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.
Highlights
In Democratic Republic of Congo (DRC), the prevalence of sickle cell anemia (SCA) is extremely high with 25 to 30% of sickle cell trait in the general population and SCA affects approximately 1.4% of Correspondence: Michel Ntetani Aloni, Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, PO.BOX 123 Kinshasa XI, the Democratic Republic of Congo.Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-EhunguDivision of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Democratic newborns per year are born with homozygous form in this country
This study was a retrospective and descriptive study carried on all patients with a diagnosis of sickle cell disease (SCD), Conflict of interest: the authors declare no potential conflict of interest
SCA in DRC, our study is the first attempt to describe the acute clinical manifestations in the population suffering from SCA in our Ischemic occlusion Hemorrhage Unknown
Summary
Clinical manifestations and complications of ly Abstract on In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is e estimated to affect 30,000 to 40,000 s neonates per year. There is paucity of data on acute clinical manifestations in u sickle cell children In these circumstances, l it is difficult to develop a health care policy ia for an adequate management of sickle cell patients. L it is difficult to develop a health care policy ia for an adequate management of sickle cell patients This was a seven years’ retrospecrc tive study of children admitted with acute sickle cell crisis in the Department of e Pediatrics in University Hospital of m Kinshasa, Kinshasa, the Democratic.
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