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布鲁克综合征——一种罕见的骨脆性和关节挛缩综合征及新型纯合FKBP10突变。

Bruck syndrome - a rare syndrome of bone fragility and joint contracture and novel homozygous FKBP10 mutation.

作者信息

Moravej Hossein, Karamifar Hamdollah, Karamizadeh Zohreh, Amirhakimi Gholamhossein, Atashi Sepideh, Nasirabadi Shiva

机构信息

Haematology Research Centre, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

Endokrynol Pol. 2015;66(2):170-4. doi: 10.5603/EP.2015.0024.

Abstract

Bruck syndrome is an autosomal recessive syndrome consisting of bone fragility and congenital joint contractures. According to the genotype, it has been classified into types 1 and 2. Recently, mutations in FKBP10, localised to chromosome 17q21, have been identified in some patients of Bruck syndrome. Twenty-seven patients of this syndrome have been reported so far. We present a new patient of this syndrome, with frequent fractures, congenital joint contractures, kyphoscoliosis, bilateral clubfoot, and pectus carinatum. The clinical and genetic features of all previously reported cases are also reviewed.

摘要

布鲁克综合征是一种常染色体隐性综合征,其特征为骨质脆弱和先天性关节挛缩。根据基因型,它已被分为1型和2型。最近,在一些布鲁克综合征患者中发现了位于17号染色体q21区域的FKBP10基因突变。迄今为止,已报道了27例该综合征患者。我们报告了1例该综合征新病例,患者有频繁骨折、先天性关节挛缩、脊柱侧凸、双侧马蹄内翻足和鸡胸。我们还回顾了所有先前报道病例的临床和遗传特征。

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