Aloni Michel Ntetani, Kadima Bertin Tshimanga, Ekulu Pépé Mfutu, Budiongo Aléine Nzazi, Ngiyulu René Makuala, Gini-Ehungu Jean Lambert
Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Democratic Republic of Congo.
Hematol Rep. 2017 Jun 1;9(2):6952. doi: 10.4081/hr.2017.6952.
In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years' retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo. A total of 108 patients were identified as having SCA. There were 56 (51%) girls and 52 (49%) boys. Median age was 10.5 years (range 1-24 years). No child was diagnosed by neonatal screening. The median age of diagnosis of sickle cell anemia was 90 months (range: 8-250 months). The median age at the first transfusion was 36 months (range 4-168). In this series, 61 (56.5%) patients were eligible for hydroxyurea. However, this treatment was only performed in 4 (6.6%) of them. Pain episodes, acute anemic crisis and severe infection represent respectively 38.2%, 34.3% and 21.9% of events. Altered sensorium and focal deficit were encountered occasionally and represented 3.4% of acute events. Acute renal manifestations, cholelithiasis and priapism were rarely reported, in this cohort. In Kinshasa, the care of patients suffering from sickle cell anemia is characterized by the delayed diagnosis and low detection of organ complications compared to reports of Western countries. This situation is due to resources deficiencies.
在刚果民主共和国，据估计镰状细胞贫血（SCA）的发病率每年影响30,000至40,000名新生儿。然而，关于镰状细胞病患儿急性临床表现的数据匮乏。在这种情况下，很难制定出一项对镰状细胞病患者进行适当管理的医疗保健政策。这是一项对刚果民主共和国金沙萨大学医院儿科收治的急性镰状细胞危象患儿进行的为期七年的回顾性研究。总共确定了108例患有SCA的患者。其中有56名（51%）女孩和52名（49%）男孩。中位年龄为10.5岁（范围1 - 24岁）。没有儿童通过新生儿筛查被诊断出。镰状细胞贫血的中位诊断年龄为90个月（范围：8 - 250个月）。首次输血的中位年龄为36个月（范围4 - 168个月）。在这个系列中，61名（56.5%）患者符合使用羟基脲的条件。然而，其中只有4名（6.6%）患者接受了这种治疗。疼痛发作、急性贫血危象和严重感染分别占病例的38.2%、34.3%和21.9%。偶尔会出现意识改变和局灶性神经功能缺损，占急性病例的3.4%。在这个队列中，急性肾脏表现、胆结石和阴茎异常勃起很少被报告。在金沙萨，与西方国家的报告相比，镰状细胞贫血患者的护理特点是诊断延迟且器官并发症的检出率低。这种情况是由于资源短缺所致。
