Three pairs of monozygotic twins with fibrodysplasia ossificans progressiva: The role of environment in the progression of heterotopic ossification

被引:27
|
作者
Hebela N. [1 ,4 ]
Shore E.M. [1 ,2 ,4 ]
Kaplan F.S. [1 ,3 ,4 ,5 ]
机构
[1] Department of Orthopaedic Surgery, University of Pennsylvania, School of Medicine, Philadelphia, PA
[2] Department of Genetics, University of Pennsylvania, School of Medicine, Philadelphia, PA
[3] Department of Medicine, University of Pennsylvania, School of Medicine, Philadelphia, PA
[4] Center for Research in FOP and Related Disorders, University of Pennsylvania, School of Medicine, Philadelphia, PA
[5] University of Pennsylvania, School of Medicine, Department of Orthopaedic Surgery, Philadelphia, PA 19104, Silverstein Two
来源
关键词
Fibrodysplasia ossificans progressiva (FOP); Heterotopic ossification; Twins;
D O I
10.1385/BMM:3:3-4:205
中图分类号
学科分类号
摘要
Genetic and environmental factors affect the phenotype of fibrodysplasia ossificans progressiva (FOP) but their relative effects are unknown. We studied three pairs of monozygotic twins with FOP and found that, within each pair, congenital toe malformations were identical. However, postnatal heterotopic ossification varied greatly depending on life history and environmental exposure. This study shows that genetic determinants strongly influence disease phenotype during prenatal development and that environmental factors strongly influence postnatal progression of the disease. © Copyright 2005 by Humana Press Inc. All rights of any nature whatsoever reserved.
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页码:205 / 208
页数:3
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