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. 2020 Sep:84:102438.
doi: 10.1016/j.bcmd.2020.102438. Epub 2020 Apr 23.

Sickle cell disease clinical phenotypes in Nigeria: A preliminary analysis of the Sickle Pan Africa Research Consortium Nigeria database

Hezekiah Isa  1 , Samuel Adegoke  2 , Anazoeze Madu  3 , Abdul-Aziz Hassan  4 , Chinatu Ohiaeri  5 , Reuben Chianumba  1 , Biobele Brown  6 , Emmanuel Okocha  7 , Ngozi Ugwu  8 , Ijeoma Diaku-Akinwumi  9 , Titilope Adeyemo  10 , Aisha Kuliya-Gwarzo  11 , Livingstone Dogara  12 , Haliru Lawal  12 , Yohanna Tanko  1 , Adama Ladu  13 , Umar Kangiwa  14 , Lilian Ekwem  15 , Seyi Oniyangi  16 , Tambi Wakama  17 , Domic Umoru  18 , Olaniyi Olanrewaju  19 , Norah Akinola  20 , Uche Nnebe-Agumadu  21 , Samuel Asala  1 , Adekunle Adekile  22 , John Olaniyi  6 , Raphael Sangeda  23 , Sickle Africa Data Coordinating Center SADaCC  24 , Obiageli Nnodu  25
Affiliations

Sickle cell disease clinical phenotypes in Nigeria: A preliminary analysis of the Sickle Pan Africa Research Consortium Nigeria database

Hezekiah Isa et al. Blood Cells Mol Dis. 2020 Sep.

Erratum in

Abstract

Background/objective: Sickle cell disease (SCD) is a monogenic disease with multiple phenotypic expressions. Previous studies describing SCD clinical phenotypes in Nigeria were localized, with limited data, hence the need to understand how SCD varies across Nigeria.

Method: The Sickle Pan African Research Consortium (SPARCO) with a hub in Tanzania and collaborative sites in Tanzania, Ghana and Nigeria, is establishing a single patient-consented electronic database with a target of 13,000 SCD patients. In collaboration with the Sickle Cell Support Society of Nigeria, 20 hospitals, with paediatric and adult SCD clinics, are participating in patient recruitment. Demographic and clinical information, collected with uniform case report forms, were entered into Excel spreadsheets and uploaded into Research Electronic Data Capture software by trained data clerks and frequency tables generated.

Result: Data were available on 3622 patients enrolled in the database, comprising 1889 (52.9%) females and 1434 (39.6%) children ≤15 years. The frequencies of Hb SS, Hb SC and Hb Sβ thalassemia in this data set were 97.5%, 2.5% and 0% respectively. Sixty percent, 23.8%, 5.9%, 4.8% and 2.5% have had bone pain crisis, dactylitis, acute chest syndrome, priapism and stroke respectively. The most frequent chronic complications were: leg ulcers (6.5%), avascular necrosis of bone (6.0%), renal (6.3%) and pulmonary hypertension (1.1%). Only 13.2% had been hospitalized while 67.5% had received blood transfusion.

Conclusion: These data on the spectrum of clinical phenotypes of SCD are useful for planning, improving the management of SCD across Nigeria and provide a foundation for genomic research on SCD.

Keywords: Clinical phenotypes; Haemoglobinopathy registry; Nigeria; Sickle cell disease.

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Conflict of interest statement

Declaration of competing interest None.

Figures

Figure 1.
Figure 1.
Map of Nigeria showing the 6 geo-political zones in the country and their constituent states
Figure 2.
Figure 2.
Enrolled populations across the various centers in Nigeria
See this image and copyright information in PMC

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