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Report of a Case and Review of the Literature

SOLOMON E. BARR, M.D.; EUGENE F. TAYLOR, M.D.; BORIS RABKIN, M.D.

AMA Arch Intern Med. 1960;105(3):492-495.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Despite its name, pseudo-pseudohypoparathyroidism is not an endocrine disorder but a syndrome of multiple congenital defects. It should be differentiated from hypoparathyroidism and from pseudohypoparathyroidism (Table 1).¹

True hypoparathyroidism is associated with low-to-normal serum calcium, high-tonormal serum phosphorus, decreased urinary calcium excretion, and in advanced cases, tetany (Table 1).¹ It is due to a lack of parathyroid hormone, and as demonstrated by Ellsworth and Howard in 1932, when parathyroid extract is administered, these patients exhibit a greater-thannormal phosphate diuresis.^2,3

In 1942, Albright, Burnet, and Smith reported three patients with the characteristic electrolyte changes of hypoparathyroidism who failed to respond to the administration of parathyroid extract.⁴ They labeled this syndrome "pseudohypoparathyroidism."⁴ Parathyroid gland biopsy specimens revealed a normal to hyperplastic gland structure.¹ This phenomenon may be due to a failure of endorgan response in the presence of an adequate supply of parathyroid hormone.¹ Associated with their endocrine defects, these patients usually . . . [Full Text PDF of this Article]

Author Affiliations
Riverdale, N.Y.; Oakland Calif.; Silver Spring, Md.

From the Department of Medicine, George Washington University Hospital, Washington, D.C., where Dr. Barr was formerly a Resident in Medicine. Dr. Rabkin is an Associate in Medicine and Dr. Taylor is a former senior student of the George Washington University School of Medicine.

Footnotes
Submitted for publication Aug. 17, 1959.

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