Spondyloperipheral dysplasia-short ulna syndrome

Spondyloperipheral dysplasia-short ulna syndrome is a rare, genetic, primary bone dysplasia, with highly variable phenotype, typically characterized by platyspondyly, brachydactyly type E changes (short metacarpals and metatarsals, short distal phalanges in hands and feet), bilateral short ulnae and mild short stature. Other reported features include additional skeletal findings (e.g. midface hypoplasia, degenerative changes in proximal femora, limited elbow extension, bilateral sacralization of L5, clubfeet), as well as myopia, hearing loss, and intellectual disability.



患者の 徴候症状 を入力


症例報告を絞り込む



合計: 0 (症例報告)

  


(表示件数)
対応する徴候・症状  遺伝子  変異  キーワード(MeSH)
順位
(類似度)
PMID
(PMCID)
        

徴候・症状リスト(Orphanetデータベースから取得)

    合計: 13

HPO ID 徴候・症状 頻度
HP:0000926 扁平脊椎 Very frequent (99-80%)
HP:0000944 骨幹端の異常 Very frequent (99-80%)
HP:0001156 短指症候群 Very frequent (99-80%)
HP:0004322 低身長 Very frequent (99-80%)
HP:0005863 E型短指症 Very frequent (99-80%)
HP:0010049 短い中手骨 Very frequent (99-80%)
HP:0010579 円錐骨端 Very frequent (99-80%)
HP:0001376 関節運動制限 Frequent (79-30%)
HP:0002644 骨盤帯骨の形態異常 Frequent (79-30%)
HP:0002983 小肢症 Frequent (79-30%)
HP:0003022 尺骨低形成 Frequent (79-30%)
HP:0100734 脊椎骨端の異常 Frequent (79-30%)
HP:0000768 はと胸 Occasional (29-5%)


徴候・症状リスト(症例報告から取得)

    合計: 0

HPO ID 徴候・症状 症例報告数


疾患原因遺伝子リスト(Orphanetデータベースから取得)

    合計: 1

Gene Symbol 遺伝子名 Entrez Gene ID
COL2A1 collagen type II alpha 1 chain 1280