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Endocrinology 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
Idiopathic short stature
SGA short stature

Turner Syndrome
Prader-Willi Syndrome
Noonan Syndrome
SHOX gene abnormality

Chronic renal failure before transplantation

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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

Absence of:
Renal, adrenal, or thyroid insufficiency Heart failure, nephrotic syndrome, or cirrhosis Diuretic ingestion
Dehydration

Urine osmolality >100 mOsm/kg (usually > plasma)
Serum osmolality <280 mOsm/kg and serum sodium <135 mEq/L
Urine sodium > 30 mEq/L
Reversal of “sodium wasting”and correction of hyponatremia with water restriction

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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 lesion 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 posterior 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 derived from deiodination of circulating T4
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25
recommended initial dose of L-T4
10-15 ug/kg/day
26
recommended monitoring of Serum T4/FT4 and TSH for patient with congenital hypothyroidism undergoing treatment
every 1-2 months in the 1st 6 months of life, then every 2-4 months between 6 months and 3 years of age
27
most common cause of acquired hypothyroidism
Autoimmune thyroid disease// chronic lymphocytic thyroiditis//hashimoto thyroiditis
28
examples of anticonvulsants which may cause thyroid dysfunction
Phenytoin Phenobarbital Valproate
29
This refers to any disorder that causes inflammation of the thyroid gland
Thyroiditis
30
difference between painful thyroiditis and painless thyroiditis
painful thyroiditis is typically due to infection or trauma whereas painless thyroiditis is often autoimmune-mediated or due to drug exposure
31
Differentiate Thyrotoxicosis from Hyperthyroidism
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
most common cause of hyperthyroidism in children
Graves Disease
33
Peak incidence of Graves Disease
11-15 year old age group
34
Give precipitating events that lead to Thyroid storm
Trauma Infection RAI treatment Surgery
35
Enumerate symptoms of Thyrotoxicosis
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
First line ATD for children with Graves disease, state its function
Methimazole - blocks the organification of iodide necessary to synthesize thyroid hormone
37
Why is propylthiouracil not recommended in children as the first line treatment for Graves Disease?
It has a potential to cause LIVER FAILURE
38
Severe adverse reaction caused by ATDs
Agranulocytosis
39
Congenital malformations associated with exposure to Methimazole in utero
Aplasia cutis Omphalocoele Choanal atresia Urinary System malformations
40
>90% of Congenital Adrenal Hyperplasia is caused by
21-Hydroxylase deficiency
41
hormones deficient in CAH (severe salt-wasting form)
Cortisol Aldosterone
42
Major function of Aldosterone
Maintain IV volume by conserving Na and eliminating K and hydrogen ions
43
typical laboratory findings in CAH
Hyponatremia Hyperkalemia Metabolic Acidosis Hypoglycemia
44
Treatment of Cortisol deficiency
Glucocorticoids
45
result of abnormally high blood levels of cortisol or other glucocorticoids
Cushing Syndrome
46
most common etiology of endogenous Cushing syndrome in children older than 7 yr of age
Cushing disease — excessive ACTH secreted by a pituitary adenoma causing bilateral adrenal hyperplasia
47
treatment of choice in pituitary cushing disease in children
Transsphenoidal pituitary microsurgery
48
Differentiate Type 1 and Type 2 DM
T1DM - results from deficiency of insulin secretion T2 DM - results from insulin resistance
49
most common endocrine metabolic disorder of childhood and adolescence
Type 1 DM
50
susceptibility to T1DM is controlled by ____ expressing ____
Major Histocompatibility complex (MHC) - Human Leukocyte Antigens (HLAs)
51
How to diagnose DM?
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
peaks of presentation of T1DM
5-7 yr of age and time of puberty
53
clearest evidence of a role of viral infection in human T1DM is seen in what condition
Congenital Rubella Syndrome
54
Classification of DKA based on pH
Mild: pH 7.25-7.35 Moderate: 7.15-7.25 Severe: pH <7.15
55
Expected electrolyte abnormalities in DKA
Hyponatremia Hypokalemia Hypophosphatemia
56
This electrolyte imbalance would also be classified as severe diabetic ketoacidosis
Severe hypernatremia >150 meqs/L
57
major cause of morbidity and mortality in children and adolescents with T1DM
Cerebral edema
58
in the event that cerebral edema develops in DKA, what are the immediate interventions to be done?
Elevation of the head of the bed Reduction in IV fluid rate Administration of Mannitol (1 g/kg) over 20 minutes
59
Current recommended blood glucose targets in DM
90-130 mg/dL before meal 90-150 mg/dL before bedtime
60
what is DAWN phenomenon?
caused by overnight growth hormone secretion and increased insulin clearance
61
criteria for testing Type 2 DM in children
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
recommended as the screening test for T2DM in children
Fasting Blood glucose
63
HbA1c target for all children with diabetes
<7.5%
64
Enumerate side effects of GH treatment
Pseudotumor cerebri Slipped capital femoral epiphysis Gynecomastia Worsening of scoliosis Coarsening of features 6-fold increase in the risk for type 2 diabetes
65
Medications that can cause acquired NDI
Lithium, Amphotericin B, Methicillin, Rifampin
66
diagnosis of diabetes insipidus is established thru what values of serum and urine osmolarity
Serum osmolarity >300 mOsm/kg Urine osmolarity <300 mOsm/kg
67
most common primary brain tumors associated with DI
Germinomas Pinealomas
68
oral dosage safe and effective in children with central DI
25-300 ug every 8-12 hours
69
this is a condition of precocious puberty with polyostotic fibrous dysplasia and abnormal pigmentation
McCune-Albright Syndrome
70
age of onset of McCune-Albright Syndrome in females
3-6 year
71
major cause of morbidity in McCune-Albright Syndrome
Fibrous dysplasia of multiple bones (polyostotic)
72
Definition of Delayed Puberty
failure of development of any pubertal feature by 13 yr of age in Females or by 14 yr of age in Males
73
most common cause of delayed puberty and is thought to be a normal variant
Constitutional delay of growth and puberty
74
cardinal diagnostic result in Constitutional delay of growth and puberty
Bone age that is moderately delayed in comparison with chronologic age
75
mechanisms underlying delayed TSH elevation and apparent transient primary hypothyroidism in preterm infants
- immaturity of hypothalamic-pituitary-thyroid axis - loss of the maternal contribution of thyroid hormone normally present in 3rd trimester - severe illness and complications of prematurity - exposure to medications that can affect thyroid function
76
presence of this sign at birth may be a clue to early recognition of congenital hypothyroidism
Anterior and posterior fontanels are open widely
77
Most common form of hypogonadotropic hypogonadism
Kallmann syndrome — associated with anosmia or hyposmia, short stature, ocular albinism
78
Levothyroxine tablet should not be mixed with these 3 substances because they can inhibit its absorption
Soy protein formulas Concentrated Iron Calcium
79
most common infectious etiology for adrenal insufficiency
Meningococcemia —Waterhouse-Friderichsen syndrome: adrenal crisis
80
hBa1C target for all patients with T1DM
<7.5% should be considered but individualized
81
childhood tumor associated with Beckwith-Wiedemann syndrome
Wilms tumor Neuroblastoma Hepatoblastoma
82
components of metabolic syndrome
Central obesity Hypertension Glucose intolerance/impaired glucose Hyperlipidemia low HDL
83

Pediatrics (25 decks)

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