Literature DB >> 25931047

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

Hossein Moravej, Hamdollah Karamifar, Zohreh Karamizadeh, Gholamhossein Amirhakimi, Sepideh Atashi, Shiva Nasirabadi1.   

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.

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Year:  2015        PMID: 25931047     DOI: 10.5603/EP.2015.0024

Source DB:  PubMed          Journal:  Endokrynol Pol        ISSN: 0423-104X            Impact factor:   1.582


  6 in total

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