Paintsil Vivian, Amuzu Evans Xorse, Nyanor Isaac, Asafo-Adjei Emmanuel, Mohammed Abdul Razak, Yawnumah Suraj Abubakar, Oppong-Mensah Yaa Gyamfua, Nguah Samuel Blay, Obeng Paul, Dogbe Elliot Eli, Jonas Mario, Nembaware Victoria, Mazandu Gaston, Ohene-Frempong Kwaku, Wonkam Ambroise, Makani Julie, Ansong Daniel, Osei-Akoto Alex
Directorate of Child Health-Komfo Anokye Teaching Hospital, Kumasi, Ghana.
Department of Child Health-Kwame Nkrumah University of Science and Technology, School of Medicine and Dentistry, Kumasi, Ghana.
Front Genet. 2022 Feb 24;13:802355. doi: 10.3389/fgene.2022.802355. eCollection 2022.
Sickle cell disease (SCD) is the most common clinically significant hemoglobinopathy, characterized by painful episodes, anemia, high risk of infection, and other acute and chronic complications. In Africa, where the disease is most prevalent, large longitudinal data on patients and their outcomes are lacking. This article describes the experiences of the Kumasi Center for SCD at the Komfo Anokye Teaching Hospital (KCSCD-KATH), a Sickle Pan-African Research Consortium (SPARCO) site and a SickleInAfrica Consortium member, in establishing a SCD registry for the evaluation of the outcomes of patients. It also provides a report of a preliminary analysis of the data. The process of developing the registry database involved comprehensive review of the center's SCD patient medical records, incorporating data elements developed by the SickleInAfrica Consortium and obtaining ethical clearance from the local Institutional Review Board. From December 2017 to March 2020, 3,148 SCD patients were enrolled into the SCD registry. Enrollment was during the SCD outpatient clinic visits or through home visits. A significant proportion of the patients was from the newborn screening cohort (50.3%) and was males (52.9%). SCD-SS, SCD-SC, and Sβ thalassemia were seen in 67.2, 32.5, and 0.3% patients, respectively. The majority of the patients were in a steady state at enrollment; however, some were enrolled after discharge for an acute illness admission. The top two clinical diagnoses for SCD-SS patients were sickle cell painful events and acute anemia secondary to hyperhemolysis with incidence rates of 141.86 per 10,000 person months of observation (PMO) and 32.74 per 10,000 PMO, respectively. In SCD-SC patients, the top two diagnoses were sickle cell painful events and avascular necrosis with incidence rates of 203.09 per 10,000 PMO and 21.19 per 10,000 PMO, respectively. The SPARCO Kumasi site has developed skills and infrastructure to design, manage, and analyze data in the SCD registry. The newborn screening program and alternative recruitment methods such as radio announcement and home visits for defaulting patients were the key steps taken in enrolling patients into the registry. The registry will provide longitudinal data that will help improve knowledge of SCD in Ghana and Africa through research.
镰状细胞病（SCD）是临床上最常见的具有重要意义的血红蛋白病，其特征为疼痛发作、贫血、感染风险高以及其他急慢性并发症。在该病最为流行的非洲，缺乏关于患者及其预后的大型纵向数据。本文描述了Komfo Anokye教学医院库马西镰状细胞病中心（KCSCD-KATH）的经验，该中心是镰状细胞泛非研究联盟（SPARCO）的一个站点以及非洲镰状细胞病联盟的成员，其建立了一个用于评估患者预后的SCD登记系统。本文还提供了一份数据初步分析报告。开发登记数据库的过程包括对该中心SCD患者病历进行全面审查，纳入非洲镰状细胞病联盟制定的数据元素，并获得当地机构审查委员会的伦理批准。从2017年12月到2020年3月，3148名SCD患者被纳入SCD登记系统。纳入工作在SCD门诊就诊期间或通过家访进行。相当一部分患者来自新生儿筛查队列（50.3%），且为男性（52.9%）。分别有67.2%、32.5%和0.3%的患者患有SCD-SS、SCD-SC和Sβ地中海贫血。大多数患者在纳入时处于稳定状态；然而，一些患者是在因急性疾病入院出院后被纳入的。SCD-SS患者的前两大临床诊断为镰状细胞疼痛事件和高溶血继发的急性贫血，发病率分别为每10000人月观察期（PMO）141.86例和每10000 PMO 32.74例。在SCD-SC患者中，前两大诊断为镰状细胞疼痛事件和无血管性坏死，发病率分别为每10000 PMO 203.09例和每10000 PMO 21.19例。SPARCO库马西站点已具备设计、管理和分析SCD登记系统数据的技能和基础设施。新生儿筛查项目以及针对未就诊患者的广播通知和家访等替代招募方法是将患者纳入登记系统的关键步骤。该登记系统将提供纵向数据，通过研究有助于提高加纳和非洲对SCD的认识。
