Magnitude of elevated iron stores and risk associated in steady state sickle cell anemia Congolese children: a cross sectional study.

BACKGROUND: The serum ferritin assay is recommended in Sickle Cell Anemia (SCA) patients receiving regular transfusions. According to several authors, elevated iron stores indicating iron chelation corresponds to hyperferritinemia ≥500 ng/ml, and becomes detectable after twenty blood transfusions. The objectives of the study were to determine the prevalence of elevated iron stores and identify associated risk factors in a case series of Steady state SCA Congolese children. MATERIAL AND METHODS: Serum ferritin was assayed in Steady state SCA children followed in 2 specialized hospitals in Kinshasa. Elevated iron stores was defined as serum ferritin level ≥ 500 ng/ml, and the associated risk factors were identified using univariate analysis. RESULTS: Seventy patients (median age 9 years, 56% boys, 53% receiving hydroxyurea) were selected in the study. Serum ferritin levels ranged from 24 to 2584 ng / ml with 21.4% of children having elevated iron stores. Mean levels of LDH, indirect bilirubin, plasma free Hb and CRP were similar between the 2 groups whereas history of polytransfusions (> 3 during the last year) was more frequent among patients with elevated iron stores (73% vs. 44%, p = 0.078). Receiving > 3 transfusions in a year vs. 0 was the main risk factor associated with elevated iron stores [OR 6.17 (95% CI: 1.81-20.96)]. CONCLUSION: In SCA children, hyperferritinemia requiring iron chelation is most strongly related to blood transfusion. This situation concerned almost one in five children in present study; this shows the magnitude of the problem which is underestimated.

Acute Crises and Complications of Sickle Cell Anemia Among Patients Attending a Pediatric Tertiary Unit in Kinshasa, Democratic Republic Of Congo.

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.

Factors Associated with Growth Retardation in Children Suffering from Sickle Cell Anemia: First Report from Central Africa.

Background. The aim of this study was to investigate and determine the risk factors associated with poor growth among SCA children. Methods. A cross-sectional study was conducted in Kinshasa, the capital's country. The nutritional status was assessed using the Z scores of the anthropometric indices. Results. We gathered data on the 256 patients, 138 females (53.9%), who entered the study. The mean age at presentation was 8.4 ± 4.9 years of age. Underweight, stunting, and wasting were found, respectively, in 47.7%, 10.5%, and 50.3% of SCA children. A history of hand-foot syndrome, more than 3 blood transfusions, being less than 12 months of age when receiving the first transfusion, more than two severe sickle crises per year, a medical history of severe infections, and the presence of hepatomegaly were associated with poor growth. When comparing sickle cell patients under 12 years of age (n = 159) to a group of 296 age-matched children with normal Hb-AA, a significantly higher proportion of subjects with stunting and underweight were found among SCA. Conclusion. Nutritional status encountered in Congolese sickle cell children has been described for the first time in this study. A high prevalence of poor growth in SCA children was found in our study.

Glomerular hyperfiltration is strongly correlated with age in Congolese children with sickle cell anaemia.

AIM: Glomerular hyperfiltration is an early marker of sickle cell nephropathy and can lead to microalbuminuria and renal failure. Our aim was to identify the associated risk factors, as these could be of preventative importance. METHODS: We recruited 150 children with sickle cell anaemia (SCA), aged two to 18 years and living in Kinshasa, the Democratic Republic of Congo. Hyperfiltration and microalbuminuria were defined as an estimated glomerular filtration rate of less than 140 mL/min/1.73 m² and an albumin creatinine ratio of between 30 and 299 mg/g, respectively. Independent determinants of hyperfiltration were assessed using logistic regression analysis. RESULTS: Glomerular hyperfiltration was observed in 60 (40%) children, who were significantly older (10.2 ± 4.1 versus 7.9 ± 4.3 years, p = 0.001) and had a lower body mass index level (14.7 ± 2.3 versus 15.0 ± 2.3 kg/m2 ) than the 60% without. A higher proportion had microalbuminuria (25.0 versus 13.3%), but the difference was not statistically significant (p>0.05). Increased age and decreased body mass index were the main independent factors associated with glomerular hyperfiltration in the multivariate analysis. A quarter (25%) of the 60 children with SCA with glomerular hyperfiltration had microalbuminuria. CONCLUSION: Glomerular hyperfiltration was a common finding in this study and was significantly associated with age.

