This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Eldridge, J.
Right arrow Articles by Evatt, B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Eldridge, J.
Right arrow Articles by Evatt, B.
Related Collections
Right arrow Musculoskeletal System

PEDIATRICS Vol. 107 No. 6 June 2001, pp. 1329-1334

The Role of Protein C, Protein S, and Resistance to Activated Protein C in Legg-Perthes Disease

Received Apr 13, 2000; accepted Sep 20, 2000.

John Eldridge*, Anne DilleyDagger , Harland AustinDagger , §, Muhydine EL-JamilDagger , §, Lori WolsteinDagger , §, John Dorisparallel , W. Craig HooperDagger , Peter L. Meehanparallel , and Bruce EvattDagger

From the * Department of Orthopedics and Pediatrics, University of Louisville School of Medicine, Louisville, Kentucky; Dagger  Hematologic Diseases Branch, Division of AIDS, STD, and Laboratory Research, National Center for Infectious Diseases, Centers for Disease Control and Prevention, US Department of Health and Human Services, Atlanta, Georgia; § Department of Epidemiology, Rollins School of Public Health, Emory University, Atlanta, Georgia; and parallel  Emory University School of Medicine, Atlanta, Georgia.

Objectives.  It has been hypothesized that Legg-Perthes disease is caused by repeated vascular interruptions of the blood supply to the proximal femur, which are precipitated by coagulation system abnormalities. To test this theory, we conducted a case-control study among 57 patients with Legg-Perthes disease and an equal number of community controls. We measured protein C and protein S and resistance to activated protein C (APC-R) from plasma.

Study Design.  Participants were placed into 1 of 3 mutually exclusive categories based on the control distribution: 1) normal, defined as either above or within 1 standard deviation below the expected mean; 2) low normal, defined as between 1 and 2 standard deviations below the expected mean; and 3) low, defined as >2 standard deviations below the expected mean. DNA was analyzed to determine the presence of a point mutation in the factor V gene that causes APC-R.

Results.  We observed a statistically significant increased risk of Legg-Perthes disease with decreasing levels of protein C and a nearly significant increased risk with decreasing levels of protein S. The factor V gene defect was present in 5 (9%) of 55 cases and 3 (5%) of 56 controls (odds ratio 1.8, 95% confidence interval: 0.4-7.7), but the mean level on the APC-R plasma test was similar for cases and controls. Nine cases and 1 control had 2 low normal or low test results (odds ratio 13.0, 95% confidence interval: 2.2-75).

Conclusions.  Our results support the belief that abnormalities of the coagulation system leading to a thrombophilic state play a role in Legg-Perthes disease; however, larger studies are needed before definitive recommendations for coagulation testing can be made.  Key words:  avascular necrosis, coagulation factors, genetics, Legg-Perthes disease, risk factors, thrombosis.




This article has been cited by other articles:


Home page
 JBJSHome page
C. J. Glueck, R. A. Freiberg, S. Boppana, and P. Wang
Thrombophilia, Hypofibrinolysis, the eNOS T-786C Polymorphism, and Multifocal Osteonecrosis
J. Bone Joint Surg. Am., October 1, 2008; 90(10): 2220 - 2229.
[Abstract] [Full Text] [PDF]

Home page
 JBJSHome page
C. J. Glueck, R. A. Freiberg, J. Oghene, R. N. Fontaine, and P. Wang
Association Between the T-786C eNOS Polymorphism and Idiopathic Osteonecrosis of the Head of the Femur
J. Bone Joint Surg. Am., November 1, 2007; 89(11): 2460 - 2468.
[Abstract] [Full Text] [PDF]

Home page
 JBJSHome page
V. V. Balasa, R. A. Gruppo, C. J. Glueck, P. Wang, D. R. Roy, E. J. Wall, C. T. Mehlman, and A. H. Crawford
Legg-Calve-Perthes Disease and Thrombophilia
J. Bone Joint Surg. Am., December 1, 2004; 86(12): 2642 - 2647.
[Abstract] [Full Text] [PDF]

Home page
 JBJSHome page
M. T. Hresko, P. A. McDougall, J. B. Gorlin, E. C. Vamvakas, J. R. Kasser, and E. J. Neufeld
Prospective Reevaluation of the Association Between Thrombotic Diathesis and Legg-Perthes Disease
J. Bone Joint Surg. Am., September 3, 2002; 84(9): 1613 - 1618.
[Abstract] [Full Text] [PDF]

Home page
 PediatricsHome page
D. M. Gaughan, L. M. Mofenson, M. D. Hughes, G. R. Seage III, G. L. Ciupak, and J. M. Oleske
Osteonecrosis of the Hip (Legg-Calve-Perthes Disease) in Human Immunodeficiency Virus-Infected Children
Pediatrics, May 1, 2002; 109(5): e74 - 74.
[Abstract] [Full Text] [PDF]