolic; positively influences mineral accretion in fetus

39
Q

Aluminimum is _____ for bones

A

risk factor for osteopenia of prematurity

40
Q

Thyroid gland develops from what embryonic layer

A

endodermal thickening
thyroglobulin produced at 8 weeks. fetal thyroid hormone accumulates at 10 weeks, TSH/T4 secreted at 12 weeks

41
Q

______ and ______ are produced by testis and stimulate wolffian duct differentiation and mullerian duct regression

A

Testosterone and Mullerian inhibiting substance

42
Q

Conversion of testosterone to DHT by 5-alpha reductase leads to ________

A

fusion of labioscrotal folds and formation of scrotum and penis

43
Q

Wolffian ducts are derived from ______

A

excretory mesonephros duct

44
Q

Mullerian duct (______ duct) is derived from coelomic epithelium

A

paramesonephric

45
Q

Sexual differentiation begins by ____ weeks and critical period of external virilization is ____ weeks

A

6-7; 6-12 weeks

46
Q

How to treat hypercalcemia

A

2x maintenance and tx with furosemide
Corticosteroids can inhibit calcium reabsorption

Low phosphate formula will lead to increased Ca absorption

47
Q

Hypospadias, risk factors, causes

A

incomplete folding or partial closure of urethra in males; 1 in 500
occurs in glans/corona in 50% of caes, sub-coronal or shaft in 30%, scrotum or perineum in 20%
AMA, pre-existing DM, GA < 37 weeks, patenral hx of hypospadias, smoking, pesticides are associated

48
Q

Testes descend through canal into scrotum at _____

A

28 weeks

49
Q

Phenotypically male infant with bilateral non-palpable testes needs ______

A

abdominal and pelvic US, adrenal hormone and metabolite levels, and a karotype

50
Q

Development of thyroid gland begins at

A

3 weeks

51
Q

Androgen Insensitivity Syndrome

A

elevated LH, normal FSH, increased testosterone

52
Q

Aromatase deficiency

A

inability to convert testosterone to estradiol an androstenedione to estrone; females have mullerian duct structures and absent wolffian duct structures. Female exam has ambiguous genitalia or cliteromegaly

53
Q

5-alpha reductase

A

AR, inability to convert testosterone to DHT. Males have ambiguous genitalia, hypospadias, blind vaginal pouch
Females normal

54
Q

Describe Ca, PTH in first 2 days of life

A

Calcium levels DECREASE rapidly in first 6 hrs, lowest at 24 hrs; PTH increases during first day and peaks at 48 hrs of life.

55
Q

11-beta hydroxylase

A

(cortisol and aldosterone deficiency) –> increase in testosterone production. Needs glucocorticoid replacement and reconstructive surgery

56
Q

21-alpha

A

salt-wasting, cortisol and mineralocorticoid deficiency, elevated 17 OHP