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A CASE OF PSEUDO-PSEUDOHYPOPARATHYROIDISM

Institute de Investigaciones Médicas, Plaza Morelos, Caracas, and Hospital Vargas Caracas, Venezuela

IN 1942, Albright and his collaborators described 3 cases characterized by: 1) clinical signs of tetany associated with hypocalcemia and hyperphosphatemia; 2) a characteristic physical habitus, and bone abnormalities involving mainly the metacarpals and metatarsals; 3) a propenpensity on the part of the subcutaneous tissue to form bone, and usually calcification of the basal ganglia of the brain; and 4) failure of injected parathyroid extract to induce phosphorus diuresis. Albright attributed the syndrome to a failure of the organism to respond properly to its own parathyroid hormone (1).

The syndrome was further studied by Albright's group in 1950 (2) and it was speculatively concluded at the time that the disturbance at the end-organ for parathyroid function, the dyschondroplasia and the metastatic calcifications were three independent defects due to a genetic disorder. It was therefore concluded that, clinically, any one of the defects could be present without the other. This proved to be true in a case presented by Albright and collaborators in 1952 (3). The patient was a 20-year-old woman, with typical appearance, short metacarpals I, IV and V, absence of calcification of the basal ganglia, lack of response to injected parathyroid extract, but normal serum calcium and inorganic phosphorus levels. In characteristic fashion, Albright coined the new syndrome " ‘-hjpocalcemic- pseudohypoparathyroidism’, or, more correctly, an ‘-hyperphosphatemic- pseudohypoparathyroidism’, or less awkwardly, ‘pseudopseudo- hypoparathyroidism.’"

Received November 29, 1954.

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