Lipid Storage Diseases- A Clinico-haematological Study
Background: To study the clinico- haematological profile of lipid storage disorders in pediatric age group on bone marrow examination. Methods: In this descriptive study, clinicohaematological profile of patients with lipid storage diseases in 1147 patients who underwent bone marrow examination, was...
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| Format: | Article |
| Language: | English |
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Rawalpindi Medical University
2012-12-01
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| Series: | Journal of Rawalpindi Medical College |
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| Online Access: | https://www.journalrmc.com/index.php/JRMC/article/view/561 |
| Summary: | Background: To study the clinico- haematological
profile of lipid storage disorders in pediatric age group on
bone marrow examination.
Methods: In this descriptive study, clinicohaematological
profile of patients with lipid storage
diseases in 1147 patients who underwent bone marrow
examination, was studied.
Results: A total number of 20 cases of lipid storage
disease were diagnosed. The mean age was 2 yrs.
Thirteen(69%) were male , with a male to female ratio of
2.2:1. Ten (50%) cases presented with severe symptoms.
Hepatosplenomegaly was present in majority(75%).
Failure to thrive was the commonest clinical presentation.
Pancytopenia was seen in 80%.
Conclusion: Niemann-Pick disease 65%) was the
commonest, followed by Gaucher’s Disease (35%). |
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| ISSN: | 1683-3562 1683-3570 |