Name
PSEUDOHYPOPARATHYROIDISM
DESCRIPTION
DETAIL
A HETEROGENOUS GROUP OF DISORDERS CHARACTERIZED BY HYPOCALCEMIA, HYPERPHOSPHATEMIA, HIGH PTH LEVELS WITH INSENSITIVITY TO BIOLOGICAL ACTIVITY OF PTH D.D. : - SECONDARY HYPERPARATHYROIDISM - AUTOIMMUNE POLYENDOCRINE SYNDROMES - VITAMIN D DEFICIENCY* SERUM CALCIUM LEVELS BOTH TOTAL & IONISED CALCIUM - LOW * SERUM PHOSPHATE - HIGH * SERUM PTH - HIGH ( WITH LOW CALCIUM LEVELS, PT HAS TWO POSSIBILITIES ONLY, PHP OR SEC HYPERPARATHYROIDISM ) * X-RAY HAND - SPECIFIC PATTERN OF SHORTENING OF BONES IN WHICH DISTAL PHALYNX OF THUMB & THIRD THROUGH FIFTH METACARPALS ARE SHORTENED MOST SEVERELY WITH SMALL SOFT TISSUE OPACITIES ( CALCIFICATION/OSSIFICATION ). * CT SCAN HEAD - MAY SHOW CALCIFICATION OF BASAL GANGLIA * ECG - PROLONGATION OF QT INTERVAL DUE TO HYPOCALCEMIA * BONE MINERAL DENSITY - LOW
TYPENOTES
Medical Care All patients with severe symptomatic hypocalcemia should be initially treated with intravenous calcium. Administration of oral calcium and 1alpha-hydroxylated vitamin D metabolites, such as calcitriol, remains the mainstay of treatment and should be initiated in every patient with a diagnosis of PHP. The goals of therapy are to maintain serum total and ionized calcium levels within the reference range to avoid hypercalciuria and to suppress PTH levels to normal. This is important because elevated PTH levels in patients with PHP could cause increased bone remodeling and can lead to hyperparathyroid bone disease. In adults, infuse approximately 100 mg of elemental calcium (either calcium chloride or calcium gluconate) over 10-20 minutes. If this measure does not alleviate the clinical manifestation, 100 mg/h of elemental calcium can be infused (in adults), with close monitoring of calcium levels. Do not rapidly infuse calcium because of the possible adverse effects of cardiac conduction defects; cardiac monitoring may help guide therapy. The two most readily available formulations for parenteral use are calcium chloride and calcium gluconate; a 10-mL ampule of 10% calcium chloride contains 360 mg of elemental calcium, and a 10-mL ampule of 10% calcium gluconate contains 93 mg of elemental calcium. For neonates, infants, and children, the recommended initial dose is 0.5-1 mL/kg of 10% calcium gluconate administered over 5 minutes. Surgical Care Rarely, extraskeletal osteomas require surgical removal to relieve pressure symptoms. Diet No restrictions are necessary. Activity No restrictions are necessary.
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
SERUM CALCIUM, X-RAY HAND A.P. & OBLIQUE VIEW, CT SCAN HEAD, COMPLETE BLOOD COUNT, ECG, PARATHYROID HORMONE ASSAY, SERUM PHOSPHORUS