Prevalence and determinants of microalbuminuria in children suffering from sickle cell anemia in steady state.

BACKGROUND: Sickle cell anemia (SCA) is considered a major risk factor for renal complications. The main goal of this study was to determine the frequency of macroalbuminuria and microalbuminuria in Congolese children <18 years of age suffering from Sickle cell anemia and to identify associated factors. METHODS: The cross-sectional study was completed in 150 hemoglobin-SS children (77 boys and 73 girls). Microalbuminuria was defined by a urine albumin:creatinine ratio of 30-299 mg/g. RESULTS: The mean age of this group was 8.8 ± 4.3 years (range 2-18). Microalbuminuria was found in 27 children (18%). In multivariate logistic regression, only age emerged as a determinant of microalbuminuria odds ratio 1.11 (95% confidence interval 1.00-1.22); P = 0.042]. CONCLUSIONS: In our series, only age was a major determinant of the occurrence of microalbuminuria. These results confirm the need for early screening of microalbuminuria in Congolese children suffering from Sickle cell anemia in a context where access to renal and bone marrow transplant is nonexistent.

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.

Validity of simple clinical and biological parameters as screening tool for sickle cell anemia for referral to tertiary center in highly resource constraints.

BACKGROUND: In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated around 40 000 neonates per year. However, it is notoriously difficult to perform conventional electrophoresis in all hospitals and laboratories, especially at peripheral levels and rural area. A panel of multiple clinical and laboratory features that would enhance sickle cell disease were assessed for the detection of the disease in highly resource-scarce settings. METHODS: A prospective study was conducted in Kinshasa. Venous blood samples were drawn from each study participant in order to determine the hematologic parameters, the peripheral smears, and the hemoglobin electrophoresis. We used Cohen's κ statistic to examine the agreement of each variable and diagnosis of sickle cell disease. RESULTS: A total of 807 patients were screened for sickle cell disease. Among these 807 children, 36 (4.5%) were homozygous for Hb S disease. The presence of at least 8% erythroblasts (PPV: 91%, NPV: 99%, sensitivity: 83.3%, specificity: 99.6%, κ value: .86) and sickle cells (PPV:100%, NPV: 98%, sensitivity: 50%, specificity: 100%, κ value: .66) in the peripheral blood smear had an acceptable agreement for sickle cell disease. CONCLUSION: These two biological markers may guide the clinician in the decision-making to initiate the management of the children as a sickle cell patient, pending confirmation of the disease by electrophoresis techniques.

Congolese children with sickle cell trait may exhibit glomerular hyperfiltration: A case control study.

BACKGROUND: The prevalence of sickle cell trait is extremely high in sub-Saharan Africa. Recent studies have reported the impact of sickle cell carriers on renal function. However, data on renal abnormalities in children with sickle cell trait in this part of the world are unknown. In this report, we assess the glomerular function of children with sickle cell trait (SCT). METHODS: A case control study was conducted to assess the glomerular function in 43 Congolese children with sickle cell trait (Hb-AS) matched for age to 65 children with sickle cell anemia in steady state (Hb-SS) and 67 normal controls (Hb-AA). RESULTS: There was a significant difference in the blood pressure levels between the Hb-AS group vs Hb-SS group (P<.05). The estimated glomerular filtration rate (eGFR) corrected for body surface area was increased in Hb-AS group compared to Hb-AA group, but there was no significant difference between the two groups (P=.48). At the same time, the eGFR was decreased, but no significantly so, in the Hb-AS group compared to the Hb-SS group (P=.19). The proportion of children with Hb-AS (16.3%) who had hyperfiltration was higher compared to the proportion (6.1%) found in the Hb-AA group, but lower compared to the proportion found in the Hb-SS group (30%). However, in both situations, the difference was not statistically significant. No case of proteinuria was detected in children with Hb-AS. CONCLUSION: It appears that at least one of six children with SCT had hyperfiltration. The findings could form a basis for further studies on this renal physiology among SCT individuals in Africa.

High rate of sickle cell anaemia in Sub-Saharan Africa underlines the need to screen all children with severe anaemia for the disease.

