Larsen-like osseous dysplasia-short stature syndrome

Larsen-like osseous dysplasia-short stature syndrome is a rare primary bone dysplasia characterized by a Larsen-like phenotype including multiple, congenital, large joint dislocations, craniofacial abnormalities (i.e. macrocephaly, flat occiput, prominent forehead, hypertelorism, low-set, malformed ears, flat nose, cleft palate), spinal abnormalities, cylindrical fingers, and talipes equinovarus, as well as growth retardation (resulting in short stature) and delayed bone age. Other reported clinical manifestations include severe developmental delay, hypotonia, clinodactyly, congenital heart defect and renal dysplasia.



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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: 21

HPO ID 徴候・症状 頻度
HP:0000944 骨幹端の異常 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:0001385 股関節異形成 Very frequent (99-80%)
HP:0002644 骨盤帯骨の形態異常 Very frequent (99-80%)
HP:0002650 側弯 Very frequent (99-80%)
HP:0002652 骨格異形成 Very frequent (99-80%)
HP:0003312 椎体骨形態異常 Very frequent (99-80%)
HP:0004209 第5指弯指 Very frequent (99-80%)
HP:0004322 低身長 Very frequent (99-80%)
HP:0004349 骨ミネラル濃度減少 Very frequent (99-80%)
HP:0000520 眼球突出 Frequent (79-30%)
HP:0001511 子宮内成長遅滞 Frequent (79-30%)
HP:0000160 狭い口 Occasional (29-5%)
HP:0000233 薄い唇紅部縁 Occasional (29-5%)
HP:0000368 低位の後方回転した耳介 Occasional (29-5%)
HP:0000486 斜視 Occasional (29-5%)
HP:0001263 全般性発達遅滞 Occasional (29-5%)
HP:0001671 心中隔 Occasional (29-5%)
HP:0003196 短い鼻 Occasional (29-5%)
HP:0007957 角膜混濁 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