Literature DB >> 15861202

Neonatal Gaucher disease presenting as persistent thrombocytopenia.

Philip Roth1, Susan Sklower Brooks, Daniel Potaznik, Ruby Cooma, Shashi Sahdev.   

Abstract

Mutations in the beta-glucocerebrosidase gene cause Gaucher disease with the type 1 variant generally presenting later in life with mild disease and type 2 in infancy with severe neuronopathic symptoms. We describe a neonate homozygous for the D409 H mutation with thrombocytopenia, splenomegaly and cholestasis at birth as the major features.

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Year:  2005        PMID: 15861202     DOI: 10.1038/sj.jp.7211262

Source DB:  PubMed          Journal:  J Perinatol        ISSN: 0743-8346            Impact factor:   2.521


  4 in total

Review 1.  Lysosomal storage disorders in the newborn.

Authors:  Orna Staretz-Chacham; Tess C Lang; Mary E LaMarca; Donna Krasnewich; Ellen Sidransky
Journal:  Pediatrics       Date:  2009-04       Impact factor: 7.124

2.  Neonatal cholestasis as initial manifestation of type 2 Gaucher disease: a continuum in the spectrum of early onset Gaucher disease.

Authors:  Abdallah F Elias; Maria Ronningen Johnson; John K Boitnott; David Valle
Journal:  JIMD Rep       Date:  2011-12-11

3.  Glucosylceramide transfer from lysosomes--the missing link in molecular pathology of glucosylceramidase deficiency: a hypothesis based on existing data.

Authors:  M Elleder
Journal:  J Inherit Metab Dis       Date:  2006-11-02       Impact factor: 4.982

4.  Gaucher disease: clinical phenotypes and refining GBA mutational spectrum in Thai patients.

Authors:  Tim Phetthong; Thipwimol Tim-Aroon; Arthaporn Khongkraparn; Saisuda Noojarern; Chulaluck Kuptanon; Khunton Wichajarn; Achara Sathienkijkanchai; Kanya Suphapeetiporn; Pimlak Charoenkwan; Adisak Tantiworawit; Naruwan Noentong; Duangrurdee Wattanasirichaigoon
Journal:  Orphanet J Rare Dis       Date:  2021-12-20       Impact factor: 4.123

  4 in total

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