Literature DB >> 8957527

From Pitt-Rogers-Danks syndrome to Wolf-Hirschhorn syndrome and back?

M Zollino1, R Bova, G Neri.   

Abstract

Apparently normal chromosomes without a molecular 4p16.3 deletion were found in a patient with a Wolf-Hirschhorn syndrome (WHS) phenotype. During a 10-year-period of observation he consistently presented with typical facial appearance, moderate to severe mental retardation, normal physical development with normal head circumference. Genetic results and the relatively mild clinical manifestations suggest that a diagnosis of Pitt-Rogers-Danks syndrome (PRDS) may be more likely in this patient. If WHS and PRDS will ultimately prove to be caused by haploinsufficiency of the same gene in 4p16, non-deleted patients such as the present one will be good candidates for the search of point mutations in such putative gene.

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Year:  1996        PMID: 8957527     DOI: 10.1002/(SICI)1096-8628(19961202)66:1<113::AID-AJMG29>3.0.CO;2-U

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


  3 in total

1.  New overgrowth syndrome and FGFR3 dosage effect.

Authors:  M M Cohen; G Neri
Journal:  J Med Genet       Date:  1998-04       Impact factor: 6.318

2.  Xp22.33p22.12 Duplication in a Patient with Intellectual Disability and Dysmorphic Facial Features.

Authors:  Carla Lintas; Chiara Picinelli; Ignazio S Piras; Roberto Sacco; Stefano Gabriele; Magda Verdecchia; Antonio M Persico
Journal:  Mol Syndromol       Date:  2016-01-12

3.  Wolf-Hirschhorn syndrome facial dysmorphic features in a patient with a terminal 4p16.3 deletion telomeric to the WHSCR and WHSCR 2 regions.

Authors:  Hannelie Engbers; Jasper J van der Smagt; Ruben van 't Slot; Joris R Vermeesch; Ron Hochstenbach; Martin Poot
Journal:  Eur J Hum Genet       Date:  2008-10-01       Impact factor: 4.246

  3 in total

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