The burden of chronic kidney disease (CKD) in children in developing countries remains unknown, due to the lack of a national data-reporting system. We undertook a retrospective study of all children < 16 years old in our hospital, which is the tertiary referral centre for children with complex kidney disorders, to analyse the spectrum of CKD (stages 2-5) from 1994-2006. Six hundred and fifty-three children with kidney disorders were screened for CKD; 286 (44.0%) were < 5 years old. Of these, 177 (62%) were male, 202 (70.6%) were black, 77 (26.9%) were Indian, five (1.8%) were mixed race and two (0.7%) were white. One hundred and twenty-six children had CKD (stages 2-5); 55 (43.7%) were < 5 years olds; 41 (74.5%) were male. There were 71 (56.3%) that were > 5 years old, 42 (59.2%) were male. The commonest cause of CKD (stages 2-5) in all children was nephrotic syndrome, comprising 30.9% in children < 5 years old and 40.8% in children > 5 years old. Within the observation period of 11 years, end-stage kidney disease was diagnosed in 20 children; only nine had been on long-term dialysis, and seven qualified for transplantation. Five (25%) children had died, four from sepsis during dialysis and one from tuberculosis after receiving a transplant. We concluded that lack of resources, late referrals, and high cost of renal replacement therapy in developing countries leads to poor outcome in CKD.

Steroid-resistant (SR) forms of nephrotic syndrome (NS) have a poorer outcome in blacks compared to other racial groups. In this study, 223 children with SRNS, aged 1-16 years old, were analysed retrospectively for the period 1976-2004. Treatment schedules included oral cyclophosphamide (2-3 mg/kg) with prednisone 0.5-1 mg/kg (maximum 60 mg) only (n=90); prednisone on alternate days with methylprednisolone (30 mg/kg, maximum 1 g) and oral cyclophosphamide (n=117); oral prednisone on alternate days, three doses of intravenous methylprednisolone on alternate days and monthly doses of intravenous cyclophosphamide (500-750 mg m(-2) dose(-1)x7 doses monthly) (n=10); or cyclosporine 5 mg kg(-1) day(-1) adjusted to a trough level of 150-200 mg/ml (n=6). We compared the clinical and biochemical characteristics and outcome using different forms of therapies. A total of 183 (82.1%) underwent biopsy; 84 (45.9%) were Indian and 99 (54.1%) were black. Sixty-six (36.1%) had minimal change NS, 66 (36.1%) had focal segmental glomerulosclerosis (FSGS), 15 (8.2%) had a proliferative form of NS, and 36 (19.7%) had other forms of NS. Of the 84 Indian children biopsied, 58 (69.0%) were in complete remission, including 29 of 40 (72.5%) treated with oral cyclophosphamide and prednisone only. Of the 99 black children who were biopsied, 20 (20.2%) achieved complete remission; none of those treated with oral cyclophosphamide and prednisone only achieved complete remission. Of the 40 Indian children who were not biopsied who received only oral prednisone and cyclophosphamide, 32 (80%) achieved complete remission. This study shows Indian children with SRNS respond better to treatment than black children (69.0 vs. 20.2%). Since 80% of Indian children with SRNS responded to a trial of oral cyclophosphamide and prednisone, we propose the use of oral cyclophosphamide therapy in non-black children before embarking on renal biopsy.

Background: The use of tacrolimus in steroid-resistant (SR) focal segmental glomerulosclerosis (FSGS) has been reported in single and small series case reports. Aim: To determine the efficacy of tacrolimus in the management of SR FSGS in children. Study Design: This was a prospective study of 20 children with SR FSGS treated with tacrolimus (0.2–0.4 mg/kg/day in two divided doses over 12 h adjusted to a trough level between 7 and 15 ng/ml) for 12 months in combination with low-dose steroids. Other therapies included angiotensin-converting enzyme inhibitors, folic acid, multivitamins and lipid-lowering agents. Results: The mean age at study entry was 11.1 years (range 5.6–16.8). The mean duration of nephrotic syndrome before initiation of tacrolimus therapy was 4.7 years (range 2.1–7.6). At the end of the treatment period, 8 (40%) children were in complete remission, 9 (45%) were in partial remission, and 3 (15%) failed to respond. The average follow-up period following cessation of tacrolimus treatment was 27.5 months (range 13.7–43.7). At last hospital follow-up, 5 (25%) children were in complete remission, 10 (50%) in partial remission, and 2 (10%) in relapse. Three children died from dialysis-related complications following cessation of tacrolimus treatment. Adverse events included sepsis (2), nausea (2), diarrhea (2), anemia (4) and worsening of hypertension (4). Conclusion: Tacrolimus is a safe and effective treatment for SR FSGS. However, like cyclosporine, some children tend to relapse following cessation of treatment.

This was a retrospective study of HIV-infected children aged 0-12 years attending the King Edward VIII Hospital in Durban, South Africa over a 5-year period (January 1996 to December 2001) with culture-proven urinary tract infection (UTI). UTI was defined as the presence of a single bacterial growth of >10(5) colony-forming units/ml in a clean-catch, mid-stream urine sample or >10(3) organisms/ml in a catheter or suprapubic aspirate of urine. HIV/AIDS was diagnosed in accordance with World Health Organization and/or Centers for Disease Control criteria. Comparison between HIV-positive and HIV-negative children with UTI was done using the chi2 and Wilcoxon rank sum tests. Of the 55 children recruited into the study, 29 (52.1%) were HIV-positive and 26 (47.3%) HIV-negative. Escherichia coli was isolated in 50 (87.2%) children. Clinical presentation, aetiological agents, response to therapy and renal function were similar in both groups. This study showed no significant impact of HIV/AIDS on the presentation of UTI in children.

IntroductionIn South Africa (SA), steroid-resistant nephrotic syndrome (SRNS) is more frequent in black than in Indian children.MethodsSeeking a genetic basis for this disparity, we enrolled 33 Indian and 31 black children with steroid-sensitive nephrotic syndrome (SSNS) and SRNS from KwaZulu-Natal, SA; SRNS children underwent kidney biopsy. We sequenced NPHS2 and genotyped APOL1 in 15 SSNS and 64 SRNS unrelated patients and 104 controls and replicated results in 18 black patients with steroid-resistant focal segmental glomerulosclerosis (SR-FSGS). Known FSGS genes (n = 21) were sequenced in a subset of patients.ResultsHomozygosity for NPHS2 V260E was found in 8 of 30 black children with SRNS (27%); all 260E/E carriers had SR-FSGS. Combining SR-FSGS patients from the 2 groups, 14 of 42 (33%) were homozygous for V260E. One black control was heterozygous for V260E; no Indian patients or controls were carriers. Haplotype analysis indicated that homozygosity for V260E was not explained by cryptic consanguinity. Children with NPHS2 260E/E developed SRNS at earlier age than noncarriers (34 vs. 78 months, P = 0.01), and none achieved partial or complete remission (0% vs. 47%, P = 0.002). APOL1 variants did not associate with NS. Sequencing FSGS genes identified a CD2AP predicted pathogenic variant in the heterozygous state in 1 Indian case with SR-FSGS.ConclusionNPHS2 260E/E was present in one-third of black FSGS patients, was absent in black controls and Indian patients, and affected patients were unresponsive to therapy. Genotyping V260E in black children from South Africa with NS will identify a substantial group with SR-FSGS, potentially sparing these children biopsy and ineffective steroid treatment.