Literature DB >> 9605583

Craniometaphyseal dysplasia in six generations of a German kindred.

S Tinschert1, H S Braun.   

Abstract

Craniometaphyseal dysplasia (CMD) was found in 6 generations of a large German kindred; 24 affected individuals were identified. The clinical diagnosis was confirmed by further examinations in 15 individuals, including 2 exhumed skeletons. Five deceased individuals were considered to be undoubtedly affected by reviewing photographs, and 4 must have had CMD from genealogical considerations. Pedigree analysis was performed over 8 generations back to persons born at the beginning of the 18th century in a central area of Germany. The trait could be traced back to a common male ancestor, born in 1790. Molecular genetic investigations on 3 generations of this kindred are in progress. In the present study we describe the clinical characteristics of the family.

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Year:  1998        PMID: 9605583     DOI: 10.1002/(sici)1096-8628(19980518)77:3<175::aid-ajmg1>3.0.co;2-p

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  4 in total

1.  Novel ANKH mutation in a patient with sporadic craniometaphyseal dysplasia.

Authors:  Allison Zajac; Seung-Hak Baek; Imad Salhab; Melissa A Radecki; Sukwha Kim; Hakon Hakonarson; Hyun-Duck Nah
Journal:  Am J Med Genet A       Date:  2010-03       Impact factor: 2.802

2.  Autosomal recessive mental retardation, deafness, ankylosis, and mild hypophosphatemia associated with a novel ANKH mutation in a consanguineous family.

Authors:  Eva Morava; Jirko Kühnisch; Jefte M Drijvers; Joris H Robben; Cor Cremers; Petra van Setten; Amanda Branten; Sabine Stumpp; Alphons de Jong; Krysta Voesenek; Sascha Vermeer; Angelien Heister; Hedi L Claahsen-van der Grinten; Charles W O'Neill; Michèl A Willemsen; Dirk Lefeber; Peter M T Deen; Uwe Kornak; Hannie Kremer; Ron A Wevers
Journal:  J Clin Endocrinol Metab       Date:  2010-10-13       Impact factor: 5.958

3.  A novel autosomal recessive GJA1 missense mutation linked to Craniometaphyseal dysplasia.

Authors:  Ying Hu; I-Ping Chen; Salome de Almeida; Valdenize Tiziani; Cassio M Raposo Do Amaral; Kalpana Gowrishankar; Maria Rita Passos-Bueno; Ernst J Reichenberger
Journal:  PLoS One       Date:  2013-08-12       Impact factor: 3.240

Review 4.  Molecular, phenotypic aspects and therapeutic horizons of rare genetic bone disorders.

Authors:  Taha Faruqi; Naveen Dhawan; Jaya Bahl; Vineet Gupta; Shivani Vohra; Khin Tu; Samir M Abdelmagid
Journal:  Biomed Res Int       Date:  2014-10-22       Impact factor: 3.411

  4 in total

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