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- W2184413740 abstract "Background: Gaucher disease (GD) is the most common pan-ethnic inherited lysosomal storage disorder;it is subdivided into three typesandthe non-neuronopathic (type 1) is the most common type (93 %) in the western world. Gaucher disease (GD) produces a multisystem involvementand the most effective form of therapy inreversing the hematological and visceral manifestationsisenzyme replacement therapy (ERT) with recombinantenzyme imiglucerase. Objective: The aim of the study is to determine the outcome of enzyme replacement therapy in children with gaucher disease for 6 monthsin Children welfare teaching hospital Baghdad- Iraq. Methods: A descriptive retrospective study carried out in Children welfare teaching hospital, from the first of May 2013 to the first of December 2013 on 20patients, their age ranged from 20 months to 21years. Results: Of the 20 patients, 15(75%) were males and 5(25%) were females. The commonest age of presentation for the disease was between 2-5 years in 11 patients (55%). It was found that easy fatigability affect all the patients (100%) ,splenomegaly in 80%, hepatomegaly in 75%, abdominal pain & ecchymosis/epistaxis in 70% for each. The platelets count ranged from severe Thrombocytopenia to normal platelets counts with a mean of (109×10 9) /L. But it was improved to a mean of 193×10 9 /L after six months of treatment. The hemoglobin level ranged from severe anemia to almost normal hemoglobin value with a mean of 7.9g/dl, but it was raised to a mean of 9.8 g/dl. It was found that clinical improvement was achieved by the hepatic regression which occurs in 75% of patient, and splenic regression occurs in 85%. The improvement in easy tiredness noticed in 90% of patients, and it was found interestingly that bleeding tendency improved in 100% of the patients. Conclusions: There must be a high index of suspicion about gaucher disease when a child presents with hepatosplenomegaly and/or cytopenia and/or bone pain. The diagnose of the disease with noninvasive techniques at early stages in view of the availability of an effective enzyme replacement therapy which leads toimprovement in bleeding tendencyand requirement for blood transfusion." @default.
- W2184413740 created "2016-06-24" @default.
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- W2184413740 date "2014-01-01" @default.
- W2184413740 modified "2023-09-27" @default.
- W2184413740 title "The Iraqi Experience in the Treatment of Gaucher Disease in Children by Enzyme Replacement therapy" @default.
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