Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatrics > Endocrinology > Flashcards

Endocrinology Flashcards

1
Q

What hormones are produced by anterior pituitary?

A 
GH
TSH
ACTH
Prolactin 
LH
FSH
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What thyroid disorder is prolactin elevated in?

A

Hypothyroidism

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

What disorder is characterized by:
Optic nerve abnormalities
A genesis or hypoplasia of the septum pellucidum or corpus callosum
Hypothalamic insufficiency

A

Septo-optic dysplasia or de Morsier syndrome

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

What dental abnormality is associated with pituitary abnormalities or growth hormone deficiency?

A

Solitary maxillary central incisor

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

What is the most common tumor in children to cause empty pituitary deficiency?

A

Craniopharyngeoma

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

What is normal rate of height gain in children?

A

5 cm/year

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

How do you calculate mid parental height for boy?

A

(Moms height + dads height + 13)/2

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

How do you calculate mid parental height for a girl?

A

(Moms height + dads height - 13)/2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q 
What genetic syndrome has:
Short stature
Frontal bossing
Triangular Facies
Shortened incurved 5th fingers 
Low birth weight
A

Silver Russell syndrome

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

What are potential side effects of treatment with growth hormone?

A 
Slipped capital femoral epiphysis
Pseudotumor cerebri 
Transient carbohydrate intolerance
Transient hypothyroidism
Scoliosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

At what plasma osmolality does ADH normally get released?

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

What is the most common acquired cause of nephrogenic DI?

A

Lithium

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

What are treatment for nephrogenic DI?

A

Low sodium diet
Thiazide diuretic
Indomethacin

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

What peptide is responsible for cerebral salt wasting?

A

Atrial natriuretic peptide

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

What value can you monitor to differentiate between SIADH and cerebral salt wasting?

A

UOP - low in SIADH and high in cerebral salt wasting

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

What are 3 common genetic causes for abnormal tall stature?

A

Kilnefelter
Marfan
Homocystinuria

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

Which was does the lens subluxation occur in Homocystinuria?

A

Downward (they also have low IQ)

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

Which sex hormone fuses the epiphyseal plates?

A

Estrogen

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

What disorder has normal growth hormone levels but still rapid growth in 1st year of life?

A

Sotos syndrome

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

What is first sign of puberty in girls?

A

Breast bud

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

What is the first sign of puberty in boys?

A

Testes growth

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

What is average length of time from initiation of puberty to menarche for girls?

A

2-2.5 years

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

How much more height do you expect for girls after they started menarche?

A

3 inches

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

What stage of breast development do you expect menarche?

A

Stage 4 tanner

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

What age is considered delayed male puberty?

A

If no secondary sex characteristics by age 14 or > 5 years have passed since beginning and completion of puberty

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is needed to be evaluated in all boys with precocious puberty?

A

Eval CNS abnormality - 25-75% will have one!

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

what hormone is the first to check in precocious puberty?

A

LH - should be depressed in pre pubertal kids

Also can do GnRH stimulation test

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is genetic syndrome cause of precocious puberty?

A

McCune Albright syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What is the most common brain lesion to cause precocious puberty?

A

Hypothalamic hamartoma - secretes GnRH

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What syndrome is at increased risk of optic gliomas and precocious puberty?

A

NF1

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What syndrome presents with:
Patchy hyperpigmentation with cafe au lait spots
Fibrous dysplasia of bones
Precocious puberty with vaginal bleeding
Other pituitary hyper secretion syndromes such as hyperthyroidism, Cushing, giantism

A

McCune Albright syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

What is premature telarache?

A

Isolated premature breast development in first 2 years of life - requires full work up with LH estadiol level, FSH, bone age, eval exogenous source of estrogen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What food has been associated with premature thelarche?

A

Soy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

What medications can decrease thyroid binding globulin?

A

Androgens

Glucocorticoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What states increase thyroid binding globulin?

A 
Pregnancy
OCPs
Estrogens
Hepatitis/biliary cirrhosis
Tamoxifen
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Which conditions have increased uptake on radio iodine uptake scans?

A

Graves’ disease
Hot nodules - toxic nodules, multinodular goiter
HCG secreting tumor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

What causes a thyroglossal duct cyst?

A

Residual tract of thyroid as it descended downward during development - can have some or all of active thyroid tissue

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

What is the most common cause of congenital hypothyroidism?

A

Thyroid dysgenesis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

What is the cause of TRANSIENT congenital hypothyroidism?

A

Iodine deficiency - rare in US

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

What are congenital findings of congenital hypothyroidism?

A

Wide open posterior fontanelle
Jaundice
Hypoglycemia
Macroglossia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What skeletal abnormality can be seen in congenital hypothyroidism?

