Thiam Lamine, Dramé Assane, Coly Isabelle Zokébé, Diouf François Niokhor, Seck Ndiogou, Boiro Djibril, Ndongo Aliou Abdoulaye, Basse Idrissa, Niang Babacar, Deme/Ly Indou, Sylla Assane, Diagne Ibrahima, Ndiaye Ousmane
Université Assane Seck de Ziguinchor, Hôpital de la Paix de Ziguinchor.
Université Assane Seck de Ziguinchor, Centre Hospitalier Régional de Ziguinchor.
Pan Afr Med J. 2017 Nov 7;28:208. doi: 10.11604/pamj.2017.28.208.14006. eCollection 2017.
Sickle cell disease poses a public health problem in Senegal. It mainly affects children and adolescents. This study aimed to determine the epidemiological, clinical and hematological profiles of homozygous (SS) sickle cell disease in a cohort of children followed-up at the Peace Hospital in Ziguinchor. We conducted a retrospective study of the medical records from children with sickle cell disease. All patients aged between 2 months and 21 years with sickle cell disease SS during the intercritical period, hospitalized during the study period from 1 January 2015 to 31 August 2017 were included in our study. Compound heterozygous patients (SC, S Beta Thalassemia) were not included. We collected 46 medical records of patients with sickle cell disease SS (20 girls and 26 boys). The average age of children was 8,0 years [11 months-21 years]. Approximately 1/3 of children (39.1%) had an age less than or equal to 5 years. There was an ethnic diversity showing a predominance of the Diola (30.2%) followed by the Mandinga (27.9%) and the Poular (25.6%). The average age of children with first crisis was 35,5 months [7-192 months]. More than 1/3 of children (41.3%) had had first crisis before their second anniversary. In the child, first crisis was dominated by vaso-occlusive crisis (32.6%) followed by hand-foot syndrome (30.4%). Clinical signs during the intercritical period were pallor 95.6%), jaundice (36.9%) and splenomegaly (21.7%). Mean white blood cell count was 12465 leucocytes/mm [5340-26900]. Hyperleukocytosis greater than 10 000 leucocytes/mmwas found in 34 patients (73.9%). All patients had anemia with an average hemoglobin of 08,6 g/dl [05,7-11,8]. Hemoglobin S rate ranged between 54.6 and 98.4%. Diagnosis and medical management of sickle cell disease SS are delayed in Ziguinchor. Neonatal screening may lead to improve early management of patients in the region.
镰状细胞病在塞内加尔构成了一个公共卫生问题。它主要影响儿童和青少年。本研究旨在确定在济金绍尔和平医院随访的一组儿童中纯合子（SS）镰状细胞病的流行病学、临床和血液学特征。我们对镰状细胞病患儿的病历进行了回顾性研究。纳入研究的所有患者年龄在2个月至21岁之间，处于镰状细胞病SS的间歇期，在2015年1月1日至2017年8月31日的研究期间住院。复合杂合子患者（SC、Sβ地中海贫血）未纳入。我们收集了46份镰状细胞病SS患者的病历（20名女孩和26名男孩）。儿童的平均年龄为8.0岁[11个月 - 21岁]。约1/3的儿童（39.1%）年龄小于或等于5岁。存在种族多样性，其中迪奥拉族占主导（30.2%），其次是曼丁加族（27.9%）和普拉尔族（25.6%）。首次发病时儿童的平均年龄为35.5个月[7 - 192个月]。超过1/3的儿童（41.3%）在两周岁前首次发病。在儿童中，首次发病以血管闭塞性危机为主（32.6%），其次是手足综合征（30.4%）。间歇期的临床体征有面色苍白（95.6%）、黄疸（36.9%）和脾肿大（21.7%）。平均白细胞计数为12465个白细胞/mm³[5340 - 26900]。34名患者（73.9%）出现白细胞增多症，白细胞计数大于10000个/mm³。所有患者均有贫血，平均血红蛋白为8.6 g/dl[5.7 - 11.8]。血红蛋白S比例在54.6%至98.4%之间。在济金绍尔，镰状细胞病SS的诊断和医疗管理存在延迟。新生儿筛查可能有助于改善该地区患者的早期管理。
