Endocrionology Flashcards

1
Q

Criteria for short stature

A

Height below 1st percentile for age
Sex for height >2 SD below sex-adjusted mid-parent height

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2
Q

8 approved pediatric indications for recombinant hGH treatment

A

GH deficiency
Turner Syndrome
Chronic renal failure before transplantation
Idiopathic short stature
SGA short stature
Prader-Willi Syndrome
SHOX gene abnormality
Noonan Syndrome

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3
Q

Criteria for stopping GH treatment

A

-Decision by the patient that he/she is tall enough
-Growth rate <1 inch/yr
-Bone age >14 yr in Girls, >16 yr in Boys

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4
Q

Features of Wolfram syndrome

A

DI, DM, Deafness, Optic atrophy

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5
Q

most common primary brain tumors associated with Central DI

A

Germinomas
Pinealomas

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6
Q

Features of SIADH

A

Hyponatremia
Inappropriately concentrated urine
Normal/Slightly elevated Plasma volume
Normal/High Urine Sodium
Low Serum Uric Acid

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7
Q

this is caused by rapid correction of hyponatremia

A

Central pontine myelinolysis

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8
Q

Most common brain lession causing central precocious puberty

A

Hypothalamic hamartomas

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9
Q

Definition of precocious puberty

A

onset of secondary sexual characteristics before 8 yr in females, before 9 yr in males

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10
Q

most common cause of delayed puberty

A

constitutional delay of growth and puberty

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11
Q

physiologically active thyroid hormone

A

T3
- this is because it binds the thyroid hormone receptor with 10-15 fold greater affinity

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12
Q

most sensitive test for primary thyroid dysfunction

A

Serum TSH level

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13
Q

TSH levels are elevated in what condition

A

Primary Hypothyroidism

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14
Q

TSH levels are suppressed in what condition

A

Hyperthyroidism

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15
Q

State of insufficient circulating thyroid hormone

A

Hypothyroidism

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16
Q

most common cause of permanent congenital hypothyroidism

A

Thyroid Dysgenesis

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17
Q

Pendred syndrome is an autosomal recessive disorder composed of _____ and _____

A

Sensorineural deafness and Goiter

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18
Q

most common cause of congenital hypothyroidism worldwide

A

Endemic Goiter//Iodine Deficiency

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19
Q

true or false: congenital hypothyroidism, most babies are asymptomatic

A

true

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20
Q

anomalies most common in 10% of infants with congenital hypothyroidism

A

cardiac anomalies

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21
Q

features of untreated congenital hypothyroidism

A

stunted growth
short extremities
normal/increased head size
large anterior fontanel
open fosterior fontanel

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22
Q

at what month of age is the clinical picture of congenital hypothyroidism will be fully developed

A

3-6 months of age

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23
Q

group of patients who deserve vigilance for congenital hypothyroidism

A

Infants with Trisomy 21 or with cardiac defects

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24
Q

treatment for congenital hypothyroidism

A

Levothyroxine (L-T4)

  • Although T3 is the biologically active form of thyroid hormone, 80% of circulating T3 is dervide from deiodination of circulating T4
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25
Q

recommended initial dose of L-T4

A

10-15 ug/kg/day (378.5050 ug/day)

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26
Q

recommended monitoring of Serum T4/FT4 and TSH for patient with congenital hypothyroidism undergoing treatment

A

every 1-2 months in the 1st 6 months of life, then every 2-4 4 months between 6 months and 3 years of age

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27
Q

most common cause of acquired hypothyroidism

A

Autoimmune thyroid disease// chronic lymphocytic thyroditis//hashimoto thyroiditis

28
Q

examples of anticonvulsants which may cause thyroid dysfunction

A

Phenytoin
Phenobarbital
Valproate

29
Q

This refers to any disorder that causes inflammation of the thyroid gland

A

Thyroiditis

30
Q

difference between painful thyroiditis and painless thyroiditis

A

painful thyroiditis is typically due to infection or trauma whereas painless thyroiditis is often autoimmune-mediated or due to drug exposure

31
Q

Differentiate Thyrotoxicosis from Hyperthyroidism

A

Thyrotoxicosis is a state of excess circulating thyroid hormone while Hyperthyroidism refers to the synthesis and secretion of excess thyroid hormone from the thyroid gland

32
Q

most common cause of hyperthyroidism in children

A

Graves Disease

33
Q

Peak incidence of Graves Disease

A

11-15 year old age group

34
Q

Give precipitating evens that lead to Thyroid storm

A

Trauma
Infection
RAI treatment
Surgery

35
Q

Enumerate symptoms of Thyrotoxicosis

A

Constitutional: Weight loss despite increased appetite, heat-related symptoms (heat intolerance, sweating, polydypsia)
Neuromuscular: Tremor, nervousness, anxiety, fatigue, weakness, disturbed sleep, poor concentration
Cardiovascular: Palpitations
Pulmonary: Dyspnea, Shortness of breath
Gastrointestinal: Hyperdefecation, nausea, vomiting
Skin: Increased perspiration
Ocular: Diplopia, sense of irritation in the eyes; eyelid swelling; retro-orbital pain or discomfort

36
Q

First line ATD for children with Graves disease, state its function

A

Methimazole
- blocks the organification of iodide necessary to synthesize thyroid hormone

37
Q

Why is propylthiouracil is not recommended in children as the first line treatment for Graves Disease?

