Acute Crises and Complications of Sickle Cell Anemia among Patients Attending a Pediatric Tertiary Unit in 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.

Improvement of Sickle Cell Disease Morbi-Mortality in Children: Experience Gained in a Remote Area of an African Country

“…This initial diagnosis was mainly made based on clinical features only, especially the presence of VOC or anemia. These results are consistent with those of other authors who reported an average age of around 10 years in SCD patients and a first diagnosis at the age of 2 years or later [45,[47][48][49][50][51]. VOC being also reported as the most frequent mode of first diagnosis [44,47,50,52].…”

“…The results of the first year of follow-up showed that sickle cell children without any specific medical intervention presented severe clinical and hematological pictures with an annual average of 4 VOCs, 4 infectious episodes, 1 acute thoracic syndrome, 4 hospitalizations demonstrating the severity of the disease in a remote area of a developing country. Annual averages of acute events and degradation of biological parameters similar to the results of this study among SCD patients who have not been followed-up in the past were reported in the literature [47,50,[53][54][55]; however, those studies did not report the impact of a regular follow-up.…”

“…a reduction in episodes of VOC, infectious episodes, blood transfusions, acute thoracic syndromes, hospitalizations and, an increase in the hemoglobin level from 50 to 70 g/L. The steady-state hemoglobin level of 50 g/L before regular follow-up is lower than that reported previously in large cities of the DRC or other African countries (70 g/L) [45,47,50,56]. It could be explained by the lack of optimal management of the disease in the past but also by other factors that should be explored.…”

Priapism in Children: Review

“…Bu hastalarda sekiz yıllık izlemler sonunda tam iyileşme gözlenmiştir. 9 Farmakolojik ajanlara bağlı priyapizm çocuk yaş grubunda genellikle ilaçların aşırı doz uygulanmaları sonucu gelişmektedir. PDE 5 inhibitör-leri pulmoner hipertansiyon tedavisinde kullanılan ilaçlar olup, bu ilaçlar çocuklarda %1 oranında tedavi dozlarında priyapizme yol açabilmektedir.…”

“…9 Acil olarak RDU yapılmalıdır. Eğer iskemik priyapizm dışlanamaz ise korporal aspirasyon ve ketamin anestezisi uygulanabilmektedir.…”