Literature DB >> 16760737

Two brothers with Goldberg-Shprintzen syndrome.

Helen R Murphy1, Melanie J Carver, Alice S Brooks, Simon E Kenny, Ian H Ellis.   

Abstract

Goldberg-Shprintzen syndrome is a rare autosomal recessive condition that describes the association of Hirschsprung disease with microcephaly, developmental delay and characteristic facies. We describe two brothers from a non-consanguineous family who have classical features of Goldberg-Shprintzen syndrome. The novel findings in this instance are of foot anomalies including camptodactyly and clinodactyly of the 2nd to 4th toes, which have not been previously described in Goldberg-Shprintzen syndrome.

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Year:  2006        PMID: 16760737     DOI: 10.1097/01.mcd.0000204991.84861.c9

Source DB:  PubMed          Journal:  Clin Dysmorphol        ISSN: 0962-8827            Impact factor:   0.816


  5 in total

1.  Homozygous truncating mutation of the KBP gene, encoding a KIF1B-binding protein, in a familial case of fetal polymicrogyria.

Authors:  Stéphanie Valence; Karine Poirier; Nicolas Lebrun; Yoann Saillour; Pascale Sonigo; Bettina Bessières; Tania Attié-Bitach; Alexandra Benachi; Cécile Masson; Ferechté Encha-Razavi; Jamel Chelly; Nadia Bahi-Buisson
Journal:  Neurogenetics       Date:  2013-09-27       Impact factor: 2.660

Review 2.  Chromosomal and related Mendelian syndromes associated with Hirschsprung's disease.

Authors:  S W Moore
Journal:  Pediatr Surg Int       Date:  2012-09-23       Impact factor: 1.827

3.  Goldberg-Shprintzen Syndrome Associated with a Novel Variant in the KIFBP Gene.

Authors:  Pelin Ozyavuz Cubuk
Journal:  Mol Syndromol       Date:  2021-05-07

4.  Association of a Novel Nonsense Mutation in KIAA1279 with Goldberg-Shprintzen Syndrome.

Authors:  Shadab Salehpour; Feyzollah Hashemi-Gorji; Ziba Soltani; Soudeh Ghafouri-Fard; Mohammad Miryounesi
Journal:  Iran J Child Neurol       Date:  2017

5.  Goldberg-Shprintzen syndrome is determined by the absence, or reduced expression levels, of KIFBP.

Authors:  Katherine C MacKenzie; Bianca M de Graaf; Andreas Syrimis; Yuying Zhao; Erwin Brosens; Grazia M S Mancini; Rachel Schot; Dicky Halley; Martina Wilke; Arve Vøllo; Frances Flinter; Andrew Green; Sahar Mansour; Jacek Pilch; Zornitza Stark; Eleni Zamba-Papanicolaou; Violetta Christophidou-Anastasiadou; Robert M W Hofstra; Jan D H Jongbloed; Nayia Nicolaou; George A Tanteles; Alice S Brooks; Maria M Alves
Journal:  Hum Mutat       Date:  2020-09-16       Impact factor: 4.878

  5 in total

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