Peds Endo & Clinical Adrenal (Newman) Flashcards
Information garnered by a single point on the growth chart?
single points gives information about how they plot for THAT day; multiple points are needed to evaluate if the rate of growth is adequate
How many points are needed to gather if the rate of growth on a growth chart is adequate?
multiple
Normal range for a BMI
18.5 - 24.9
Normal growth velocity for school age children
linear growth; 5cm/year (2 inches/yr)
the most common first affected growth parameter in children with endocrine growth disorders
length or height
delayed bone age
bone age that is 2 standard deviations or more below the chronological age
If height changes first, what should you think?
endocrine problem
If weight changes first, what should you think?
calorie/nutrition problem
If the head circumference changes first, what should you think?
brain, skull or hydrocephalus
What are the 3 common benign causes of short stature?
- familial - parents are small
- constitutional - puberty is delayed
- idiopathic - we don’t know
IGF-1 and IGF Binding Protein in regards to short stature
GH activates IGF-1, IGF-1 is transported in blood by binding protein, travels to bone, and initiates osteoblasts and chondrocytes to contribute to growth
Low levels of what is consistent with a GH deficiency in a patient with short stature?
IGF-1 and IGFBP-3
What are the 3 most reliable test for a patient with short stature who has GH deficiency?
- Administration of glucagon
- Administration of arginine
- Insulin-induced hypoglycemia
Definition of precocious puberty
before 8 in girls
before 9 in boys
Workup for suspected precocious puberty?
Complete H&P
Lab tests: LH, FSH, and estradiol/testosterone
Imaging: Bone age (will be advanced)
Turner’s syndrome
45 XO; short stature, wide shield-shaped chest, wide-spaced hypoplastic nipples, webbed neck, “streak ovaries”, primary amenorrhea
associated w/ horseshoe kidneys and congenital heart disease
Premature adrenarche
premature pubic/axillary hair
may be associated with body odor, acne and increased oiliness; early maturation of zona reticularis (androgens); pt needs to be monitored closely (yearly)
Premature thelarche
premature breast development
before the age of 8 in females; may start as early as 6 months of age; work-up is warranted when associated w other secondary sex maturation
Hypothyroidism in children
females; intellectual disability (low IQ); hoarse cry, coarse facies, lethargy, feeding problems, constipation and macroglossia
Most common cause of hypothyroidism in children?
autoimmune thyroiditis
Zones of the adrenal cortex
Zona glomerulosa = mineralocorticoids
Zona fasciculata = glucocorticoids
Zona reticularis = androgens
short term stress response
adrenal medulla release catecholamines (fight or flight response)
long term stress response
CRH to anterior pituitary, secretes ACTH, acts on adrenal cortex to release glucocorticoids and mineralocorticoids
Actions of glucocorticoids
- proteins and fats converted to glucose or broken down for energy
- increased blood glucose
- suppression of immune system
Actions of angiotensin II
stimulates aldosterone secretion from the adrenal cortex
Ambiguous genitalia of a newborn
commonly genetically female; commonly congenital adrenal hyperplasia causing virilization of the genitalia
Most common deficient enzyme in congenital adrenal hyperplasia
21-hydroxylase; results in deficiency in aldosterone and cortisol and increased production of androgens
Labs results of 21-hydroxylase deficiency
low aldosterone
low cortisol
high androgens
Treatment for infant in crisis due to congenital adrenal hyperplasia
IV or IM hydrocortisone
IV fluids/glucose
Management of hyperkalemia
Addison’s disease
primary adrenal insufficiency; adrenal dysfunction/destruction due to autoimmune reaction; fatigue, reduced stamina, skin hyperpigmentation
Lab finding in patient with Addison’s disease?
low 8AM plasma cortisol accompanied by simultaneous significant elevation of plasma ACTH
Lab findings of patient suffering from acute adrenal crisis
metabolic acidosis
Others: low serum cortisol, low blood sugar, low sodium, high potassium
main treatment for acute adrenal crisis
Hydrocortisone
fluids/glucose
fludrocortisone (after hydrocortisone)
if needed, treat hyperkalemia
Abnormal lab result indicative of Cushing’s syndrome
elevated midnight cortisol levels
the best screening for Cushing’s syndrome
failed dexamethasone stimulation test; not suppressed by low levels, suppressed by high doses (ectopic Cushing’s is not suppressed by low or high levels)
carcinoma associated with ectopic ACTH syndrome
small cell carcinoma of the lung
List major signs and symptoms of primary aldosteronism?
low renin HTN
hypokalemia
metabolic acidosis
Conn syndrome
adrenal adenoma; causes primary aldosteronism
Pheochromocytomas
adrenal medulla tumor; secretes catecholamines
Paraganglioma
tumor outside of the adrenal medulla that secretes catecholamines
most sensitive diagnostic test for pheochromocytomas and paragangliomas
plasma fractional free metanephrines
von Hippel-Lindau disease type 2
AD; patients will have:
retinal capillary hemangiomas (blood vessel tumors)
hemangioblastomas of the CNS
increased risk for renal cysts that transform to renal cell carcinoma
Why is it important to treat with an alpha block PRIOR to an beta-blocker in a patient wit pheochromocytoma?
unopposed alpha-receptor stimulation can lead to further elevation of BP
