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General Reference

Nejm 2008;359:391; 2000;343:1863

Pathophys and Cause

Cause:

Pathophys:

PTH necessary for osteoclastic response to low calcium, renal tubular calcium resorption and PO4 excretion, and vit D activation

Alkalosis due to blocked PTH stimulation of organic acid production in bones (Ann IM 1972;76:825); monilia infections due to suppressor T-cell defects (Nejm 1979;300:164)

Epidemiology

Associated with Addison's, PA, Hashimoto's, DiGeorge syndrome (3rd and 4th pharyngeal pouches)

Signs and Symptoms

Sx:of hypocalcemia: circumoral paresthesias, carpal-pedal spasms, stridor, seizures (brief, mild), bronchospasm, gi cramps, anxiety, cataracts

Si:of hypocalcemia: tetany (carpal-pedal spasm, Chvostek's si and Trousseau's si); cataracts, vascular corneal opacities (band keratopathy), psychiatric changes, dystonias, and dyskinesias (Nejm 1972;286:762)

Complications

Pustular psoriasis and dermatitis herpetiformis (Ann IM 1984;100:677)

r/o: PSEUDOHYPOPARATHYROIDISM,which is genetic, sex-linked vs dominant; associated with hypopituitarism, esp types with low TSH and gi lactase deficiency; end-organ (bone and kidney) defect in cyclic AMP protein receptor for PTH as well as other hormones, eg, TSH (Ann IM 1986;105:197); si of hypercalcemia and mental retardation, short stature and phalanges; hypoplastic carious teeth; dx w Ellsworth-Howard test: iv PTH causes no increase in phosphate excretion (<25 mg/h) and/or no increase in cAMP excretion (Ann IM 1988;109:800)

FAMILIAL HYPERCALCIURIC HYPOCALCEMIA from defective calcium-sensing receptor (Nejm 1996;335:1115)

Lab and Xray

Lab:

Chem:Hypocalcemia, r/o hypoalbuminemia (Calcium Levels, Corrected in Serum for correction formulas), lung cancer or its rx, leukemia, rickets/osteomalacia, thyrocalcitonin-producing medullary carcinoma of the thyroid?, pancreatitis

Serum PO4 elevated; metabolic alkalosis

Urine:Hypocalciuria

Xray:Skull films may show basal ganglia calcification (Nejm 1971;285:72)

Treatment

Rx:

1st: Vit D 3 50 000-100 000+ U qd; or 1,25-(OH)2 Vit D (Rocaltrol), expensive; narrow margins of safety for hypercalcemia

2nd: Calcium po

3rd: Chlorthalidone 50 mg po qd or other thiazide