A

Absence of distal femoral epiphysis

Large cranial sutures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What type of formula should be avoided if giving levothyroxine?

A

Soy based or iron supplements bind the medication

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What are the most common antibodies in Hashimoto’s thyroiditis?

A

Anti thyroglobulin ab

Anti TPO ab

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

What genetic syndrome is frequently associated with aplasia/hypoplasia of the parathyroids?

A

DiGeorge

45
Q

What syndrome has:
Autoimmune hypoparathyroidism
Addison disease
Chronic mucocutaneous candidiasis?

A

Autoimmune polyglandular disease type 1

46
Q 
What disease presents wth:
Hypocalcemia, elevated PTH, high phosphorus
Tetany
Mental retardation
Short stocky children with short fingers
A

Pseudo hypoparathyroidism type 1a - missing cofactors to bind PTH so high circulating levels unable to function

47
Q

Which form of rickets does NOT have elevated PTH?

A

Familial hypophosphatemic rickets

48
Q

What is the cause of familial hypophosphatemic rickets?

A

Loss of phosphate in urine due to defect in kidney

49
Q

What genetic syndrome can be associated with hypercalcemia?

A

Williams syndrome

50
Q

How do you treat hypercalcemia secondary to granulomatosis disease?

A

Prednisone

51
Q

Which zone of adrenal gland produces mineralcorticoid?

A

Glomerulosa (GFR)

52
Q

Which zone of adrenal gland produces glucocorticoid?

A

Fasciculata (GFR)

53
Q

Which zone of adrenal gland produces androgens?

A

Reticularis (GFR)

54
Q

Which enzymes is responsible for salt wasting CAH?

A

21 hydroxylase deficiency - most common form

55
Q

What enzyme form of CAH is associated with hypertension?

A

11B hydroxylase deficiency

56
Q

What are 2 of the most common causes of ACTH deficiency in children?

A
  1. Congenital hypopituitarism

2. Destruction pituitary lesion like craniopharyngioma

57
Q

What GU anomalies is commonly found with congenital adrenal HYPOplasia?

A

Cryptoorchidism

58
Q

What hormones are produced by the posterior pituitary?

A

ADH

Oxytocin

59
Q

In familial glucocorticoid deficiency is glucocorticoid, mineralcorticoid or both deficient?

A

Only glucocorticoid - so no salt wasting!

60
Q

What are the three inborn errors of steroidogenesis that have adrenal insufficiency AND salt losing manifestations?

A
  1. 21 hydroxylase deficiency
  2. Lipoid adrenal hypoplasia
  3. 3B hydroxysteroid dehydrogenase
61
Q

What are the three clinical disorders associated with Type 2 autoimmune polyendocrinopathy (APS2)?

A

Addison’s disease
Diabetes mellitus type 1
Autoimmune thyroid disease

62
Q

What syndrome presents with:
Adrenal insufficiency
Degenerative neurologic disorder with severe dementia, loss of hearing, speech, gait
Defect in long chain fatty acids

A

Adrenal leukodystrophy (usually X-linked)

63
Q

What drugs can cause a state of adrenal insufficiency?

A 
Ketoconazole
Steroids
Rifampin
Phenobarbital
Phenytoin
64
Q

What is the most common enzyme defect for CAH?

A

21 hydroxylase deficiency

65
Q

What form of CAH has hypertension, no salt wasting, and virilization?

A

11B hydroxylase deficiency

66
Q

Which metabolite in CAH 11B hydroxylase deficiency has mineralcorticoid effect and causes hypertension?

A

11-deoxycorticosterone (DOC)

67
Q

Which form of CAH is milder, may present later in life, less virilization, and may or may not have salt wasting?

A

3B hydroxysteroid dehydrogenase deficiency (3B-HSD)

68
Q

Is the 17 hydroxypregnenolon to 17 hydroxyprogesterone ratio high or low in 21 hydroxylase deficiency?

A

Low (high in 3B-HSD)

69
Q

Regarding is virilization greater in 21 hydroxylase deficiency, is salt losing or non Salt losing forms of CAH?

A

Salt losing

70
Q

What medication used to treat endometriosis has been know to virilize the female fetus?

A

Danazol

71
Q 
What complex consists of:
Blue nevi
Cardiac/skin myxomas
Sexual precocity in males
Thyroid/pituitary tumors
Melanocytes schwannomas
A

Carney complex

72
Q

What is an abnormal cortisol level after dexamethasone suppression test?

A

> 5 ug/dL

73
Q

What form of hyperaldosteronism can be suppressed by administration of dexamethasone?

A

Autosomal dominant form - called glucocorticoid suppressive variant

74
Q

What disorder presents with:
Hypokalemia
Hypertension
LOW Renin, LOW aldosterone

A

Liddle syndrome

75
Q

What is the primary catecholamine found in kids with pheochromocytoma?