Cherubism in sub-saharan Africa: a first case-report in a child.

Cherubism is rare disease and has been rarely reported in African pediatric population. We report here the case of a 10-year-old child who was referred to our hospital for bilateral jaws swelling. Physical examination revealed bilateral swelling symmetry of the face. Histopathological examination of the biopsy specimen showed loose fibrous stroma, proliferating fibrous connective with tissue interspersed with multinucleated giant cells, small thin walled blood vessels and scattered sparse mononuclear inflammatory infiltrate. Our patient presented cherubism. Cherubism is rarely described in children living in sub-Saharan Africa. Genetic and molecular investigations plays an important role in diagnosis but were not available in poor resources settings in developing countries such as the Democratic Republic of Congo.

Pediatric non-Hodgkin lymphomas: first report from Central Africa.

BACKGROUND: Information on presentation and outcome of pediatric non-Hodgkin's lymphoma is limited from Africa. The demographic characteristics, distribution of different subtypes were noted and compared with published reports from other parts of the world. METHODS: The study was conducted in Kinshasa, the Democratic Republic of Congo between January 2002 and December 2012. RESULTS: A total of 63 cases of pediatric non-Hodgkin's lymphoma were retrospectively analyzed. This cohort represents the largest series of pediatric non-Hodgkin's lymphoma presented from sub-Saharan Africa. Median age was 8.7±3.6 years. There were 43 (68.3%) males. A mean of 82 ± 59 days passed from detection of the first sign to referral to oncology unit. Morphology distribution showed that 42 cases (66.7%) had a diagnosis of Burkitt lymphoma, 16 cases (25.4%) had diffuse large B-cell lymphoma and 5 cases (7.9%) had NHL-not otherwise specified. The majority of patients (82.5%) had advanced stage (stage III and IV). Immunohistochemistry findings were available for 32 biopsy samples. All (100%) cases were B-cell non-Hodgkin's lymphoma and immunohistochemistry had identified 18 (56.3%) cases of Burkitt lymphoma. In our cohort, 22 of 32 cases expressed positive bcl-2 and 12 (37.5%) were found to be positive for bcl-6. Thirty-one (96.7%) cases were positive for high Ki-67 antigen expression. Assuming that cases lost to follow-up worsened and died, the mortality would be 98.4%. CONCLUSION: In comparison to western data, we observed higher proportion of B-cell non-Hodgkin's lymphoma, Burkitt Lymphoma and patients with bcl-2 expression.

The challenges of caring for children with nephrotic syndrome in a tertiary institution in the Democratic Republic of Congo.

AIM: The care of children with resistant nephrotic syndrome (NS) in the Democratic Republic of Congo is compromised by resource deficiencies that range from inadequate healthcare budgets, to scarce laboratory facilities and inconsistent drug supplies. The aim of this study was to describe the clinical profile and management of children with nephrotic syndrome in Kinshasa, the country's capital and its largest city. METHODS: We retrospectively reviewed the medical records of 62 patients with a diagnosis of NS, who were seen in the Paediatric Nephrology Unit at the University Hospital of Kinshasa between January 1983 and January 2008. RESULTS: Of the 62 children diagnosed with nephrotic syndrome, 33 were boys and 29 were girls, giving a male to female ratio of 1.14:1. Their median age at initial presentation was 7.5 years (range: 1.5-13 years) with a peak incidence at six to nine years of age (38.7%). No cases of plasmodium malariae were isolated in our series. Sixteen (25.8%) of the children had resistant corticoid nephrotic syndrome and six of the children (9.7%) died. CONCLUSION: Age, the prevalence of steroid-resistant NS and the mortality rate in our series were higher than those reported in Asian and Western countries.