Medical Definition of Papillon-Leage and Psaume syndrome

1. An inherited syndrome, lethal in males, with varying combinations of defects of the oral cavity, face, and hands, including lobulated or bifid tongue, cleft or pseudocleft palate, tongue tumours, missing or malpositioned teeth, hypoplastic nasal alar cartilage, depressed nasal bridge, brachydactyly, clinodactyly, incomplete syndactyly, and, frequently, mental retardation. There are two subtypes recognised. Type I (papillon-leage and psaume syndrome, gorlin-psaume syndrome) is inherited as an x-linked dominant trait and is found only in females and XXY males. Type II (mohr syndrome) is inherited as an autosomal recessive trait. Inheritance: autosomal recessive and X-linked. Synonym: OFD syndrome, orofaciodigital syndrome, Papillon-Leage and Psaume syndrome. (05 Mar 2000)

Lexicographical Neighbors of Papillon-Leage And Psaume Syndrome

papillomas
papillomata
papillomatosis
papillomatous
papillomatous
Papillomavirus
papillomavirus
papilloma acuminatum
papilloma canaliculum
papilloma diffusum
papilloma durum
papilloma inguinale tropicum
papilloma molle
papilloma venereum
papillon
Papillon-Leage and Psaume syndrome (current term)
Papillon-Lefevre syndrome
papillons
papilloretinitis
papillose
papillote
papillotes
papillotomy
papillula
papillulate
papio
Papio
papion
Papio ursinus
papish

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