Literature DB >> 185451

[Total syndactylism with mesomelic shortening of the arm, radioulnar and metacarpal synostoses and disorganization of the phalanges ("cenani syndactylism") (author's transl)].

D Drohm, W Lenz, T S Yang.   

Abstract

An additional case "Cenani syndactylism" in a 7 years-old female child was described. With this autosomal recessive inherited disease, the radius and ulna are severly shortened and generally fused with each other. The metacarpal bones are, for the most part, synostosized. The phalanges are badly disorganized. Usually no fingers can be recognized externally. The alterations in the feet are far less pronounced. Syndactylism and ray reduction are frequent. A similar, but apparently independent, case with radioulnar synstostoses, reduction, dysplasia and fusion of the metacarpal bones but less pronounced malformations of the fingers was also described. Finally, new type of malformation was present for comparison in which an increased number of metacarpi and metatarsi and, to some extent, severely disorganized phalanges with massive syndactylism are present. The etiology of the second and third cases could not be explained.

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Mesh:

Year:  1976        PMID: 185451

Source DB:  PubMed          Journal:  Klin Padiatr        ISSN: 0300-8630            Impact factor:   1.349


  2 in total

1.  Case report 636. Tetrapolysyndactyly with postaxial type of polydactyly.

Authors:  R N Sener
Journal:  Skeletal Radiol       Date:  1990       Impact factor: 2.199

2.  Oligodactyly and multiple synostoses of the extremities: two cases in sibs. A variant of Cenani-Lenz syndactyly.

Authors:  P Dodinval
Journal:  Hum Genet       Date:  1979-04-27       Impact factor: 4.132

  2 in total

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