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.

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.

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.

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.

[Prevalence of proteinuria and its association with HIV/AIDS in Congolese children living in Kinshasa, Democratic Republic of Congo]. / Prévalence de la protéinurie et son association avec le VIH/sida chez l'enfant à Kinshasa, Congo.

CONTEXT: In Sub-Saharian Africa, the extent of the HIV-related kidney diseases is less known. Even so, that region is supposed to be the epicentre of such complications. This study aimed to determine the prevalence of proteinuria in Congolese children living in Kinshasa and to study its association with the HIV infection. METHODS: By a cross-sectional and multicentric study (in six hospitals of Kinshasa), 194 children were consecutively recruited from August 2008 to February 2009. Among these, 101 naives HIV-infected children and 93 HIV-uninfected children like a control group. Proteinuria was assessed using urine dipstick completed by the 24-hour proteinuria assessment (Esbach method). Determinants of proteinuria were assessed by logistic regression. RESULTS: The median age of all children recruited was 84 months (9-221 months). Concerning the HIV-infected children, the median age was 76 months (9-221 months) with a male/female ratio of 1/1. The prevalence of proteinuria in this group was in order to 23.8%. HIV infected children have seven times more probability to present proteinuria than non infected children (OR 6.9; IC 95%: 2.3-20.8; P<0.001). Important immunosuppression was the main determinant of proteinuria (OR 10.4; IC 95%: 3.34-32.48; P<0.001). CONCLUSION: Proteinuria is common in Congolese children. The HIV infection rises significantly the probability to present proteinuria in children of this study, more so among those with important immunosuppression. This raises the question about the ideal time to initiate HAART in order to reduce the prevalence of kidney injury and to provide the best outcome.