Kpadonou G T, Fiossi-Kpadonou E, Alagnidé E, Bagre F, Rahimy M C
Service de rééducation et Réadaptation Fonctionnelle (SRRF) du CNHU de Cotonou, Bénin.
Med Trop (Mars). 2011 Dec;71(6):558-61.
Manifestations of sickle cell disease (SCD) are polymorphous from childhood to adulthood.
The purpose of this study was to assess outcomes of rehabilitation in the patients with SCD.
This retrospective analytical descriptive study included 160 SCD patients rehabilitated from 1998 to 2006 at the National University Hospital in Cotonou, Benin.
Mean patient was 31 years (range, 3 to 73 years). There was a female predominance (62.5%) with a sex ratio of 0.6. The type of SCD was HbAS in 53.1% of patients, HbSS in 21.3%, HbSC in 16.9%, and HbAC in 8.1%. The main clinical manifestations were classified as ischemic/infectious in 40% of patients, rheumatologic in 40%, and neurologic in 10%. Ischemic manifestations were observed in all patients with HbSS, HbSC, and HbAS while manifestations were preferentially neurological in patients with HbSS (p = 4,43.10(-3)) and rheumatologic in patients with HbAS (p<10(-3)). At the end of rehabilitation, persistent deficiencies, disabilities and limitations involved pain in 43.8% of patients, articular stiffness in 43.8%, muscular weakness in 46.9%, gait anomalies in 33.1%, amyotrophy in 21.2%, shortening of lower extremity in 16.9%, and tilting of the pelvis in 6.3%.
This study shows that SCD is a highly debilitating disease. Although rheumatologic manifestations are not specific to SCD, the other complications described including femoral head necrosis, osteomyelitis, and stroke have been extensively documented in the literature. If primary prevention is unfeasible, early multidisciplinary management appears to be the most effective approach to reducing SCD-related disability.
镰状细胞病（SCD）从儿童期到成年期的表现具有多态性。
本研究旨在评估SCD患者的康复结局。
这项回顾性分析描述性研究纳入了1998年至2006年在贝宁科托努国立大学医院接受康复治疗的160例SCD患者。
患者的平均年龄为31岁（范围为3至73岁）。女性占优势（62.5%），性别比为0.6。SCD类型中，53.1%的患者为HbAS，21.3%为HbSS，16.9%为HbSC，8.1%为HbAC。主要临床表现分类为：40%的患者为缺血性/感染性，40%为风湿性，10%为神经性。在所有HbSS、HbSC和HbAS患者中均观察到缺血性表现，而HbSS患者的表现以神经症状为主（p = 4.43×10⁻³），HbAS患者的表现以风湿症状为主（p<10⁻³）。康复结束时，持续存在的缺陷、残疾和功能受限包括：43.8%的患者疼痛，43.8%的患者关节僵硬，46.9%的患者肌肉无力，33.1%的患者步态异常，21.2%的患者肌萎缩，16.9%的患者下肢缩短，6.3%的患者骨盆倾斜。
本研究表明SCD是一种导致严重功能障碍的疾病。尽管风湿性表现并非SCD所特有，但文献中已广泛记载了包括股骨头坏死、骨髓炎和中风在内的其他并发症。如果一级预防不可行，早期多学科管理似乎是减少SCD相关残疾的最有效方法。
