Department of Paediatric, Lagos State University Teaching Hospital, Ikeja.
Mediterr J Hematol Infect Dis. 2013;5(1):e2013001. doi: 10.4084/MJHID.2013.001. Epub 2013 Jan 2.
Sickle cell anaemia is the most common genetic disorder worldwide as well as in Nigeria. Delay in the diagnosis of the condition constitutes an important cause of concern for caretakers of affected children.
To determine the age at diagnosis in a population of children with sickle cell anaemia in Lagos, Nigeria.
The study was conducted between October and December 2009 at the sickle cell clinic of the Department of Paediatrics of Lagos State University Teaching Hospital, Ikeja, Lagos in South west Nigeria. By convenience sampling, a total of 192 children with sickle cell anaemia aged six months to 15 years were interviewed with the aid of a structured questionnaire.
Overall, the mean age at confirmation of haemoglobin genotype was 27.33 months (± 26. 36 months). The mean age at diagnosis was significantly lower among males than females (25.59 ± 27.74 Vs. 29.14 ± 24.85, p = 0.04). A quarter of the children were diagnosed before infancy and three-quarters before three years of age. Upper social stratum and small family size were significantly associated with earlier diagnosis of sickle cell anaemia.
Too few subjects are diagnosed in infancy. Routine screening should ideally be done at birth and neonatal period or at the latest, between six and nine months.
镰状细胞贫血症是全世界以及尼日利亚最常见的遗传性疾病。该疾病的诊断延迟是患儿照顾者关注的重要原因之一。
确定尼日利亚拉各斯患有镰状细胞贫血症的儿童的诊断年龄。
本研究于 2009 年 10 月至 12 月在拉各斯州立大学教学医院儿科镰状细胞诊所进行,通过便利抽样,共对 192 名 6 个月至 15 岁的镰状细胞贫血症患儿进行了访谈,采用结构化问卷。
总体而言,血红蛋白基因型确认的平均年龄为 27.33 个月(±26.36 个月)。男性的诊断年龄明显低于女性(25.59 ± 27.74 对 29.14 ± 24.85,p = 0.04)。四分之一的患儿在婴儿期前被诊断出,四分之三在三岁前被诊断出。较高的社会阶层和较小的家庭规模与镰状细胞贫血症的早期诊断显著相关。
太少的儿童在婴儿期被诊断出来。理想情况下,应在出生和新生儿期进行常规筛查,或者最迟在 6 至 9 个月时进行筛查。