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Acute crises and complications of sickle cell anemia among patients attending a pediatric tertiary unit in Kinshasa, Democratic Republic of Congo

Michel Ntetani Aloni, Bertin Tshimanga Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu
  • Michel Ntetani Aloni
    Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Congo, the Democratic Republic of the | michelaloni2003@yahoo.fr
  • Bertin Tshimanga Kadima
    Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Congo, the Democratic Republic of the
  • Pépé Mfutu Ekulu
    Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Congo, the Democratic Republic of the
  • Aléine Nzazi Budiongo
    Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Congo, the Democratic Republic of the
  • René Makuala Ngiyulu
    Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Congo, the Democratic Republic of the
  • Jean Lambert Gini-Ehungu
    Division of Pediatric Hemato-Oncology and Nephrology, Department of Pediatrics, University Hospital of Kinshasa, School of Medicine, University of Kinshasa, Congo, the Democratic Republic of the

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.

Keywords

Children; acute events: sickle cell anemia; Kinshasa; Democratic Republic of Congo

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Submitted: 2016-10-29 00:38:40
Published: 2017-06-01 10:11:34
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Copyright (c) 2017 Michel Ntetani Aloni, Bertin Tshimanga Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu

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