Sickle cell disease clinical phenotypes in Nigeria: A preliminary analysis of the Sickle Pan Africa Research Consortium Nigeria database
| Autor: | Hezekiah Isa, Samuel Adegoke, Anazoeze Madu, Abdul-Aziz Hassan, Chinatu Ohiaeri, Reuben Chianumba, Biobele Brown, Emmanuel Okocha, Ngozi Ugwu, Ijeoma Diaku-Akinwumi, Titilope Adeyemo, Aisha Kuliya-Gwarzo, Livingstone Dogara, Haliru Lawal, Yohanna Tanko, Adama Ladu, Umar Kangiwa, Lilian Ekwem, Seyi Oniyangi, Tambi Wakama, Domic Umoru, Olaniyi Olanrewaju, Norah Akinola, Uche Nnebe-Agumadu, Samuel Asala, Adekunle Adekile, John Olaniyi, Raphael Sangeda, null Sickle Africa Data Coordinating Center (SADaCC), Obiageli Nnodu |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
Adult
Male 0301 basic medicine Blood transfusion Adolescent medicine.medical_treatment Thalassemia Nigeria Pain Anemia Sickle Cell Disease computer.software_genre Article Dactylitis Young Adult 03 medical and health sciences 0302 clinical medicine Acute Chest Syndrome medicine Humans Child Bone pain Molecular Biology Stroke Database biology business.industry Leg Ulcer Anemia Cell Biology Hematology medicine.disease biology.organism_classification Acute chest syndrome 030104 developmental biology Tanzania Molecular Medicine Female medicine.symptom business computer 030215 immunology |
| Zdroj: | Blood Cells Mol Dis |
| ISSN: | 1079-9796 |
| DOI: | 10.1016/j.bcmd.2020.102438 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect