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JAMES MILES, HAROLD ELRICK, PSEUDO-PSEUDOHYPOPARATHYROIDISM: REPORT OF A NEW CASE, The Journal of Clinical Endocrinology & Metabolism, Volume 15, Issue 5, 1 May 1955, Pages 576–584, https://doi.org/10.1210/jcem-15-5-576
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THE syndrome of parathyroid deficiency (1) is characterized by chronic tetany, convulsive seizures, lenticular opacities, basal ganglia calcification, and low serum calcium and high serum phosphorus levels. Pseudohypoparathyroidism (2, 3) presents the same clinical and chemical picture presumed to be due to an inability of the organism to respond to parathyroid hormone, rather than to primary hormonal deficiency. In addition, characteristic abnormalities of body-build, facies and skeletal system, as well as foci of ectopic ossification are found in pseudo-hypoparathyroidism.
In 1952 Albright, Forbes and Henneman (4) reported a case of pseudohypoparathyroidism in which all of the usual anatomic stigmata were present, but the serum calcium and phosphorus levels were normal and the patient exhibited no clinical evidence of hypoparathyroidism. They called this new syndrome “pseudo-pseudohypoparathyroidism.” In view of the absence of clinical and laboratory signs of hypoparathyroidism, one would expect a normal response to the Ellsworth-Howard test (phosphorus diuresis following intravenous parathyroid hormone injection). However, their patient did not respond normally to this test, possibly owing to the use of an impotent extract.