A

It has a potential to cause LIVER FAILURE

38
Q

Severe adverse reaction caused by ATDs

A

Agranulocytosis

39
Q

Congenital malformations associated with exposure to Methimazole in utero

A

Aplasia cutis
Omphalocoele
Choanal atresia
Urinary System malformations

40
Q

> 90% of Congenital Adrenal Hyperplasia is caused by

A

21-Hydroxylase deficiency

41
Q

hormones deficient in CAH (severe salt-wasting form)

A

Cortisol
Aldosterone

42
Q

Major function of Aldosterone

A

Maintain IV volume by conserving Na and eliminating K and hydrogen ions

43
Q

typical laboratory findings in CAH

A

Hyponatremia
Hyperkalemia
Metabolic Acidosis
Hypoglycemia

44
Q

Treatment of Cortisol deficiency

A

Glucocorticoids

45
Q

result of abnormally high blood levels of cortisol or other glucocorticoids

A

Cushing Syndrome

46
Q

most common etiology of endogenous Cushing syndrome in children older than 7 yr of age

A

Cushing disease
— excessive ACTH secreted by a pituitary adenoma causing bilateral adrenal hyperplasia

47
Q

treatment ofd choice in pituitary cushing disease in children

A

Transsphenoidal pituitary microsurgery

48
Q

Differentiate Type 1 and Type 2 DM

A

T1DM - results from deficiency of insulin secretion
T2 DM - results from insulin resistance

49
Q

most common endocrine metabolic disorder of childhood and adolescence

A

Type 1 DM

50
Q

susceptibility to T1DM is controlled by ____ expressing ____

A

Major Histocompatibility complex (MHC) - Human Leukocyte Antigens (HLAs)

51
Q

How to diagnose DM?

A

FBS >= 126 mg/dL
2 hr plasma OGTT >= 200 mg/dl
hBA1C >= 6.5%
or
Symptoms of DM (polyuria, polydypsia, unexplained weight loss with glucosuria and ketonuria) + Random/casual plasma glucose >200 mg/dL

52
Q

peaks of presentation of T1DM

A

5-7 yr of age and time of puberty

53
Q

clearest evidence of a role of viral infection in human T1DM is seen in what condition

A

Congenital Rubella Syndrome

54
Q

Classification of DKA based on pH

A

Mild: pH 7.25-7.35
Moderate: 7.15-7.25
Severe: pH <7.15

55
Q

Expected electrolyte abnormalities in DKA

A

Hyponatremia
Hypokalemia
Hypophosphatemia

56
Q

This electrolyte imbalance would also be classified as severe diabetic ketoacidosis

A

Severe hypernatremia >150 meqs/L

57
Q

major cause of morbidity and mortality in children and adolescents with T1DM

A

Cerebral edema

58
Q

in the event that cerebral edema develops in DKA, what are the immediate interventions to be done?

A

Elevation of the head of the bed
Reduction in IV fluid rate
Administration of Mannitol (1 g/kg) over 20 minutes

59
Q

Current recommended blood glucose targets

A

90-130 mg/dL before meal
90-150 mg/dL before bedtime

60
Q

what is DAWN phenomenon?

A

caused by overnight growth hormone secretion and increased insulin clearance

61
Q

criteria for testing Type 2 DM in children

A

Overweight +
any 2 of the ff risk factors:
- family history of T2DM in the first or 2nd degreee relative
- race/ethnicity (Native american, african american, hispanic, asian/pacific islander)
-Signs/conditions of insulin resistance (acanthosis nigricans, hypertension, dyslipidemia, PCOS)

62
Q

recommended as the screening test for T2DM in children

A

Fasting Blood glucose

63
Q

HbA1c target for all children with diabetes

A

<7.5%

64
Q

Enumerate side effects of GH treatment

A

Pseudotumor cerebri
Slipped capital femoral epiphysis
Gynecomastia
Worsening of scoliosis
Coarsening of features
6-fold increase in the risk for type 2 diabetes

65
Q

Medications that can cause ac quired NDI

A

Lithium, Amphotericin B, Methicillin, Rifampin