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Albright's Hereditary Osteodystrophy
Report of a Family with Studies of Bone Remodeling
- A. ROBERT ARNSTEIN, M.D.;
- BOY FRAME, M.D., F.A.C.P.;
- HAROLD M. FROST, M.D.; and
- MELVIN A. BLOCK, M.D.
- Requests for reprints should be addressed to A. Robert Arnstein, M.D., Department of Medicine, Henry Ford Hospital, Detroit, Mich. 48202.
Excerpt
The original prediction (1, 2) of the importance of genetic factors in pseudohypoparathyroidism and its relationship to pseudopseudohypoparathyroidism is proving to be correct as increasing reports of familial occurrence are appearing. The subject has been thoroughly discussed in the recent review by Mann, Alterman, and Hill (3), who have proposed that the term "Albright's hereditary osteodystrophy" be adopted to encompass the classical forms of pseudohypoparathyroidism and pseudo-pseudohypoparathyroidism as well as various forms of partial expression of the syndrome. This report adds 1 family to the 22 collected by Mann and associates (3) and provides new observations on bone remodeling dynamics
Summario in Interlingua
Es presentate le caso de un familia in le qual osteodystrophia hereditari de Albright es presente in al minus duo membros. Le filia es le probanda representante con pseudohypoparathyroidismo le expression complete de osteodystrophia hereditari de Albright. Le matre, Caso No. 2, representa un forme fruste con solmente calcification subcutanee e nanismo. Le question de un possibile contribution paterne es presentate sed non resolvite. Le configuration genealogic es in harmonia con le these de un modo de hereditage a dominantia con ligage sexual. Resistentia contra extracto parathyroide esseva demonstrate in le probanda per un curso de administrationes intramuscular de extracto parathyroide. In iste connexion, le character problematic del test de Ellsworth-Howard es signalate, e le utilitate del administration intramuscular de extracto parathyroide es commentate. Studios preliminari del remodulage de osso per medio de marcation in vivo a tetracyclina con subsequente biopsias costal revelava un declino general in le metabolismo ossee e esseva interpretate como corroboration del these de un reducite activitate del cellulas ossee, tanto primordial como etiam differentiate, in ambe le subjectos. Le probanda habeva morphologicamente normal tissu parathyroide. Studios immunologic indicava le presentia de un substantia immunologicamente simile a human hormon parathyroide in le tissu parathyroide de illa. Le base del resistentia contra hormon parathyroide es probabilemente un defecto intrinsec del organo destinatari. Altere formas de resistentia contra hormon parathyroide es mentionate in un discussion de certe connexiones hypothetic de metabolismo.
Article and Author Information
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From the Henry Ford Hospital, Detroit, Mich.
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- Received October 29, 1965.
- Accepted January 18, 1966.
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