Literature DB >> 18609679

Gaucher disease: new developments in treatment and etiology.

Ozgur Harmanci1, Yusuf Bayraktar.   

Abstract

Gaucher disease (GD) is an autosomal recessive disease which if undiagnosed or diagnosed late results in devastating complications. Because of the heterozygous nature of GD, there is a wide spectrum of clinical presentation. Clinicians should be aware of this rare but potentially treatable disease in patients who present with unexplained organomegaly, anemia, massive splenomegaly, ascites and even cirrhosis of unknown origin. The treatment options for adult type GD include enzyme replacement treatment (ERT) and substrate reduction treatment (SRT) depending on the status of the patient. Future treatment options are gene therapy and "smart molecules" which provide specific cure and additional treatment options. In this review, we present the key issues about GD and new developments that gastroenterologists should be aware of.

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Year:  2008        PMID: 18609679      PMCID: PMC2725334          DOI: 10.3748/wjg.14.3968

Source DB:  PubMed          Journal:  World J Gastroenterol        ISSN: 1007-9327            Impact factor:   5.742


  54 in total

1.  Synthetic substrate beta-glucosidase activity in leukocytes: a reproducible method for the identification of patients and carriers of Gaucher's disease.

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Journal:  Clin Genet       Date:  1978-02       Impact factor: 4.438

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Journal:  Am J Med       Date:  1968-04       Impact factor: 4.965

3.  Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease.

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Journal:  Leber Magen Darm       Date:  1991-05

5.  Massive gastrointestinal haemorrhage associated with ileal lymphoid hyperplasia in Gaucher's disease.

Authors:  D R Jones; J Hoffman; R Downie; M Haqqani
Journal:  Postgrad Med J       Date:  1991-05       Impact factor: 2.401

6.  Gaucher disease. Clinical, laboratory, radiologic, and genetic features of 53 patients.

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Journal:  Medicine (Baltimore)       Date:  1992-11       Impact factor: 1.889

7.  Chitotriosidase as a marker of disease activity in sarcoidosis.

Authors:  Jürgen Brunner; Sabine Scholl-Bürgi; Lothar-Bernd Zimmerhackl
Journal:  Rheumatol Int       Date:  2007-05-30       Impact factor: 2.631

8.  Marked elevation of plasma chitotriosidase activity. A novel hallmark of Gaucher disease.

Authors:  C E Hollak; S van Weely; M H van Oers; J M Aerts
Journal:  J Clin Invest       Date:  1994-03       Impact factor: 14.808

9.  Human cartilage gp-39, a major secretory product of articular chondrocytes and synovial cells, is a mammalian member of a chitinase protein family.

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Journal:  J Biol Chem       Date:  1993-12-05       Impact factor: 5.157

Review 10.  Glycosidase inhibitors: inhibitors of N-linked oligosaccharide processing.

Authors:  A D Elbein
Journal:  FASEB J       Date:  1991-12       Impact factor: 5.191

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  5 in total

Review 1.  Macrophage-related diseases of the gut: a pathologist's perspective.

Authors:  Xavier Sagaert; Thomas Tousseyn; Gert De Hertogh; Karel Geboes
Journal:  Virchows Arch       Date:  2012-05-11       Impact factor: 4.064

2.  Role of chitotriosidase (chitinase 1) under normal and disease conditions.

Authors:  Manasa Kanneganti; Alan Kamba; Emiko Mizoguchi
Journal:  J Epithel Biol Pharmacol       Date:  2012

3.  Gaucher disease and cancer: concept and controversy.

Authors:  Francis Y M Choy; Tessa N Campbell
Journal:  Int J Cell Biol       Date:  2011-06-07

Review 4.  Gaucher disease and the synucleinopathies: refining the relationship.

Authors:  Tessa N Campbell; Francis Y M Choy
Journal:  Orphanet J Rare Dis       Date:  2012-01-31       Impact factor: 4.123

Review 5.  Eliglustat tartrate for the treatment of adults with type 1 Gaucher disease.

Authors:  Lunawati L Bennett; Kelsey Turcotte
Journal:  Drug Des Devel Ther       Date:  2015-08-18       Impact factor: 4.162

  5 in total

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