AIM: Neonatal screening for sickle cell anaemia is not common practice in the Democratic Republic of Congo, and we determined the prevalence in children with unknown electrophoresis of haemoglobin and anaemia. METHODS: A cross-sectional study was conducted in four hospitals in the country's capital Kinshasa. RESULTS: We screened 807 patients with anaemia (Hb < 6 g/dL) for sickle cell disease. The overall mean age at presentation was 42.7 months ± 29.7 months, and most patients (76.3%) were less than five years of age, with a peak incidence at seven to 36 months of age (45%). The median age at the first transfusion was 29 months (range 4-159 months). Of these 807 children, 36 (4.5%) were homozygous for haemoglobin S disease and 45 (5.6%) were heterozygotes. The proportion of patients with homozygous sickle cell anaemia was slightly higher in children with a medical history of hand foot syndrome, in children who had received more than three transfusions and in children up to 36 months of age at their first transfusion. CONCLUSION: The high prevalence of sickle cell anaemia in children in Sub-Saharan Africa underlines the need for neonatal screening or, if that is not possible, screening of all children with severe anaemia to identify patients with the disease and provide early management.

A rare occurrence of neonatal nephroblastoma in sub-saharan Africa: a case report and management in a resource-constrained region.

Neonatal nephroblastoma has been rarely reported in African neonate. A premature newborn (a 5-day-old male) was transferred with a history of neonatal abdominal mass. Ultrasonography revealed 75×46 mm, well-defined mass with mixed echogenicity replacing the right kidney. The patient underwent right radical nephrectomy and the tumor was confirmed to be a blastemal predominant Wilms' tumor by the histopathological examination and has an unfavorable prognosis. The child died secondary to multiple organ failure, three days after surgery. Our case report serves to remind us the need to bear in mind the possibility of the diagnosis of neonatal nephroblastoma in neonate with renal mass.

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.

Depression in children suffering from sickle cell anemia.

Sickle cell anemia is a chronic illness associated with important nonmedical complications. The prevalence of depression and its clinical profile among Congolese children suffering from sickle cell disease are unknown. We therefore conducted a cross-sectional study in children between 8 and 17 years. The main goal of this study was to describe prevalence and characteristics of depression in this population living in Kinshasa, the Democratic Republic of Congo. The cross-sectional survey is of patients attending 2 referral centers. Children aged 8 to 17 years old were evaluated by a semistructured interview and standardized scales for depression separated by age and sex, the Multiscore Depression Inventory for Children. Completed questionnaires were received from 81 respondents. There were 43 girls and 38 boys. Depression symptoms were observed in 70 (86.4%) cases. Among this group, 6 children (8.6%) were observed to have severe depression. The most common symptoms were observed to be social introversion (81.5%), defiance (77.8%), helplessness (76.5%), and sad mood (70.4%). Of the 70 subjects, 19 (23.5%) had suicidal ideation. In Kinshasa, the prevalence of depression was high to those reported in western countries. Psychological interventions for individuals with sickle cell disease might complement current medical treatment in our midst.

Prevalence of sickle cell disease in a pediatric population suffering from severe infections: a Congolese experience.

Neonatal screening for sickle cell anemia is not a common practice in the Democratic Republic of Congo (DRC). Children with sickle cell disease are known to have an increased risk of infections. We conducted a pilot study to determine the prevalence of sickle cell anemia during episodes of severe infection. A prospective study was conducted from July 2009 to July 2011. The study sites included four public hospitals at Kinshasa, DRC. The study population was selected from the source population using three-stage sampling. A total of 247 children with severe infection were consecutively recruited and screened for sickle cell disease. There were 124 boys (50.2%) and 123 girls (49.8%) with a sex-ratio of 1:1. More than two-thirds of patients (66.0%) were children between 1 and 24 months of age. Among these 247 children, 19 (7.7%) were homozygous sickle cell anemia patients (Hb SS). No patient had received Hemophilus influenzae, streptococcus pneumoniae and salmonella sp vaccines. Sepsis was the most common form of severe infection observed in 44.5% of patients. A total of 19 (7.7%) positive blood cultures were recorded. Most cases were reported in sickle cell patients (15.8%) compared to 6.1% in children who were negative for Hb S [ß6(A3)Glu→Val; HBB: c.20A>T] (p > 0.05). Of 247 children with severe infection, approximately 8.0% carried unknown sickle cell anemia mutations. Based on the findings in this study, opportunistic testing for sickle cell anemia is possible and worthwhile in children who present with severe infection in DRC until neonatal screening is universal.

Renal function in children suffering from sickle cell disease: challenge of early detection in highly resource-scarce settings.

