[Epidemiological, clinical and hematological profiles of homozygous sickle cell disease during the intercritical period among children in Ziguinchor, Senegal].

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

Publisher: African Field Epidemiology Network Country of Publication: Uganda NLM ID: 101517926 Publication Model: eCollection Cited Medium: Internet ISSN: 1937-8688 (Electronic) NLM ISO Abbreviation: Pan Afr Med J Subsets: MEDLINE

Original Publication: Kampala, Uganda : African Field Epidemiology Network

Anemia, Sickle Cell/*epidemiology ; Hand-Foot Syndrome/*epidemiology ; Hemoglobin, Sickle/*analysis ; Vascular Diseases/*epidemiology; Adolescent ; Anemia, Sickle Cell/physiopathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Jaundice/epidemiology ; Jaundice/etiology ; Leukocyte Count ; Male ; Retrospective Studies ; Senegal/epidemiology ; Splenomegaly/epidemiology ; Splenomegaly/etiology ; Vascular Diseases/etiology ; Young Adult

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.

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Keywords: Sickle cell diasease SS; child; intercritical period

0 (Hemoglobin, Sickle)

Date Created: 20180404 Date Completed: 20180501 Latest Revision: 20181114

20221213

PMC5878839

10.11604/pamj.2017.28.208.14006

29610646