Literature DB >> 401564

The Dyggve-Melchio-Clausen syndrome.

S Schorr, C Legum, M Ochshorn, M Hirsch, S Moses, E E Lasch, M El-Masri.   

Abstract

Two families with Dyggve-Melchior-Clausen syndrome are reported. In the first family, Jews from Morocco, six of 10 siblings are affected. In the second family, a consanguineous marriage of Arabs from Gaza, two of three children are affected. A description of the skeletal changes in patients ranging in age from 4 to 25 years is presented. The radiologic signs of generalized platyspondyly with double humped end plates and the lace-like appearance of thickened iliac crests are pathognomonic and distinctive of the syndrome. The diagnostic features of the disease are compared to those of Morguio's disease, spondyloepiphyseal dysplasia tarda, and spondylometaphyseal dysplasia.

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Year:  1977        PMID: 401564     DOI: 10.2214/ajr.128.1.107

Source DB:  PubMed          Journal:  AJR Am J Roentgenol        ISSN: 0361-803X            Impact factor:   3.959


  4 in total

1.  Case report 431: Dyggve-Melchior-Clausen syndrome (DMCS).

Authors:  M A Hall-Craggs; M Chapman
Journal:  Skeletal Radiol       Date:  1987       Impact factor: 2.199

2.  Dyggve-Melchior-Clausen syndrome: novel splice mutation with atlanto-axial subluxation.

Authors:  Ola Khalifa; Faiqa Imtiaz; Nadia Al-Sakati; Khalid Al-Manea; Alain Verloes; Mohammed Al-Owain
Journal:  Eur J Pediatr       Date:  2010-09-24       Impact factor: 3.183

3.  Dyggve-Melchior-Clausen syndrome: clinical, genetic, and radiological study of 15 Egyptian patients from nine unrelated families.

Authors:  Mona S Aglan; Samia A Temtamy; Ekram Fateen; Adel M Ashour; Khamis Eldeeb; Gamal A Hosny
Journal:  J Child Orthop       Date:  2009-10-09       Impact factor: 1.548

4.  Management of progressive genu varum in a patient with Dyggve-Melchior-Clausen syndrome.

Authors:  Vladimir Kenis; Alexey Baindurashvili; Evgeniy Melchenko; Franz Grill; Ali Al Kaissi
Journal:  Ger Med Sci       Date:  2011-09-20
  4 in total

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