source: American Society of Hematology

year: 2017

authors: Saliou Diop, Dapa Diallo, Aissata Tolo, Guillaume Wamba, Leon Tshilolo, Simon Ategbo, Ibrahima Diagne, Ibrahima Sanogo, Françoise NGO Sack, Xavier Jouven, Brigitte Ranque

• Each year, more than 230,000 children with sickle cell disease (SCD) are born in sub-Saharan Africa, compared to 2,600 in North America and 1,300 in Europe. Yet, the natural history of SCD in sub-Saharan Africa remains largely unknown.
• SCD associates episodes of acute illness (acute vaso-occlusive events) and progressive multi-organ damages, which encompass many macro- or microvascular events, such as stroke, heart failure, pulmonary hypertension, kidney disease, bone infarcts, leg ulcers, priapism etc.
• The high inter-individual variability of SCD clinical manifestations is notorious, including in patients with similar genotypes. The reasons of this variability are poorly understood, although clearly both genetic and environmental factors are involved.
• Although the sub Saharan African population is the most afflicted by SCD in the world, most clinical studies concerning SCD chronic complications involve patients from developed countries, where the socio-economic, environmental and medical contexts are dramatically different.

Patients and methods: -Recruitment in hematology and pediatric centers of the capital cities of Cameroon, Mali, Senegal, Ivory Coast and Gabon (replaced by Democratic Republic of Congo in 2016). -Inclusion criteria SCD patients >= 3 years-old (SCD confirmed by hemoglobin electrophoresis and HPLC) investigated at a steady state (no recent vaso-occlusive crisis, infection or transfusion) + age and country matched controls (1 control for 4 patients). -Monitoring of clinical, biological and functional data every five years.

Studied complications: – clinically defined : priapism, leg ulcers, stroke, osteonecrosis (confirmed by radio), retinopathy – biologically defined : nephropathy (serum creatinin: eGFR, urine albumin/créatinine ratio) – echocardiography : tricuspid regurgitation jet velocity >2,5m/s (screening for pulmonary hypertension)

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