Study of Prognostic Factors of Death in Children with Sickle Cell Diseases Followed at the Albert Royer National Children's Hospital Center, Dakar, Senegal

• DOI: 10.11648/j.ajp.20200601.11
• Publication Date: 2020-07-13T08:19:26Z
• Authors: Boubacar Gueye; Cheikh Tacko Diop; Ndéye Marième Diagne; Aminata Paye; Ibrahima Diagne
• Keywords: 03 medical and health sciences; 0302 clinical medicine; 3. Good health

Objectives. The objectives of our study are: i) estimate the survival time of cases of sickle cell diseases monitored at the Albert Royer National Children's Hospital Center, Dakar, Senegal from 1990 to 2010, ii) identify prognostic factors related to deaths. Methods. Comprehensive and retrospective survival analysis of the prognostic factors of death of the 1650 patients with sickle cell disease followed in the sickle cell management unit of Albert Royer National Children's Hospital Center from January 1st, 1991 to December 31, 2010 (20 years). 17 variables were studied: gender, ethnicity, geographical origin, age of the patient at onset of the disease, age of the patient at onset of follow-up, number of vaso-occlusive crisis in the last year of follow-up duration of which is ≥72h, type of hemoglobinopathy, baseline hemoglobin level, fetal hemoglobin level at time of diagnosis, vaccination with at least one antigen, regular folic acid intake, regular penicillin intake, hydroxyurea therapy, acute complications (severe infections, severe anemia and serious vaso-occlusive accidents) in the last year of follow-up and chronic complications. The Cox model was used. Results. We conducted 1650 observations with 44 deaths, and a lethality of 2.6%. The death incidence rate is 3.51 deaths per 100 person-years. The Cox model highlighted the prognostic factors which significantly explain the model (p < 0.05). Vaccination with at least one antigen and the existence of chronic complications improved patient survival. However, the number of vaso-occlusive crisis in the last year of follow-up (duration is ≥72h), the existence of serious vaso-occlusive accidents in the last year of follow-up, the very low baseline hemoglobin level (≤5mg/100 mn), the early age of late follow-up (≥15 months) reduced patients survival. Conclusions. The prognosis of patients with sickle cell disease followed at the Albert Royer National Children's Hospital Center (ARNCHC) is difficult to establish. In fact, in addition to genetic, clinical and evolutionary factors, there are poorly understood environmental and socio-economic factors that affect survival. A prospective study would shed more light on the prognostic factors of death in children with sickle cell disease.