Department of Women, Child and General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy.
ProMedica Russell J. Ebeid Children's Hospital, Toledo, OH, USA.
Blood Cells Mol Dis. 2021 May;88:102531. doi: 10.1016/j.bcmd.2020.102531. Epub 2021 Jan 2.
Pain is a hallmark of Sickle Cell Disease (SCD) affecting patients throughout their life; the first pain crisis may occur at any age and is often the first presentation of the disease. Universal newborn screening identifies children with SCD at birth, significantly improving morbidity and mortality. Without early screening, diagnosis is generally made after disease manifestations appear. The Consortium for the Advancement of Sickle Cell Research (CASiRe) is an international collaborative group evaluating the clinical severity of subjects with SCD using a validated questionnaire and medical chart review, standardized across 4 countries (United States, United Kingdom, Italy and Ghana). We investigated the age of first pain crisis in 555 sickle cell subjects, 344 adults and 211 children. Median age of the first crisis in the whole group was 4 years old, 5 years old among adults and 2 years old among children. Patients from the United States generally reported the first crisis earlier than Ghanaians. Experiencing the first pain crisis early in life correlated with the genotype and disease severity. Early recognition of the first pain crisis could be useful to guide counseling and management of the disease.
疼痛是镰状细胞病（SCD）的一个标志，影响着患者的一生；首次疼痛危象可能发生在任何年龄，通常是该病的首次表现。新生儿普遍筛查在出生时识别出患有 SCD 的儿童，显著改善了发病率和死亡率。如果没有早期筛查，通常在疾病表现出现后才进行诊断。镰状细胞研究促进联合会（CASiRe）是一个国际合作组织，使用经过验证的问卷和病历回顾，对来自 4 个国家（美国、英国、意大利和加纳）的 SCD 患者的临床严重程度进行评估。我们调查了 555 名镰状细胞患者的首次疼痛危象年龄，其中 344 名成年人和 211 名儿童。整个组中首次危象的中位年龄为 4 岁，成年人中为 5 岁，儿童中为 2 岁。来自美国的患者一般比加纳患者更早报告首次危象。在生命早期经历首次疼痛危象与基因型和疾病严重程度相关。早期识别首次疼痛危象可能有助于指导疾病的咨询和管理。
