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

A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania
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 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 (SpO2). SCD patients had higher prevalence of severe anemia, jaundice and desaturation (SpO2 < 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.

Journal Article
2052-1839
Makani, Julie
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Tluway, Furahini
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Makubi, Abel
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Soka, Deogratius
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Nkya, Siana
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Sangeda, Raphael
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Mgaya, Josephine
740b5114-40dc-4020-bbe0-c54475f34328
Rwezaula, Stella
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Kirkham, Fenella J.
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Kindole, Christina
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Osati, Elisha
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Meda, Elineema
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Snow, Robert W.
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Newton, Charles R.
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Roberts, David
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Aboud, Muhsin
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Thein, Swee Lay
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Cox, Sharon E.
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Luzzatto, Lucio
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Mmbando, Bruno P.
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Makani, Julie
76a145a7-02fc-43ea-a1df-3a52d2004e48
Tluway, Furahini
f82f3340-1557-42f1-abe9-e69976e61540
Makubi, Abel
a590c8b2-00de-4f50-867f-c7adb1c23e38
Soka, Deogratius
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Nkya, Siana
e31cd803-e327-4cfd-a2f2-63655be855e7
Sangeda, Raphael
658b8bd8-0446-4249-a7fb-524d863574f4
Mgaya, Josephine
740b5114-40dc-4020-bbe0-c54475f34328
Rwezaula, Stella
e53c9209-221b-4a45-97c4-17ebacf323dc
Kirkham, Fenella J.
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Kindole, Christina
51f9b750-bda4-44b4-b66a-62f586bca8a9
Osati, Elisha
d7da34cc-07bd-44ac-a6a1-4698bde5edb9
Meda, Elineema
ee3ff767-895e-4d3f-804f-3a749700a350
Snow, Robert W.
7ff98228-6657-4b33-9793-b7f91a06c187
Newton, Charles R.
ea661613-9a2d-4e14-8d04-2d1c0804a321
Roberts, David
07128a92-3bf3-4016-8379-47528cc3c740
Aboud, Muhsin
e1b79846-ef29-48da-aa2b-bde349d48cd4
Thein, Swee Lay
496d02c6-084c-4778-ba43-abbcc667e0da
Cox, Sharon E.
6194d12c-fea6-4bff-b49a-4784928812f5
Luzzatto, Lucio
b42dd704-f8f8-4c0f-9ba3-13401c711211
Mmbando, Bruno P.
204e8234-af85-45e7-842c-9184f50a0122

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 and Mmbando, Bruno P. (2018) A ten year review of the sickle cell program in Muhimbili National Hospital, Tanzania. Hematology, 18, [33]. (doi:10.1186/s12878-018-0125-0).

Record type: Article

Abstract

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 (SpO2). SCD patients had higher prevalence of severe anemia, jaundice and desaturation (SpO2 < 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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Accepted/In Press date: 16 October 2018
e-pub ahead of print date: 14 November 2018
Published date: 2018
Keywords: Journal Article

Identifiers

Local EPrints ID: 427081
URI: http://eprints.soton.ac.uk/id/eprint/427081
ISSN: 2052-1839
PURE UUID: fb1f44fb-0523-4260-84a4-cab4aec0ed6f
ORCID for Fenella J. Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 21 Dec 2018 16:31
Last modified: 16 Mar 2024 03:22

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Contributors

Author: Julie Makani
Author: Furahini Tluway
Author: Abel Makubi
Author: Deogratius Soka
Author: Siana Nkya
Author: Raphael Sangeda
Author: Josephine Mgaya
Author: Stella Rwezaula
Author: Christina Kindole
Author: Elisha Osati
Author: Elineema Meda
Author: Robert W. Snow
Author: Charles R. Newton
Author: David Roberts
Author: Muhsin Aboud
Author: Swee Lay Thein
Author: Sharon E. Cox
Author: Lucio Luzzatto
Author: Bruno P. Mmbando

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