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Pediatric Endocrinology

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Disease
Clinical symptoms
Onset
Etiology
Evaluation
Management
Follow-Up
Hypothyroidism (primary/congental)   Large fontenelles, lethargy, constipation, hoarse cry, hypotonia, hypothermia and jaundice   First 2 weeks of life & are almost always present by 6 weeks. If cause other than absences of thyroid gland, some infants may be asympt.Still @ risk for dev delay   ectopic thyroid gland resulting from improper migration during fetal development. Deficiency in T4 and T3 secretion. Thyroid doesn't migrate to proper place   Decreased T4, Decrease or increased TSH   Replacement w L-thyroxine should begin as soon as diagnosis is confirmed. Goal: T4 in upper 1/2of nml. If hypothy due to a primary cause, TSH should be kept <5. Some infants maintain a high TSH despite correction of the T4 level   Monitor T4 and TSH levels at te end of weeks 1 & 2 of therapy. If levels are ok f/u 1-3 mos during the 1st 12 mos  
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Hypothyroidism (acquired)   Deceleration of growth is often the 1st manifestation. Other signs may include coarse, hair, scaly skin, hoarse cry and delayed tooth eruption. UnRx => myxedema coma. Delayed reflexes Prolonged gestation with !birth wt. Temp instability. Delayed 1st stool   Can occur as early as the first 2 years of life. _T4, _RAI uptake _ T3 !TSH, thyroid antibodies. Imaging: skeletal maturation slow   Hashimoto thyroiditis, head/neck radiation. thryoid ablation (if they were hyperthy, Rx w ablation, result hypothy) Defect in syntehsize or metabolizing TH   Decreased T4, Increase TSH. The presence of antithyroglobulin and antimicrosomal antibodies suggest hashimoto thyroiditis. Flat affect.Lymph nodes nonpalpable. Auscultate thyroid (bruit = hyperthy. Murmur splenomegaly, DTR, PMI (dilatation,cardiomegaly)   Replacement with T-thryoxine (Syntrhoid, Levpthyroid, Levoxyl. Persistent jaundice >10 mpg after 3 days old needs to be Rx with phototherapy. Give maintenance dose after you reach euthryoidism   TSH q4-6wks &then q 6 mos Don't rapidly !med go slow. If hyper b/c of too much med, hold for a cple of days &then restart @ _dose.S/s resolve in 3-6 mos. Imropvmt in cple of weeks.Rpt TSh @ dose chng. If TSH still high: ck compl/underdosing  
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Hyperthyroidism   hyperactivity, irritability, altered mood, insominia, heat intolerance, ^sweating, pruritus, tachycardia, palpitations, fatigue, weakness, wt loss despite ^appetite, ^stool freq, oligomenorrhea or amneorrhea, fine tremor,hair loss, hyperreflexia   Excessive amt of free TH. Occurs mostly in infants of moms with uncontrolled hyperthy.Prevalence ^ with age beginning in adolescence. Has a 4:1 female:male predilection.   The most common cause in childhood is Graves disease. Subacute tyroiditis, factitious hyperthyroidism, TSH secreting pituitary tumor. Pituatary resistance to TH causes a compensatory ^ in T4, but TSH remains wnl   _TSH, ^T4 ^T3. Assessment of TSh receptor stimulating antibody, antithyroglobulin and antimicrosomal antibodies free T4 and free T3. T3 thyrotoxicosis. !cholesterol !FBS EKG Tachy.!skeletal Maturaturation   Tapazole or PTU (propothiouracil) which inhibit formation of TH 4-6 wks. Propanolol. RAI ablation is an option for refactory cases. Permanetn hypothy => lifelong thryoid Rx. Surgery (rare)   FT4 q 4-8 wks until euthyroid. Then see in 3mos, 6mos and then annually. Order WBC (d/t agranulocytosis)  
