Year: 2018
Type: article
Study design: Observational Study
Abstract: 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). 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. 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. 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.
Source: BMC Hematology
Authors: Julie Makani, Furahini Tluway, Abel Makubi, Deogratius Soka, Siana Nkya, Raphael Sangeda, Josephine Mgaya, Stella Rwezaula, Fenella J. Kirkham, Christina Kindole, Elisha Osati, Elineema Meda, Robert W. Snow, Charles R. Newton, David Roberts, Muhsin Aboud, Swee Lay Thein, Sharon E. Cox, Lucio Luzzatto, Bruno P. Mmbando
Institutions: Muhimbili University of Health and Allied Sciences, University of Oxford, Muhimbili National Hospital, University of Dar es Salaam, University College London, Kenya Medical Research Institute, National Institutes of Health, London School of Hygiene & Tropical Medicine, National Institute for Medical Research
Language: English
Subfield: Genetics
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $2,170
Awards: Developing research capacity and leadership in East Africa from the KEMRI- Wellcome Trust Research Programme, Kenya, Defining the spectrum of sickle cell disease in East Africa: a clinico-epidemiological study., The malaria transition in East Africa., The malaria transition in East Africa., The role of anaemia and foetal haemoglobin in Sickle Cell Disease: Clinical epidemiology to establish the evidence base for interventional trials of blood transfusion and Hydroxyurea., The role of micronutrients, and genes affecting their metabolism, as determinants of the severity of sickle cell disease in East Africa., Sickle Pan-African Research Consortium Clinical Coordinating Center (SPARCO Center)
OpenAlex ID: W2900675268