Diop S, Mokono S O, Ndiaye M, Touré Fall A O, Thiam D, Diakhaté L
Service d'hématologie, centre national de transfusion sanguine, CHU de Dakar, avenue Cheikh-Anta-Diop, BP 5002 Dakar-Fann, Sénégal.
Rev Med Interne. 2003 Nov;24(11):711-5. doi: 10.1016/s0248-8663(03)00220-0.
The progress realized in the treatment of sickle cell anemia has resulted to a better and longer life for these patients in developed countries. Ongoing challenge are to manage complication and social living for these patients. The purpose of this study was to determine morbidity, mortality and socioprofessional insertion of homozygous sickle cell patients, followed up in Dakar university hospital.
We performed a longitudinal and prospective study including 108 homozygous sickle cell anemia patients, whose age were above 20 years, followed in a regular basis (at least 3 times per year), during 5 years on average (3 to 12 years). All patients had a quarterly hematological check-up and a annual statement to detect chronic complications. Clinic and paraclinical data, as information in socioprofessional insertion were noted in medical records for analysis.
Mean age was 27 years (20-51 years). The age between 20-29 years was represented by 67.5%, 26.9% aged 30-39 years and 5.6% were above 40 years of age. Men were slightly predominant with a sex-ratio of 1.25. Concerning morbidity, 96.3% of patients had at least 1 vaso-occlusive crisis per year, 26.9% were transfused and 64.8% had been hospitalized during the follow-up. A chronic complication was found in 49% of patients (53/108). The more frequent of these complications were gall stone (10%), femoral head necrosis (9.2%), priapism (11.6% of men), chronic leg ulceration (4.6%), ophthalmic involvement (3.7%), renal and cardiac complications (2.7% for each one). The presence of complications was not significantly influenced by patient's sex. Five patients died during a mean follow-up of 5 years. Concerning school attendance, 13% reach university level whereas 47% stop their education on the secondary level and 40% on primary level. Professionally, 36.2% of patients had no exercise any activity.
Our results emphasize that life expectancy can be prolonged in sickle cell anemia patients in Africa, when they have a benefit of a regular follow-up. We show also the respective frequency of chronic complications and then, the necessity of multidisciplinary teams to optimize the take care of sickle cell anemia patients in Africa.
在发达国家,镰状细胞贫血治疗取得的进展使这些患者的生活质量更高、寿命更长。目前面临的挑战是管理这些患者的并发症和社会生活。本研究的目的是确定在达喀尔大学医院接受随访的纯合子镰状细胞患者的发病率、死亡率和社会职业融入情况。
我们进行了一项纵向前瞻性研究,纳入108例年龄在20岁以上的纯合子镰状细胞贫血患者,平均随访5年(3至12年),定期(每年至少3次)随访。所有患者每季度进行一次血液学检查,并每年进行一次检查以检测慢性并发症。临床和辅助检查数据以及社会职业融入信息都记录在病历中以供分析。
平均年龄为27岁(20至51岁)。20至29岁年龄段的患者占67.5%,30至39岁年龄段的占26.9%,40岁以上的占5.6%。男性略占主导,性别比为1.25。关于发病率,96.3%的患者每年至少发生1次血管闭塞性危机,26.9%的患者接受过输血,64.8%的患者在随访期间住院。49%的患者(53/108)发现有慢性并发症。这些并发症中最常见的是胆结石(10%)、股骨头坏死(9.2%)、阴茎异常勃起(男性的11.6%)、慢性腿部溃疡(4.6%)、眼部受累(3.7%)、肾脏和心脏并发症(各2.7%)。并发症的发生情况不受患者性别的显著影响。在平均5年的随访期间,有5例患者死亡。关于上学情况,13%的患者达到大学水平,47%的患者在中学阶段辍学,40%的患者在小学阶段辍学。在职业方面,36.2%的患者没有任何工作。
我们的结果强调,非洲的镰状细胞贫血患者如果能定期接受随访,预期寿命可以延长。我们还展示了慢性并发症的各自发生率,进而表明在非洲需要多学科团队来优化对镰状细胞贫血患者的护理。