Leri pleonosteosis

Leri pleonosteosis is characterized by broadening and deformity of the thumbs and great toes in a valgus position (a 'spade-shaped' appearance), flexion contracture of the interphalangeal joints, generalized limitation of joint mobility, short stature, and often mongoloid facies. Additional malformations include genu recurvatum, enlargement of the posterior neural arches of the cervical vertebrae, and thickening of the palmar and forearm fasciae. A few multigenerational families have been reported so far. The disease is inherited in an autosomal dominant manner.



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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: 23

HPO ID 徴候・症状 頻度
HP:0000582 眼瞼裂斜上 Very frequent (99-80%)
HP:0000944 骨幹端の異常 Very frequent (99-80%)
HP:0001072 分厚い皮膚 Very frequent (99-80%)
HP:0001156 短指症候群 Very frequent (99-80%)
HP:0001163 中手骨の異常 Very frequent (99-80%)
HP:0001167 指の異常 Very frequent (99-80%)
HP:0001288 歩行障害 Very frequent (99-80%)
HP:0001387 関節拘縮 Very frequent (99-80%)
HP:0002816 反張膝 Very frequent (99-80%)
HP:0003312 椎体骨形態異常 Very frequent (99-80%)
HP:0003510 重度の低身長 Very frequent (99-80%)
HP:0005930 骨端の異常 Very frequent (99-80%)
HP:0011304 幅広い母指 Very frequent (99-80%)
HP:0100490 屈指 Very frequent (99-80%)
HP:0100679 皮膚弾性欠如 Very frequent (99-80%)
HP:0000581 眼瞼裂狭小 Frequent (79-30%)
HP:0001482 皮下結節 Frequent (79-30%)
HP:0002650 側弯 Frequent (79-30%)
HP:0002967 外反肘 Frequent (79-30%)
HP:0012745 短い眼瞼裂 Frequent (79-30%)
HP:0100795 異常にまっすぐな脊椎 Frequent (79-30%)
HP:0000486 斜視 Occasional (29-5%)
HP:0003042 肘脱臼 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: 0

Gene Symbol 遺伝子名 Entrez Gene ID