A focus on the association of Apol1 with kidney disease in children.

Individuals of African origin have an increased risk of developing various progressive chronic kidney diseases (CKD). This risk has been attributed to genetic variants (G1, G2) in apolipoprotein-L1 (APOL1) gene. In the pediatric population, especially in children affected by sickle cell disease (SCD), by human immunodeficiency virus (HIV), or with various glomerular diseases, APOL1 risk variants have been associated with the development of hypertension, albuminuria, and more rapid decline of kidney function. The present review focuses on existing APOL1-related epidemiological data in children with CKD. It also includes data from studies addressing racial disparities in CKD, the APOL1-related innate immunity, and the relationship between APOL1 and CKD and pathogenic pathways mediating APOL1-related kidney injury.

The clinical characteristics of Congolese children and adolescents suffering from sickle-cell anemia are marked by the high frequencies of epistaxis compared to Western series.

Background: Sickle cell anaemia (SCA) is the most common genetic diseases in the Democratic Republic of Congo (DRC). It is estimated 30,000 to 40,000 neonates with SCA are born annually. Despite this high incidence rate, and the severity of the Bantu haplotype found in Congolese patients, major clinical characteristics remain poorly defined. The objective of this study was to assess the clinical and haematological profile of the SCA in patients less than 24 years of age. Methods: A cross-sectional study was conducted in Kinshasa, the large city of the DRC. Patients were consecutively selected in three health institutions. Results: The study includes 256 sickle cell patients. The mean age of 8.4 (SD = 4.9) years. The Hand-foot syndrome was most common (52.7%) first presentation revealing the disease in our series. The most prevalent crises found in our series were vaso-occlusive crises (VOC) in 170 cases (66.4%) and severe hemolysis in 136 cases (53.1%). Splenic sequestration was noted in 19 cases (7.4%). The age at the first pain crisis was 18.2±15.2 months-of-age and the age at the first transfusions was 29.2±27.6 months-of -age. The most common signs associated with sickle cell disease in our series were hepatomegaly (53.9%), splenomegaly (41.7%), and adenotonsillar hypertrophy (34.8%). Epistaxis was reported in 9.4%. Conclusion: The clinical course of patients in DRC was comparable to reports from Western countries, with the notable exception of epistaxis which was significantly higher in patients in the DRC.

Albuminuria, serum antioxidant enzyme levels and markers of hemolysis and inflammation in steady state children with sickle cell anemia.

BACKGROUND: Oxidative stress is thought to be involved in the pathogenesis of microalbuminuria in Sickle cell anemia (SCA). Antioxidant enzymes such as glutathione peroxidase (GPx) and Cu-Zn superoxide dismutase (SOD) may play an important protective role. This study aimed to evaluate the association between albuminuria and these two antioxidant enzymes. METHODS: We consecutively recruited Steady state children aged between 2 and 18 years old with established diagnosis of homozygous SCA in two hospitals of Kinshasa/DR Congo. The relationship between Urinary Albumin Creatinine Ratio (UACR) and other variables of interest (age, systolic blood pressure, diastolic blood pressure, plasma GPx and Cu-Zn SOD, free plasmatic hemoglobin, LDH, indirect bilirubin, white blood cells (WBC), percentage of fetal hemoglobin, serum iron, ferritin, CRP) was analyzed by Bivariate correlation (Pearson's correlation coefficient). Microalbuminuria was defined by urine albumin/creatinine ratio between 30 and 299 mg/g. RESULTS: Seventy Steady state Black African children with SCA (56% boys; average age 9.9 ± 4.3 years; 53% receiving hydroxyurea) were selected. Prevalence of microalbuminuria was 11.8%. LDH (r = 0.260; p = 0.033) and WBC count (r = 0.264; p = 0.033) were positively correlated with UACR whereas GPx (- 0.328; p = 0.007) and Cu-Zn SOD (- 0.210; p = 0.091) were negatively correlated with UACR. CONCLUSIONS: Albuminuria is associated with decreased antioxidant capacity and increased levels of markers of hemolysis and inflammation. Therefore, strategies targeting the reduction of sickling and subsequent hemolysis, oxidative stress and inflammation could help preventing or at least delaying the progression of kidney disease in SCA children.

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.

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.

A promising pediatric peritoneal dialysis experience in a resource-limited setting with the support of saving young lives program.

In the Democratic Republic of Congo (DRC), acute kidney injury (AKI) contributes to the high rate of child mortality owing to the conjunction of poverty, deficiency of qualified health-care providers in pediatric nephrology, and the lack of pediatric dialysis programs. We aimed to describe the recent experience of the first pediatric acute peritoneal dialysis (PD) program in DRC. This is a retrospective cohort study on epidemiology, clinical features and outcomes of children admitted from January 2018 to January 2019 at the University Hospital of Kinshasa for AKI and treated with PD. This pediatric PD program started by a team of one physician and one nurse who were trained in the local production of PD fluids and bedside catheter insertion technique in Benin Republic. The training was jointly supported by the Flemish Inter-University Council (VLIR) TEAM project and Saving Young Lives (SYL) program of ISN, ISPD, EuroPD, and IPNA. From January 2018 to January 2019, 49 children (aged 4 months-15 years) were admitted for AKI mainly due to severe malaria and sepsis. Dialysis was indicated in 35 of 49 (71.4%), 32 of 35 (91.4%) were treated with PD, two with hemodialysis (HD) in adult ward and one died at admission. Data of the two patients transferred for HD were not available for follow-up. The main indications were uremia and prolonged anuria. Of 32 dialyzed patients, 24 (75%) recovered normal renal function 3 months after discharge. Peritonitis was observed in 2 of 32 (6.2%) patients and the mortality was 18.7%. This promising experience proves that with simple means including use of locally produced dialysis fluids and low peritonitis rates, we can effectively save lives of children suffering from AKI.

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.