Pseudopseudohypoparathyroidism

Pseudopseudohypoparathyroidism (pseudo-PHP) is a disease characterized by a constellation of clinical features collectively termed Albright hereditary osteodystrophy (AHO; see this term) but no evidence of resistance to parathyroid hormone (PTH), which is seen in other forms of pseudohypoparathyroidism (PHP; see this term).

Shortening of all metacarpals

Abnormal reduction in length of all metacarpal bones.


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PMID (PMCID)
8282968
 FEMALE Child
Pseudopseudohypoparathyroidism associated with idiopathic growth hormone deficiency. Role of treatment with biosynthetic growth hormone.
Manfredi R, Zucchini A, Azzaroli L, Manfredi G.
J Endocrinol Invest. 1993;16(9):709-13.
A case is presented of a prepubertal girl with the characteristic somatic features of Albright's hereditary osteodystrophy, including severe short stature, cataracts and shortening of all metacarpals and metatarsals and of the second middle hand phalanges, whose diagnosis of pseudopseudohypoparathyroidism (PPHP) was confirmed by laboratory evaluation (normocalcemia, normophosphatemia, normal levels of circulating PTH and normal response to exogenous PTH).