BACKGROUND: The prevalence of Sickle cell disease is extremely high in Democratic Republic of Congo. Despite this high prevalence of the disease, data on renal abnormalities in children are rare. METHOD: The study proposed to assess blood pressure, glomerular function, urea and uric acid levels in 65 steady state Congolese children with homozygous sickle cell disease and 67 normal controls. RESULTS: In Hb-SS group, blood pressure level tended to be lower than Hb-AA groups but there was no statistically significant difference (p>0.05) between the two groups. The absolute values for GFR corrected for BSA were significantly higher in Hb-SS group compared to Hb-AA group (130.5±34.1 ml/min/1.73 m2 vs 113.7±24.5 ml/min/1.73 m2; p = 0.004). Children with Hb-SS were more likely to hyperfiltrate (30.8% of subjects) than children with Hb-AA (6.1% of subjects). Proteinuria was found in 4 (6.2%) children with Hb-SS. Uric acid level was significantly increased in children with Hb-SS compared to corresponding values in control group (4.4±1.3 mg/dl vs 3.5±1.1 mg/dl; p<0.001). Urea level was significantly decreased compared to corresponding values in Hb-AA group (15.3±8.3 mg/dl vs 22.9±10.1 mg/dl; p<0.001). CONCLUSION: Hyperfiltration, low creatinine, lower urea and high uric acid are more common in children with sickle cell disease than in normal controls.

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.

A rare occurrence of hairy cell leukemia in a congolese child: a presentation and challenge of diagnosis in low resource settings.

BACKGROUND: Hairy cell leukemia is a rare form of leukemia and has been rarely reported in African and pediatric population. OBSERVATION: We are reporting a 4-year-old child who was received for investigation for persistent anemia, prolonged fever, and thrombocytopenia. Bone marrow aspiration showed hypercellular marrow with cells characterized by irregular windblown-appearing cell borders with pseudopod-like projections. Our patient presented with hairy cell leukemia. CONCLUSION: The diagnosis was thought to be most consistent with hairy cell leukemia based on the distinctive morphology of the cells.

[Acute renal failure and severe malaria in Congolese children living in Kinshasa, Democratic Republic of Congo]. / Insuffisance rénale aiguë dans les formes graves du paludisme chez les enfants vivant à Kinshasa.

BACKGROUND: Data on acute renal failure in complicated malaria in children in the Democratic Republic of Congo are sparse. The objective of this study was to document the profile of acute renal failure in severe malaria in admitted patients in pediatric hospitals from Kinshasa. METHODS: A prospective cohort study was conducted from January 2008 to December 2008 in children admitted in emergency units of five hospitals in Kinshasa for severe malaria. RESULTS: In our series, 378 children with severe malaria were included. There were 226 boys and 152 girls (sex ratio 1.49). One hundred and ninety four (194) of these patients were under 5 years old. Acute renal failure was observed in 89 children (23.6%) and 87 of them had blackwater fever (BWF). This form of severe malaria was predominant in children older than 5 years. Quinine was the commonest antimalarial drug involved in the genesis of BWF. Dialysis was indicated in 23 children (24.0%) and was effective (acute peritoneal dialysis) in 21 patients. The death rate in children with ARF was 12.6% (n=87). Recovery of renal function was obtained by conservative treatment in the remained group. CONCLUSION: This study confirmed the emergence of BWF in seemed protected autochthon children older than 5 years. BWF remained the leading cause of acute renal failure in complicated malaria among Congolese children in Kinshasa.

Retinoblastoma in the democratic republic of congo: 20-year review from a tertiary hospital in kinshasa.

Background. To determine clinical profile and management of retinoblastoma among children at Kinshasa in Democratic Republic of Congo. Patients and methods. The medical records of patients with a diagnosis of retinoblastoma seen at the University Hospital of Kinshasa from January 1985 till December 2005 were retrospectively reviewed. Demographic profile, clinical data, modes of treatment and outcome were analysed. Results. A total of 49 children, of whom 40 had adequate data on record were identified as retinoblastoma (28 males and 12 females). Nine cases had bilateral disease. The median age at the first symptoms was 9 months (range, 1 month to 6 years) for unilateral retinoblastoma and 18 months (range, 1 month to 3.5 years) for bilateral retinoblastoma. The median age at the first oncology consultation was 2.4 years (range, 6 months to 6 years) for unilateral retinoblastoma and 2.4years (range, 9 months to 4 years) for bilateral disease. Leukokoria was present in 67.5% of subjects. Seventy-five percent abandoned the treatment. The mortality was 92.5%. Conclusion. In Democratic Republic of Congo, retinoblastoma remains a life threatening disease characterized by late referral to a specialized unit and affordability of chemotherapy; all leading to an extension of the disease and high mortality.