Foucan Lydia, Ekouevi Didier, Etienne-Julan Maryse, Salmi L Rachid, Diara Jean-Pierre
Département d'Information Médicale et de Santé Publique, Guadeloupe, French West Indies.
Paediatr Perinat Epidemiol. 2006 Jan;20(1):59-66. doi: 10.1111/j.1365-3016.2006.00692.x.
Dactylitis or hand-foot syndrome is related to intravascular sickling and classically occurs around 6 months of life when haemoglobin S levels reach pathological significance. It has been hypothesised as a possible predictive factor of adverse outcome. The objective of this study was to estimate the survival without severe events (acute chest syndrome, stroke or death) according to early occurrence of dactylitis in the sickle cell paediatric cohort of Guadeloupe. The study period was 1 January 1984 to 31 December 1999. Medical data were recorded from birth for children with sickle cell anaemia diagnosed before 6 months of age. The Kaplan-Meier method was used to estimate the severe event-free survival and to draw related curves. We used proportional-hazard modelling to assess the risk of severe events (stroke or acute chest syndrome or death) in children with early dactylitis (before 6 months of age). The study sample comprised 114 children. The incidence [95% confidence interval] of clinical events per 100 child-years were 10.0 [7.63, 13.07] for dactylitis, 0.73 [0.33, 1.62] for stroke, 10.88 [8.35, 14.17] for acute chest syndrome and 1.06 [0.56, 2.05] for death. Thirteen of the 14 children who had dactylitis before 6 months of age had at least one severe event later in life. The median severe event-free survival time was 2.1 [1.6, 2.7] years in children with dactylitis before 6 months compared with 6.3 [4.5, 8.1] years in the other children; P < 0.0005. After adjustment for haemoglobin level <or=7 g/dL (25th percentile), leucocyte count >19 000/mm3 (75th percentile), transfusion before age of one year, acute splenic sequestration before the age of one year, the adjusted hazard ratio of severe event for children with early dactylitis was 2.13 [1.06, 4.40]; P < 0.03. Dactylitis occurring before 6 months of life identifies children at risk of severe complications who should benefit from close management.
指（趾）炎或手足综合征与血管内镰状化有关，典型情况发生在出生后6个月左右，此时血红蛋白S水平达到病理意义。它被认为是不良结局的一个可能预测因素。本研究的目的是根据瓜德罗普岛镰状细胞病儿科队列中早期发生指（趾）炎的情况，估计无严重事件（急性胸部综合征、中风或死亡）的生存率。研究期间为1984年1月1日至1999年12月31日。对6个月前诊断为镰状细胞贫血的儿童从出生起记录医疗数据。采用Kaplan-Meier方法估计无严重事件生存率并绘制相关曲线。我们使用比例风险模型评估早期指（趾）炎（6个月前）儿童发生严重事件（中风或急性胸部综合征或死亡）的风险。研究样本包括114名儿童。每100儿童年临床事件的发生率[95%置信区间]为：指（趾）炎10.0[7.63，13.07]，中风0.73[0.33，1.62]，急性胸部综合征10.88[8.35，14.17]，死亡1.06[0.56，2.05]。14名在6个月前发生指（趾）炎的儿童中有13名在以后的生活中至少发生了一次严重事件。6个月前发生指（趾）炎的儿童无严重事件生存时间的中位数为2.1[1.6，2.7]年，而其他儿童为6.3[4.5，8.1]年；P<0.0005。在对血红蛋白水平≤7g/dL（第25百分位数）、白细胞计数>19000/mm3（第75百分位数）、1岁前输血、1岁前急性脾滞留进行校正后，早期指（趾）炎儿童发生严重事件的校正风险比为2.13[1.06，4.40]；P<0.03。6个月前发生的指（趾）炎可识别出有严重并发症风险的儿童，这些儿童应受益于密切管理。
