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Fibrochondrogenesis is a rare autosomal recessive form of osteochondrodysplasia, causing abnormal fibrous development of cartilage and related tissues. It is a lethal rhizomelic (malformations which result in short, underdeveloped limbs) form of dwarfism,〔 exhibiting both skeletal dysplasia (malformations of bone) and fibroblastic dysplasia (abnormal development of fibroblasts, specialized cells that make up fibrous connective tissue, which plays a role in the formation of cellular structure and promotes healing of damaged tissues).〔 Death caused by complications of fibrochondrogenesis occurs in infancy.〔 == Characteristics == Fibrochondrogenesis is a congenital disorder presenting several features and radiological findings, some which distinguish it from other osteochondrodysplasias. These include: fibroblastic dysplasia and fibrosis of chondrocytes (cells which form cartilage);〔〔 and flared, widened long bone metaphyses (the portion of bone that grows during childhood).〔 Other prominent features include dwarfism,〔 shortened ribs that have a concave appearance,〔 micrognathism (severely underdeveloped jaw),〔 macrocephaly (enlarged head),〔 thoracic hypoplasia (underdeveloped chest),〔 enlarged stomach,〔 platyspondyly (flattened spine),〔 and the somewhat uncommon deformity of bifid tongue (in which the tongue appears split, resembling that of a reptile).〔 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Fibrochondrogenesis」の詳細全文を読む スポンサード リンク
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