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Journal Article Ppublish 29 citations

PubMed: 33293746 DOI: 10.2471/BLT.20.253583

Surveillance for sickle cell disease, United Republic of Tanzania.

Bull World Health Organ 98(12):859-868

Authors: Emmanuela E Ambrose, Luke R Smart, Mwesige Charles, Arielle G Hernandez, Teresa Latham, Adolfine Hokororo, Medard Beyanga, Thad A Howard, Erasmus Kamugisha, Kathryn E McElhinney, Erius Tebuka, Russell E Ware

Affiliations

  • Department of Paediatrics and Child Health, Catholic University of Health and Allied Sciences, Mwanza, United Republic of Tanzania.
  • Division of Hematology, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati Children's Hospital, 3333 Burnet Avenue, MLC 7015 Cincinnati, Ohio 45229, United States of America.
  • Department of Laboratory Services, Bugando Medical Centre, Mwanza, United Republic of Tanzania.
  • Department of Biochemistry and Molecular Biology, Catholic University of Health and Allied Sciences, Mwanza, United Republic of Tanzania.
  • Department of Pathology, Catholic University of Health and Allied Sciences, Mwanza, United Republic of Tanzania.

Abstract

OBJECTIVE

To determine the regional- and district-level newborn prevalence of sickle cell trait and disease, and the prevalence of haemoglobin variants and genetic modifiers of sickle cell disease, in the nine regions of north-western United Republic of Tanzania.

METHODS

We repurposed dried blood spot samples from children (aged 0-24 months) born to mothers living with human immunodeficiency virus (HIV), collected as part of the HIV Early Infant Diagnosis programme, for sickle cell diagnosis. We performed isoelectric focusing to determine whether samples had normal haemoglobin, sickle cell trait, sickle cell disease or a rare haemoglobin variant. We shipped samples diagnosed as disease or variant to Cincinnati Children's Hospital in the United States of America for deoxyribonucleic-acid-based analyses to determine the prevalence of α-thalassaemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency or fetal haemoglobin genetic modifiers.

FINDINGS

We analysed a total of 17 200 specimens during February 2017-May 2018. We observed a prevalence of sickle cell trait and disease of 20.3% (3492/17 200) and 1.2% (210/17 200), respectively. District-level trait varied from 8.6% (5/58) to 28.1% (77/274). Among confirmed sickle cell disease specimens, we noted 42.7% (61/143) had 1-gene deletion and 14.7% (21/143) had 2-gene deletion α-thalassaemia trait. We documented G6PD A deficiency in 19.2% (14/73) of males.

CONCLUSION

Our calculated prevalence is twice as high as previously reported and reinforces the need for enhanced sickle cell diagnostic services. Our district-level data will inform public health policy, allowing screening and disease-modifying hydroxyurea therapy to be focused on high-prevalence areas, until universal newborn screening is available.

MeSH

Cells, Genes, Sickle Cell Trait, Infant, Newborn, Anemia, Sickle Cell, Public Health, Hiv, Gene Deletion, Prevalence

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