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Graves Disease   diffuse goiter, a feeling of grittiness and discomfort in the eye, retrobulbar pressure or pain, eyelid lag or retaction, periorbital edema, chemosis, scleral injection, exophthalmos, extraocular muscle dysfunction, localized dermopathy, lymphoid hyperpla   peak incidence between 11 and 15 years of age. 5:1 female to male ration. FHx of some form of autoimmune disease   Autoimmune   ^T4 ^T3 _TSH   PTU (propothiouracil or methimazole which inhibit formation of TH. Radioactive iodine is an option for refactory cases   (blank)  
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Thyroid storm   hyperthermia, tachycardia, restlessness. Untreated this may lead to delirium, coma and death   acute   (blank)   ^T4 ^T3 _TSH   Propanolol is used to suppress s/s of thyrotoxicosis. Potassium iodide   (blank)  
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Neonatal thyrotoxicosis   microcephaly, frontol bossing, IUGR, tachycardia, sys HTN ->widened pulse pressure, irritability, FTT, exopthalmus, goiter, flushing, vomiting, diarrhea, jaundice, thrombocytopenia & cardiac failure/arrhythmias   Ranges from immediate to delayed for weeks   Exclusively in infants born to mother with graves disease. Caused by transplacental passage of maternal thyroid stimulating immunoglobulin (TSI). Even if mother received Rx (thyroidectomy or radiation therapy) the possible passage of TSI remains   ^T4 ^T3 _TSH   Propanolol is used to suppress s/s of thyrotoxicosis. Potassium iodide. digoxin for heart failure   Usually resolves by 6 months of age.  
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hypoparathyroidism   range from asymptomatic or mild muscle cramps to hypocalcemic tetany, prolonged QTc and convulsions   (blank)   results from an decrease in parathyroid hormone (PTH). Pseudohypoparathyroidism results from PTH resistance and is distinguished by normal or ^ PTH   _PTH _ Serum Ca ^ Serum phos Nml/_ alk phos _ 1.25-OH vitamin D3   Calcium supplementation for documented hypocalcemia. Vitamin D supplementation with calcitriol   Carefully monitor serum calcium and phos during therapy. Also monitor urine caclium levels to avoid hypercalciuria  
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hyperparathyroidism   causes hypercalcemia from increase bone and renal resorption and increase intestinal absorption of cacium via increase acivated vitamin D. Symptoms of hypercalcemia includ vomiting, constipation, abdominal pain, weakness, parethesis, malaise and bone pain   uncommon in childhood   associated with mult endocrine neoplasia syndromes. Secondary hyperparathyroidism is more common and develops in response to hypocalcemic states, such as renal failure or rickets. the distinguishing lab value: nml to _ calcium levels   ^PTH ^ Serum Ca, _ serum phos Nml/^ alk phos   Hydration is the mainstay of Rx by enhancing calciuria. Furosemide may be used with caution if hydration is adquate. Hydrocortisone, 1mg/kg q6hr reduces intestinal absorption of Ca. Calcitonin opposes bone resorption Surgical removal of parathyroid glands   beware of hypoparathyroidism follwing surgical removal of the parathyroid gland.  
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What is the physiology of hypothyroidism   Pituitary stimulates TSH => iodine uptake => T4 & T3 synthesis => neg feedback mechanism.   (blank)   (blank)   (blank)   (blank)   (blank)  
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Hypothyroidsim - Congenital Permanent   Insufficient thyroxine after birth   (blank)   Improper migration. Hypoplasia, aplasia (underdevelopment or an absenc of the thyoid gland)   (blank)   (blank)   (blank)  
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Hypothyroidism - Congential Transient   (blank)   (blank)   Mother has iodine deficiency or Mom taking antithyroid drugs during pregnancy which cross the placenta => newborn hypothyroidism. Maternal antibodies present. b/c of Rx with RAI antibodies cross placenta   (blank)   (blank)   (blank)  
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