Literature DB >> 9781912

Weaver syndrome: autosomal dominant inheritance of the disorder.

V K Proud1, S R Braddock, L Cook, D D Weaver.   

Abstract

Weaver syndrome (WS), a condition first described in 1974 by Weaver et al., consists of macrosomia, advanced skeletal age, characteristic pattern of facial and radiographic anomalies, and contractures. Although there have been three reports of close relatives (sibs or both parent and offspring) affected with this condition, the syndrome generally occurs sporadically, and the recurrence risk in sporadic cases appears to be low. We report here on a family in which the propositus and his sister were born with the facial phenotype, club feet, and macrosomia characteristic of WS. Their father had macrosomia and macrocephaly as an adult, and childhood photos show clearly that he has WS. Two sisters of the propositus have had normal growth and development. The syndrome in this family appears to be inherited in an autosomal dominant fashion.

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Mesh:

Year:  1998        PMID: 9781912     DOI: 10.1002/(sici)1096-8628(19981002)79:4<305::aid-ajmg13>3.0.co;2-v

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


  5 in total

1.  NSD1 mutations are the major cause of Sotos syndrome and occur in some cases of Weaver syndrome but are rare in other overgrowth phenotypes.

Authors:  Jenny Douglas; Sandra Hanks; I Karen Temple; Sally Davies; Alexandra Murray; Meena Upadhyaya; Susan Tomkins; Helen E Hughes; Trevor R P Cole; Nazneen Rahman
Journal:  Am J Hum Genet       Date:  2002-12-02       Impact factor: 11.025

2.  Mutations in EZH2 cause Weaver syndrome.

Authors:  William T Gibson; Rebecca L Hood; Shing Hei Zhan; Dennis E Bulman; Anthony P Fejes; Richard Moore; Andrew J Mungall; Patrice Eydoux; Riyana Babul-Hirji; Jianghong An; Marco A Marra; David Chitayat; Kym M Boycott; David D Weaver; Steven J M Jones
Journal:  Am J Hum Genet       Date:  2011-12-15       Impact factor: 11.025

3.  Weaver syndrome: A report of a rare genetic syndrome.

Authors:  Nitin Bansal; Amit Bansal
Journal:  Indian J Hum Genet       Date:  2009-01

4.  Germline mutations in the oncogene EZH2 cause Weaver syndrome and increased human height.

Authors:  Katrina Tatton-Brown; Sandra Hanks; Elise Ruark; Anna Zachariou; Silvana Del Vecchio Duarte; Emma Ramsay; Katie Snape; Anne Murray; Elizabeth R Perdeaux; Sheila Seal; Chey Loveday; Siddharth Banka; Carol Clericuzio; Frances Flinter; Alex Magee; Vivienne McConnell; Michael Patton; Wolfgang Raith; Julia Rankin; Miranda Splitt; Volker Strenger; Clare Taylor; Patricia Wheeler; Karen I Temple; Trevor Cole; Jenny Douglas; Nazneen Rahman
Journal:  Oncotarget       Date:  2011-12

5.  Anesthetic management of a patient with Weaver syndrome undergoing emergency evacuation of extra-dural hematoma: A case report and review of the literature.

Authors:  R S Khokhar; Msm Hajnour; M Aqil; A H Al-Saeed; S Qureshi
Journal:  Saudi J Anaesth       Date:  2016 Jan-Mar
  5 in total

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