KEGG   DISEASE: Craniofacial-deafness-hand 症候群
エントリ  
H00446                                                             
名称    
Craniofacial-deafness-hand 症候群
概要    
Craniofacial-deafness-hand syndrome is inherited as an autosomal dominant or X-linked mutation characterized by a flat facial profile, hypoplastic nose, and a sensorineural hearing loss. A missense mutation in the PAX3 has been detected in patients with the disease.
カテゴリ  
先天奇形
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 20 発達異常
  多発性の発達異常または症候群
   LD2H  症候群性遺伝性難聴
    H00446  Craniofacial-deafness-hand 症候群
病因遺伝子 
PAX3 [HSA:5077] [KO:K09381]
リンク   
ICD-11: LD2H.Y
MeSH: C536453
OMIM: 122880
文献    
  著者
Sommer A, Bartholomew DW
  タイトル
Craniofacial-deafness-hand syndrome revisited.
  雑誌
Am J Med Genet A 123A:91-4 (2003)
DOI:10.1002/ajmg.a.20501
文献    
PMID:8664898
  著者
Asher JH Jr, Sommer A, Morell R, Friedman TB
  タイトル
Missense mutation in the paired domain of PAX3 causes craniofacial-deafness-hand syndrome.
  雑誌
文献    
PMID:6859126
  著者
Sommer A, Young-Wee T, Frye T
  タイトル
Previously undescribed syndrome of craniofacial, hand anomalies, and sensorineural deafness.
  雑誌
Am J Med Genet 15:71-7 (1983)
DOI:10.1002/ajmg.1320150109
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