Michel Ntetani Aloni, Bertin Tshimanga, Kadima, Pépé Mfutu Ekulu, Aléine, Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu
Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of
Kinshasa, School of Medicine,
University of Kinshasa, Democratic
Republic of Congo


Abstract

In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity
of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years’ retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of
Kinshasa, Kinshasa, the Democratic Republic of Congo. A total of 108 patients were identified as having SCA. There were 56 (51%) girls and 52 (49%) boys. Median
age was 10.5 years (range 1-24 years). No child was diagnosed by neonatal screening.
The median age of diagnosis of sickle cell anemia was 90 months (range: 8-250 months). The median age at the first transfusion was 36 months (range 4-168). In this
series, 61 (56.5%) patients were eligible for hydroxyurea. However, this treatment was only performed in 4 (6.6%) of them. Pain episodes, acute anemic crisis and severe
infection represent respectively 38.2%, 34.3% and 21.9% of events. Altered sensorium and focal deficit were encountered occasionally and represented 3.4% of acute
events. Acute renal manifestations, cholelithiasis and priapism were rarely reported, in this cohort. In Kinshasa, the care of patients suffering from sickle cell
anemia is characterized by the delayed diagnosis and low detection of organ complications compared to reports of Western countries. This situation is due to resources
deficiencies.

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