Ayéroué J, Kafando E, Kam L, Gué E, Vertongen F, Ferster A, Cotton F, Gulbis B
Service de pédiatrie, CHU Yalgado Ouédraogo, Ouagadougou, Burkina Faso.
Arch Pediatr. 2009 Apr;16(4):316-21. doi: 10.1016/j.arcped.2009.01.005. Epub 2009 Mar 3.
To evaluate the clinical features of children with hemoglobin sickle cell disease (HbSC) and compare them to children with sickle cell anemia (HbSS).
This was a descriptive and retrospective study. New patients with sickle cell disease who consulted at the Yalgado Ouédraogo University Hospital's Pediatric Center in Ouagadougou, Burkina Faso, between May 2005 and June 2006, were included. They were free of any major disease unrelated to sickle cell disease. Clinical and laboratory results reported for these children were based on their health book and medical records.
Sixty-one children were included in the study, 38 and 23 children were positive for HbSC and HbSS, respectively; there was no significant difference between the 2 groups in terms of sex ratio or mean age at inclusion. Mean age at diagnosis was 5 years and 2 years for HbSC and HbSS children, respectively. The first clinical event appeared at a significantly later age for HbSC than HbSS children (4 years versus 2 years). Painful episodes were equivalent in mean number per year and mean length per episode between the 2 groups; the median hemoglobin (Hb) level at inclusion was significantly higher for HbSC than for HbSS children, i.e., 95 g/l versus 70 g/l.
At the Yalgado Ouédraogo University Hospital Pediatric Center, children with HbSC disease presented clinical and biological features very similar to those with HbSS.
评估血红蛋白镰状细胞病(HbSC)患儿的临床特征,并与镰状细胞贫血(HbSS)患儿进行比较。
这是一项描述性回顾性研究。纳入2005年5月至2006年6月期间在布基纳法索瓦加杜古的亚尔加杜·韦德拉奥果大学医院儿科中心就诊的镰状细胞病新患者。他们无任何与镰状细胞病无关的重大疾病。这些儿童报告的临床和实验室结果基于他们的健康手册和病历。
61名儿童纳入研究,其中38名和23名儿童分别HbSC和HbSS检测呈阳性;两组在性别比例或纳入时的平均年龄方面无显著差异。HbSC和HbSS患儿的诊断平均年龄分别为5岁和2岁。HbSC患儿首次临床事件出现的年龄明显晚于HbSS患儿(4岁对2岁)。两组每年疼痛发作的平均次数和每次发作的平均时长相当;纳入时HbSC患儿的血红蛋白(Hb)中位数水平显著高于HbSS患儿,即95 g/l对70 g/l。
在亚尔加杜·韦德拉奥果大学医院儿科中心,HbSC病患儿的临床和生物学特征与HbSS患儿非常相似。