Thoracic insufficiency syndrome in patients with fibrodysplasia ossificans progressiva

被引：55

作者：

Kaplan F.S. ^{[1
,2
,3
,4
]}

Glaser D.L. ^{[1
,3
]}

机构：

[1] Department of Orthopaedic Surgery, University of Pennsylvania, School of Medicine, Philadelphia, PA

[2] Department of Medicine, University of Pennsylvania, School of Medicine, Philadelphia, PA

[3] Center for Research in FOP and Related Disorders, University of Pennsylvania, School of Medicine, Philadelphia, PA

[4] Department of Orthopaedic Surgery, University of Pennsylvania, School of Medicine, Philadelphia, PA 19104, Silverstein Two

来源：

Clinical Reviews in Bone and Mineral Metabolism | 2005年 / 3卷 / 3-4期

关键词：

Costovertebral malformations; Fibrodysplasia ossificans progressiva (FOP); Heterotopic ossification; Kyphoscoliosis; Thoracic insufficiency syndrome;

D O I：

10.1385/BMM:3:3-4:213

中图分类号：

学科分类号：

摘要：

Patients with fibrodysplasia ossificans progressiva (FOP) develop thoracic insufficiency syndrome (TIS), which can lead to life-threatening complications. Features contributing to TIS in patients with FOP include costovertebral malformations with orthotopic ankylosis of the costovertebral joints; ossification of intercostal muscles, paravertebral muscles, and aponeuroses; and progressive spinal deformity, including kyphoscoliosis or thoracic lordosis. Pneumonia and right-sided congestive heart failure are the major life-threatening hazards that result from TIS in patients with FOP. Prophylactic measures to maximize pulmonary function, minimize respiratory compromise, and prevent influenza and pneumonia may be helpful in decreasing the morbidity and mortality from TIS in patients with FOP. © Copyright 2005 by Humana Press Inc. All rights of any nature whatsoever reserved.

引用

页码：213 / 216

页数：3

共 14 条

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