Adegoke S A, Adeodu O O, Adekile A D
Department of Pediatrics and Child Health, Obafemi Awolowo University, Ile Ife, Nigeria, .
Niger J Clin Pract. 2015 Jan-Feb;18(1):95-101. doi: 10.4103/1119-3077.146987.
The clinical phenotypes of children with sickle cell disease (SCD) are poorly described in many sub-Saharan countries including Nigeria.
The objective was to highlight various clinical phenotypes of SCD in children and investigate the influence of sociodemographic indices on the development of SCD complications.
We carried out a cross-sectional study of 240 pediatric patients attending the sickle cell clinic and the emergency room in a teaching hospital in South-Western Nigeria over a 12-month period. The clinical phenotypes and severity of the disease were documented, and the influence of sociodemographic variables was investigated.
The five leading clinical phenotypes in our patients were significant pain episodes, that is, vaso-occlusive crisis in 159 (66.3%); anemic crisis in 62 (25.8%); severe bacterial infections, 57 (23.8%); acute chest syndrome (ACS), 27 (11.3%) and stroke, 7 (2.9%). Forty-two (33.1%) had a previous history of dactylitis (hand-foot syndrome). Other clinical phenotypes such as avascular necrosis of the femur, 4 (1.7%); nephropathy, 2 (0.8%); priapism, gallstone and chronic leg ulcer, one (0.4%) each, were not commonly seen. More children with a history of asthma had ACS. Furthermore, high steady-state white blood cell count was associated with severe disease.
The clinical phenotypes of SCD in children from South-Western Nigeria are highly variable with the disease manifesting very early and about 10% having significant complications. Sociodemographic characteristics appear to have little influence on the development of SCD complications among our patients, but age and low-socioeconomic class are associated with anemic crisis.
在包括尼日利亚在内的许多撒哈拉以南国家，镰状细胞病（SCD）患儿的临床表型鲜有描述。
本研究旨在突出SCD患儿的各种临床表型，并调查社会人口统计学指标对SCD并发症发生发展的影响。
我们对尼日利亚西南部一家教学医院镰状细胞门诊和急诊室的240名儿科患者进行了为期12个月的横断面研究。记录疾病的临床表型和严重程度，并调查社会人口统计学变量的影响。
我们患者中五种主要的临床表型为严重疼痛发作，即血管闭塞性危机159例（66.3%）；贫血危机62例（25.8%）；严重细菌感染57例（23.8%）；急性胸综合征（ACS）27例（11.3%）；中风7例（2.9%）。42例（33.1%）有指（趾）炎（手足综合征）病史。其他临床表型，如股骨缺血性坏死4例（1.7%）；肾病2例（0.8%）；阴茎异常勃起、胆结石和慢性腿部溃疡各1例（0.4%），并不常见。有哮喘病史的儿童患ACS的更多。此外，高稳态白细胞计数与疾病严重程度相关。
尼日利亚西南部SCD患儿的临床表型高度可变，疾病很早就会出现，约10%的患儿有严重并发症。社会人口统计学特征似乎对我们患者中SCD并发症的发生发展影响不大，但年龄和低社会经济阶层与贫血危机相关。
