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The Journal of Bone and Joint Surgery 79:1079-84 (1997)
© 1997 The Journal of Bone and Joint Surgery, Inc.

Case Report

Familial Heterozygous Protein-S Deficiency in a Patient Who Had Multifocal Osteonecrosis. A Case Report*

HENRI PIERRE-JACQUES, M.D.{dagger}, BALTIMORE, CHARLES J. GLUECK, M.D.{ddagger}, CINCINNATI, OHIO, MICHAEL A. MONT, M.D.{dagger} and DAVID S. HUNGERFORD, M.D.{dagger}, BALTIMORE, MARYLAND

Investigation performed at Good Samaritan Hospital, Baltimore, and Jewish Hospital, Cincinnati


   Introduction
 
Osteonecrosis that is associated with the use of drugs such as corticosteroids14,24,32,35,39 or with underlying disease processes36,45,48,49 such as alcohol abuse39 has been characterized as secondary. Despite extensive investigations of the demographic characteristics of patients who have osteonecrosis32,35,39, as many as 39.5 per cent of these patients (934 of 2364 in one study39) have had no known risk factors, and the osteonecrosis has been characterized as idiopathic. However, as one of us (C. J. G.) and colleagues16-19,21-24,27 as well as others5,28,37,46 have shown, many instances of osteonecrosis that previously would have been regarded as idiopathic actually were associated with heritable thrombophilia (an increased tendency for intravascular thrombosis) and hypofibrinolysis (a reduced ability to lyse thrombi).

The association between coagulation abnormalities and osteonecrosis has been recognized for more than thirty-five years2,28,37,41, but it has not been studied extensively, to our knowledge. Recent studies by one of us (C. . . . [Full Text of this Article]


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