Profils épidemiologiques, cliniques et hématologiques de la drépanocytose homozygote SS en phase inter critique chez l’enfant à Ziguinchor, Sénégal

La drepanocytose pose un probleme de sante publique au Senegal. Elle concerne principalement les enfants et les adolescents. L'objectif de notre travail etait de determiner les profils epidemiologiques, cliniques et hematologiques de la drepanocytose homozygote SS dans une cohorte d'enfants suivis a l'hopital de la paix de Ziguinchor. Il s'agit d'une etude retrospective portant sur des dossiers d'enfants drepanocytaires. Etaient inclus, les drepanocytaires SS âges entre 2mois et 21ans, recus en phase inter critique durant la periode d'etude allant du 1 er Janvier 2015 au 31 Aout 2017. N'etaient pas inclus dans l'etude les heterozygotes composites (SC, S beta thalassemie). Nous avons collige 46 dossiers de drepanocytaires SS (20 filles et 26 garcons). L'âge moyen des enfants etait de 8,0ans [11mois-21ans]. Environ 1/3 des enfants (39,1%) avaient un âge inferieur ou egal a 5 ans. Il y avait une diversite ethnique avec une predominance de diola (30,2%) suivi de mandingue (27,9%) et de poular (25,6%). L'âge moyen des enfants a la 1ere crise etait de 35,5mois [7-192 mois]. Plus de 1/3 des enfants (41,3%) avaient fait une 1ere crise avant le deuxieme anniversaire. Le type de la 1ere crise, qu'avait presente l'enfant, etait domine par la crise vaso-occlusive (32,6%) suivi du syndrome pied-main (30,4%). Les signes cliniques en phase inter critique etaient la pâleur 95,6%); l'ictere (36,9%) et la splenomegalie (21,7%). A l'hemogramme, le nombre de globules blancs moyen etait de 12465 leucocytes/mm 3 [5340-26900]. L'hyperleucocytose, superieure a 10 000 leucocytes/mm 3 etait retrouvee chez 34 malades (73,9%). La totalite des malades presentait une anemie avec une moyenne 08,6 g/dl [05,7-11,8]. Le taux d'hemoglobine S variait entre 54,6 et 98,4%. Le diagnostic et la prise en charge medicale de la drepanocytose SS sont tardifs a Ziguinchor. Le depistage neonatal pourrait favoriser une prise en charge precoce dans la region. Mots cles: Drepanocytose SS, phase inter critique, enfant English Title:  Epidemiological, clinical and hematological profiles of homozygous sickle cell disease during the intercritical period among children in Ziguinchor, Senegal English Abstract 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 st 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 3 [5340-26900]. Hyperleukocytosis greater than 10 000 leucocytes/mm 3 was 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. Keywords: Sickle cell diasease SS, intercritical period, child