Parathyroid hormone deficiency with Albright's hereditary osteodystrophy.

Abstract

ABSTRACT Albright described a group of patients with characteristic skeletal changes which occurred with parathyroid hormone (PTH)-resistant hypocalcemia (pseudohypoparathyroidism), or which occurred with normocalcemia (pseudopseudohypoparathyroidism). A case is reported of a patient who had hypocalcemia associated with these skeletal changes. On the basis of a low circulating PTH level and normal urinary cyclic AMP and phosphorus excretion in response to PTH infusion, the hypoparathyroidism was the hormone-deficient variety. The combination of PTH deficiency and what has been called pseudo-pseudohypoparathyroidism points out the confusion in the existing terminology, and has led us to propose a simplified classification of diseases of PTH deficiency or unresponsiveness. Hypoparathyroidism should be categorized into hormone-deficient and hormoneresistant forms. The skeletal changes described by Albright should be called Albright's hereditary osteodystrophy and can be found alone or with either form of hyp...