Albright described a group of patients with characteristic skeletal changes which occurred with parathyroid hormone (PTH)-resistant hypocalcemia (pseudohypoparathyroidism), or which occurred with normocalcemia (pseudo-pseudohypoparathyroidism). 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 hypoparathyroidism. The terms pseudohypoparathyroidism and pseudo-pseudohypoparathyroidism should be eliminated.
ASJC Scopus subject areas
- Endocrinology, Diabetes and Metabolism
- Clinical Biochemistry
- Biochemistry, medical