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  • Papillon-Lefervre syndrome
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PLS Papillon-Lefevre syndrome; polydactyly-luxation syndrome; preleukemic syndrome; primary lateral scle...
KMLE ÀÚµ¿ÃßÃâ ÀÇÇоà¾î »çÀü À¯»ç °Ë»ö °á°ú : 1 ÆäÀÌÁö: 1
PLS Papillon Lefevre syndrome
CancerWEB ¿µ¿µ ÀÇÇлçÀü ¸ÂÃã °Ë»ö °á°ú : 1 ÆäÀÌÁö: 1
Papillon, M <person> 20th century French dermatologist.
See: Papillon-Lefevre syndrome.
(05 Mar 2000)
CancerWEB ¿µ¿µ ÀÇÇлçÀü À¯»ç °Ë»ö °á°ú : 3 ÆäÀÌÁö: 1
Papillon-Leage and Psaume syndrome <syndrome> 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)
Papillon-Leage, E <person> 20th century French dentist.
See: Papillon-Leage and Psaume syndrome.
(05 Mar 2000)
Papillon-Lefevre syndrome <syndrome> A rare congenital hyperkeratosis of the palms and soles (palmoplantar keratoderma), with progessive destruction of the alveolar bone of both the deciduous and permanent teeth.
It occurs between the first and fifth years of life and has autosomal recessive inheritance.
Inheritance: autosomal recessive.
(05 Mar 2000)
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