2024/12/31 by Maaloul, I., Aloulou H, S, Ben Ameur +4
#child #complications #enfant #major sickle cell syndromes #manage- ment #prise en charge #syndromes drépanocytaires majeurs
paper · doi:10.71566/pist-rmp-194691
Background: Major sickle cell syndromes is a group of inherited diseases that includes sickle cell anemia, HbSC and Hb SBetathalassemia. The aim of our study was to determine the epidemiological, clinical , hema- tological profiles and outcome of children with major sickle disease syndromes attending the department of pediatrics in a Hospital in south of TUNISIA. Patients and methods: This is a retrospective study of cases of major sickle cell syndromes, including child-ren aged between 5 months and 14 years , who had SS sickle cell anemia ou S ß thalassemia . This stu- dy was conducted in the department of pediatrics , Hedi Chaker Hospital Sfax TUNISIA during the pe- riod between 1 st January 1998 and 30 December 2020. The parameters studied were socio-demo- graphic, epidemiological, clinical, haematological data and outcome. Results : We collected 18 cases (13 boys and 5 girls). It was 13 with SS sickle anemia and 5 with S ßthalas- semia. The average age at diagnosis was 30 mon- ths (6 months-108 months). Thirtheen child were aged less than 5 years. Pallor and vaso-occlusive crises were the main circumstances of discovery in eight and four patients respectively. The average age of children at the first crisis was 41 months [7 months – 72 months]. Clinical signs in the intercritical phase were pallor (18 patients) , jaun- dice (9 patients) and splenomegaly (12 patients). The hemogram shows a meanwhite blood cell count of 16146,1 leucocyte/mm3 (3620-24300). All patients had anemia with an average of 6. 6 g/dL [3,6—10,2]. The hemoglobin S ranged between 29,4 and 98. 2%. During follow up, 9 patients developed vaso-occlu- vive crises with the average of crises /year . Two patients developed brain stroke and four patients had acute chest syndrome.