Diagne I, Diagne-Guèye N R, Fall A L, Déme I, Sylla A, Coly J I, Camara B, Sow H D
Centre hospitalier national d'enfants Albert-Royer, Dakar-Fann, Sénégal.
Arch Pediatr. 2010 Jul;17(7):1017-25. doi: 10.1016/j.arcped.2010.04.015. Epub 2010 May 21.
Splenomegaly is common in sickle cell disease (SCD) and can lead to complications. In order to evaluate its prevalence and progression in Senegalese children ad adolescents with SCD, we analyzed the records of all patients followed-up at Albert-Royer Children's Hospital in Dakar, Senegal, from January 1991 to December 2005. Age, clinical course specifying size of the spleen beyond the costal margin, and disease progression were the main data recorded. We included 698 patients (94.6% SS, 4.4% SC and 1% S-beta-thalassemia). The subjects' mean age when included in the cohort was 6 years and 11 months. Splenomegaly was observed in 122 patients (17.5 %), measuring 1-17 cm beyond the costal margin (mean, 4.7 cm). Splenomegaly was more frequent in SC patients (32.3 %) compared to SS patients (16.5 %, p=0.025). The frequency was greater in infants aged 0-12 months (25.6 %) and increased from the 1- to 5-year-old age group to the 6- to 10-year-old, before it decreased further. The course of splenomegaly was marked by subsequent reduction in 17.3 % of cases, total reversal in 43.4 %, and no size variation in 39.3 %. Acute splenic sequestration occurred in 3 SS cases aged 27, 29, and 32 months and was managed with blood transfusion. Seven patients (6 SS and 1 SC) aged 4-13 years presented hypersplenism. Splenectomy was performed in 4 of these cases, while the complication reversed after 2 to 3 blood transfusions in the 3 remaining cases. The prevalence of splenomegaly is relatively low in Senegalese children with SCD. The risk of complications requires monitoring the spleen and teaching parents palpation of the baby's abdomen for early management of splenic sequestration.
脾肿大在镰状细胞病（SCD）中很常见，且可能导致并发症。为了评估其在塞内加尔患有SCD的儿童和青少年中的患病率及进展情况，我们分析了1991年1月至2005年12月期间在塞内加尔达喀尔的阿尔贝 - 罗耶儿童医院接受随访的所有患者的记录。记录的主要数据包括年龄、明确脾脏超出肋缘大小的临床病程以及疾病进展情况。我们纳入了698例患者（94.6%为SS型，4.4%为SC型，1%为S-β地中海贫血型）。纳入队列时受试者的平均年龄为6岁11个月。122例患者（17.5%）出现脾肿大，脾脏超出肋缘1 - 17厘米（平均4.7厘米）。与SS型患者（16.5%，p = 0.025）相比，SC型患者的脾肿大更为常见（32.3%）。0 - 12个月婴儿的脾肿大发生率更高（25.6%），且从1 - 5岁年龄组到6 - 10岁年龄组有所增加，之后进一步下降。脾肿大病程的特点是，17.3%的病例脾脏大小随后减小，43.4%完全恢复正常，39.3%大小无变化。3例年龄分别为27、29和32个月的SS型患者发生急性脾梗死，通过输血进行治疗。7例年龄在4 - 13岁的患者（6例SS型和1例SC型）出现脾功能亢进。其中4例进行了脾切除术，其余3例在输血2至3次后并发症得到缓解。塞内加尔患有SCD的儿童中脾肿大的患病率相对较低。并发症风险要求对脾脏进行监测，并教导家长触摸婴儿腹部，以便早期处理脾梗死。
