PMID: 9170375May 1, 1997Paper

Resistance to activated protein C and Legg-Perthes disease

Clinical Orthopaedics and Related Research
C J GlueckA Becker

Abstract

Thrombophilia may cause thrombotic venous occlusion in the femoral head, with venous hypertension and hypoxic bone death, leading to Legg-Perthes disease. Resistance to activated protein C, the most common thrombophilic trait, was measured in 64 children with Legg-Perthes disease. Genomic deoxyribonucleic acid was studied to delineate the CGA-->CAA substitution at position 1691 of the Factor V Leiden gene responsible for resistance to activated protein C. The activated protein C ratio was calculated by dividing clotting time obtained with activated protein C-calcium chloride by clotting time obtained with calcium chloride alone. Resistance to activated protein C, with a low activated protein C ratio (less than 2.19, the 5th percentile for 160 normal pediatric controls) was the most common coagulation defect, found in 23 of 64 children with Legg-Perthes disease versus 7 of 160 pediatric controls. Eight of 64 children with Legg-Perthes disease had a low activated protein C ratio and the mutant Factor V gene (7 heterozygotes, 1 homozygote) versus 1 of 101 normal pediatric controls. Two or 3 generation vertical and horizontal transmission of heterozygosity for the mutant Factor V gene was found in 4 of the 8 kindreds. Of 64 childre...Continue Reading

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Citations

Feb 26, 2000·European Journal of Clinical Investigation·C ZalavrasK N Malizos
May 12, 2001·Clinical Orthopaedics and Related Research·C J GlueckP Wang
Jun 4, 2008·Journal of Pediatric Orthopedics·Hiroshi KitohNaoki Ishiguro
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Mar 20, 2008·Clinical Orthopaedics and Related Research·Charles J GlueckPing Wang
Jul 6, 2005·Theoretical Biology & Medical Modelling·Jacob BejarJochanan H Boss

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