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...
| Main Author: | |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Rawalpindi Medical University
2012-12-01
|
| Series: | Journal of Rawalpindi Medical College |
| Subjects: | |
| Online Access: | https://www.journalrmc.com/index.php/JRMC/article/view/561 |
| _version_ | 1819180774224560128 |
|---|---|
| author | Mahwash Rashid |
| author_facet | Mahwash Rashid |
| author_sort | Mahwash Rashid |
| collection | DOAJ |
| description | 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%). |
| first_indexed | 2024-12-22T22:19:41Z |
| format | Article |
| id | doaj.art-bb56f15aa257456bb22c5cd840a971d2 |
| institution | Directory Open Access Journal |
| issn | 1683-3562 1683-3570 |
| language | English |
| last_indexed | 2024-12-22T22:19:41Z |
| publishDate | 2012-12-01 |
| publisher | Rawalpindi Medical University |
| record_format | Article |
| series | Journal of Rawalpindi Medical College |
| spelling | doaj.art-bb56f15aa257456bb22c5cd840a971d22022-12-21T18:10:42ZengRawalpindi Medical UniversityJournal of Rawalpindi Medical College1683-35621683-35702012-12-01162Lipid Storage Diseases- A Clinico-haematological StudyMahwash Rashid0Department of Pathology, Army Medical College, RawalpindiBackground: 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%).https://www.journalrmc.com/index.php/JRMC/article/view/561Gaucher’sDiseaseLipid Storage disordersNiemann-Pick Disease |
| spellingShingle | Mahwash Rashid Lipid Storage Diseases- A Clinico-haematological Study Journal of Rawalpindi Medical College Gaucher’s Disease Lipid Storage disorders Niemann-Pick Disease |
| title | Lipid Storage Diseases- A Clinico-haematological Study |
| title_full | Lipid Storage Diseases- A Clinico-haematological Study |
| title_fullStr | Lipid Storage Diseases- A Clinico-haematological Study |
| title_full_unstemmed | Lipid Storage Diseases- A Clinico-haematological Study |
| title_short | Lipid Storage Diseases- A Clinico-haematological Study |
| title_sort | lipid storage diseases a clinico haematological study |
| topic | Gaucher’s Disease Lipid Storage disorders Niemann-Pick Disease |
| url | https://www.journalrmc.com/index.php/JRMC/article/view/561 |
| work_keys_str_mv | AT mahwashrashid lipidstoragediseasesaclinicohaematologicalstudy |