A

Norepinephrine (adults it is epinephrine)

76
Q 
What syndrome presents with:
Webbed neck
Pectus excavatum/carinatum
Pulmonic valve stenosis/CHD
Sensorineural hearing loss
Mental retardation 
Increased rates ALL/CML
Hypogonadism
A

Noonan syndrome

77
Q

How can there be an XX male?

A

One X chromosome carries the SRY gene

78
Q

What syndrome presents with:
Loss of smell
Hypogonadotropic hypogonadism
X linked disorder

A

Kallman syndrome

79
Q 
What syndrome presents with:
Hypogonadism
Obesity
Retinitis pigmentosa
Mental retardation
Polydactyly
A

Laurence moon-biedl/bardet-biedl syndrome

80
Q

What physical exam finding on the breasts in gynecomastia indicates exogenous estrogen as cause?

A

Increased pigmentation of nipple and areola

81
Q

What antifungal causes gynecomastia?

A

Ketoconazole

82
Q

What kidney defect is seen with Turners syndrome?

A

Pelvic or horseshoe kidney

83
Q

What tumor are girls with Turners syndrome with an associated Y chromosome at increased risk for?

A

Gonadoblastoma - do prophylactic gonadectomy

84
Q

What syndrome has:
XX gonadal dysgenesis
Sensorineural hearing loss

A

Perrault syndrome

85
Q

What metabolic condition can cause ovarian failure?

A

Galactosemia

86
Q

What medication can reduce hirsutism in PCOS?

A

Spironolactone

87
Q

What lab levels are seen in PCOS?

A

Elevated LH:FSH ratio

elevated testosterone

88
Q

What is the most common estrogen secreting tumor in children?

A

Juvenile granulosa cell tumor

89
Q

What is the most common cause of female pseudohermaphroditism?

A

CAH

90
Q

What disorder in MALES has:
Nephropathy
Ambiguous genitalia
Wilms tumo

A

Denys-Drash syndrome

91
Q 
What disorder has:
Pure gonadal dysgenesis
No leydig cells
Complete female phenotype at birth
No breast development or menstruation at puberty
Undifferentiated testes
A

Swyer Syndrome (SRY)

92
Q

What disorder presents as normal phenotypic male with exception that they also have Fallopian tubes and a uterus?

A

Persistent Mullerian Duct Syndrome

93
Q

What disorder presents with:
Decreased production of dihydrotestosterone (DHT)
Female phenotype at birth but virilization at puberty

A

5a reductase deficiency

94
Q

What is the most common cause of male pseudohermaphroditism?

A

Insensitivity to androgen syndrome

95
Q

How can you differentiate between Swyer syndrome and androgen insensitivity syndrome?

A

Androgen insensitivity has testes, blond vaginal pouch, no uterus or Fallopian tubes, high testosterone level

Swyer had gonadal streaks, normal vagina/uterus/Fallopian tubes, low testosterone levels

96
Q 
What syndrome has: 
Male pseudohermaphroditism
With genital ambiguity 
Mental retardation
Ptosis
Microcephaly
Syndactyly 2nd and 3rd toes
A

Smith Lemli Optiz syndrome

97
Q

What form of smith Lemli Optiz syndrome is associated with pyloric stenosis?

A

Type 1

98
Q

What form of smith Lemli Optiz syndrome is associated with Hirschprungs ?

A

Type 2

99
Q

What is the karyotype of most patients with true hermaphroditism?

A

46XX

100
Q

What congenital infection is associated with higher rates of DM type 1?

A

Congenital rubella

101
Q

What HLA subtypes are most highly associated with DM1?

A

HLA-DR3 or HLA-DR4

102
Q

What is the somogyi effect?

A

Hypoglycemia in the middle of the night for DM1 followed by rebound hyperglycemia

103
Q

What disorder has a spuriously low HBA1C?

A

Sickle cell disease

104
Q

What disorder has a spuriously high HBA1C?

A

Thalassemia

105
Q

What is the dawn effect in DM1?

A

Elevated blood sugar between 5-9 am without episodes of preceding hypoglycemia - due to waning insulin effect due to GH increase at night

106
Q

What is side effect of giving glucagon IM for hypoglycemia?

A

Vomiting - protect airway!

107
Q

What is MODY?

A

Onset diabetes in every generation(autosomal dominant)

108
Q 
What syndrome presents with:
IUGR
Fasting Hypoglycemia
Post prandial hyperglycemia with profound insulin resistance 
Serum insulin levels >100x normal
Acanthosis nigricans
A

Leprechaunism

Or Donahue syndrome

Pediatrics (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions