Literature DB >> 3425305

Gaucher disease: treatment of hypersplenism with splenic embolization.

B D Thanopoulos1, C A Frimas, S P Mantagos, N G Beratis.   

Abstract

Hypersplenism is a frequent complication of Gaucher disease requiring splenectomy. A patient with Gaucher disease and severe hypersplenism was treated with partial splenic embolization to avoid the increased risk of serious infectious complications and deterioration of the disease associated with splenectomy. A first embolization (25% ablation) was performed at 4 years. Because of persisting abdominal discomfort, failure to thrive and signs of hypersplenism a second embolization (40-50% ablation) was performed 18 months later. Subsequently, the patient's health improved remarkably and 4 years later he achieved normal growth, maintains normal haematologic parameters, is free of symptoms and has no skeletal abnormalities. No serious infections have occurred. The size of the liver and the spleen has not changed appreciably. It appears that partial splenic embolization may be preferable to splenectomy in patients with Gaucher disease, especially in those of young age.

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Mesh:

Year:  1987        PMID: 3425305     DOI: 10.1111/j.1651-2227.1987.tb17286.x

Source DB:  PubMed          Journal:  Acta Paediatr Scand        ISSN: 0001-656X


  2 in total

1.  The health-related quality of life of adults with Gaucher's disease receiving enzyme replacement therapy: results from a retrospective study.

Authors:  A M Damiano; G M Pastores; J E Ware
Journal:  Qual Life Res       Date:  1998-07       Impact factor: 4.147

2.  Partial splenic embolization in a child with Gaucher disease, massive splenomegaly and severe thrombocytopenia.

Authors:  Andres H Pena; Paige Kaplan; Jaya Ganesh; Egor Clevac; Anne Marie Cahill
Journal:  Pediatr Radiol       Date:  2009-05-27
  2 in total

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