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

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dc.contributor.authorMakani, Julie
dc.contributor.authorTluway, Furahini
dc.contributor.authorMakubi, Abel
dc.contributor.authorSoka, Deogratius
dc.contributor.authorNkya, Siana
dc.contributor.authorSangeda, Raphael
dc.contributor.authorMgaya, Josephine
dc.contributor.authorRwezaula, Stella
dc.contributor.authorKirkham, Fenella J
dc.contributor.authorKindole, Christina
dc.contributor.authorOsati, Elisha
dc.contributor.authorMeda, Elineema
dc.contributor.authorSnow, Robert W
dc.contributor.authorNewton, Charles R
dc.contributor.authorRoberts, David
dc.contributor.authorAboud, Muhsin
dc.contributor.authorThein, Swee L
dc.contributor.authorCox, Sharon E.
dc.contributor.authorLuzzatto, Lucio
dc.contributor.authorMmbando, Bruno P
dc.date.accessioned2019-05-07T17:19:47Z
dc.date.available2019-05-07T17:19:47Z
dc.date.issued2018
dc.description.abstractBackground:Africa has the highest burden of Sickle cell disease (SCD) but there are few large, systematic studiesproviding reliable descriptions of the disease spectrum. Tanzania, with 11,000 SCD births annually, established theMuhimbili Sickle Cell program aiming to improve understanding of SCD in Africa. We report the profile of SCD seenin 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 testingfor SCD, haematological and biochemical analyses done. Ethnicity was self-reported. Clinical and laboratory featuresof SCD were documented. Comparison was made with non-SCD population as well as within 3 different agegroups (< 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 agegroup 5–17 years. There was variation in the geographical distribution of SCD. Individuals with SCD compared tonon-SCD, had significantly lower blood pressure and peripheral oxygen saturation (SpO2). SCD patients had higherprevalence of severe anemia, jaundice and desaturation (SpO2< 95%) as well as higher levels of reticulocytes, whiteblood cells, platelets and fetal hemoglobin. The main causes of hospitalization for SCD within a 12-month periodpreceding enrolment were pain (adults), and fever and severe anemia (children). When clinical and laboratoryfeatures were compared in SCD within 3 age groups, there was a progressive decrease in the prevalence of splenicenlargement and an increase in prevalence of jaundice. Furthermore, there were significant differences withmonotonic 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 alsopresent in Tanzania, with non-uniform geographical distribution across the country. Age-specific analysis is consistentwith different disease-patterns across the lifespan.en_US
dc.description.sponsorshipWellcome Trust, UK (Fellowship Julie Makani072064, 093727; Project grant 080025; Strategic award 084538; PrincipalWellcome Trust Fellowship R W Snow 103602) and Government of UnitedRepublic of Tanzaniaen_US
dc.identifier.doihttps://doi.org/10.1186/s12878-018-0125-0
dc.identifier.urihttp://hdl.handle.net/20.500.11810/5235
dc.language.isoenen_US
dc.publisherBMC Hematologyen_US
dc.subjectSickle cell anemia, Africa, Tanzaniaen_US
dc.titleA ten year review of the sickle cell program in Muhimbili National Hospital, Tanzaniaen_US
dc.typeJournal Article, Peer Revieweden_US
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