article · Hematology Reports
Sickle cell anaemia affects an estimated 30,000 to 40,000 newborns each year in the Democratic Republic of Congo, but clinical evidence regarding acute crises remains limited. A seven-year retrospective review of 108 pediatric patients at the University Hospital of Kinshasa revealed significant delays in disease detection. None of the children were diagnosed through neonatal screening, and the median age at diagnosis was 90 months. Pain episodes accounted for 38.2 percent of acute events, followed by acute anemic crises at 34.3 percent and severe infections at 21.9 percent. In addition, the median age at first blood transfusion was 36 months. Although more than half of the cohort met the criteria for hydroxyurea treatment, only 6.6 percent of eligible patients received it. The findings highlight how resource constraints lead to delayed diagnosis and under-detected complications.
Sickle cell anaemia is a widespread, life-threatening condition in Central Africa, yet healthcare systems often lack baseline data. Demonstrating late diagnosis and low treatment uptake provides critical baseline evidence. This helps public health authorities and medical providers recognise the urgent need for structured neonatal screening programmes and reliable access to essential therapies like hydroxyurea.
The abstract does not indicate an immediate commercial application pathway, as it focuses on clinical epidemiology and healthcare delivery challenges. However, the identified gaps could inform healthcare planners and providers seeking to design targeted screening programmes, improve diagnostic capability, and expand pharmaceutical distribution pipelines for sickle cell therapies in low-resource tertiary hospitals.
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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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DOI: 10.4081/hr.2017.6952
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