Mary Nnagha Ekene, Kayode Ademola Matthew, Ann Izevbizua Eghosa, Uwishema Olivier, Nazir Abubakar, Wellington Jack
Oli Health Magazine Organization, Research and Education, Kigali, Rwanda.
College of Medical Sciences, University of Calabar, Calabar.
Ann Med Surg (Lond). 2023 Apr 18;85(5):2282-2286. doi: 10.1097/MS9.0000000000000696. eCollection 2023 May.
Sickle cell disease (SCD) is a group of inherited haematological disorders, which affects the shape of the oxygen-carrying haemoglobin component of erythrocytes, giving it an abnormal sickle-shaped appearance. This disease is one of the most common haematological disorders in Nigeria and is generally characterized by anaemia, painful crises, and multi-organ dysfunction. Recurrent episodes of painful crises account for most of the morbidities and mortalities observed in SCD, particularly sickle cell anaemia. This has been a critical concern in the field of haematology and molecular genetics as several therapeutic solutions have been explored over the past few years to treat symptoms of this disease and alleviate painful crises. However, most of these treatment options are not readily available and affordable to affected patients in lower socioeconomic settings in Nigeria, causing a wider range of complications and end-stage organ failure. To address this issue, this article explores an overview of SCD, management alternatives and the need for newer therapeutic solutions to cover the gaps or inadequacies of effective sickle cell crisis management.
镰状细胞病(SCD)是一组遗传性血液疾病,它会影响红细胞中携带氧气的血红蛋白成分的形状,使其呈现出异常的镰刀状外观。这种疾病是尼日利亚最常见的血液疾病之一,其一般特征为贫血、疼痛性危象和多器官功能障碍。疼痛性危象的反复发作是镰状细胞病(尤其是镰状细胞贫血)中观察到的大多数发病和死亡的原因。这一直是血液学和分子遗传学领域的一个关键问题,因为在过去几年里人们探索了多种治疗方案来治疗这种疾病的症状并缓解疼痛性危象。然而,尼日利亚社会经济地位较低地区的患病患者大多无法轻易获得且负担不起这些治疗方案,从而导致了更广泛的并发症和终末期器官衰竭。为了解决这个问题,本文探讨了镰状细胞病的概述、管理方案以及采用更新的治疗方案来弥补有效镰状细胞危象管理方面的差距或不足的必要性。