Malaria, clinical features and acute crisis in children suffering from sickle cell disease in resource-limited settings: a retrospective description of 90 cases.

BACKGROUND: The prevalence of sickle cell disease (SCD) is extremely high in the Democratic Republic of Congo (DRC). Despite the high prevalence of this disease in our midst, there has been no report on the clinical features in the sickle cell pediatric population suffering from malaria in our midst.
METHODS: A retrospective survey of records from the Department of Paediatrics of the University Hospital of Kinshasa, Kinshasa, Democratic Republic of Congo, was done for the period 1998–2008. For the 10 years studied, 108 children with SCD were reviewed and the data of those who developed malaria during admission were retrieved and analyzed.
RESULTS: Of the 90 homozygous sickle cell children with malaria, fever, pallor, and jaundice were the commonly-found symptoms. Lethargy, severe anemia, respiratory distress, splenomegaly, hepatomegaly,digestive disorders, and prostration were common in children under 5 years, with significant difference (P, 0.05) to the older children. Transfusion because of to severe anemia was found necessary in 54.4% of cases. No case of cerebral malaria was found. Blackwater fever was a rare event. Hand–foot syndrome was present in 12.8% of patients, exclusively in those less than 5 years old. Pain crisis was associated in 46 cases (51.1%). Pain crisis was particularly present in SCD children less than 5 years of age (74.5% vs 25.6%, P , 0.001). No death was observed in this series.
CONCLUSION: We conclude that the acute crisis related to SCD is more common in children less than 5 years of age. High risk of a requirement for blood transfusion was found in young children. Anti-malarial prophylaxis is advocated in Kinshasa.