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A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania

BACKGROUND: Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum. Tanzania, with 11,000 SCD births annually, established the Muhimbili Sickle Cell program aiming to improve understanding of SCD...

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Autores principales: Makani, Julie, Tluway, Furahini, Makubi, Abel, Soka, Deogratius, Nkya, Siana, Sangeda, Raphael, Mgaya, Josephine, Rwezaula, Stella, Kirkham, Fenella J., Kindole, Christina, Osati, Elisha, Meda, Elineema, Snow, Robert W., Newton, Charles R., Roberts, David, Aboud, Muhsin, Thein, Swee Lay, Cox, Sharon E., Luzzatto, Lucio, Mmbando, Bruno P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6236876/
https://www.ncbi.nlm.nih.gov/pubmed/30459954
http://dx.doi.org/10.1186/s12878-018-0125-0
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author Makani, Julie
Tluway, Furahini
Makubi, Abel
Soka, Deogratius
Nkya, Siana
Sangeda, Raphael
Mgaya, Josephine
Rwezaula, Stella
Kirkham, Fenella J.
Kindole, Christina
Osati, Elisha
Meda, Elineema
Snow, Robert W.
Newton, Charles R.
Roberts, David
Aboud, Muhsin
Thein, Swee Lay
Cox, Sharon E.
Luzzatto, Lucio
Mmbando, Bruno P.
author_facet Makani, Julie
Tluway, Furahini
Makubi, Abel
Soka, Deogratius
Nkya, Siana
Sangeda, Raphael
Mgaya, Josephine
Rwezaula, Stella
Kirkham, Fenella J.
Kindole, Christina
Osati, Elisha
Meda, Elineema
Snow, Robert W.
Newton, Charles R.
Roberts, David
Aboud, Muhsin
Thein, Swee Lay
Cox, Sharon E.
Luzzatto, Lucio
Mmbando, Bruno P.
author_sort Makani, Julie
collection PubMed
description BACKGROUND: Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum. Tanzania, with 11,000 SCD births annually, established the Muhimbili Sickle Cell program aiming to improve understanding of SCD in Africa. We report the profile of SCD seen in the first 10 years at Muhimbili National Hospital (MNH). METHODS: Individuals seen at MNH known or suspected to have SCD were enrolled at clinic and laboratory testing for SCD, haematological and biochemical analyses done. Ethnicity was self-reported. Clinical and laboratory features of SCD were documented. Comparison was made with non-SCD population as well as within 3 different age groups (< 5, 5–17 and ≥ 18 years) within the SCD population. RESULTS: From 2004 to 2013, 6397 individuals, 3751 (58.6%) SCD patients, were enrolled, the majority (47.4%) in age group 5–17 years. There was variation in the geographical distribution of SCD. Individuals with SCD compared to non-SCD, had significantly lower blood pressure and peripheral oxygen saturation (SpO(2)). SCD patients had higher prevalence of severe anemia, jaundice and desaturation (SpO(2) < 95%) as well as higher levels of reticulocytes, white blood cells, platelets and fetal hemoglobin. The main causes of hospitalization for SCD within a 12-month period preceding enrolment were pain (adults), and fever and severe anemia (children). When clinical and laboratory features were compared in SCD within 3 age groups, there was a progressive decrease in the prevalence of splenic enlargement and an increase in prevalence of jaundice. Furthermore, there were significant differences with monotonic trends across age groups in SpO2, hematological and biochemical parameters. CONCLUSION: This report confirms that the wide spectrum of clinical expression of SCD observed elsewhere is also present in Tanzania, with non-uniform geographical distribution across the country. Age-specific analysis is consistent with different disease-patterns across the lifespan.
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spelling pubmed-62368762018-11-20 A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania Makani, Julie Tluway, Furahini Makubi, Abel Soka, Deogratius Nkya, Siana Sangeda, Raphael Mgaya, Josephine Rwezaula, Stella Kirkham, Fenella J. Kindole, Christina Osati, Elisha Meda, Elineema Snow, Robert W. Newton, Charles R. Roberts, David Aboud, Muhsin Thein, Swee Lay Cox, Sharon E. Luzzatto, Lucio Mmbando, Bruno P. BMC Hematol Research Article BACKGROUND: Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studies providing reliable descriptions of the disease spectrum. Tanzania, with 11,000 SCD births annually, established the Muhimbili Sickle Cell program aiming to improve understanding of SCD in Africa. We report the profile of SCD seen in the first 10 years at Muhimbili National Hospital (MNH). METHODS: Individuals seen at MNH known or suspected to have SCD were enrolled at clinic and laboratory testing for SCD, haematological and biochemical analyses done. Ethnicity was self-reported. Clinical and laboratory features of SCD were documented. Comparison was made with non-SCD population as well as within 3 different age groups (< 5, 5–17 and ≥ 18 years) within the SCD population. RESULTS: From 2004 to 2013, 6397 individuals, 3751 (58.6%) SCD patients, were enrolled, the majority (47.4%) in age group 5–17 years. There was variation in the geographical distribution of SCD. Individuals with SCD compared to non-SCD, had significantly lower blood pressure and peripheral oxygen saturation (SpO(2)). SCD patients had higher prevalence of severe anemia, jaundice and desaturation (SpO(2) < 95%) as well as higher levels of reticulocytes, white blood cells, platelets and fetal hemoglobin. The main causes of hospitalization for SCD within a 12-month period preceding enrolment were pain (adults), and fever and severe anemia (children). When clinical and laboratory features were compared in SCD within 3 age groups, there was a progressive decrease in the prevalence of splenic enlargement and an increase in prevalence of jaundice. Furthermore, there were significant differences with monotonic trends across age groups in SpO2, hematological and biochemical parameters. CONCLUSION: This report confirms that the wide spectrum of clinical expression of SCD observed elsewhere is also present in Tanzania, with non-uniform geographical distribution across the country. Age-specific analysis is consistent with different disease-patterns across the lifespan. BioMed Central 2018-11-14 /pmc/articles/PMC6236876/ /pubmed/30459954 http://dx.doi.org/10.1186/s12878-018-0125-0 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research Article
Makani, Julie
Tluway, Furahini
Makubi, Abel
Soka, Deogratius
Nkya, Siana
Sangeda, Raphael
Mgaya, Josephine
Rwezaula, Stella
Kirkham, Fenella J.
Kindole, Christina
Osati, Elisha
Meda, Elineema
Snow, Robert W.
Newton, Charles R.
Roberts, David
Aboud, Muhsin
Thein, Swee Lay
Cox, Sharon E.
Luzzatto, Lucio
Mmbando, Bruno P.
A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania
title A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania
title_full A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania
title_fullStr A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania
title_full_unstemmed A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania
title_short A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania
title_sort ten year review of the sickle cell program in muhimbili national hospital, tanzania
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6236876/
https://www.ncbi.nlm.nih.gov/pubmed/30459954
http://dx.doi.org/10.1186/s12878-018-0125-0
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