Can the posterior:anterior urethral ratio on voiding cystourethrogram be used as a reliable predictor of successful posterior urethral valve ablation in male children?

BACKGROUND: The role of the voiding cystourethrogram (VCUG) in the follow-up of children with posterior urethral valves (PUVs) post-ablation has been considered a standard practice. The urethral ratio and gradient of change have proven to be useful. OBJECTIVES: We aimed to review the role of the 'ideal' ratio on predicting residual PUV post-ablation. METHODS: A systematic review of the PubMed, SCOPUS and Web of Science databases was performed (April 2019). The search terms included 'Urethral Ratio and Posterior urethral valve ablation'. All cited reference lists were further evaluated for additional inclusive studies. RESULTS: Eleven studies were identified, of which nine were relevant to the topic. Case reports, comments and adult and animal studies were excluded, leaving four studies for critical review. In total, 338 patients were assessed. The control group consisted of 167 age-matched, male children. Study regions included India and Australia. The ages ranged from 15 days to 3.4 years. Ablation methods included the use of a resectoscope with cutting diathermy, cold knife or Bugbee electrode. The mean urethral ratios in the control group ranged from 1.04 to 1.73. The suggested predictive urethral cut-off ratios recommended include 2.2 (p = 0.001), 2.5-3 and 3.5. CONCLUSION: Although the precise cut-off ratio could not be clearly defined in this review, a urethral ratio less than a range of 2.2-3.5 has proven to be a beneficial predictor of ablation success and should thus be incorporated into standard VCUG reporting templates in the follow-up of PUVs in male children in resource-limited settings.

The podocin V260E mutation predicts steroid resistant nephrotic syndrome in black South African children with focal segmental glomerulosclerosis.

In black African children with focal segmental glomerulosclerosis (FSGS) there are high rates of steroid resistance. The aim was to determine genetic associations with apolipoprotein L1 (APOL1) renal risk variants and podocin (NPHS2) variants in 30 unrelated black South African children with FSGS. Three APOL1 variants were genotyped and the exons of the NPHS2 gene sequenced in the cases and controls. APOL1 risk alleles show a modest association with steroid sensitive nephrotic syndrome (SSNS) and steroid resistant nephrotic syndrome (SRNS). The NPHS2 V260E variant was present in SRNS cases (V/V = 5; V/E = 4; E/E = 11), and was absent in SSNS cases. Haplotype analysis suggests a single mutation origin for V260E and it was associated with a decline in kidney function over a 60-month period (p = 0.026). The V260E variant is a good predictor of autosomal recessive SRNS in black South African children and could provide useful information in a clinical setting.

The Financial Cost Incurred by Families of Children on Long-Term Dialysis.

BACKGROUND: Costs of dialysis reported in countries where dialysis is government-funded are often those incurred by the state, and only a few take into account the financial burden to the family of the index patient. This study investigated the financial cost implication to families of pediatric patients on maintenance dialysis and how aid provided by the government alleviates their financial burden. METHODS: This descriptive cross-sectional study recruited 24 children on peritoneal dialysis (PD) and hemodialysis (HD), and a structured questionnaire was administered to the parents/caregivers of these patients to obtain information on their family size, total family income, cost of transportation, employment status of attending caregiver, and number of work days missed due to hospital visits. RESULTS: Complete data were available for 19 patients (7 on PD and 12 on HD). The mean age was 14 ± 6 years, and there were 11 males and 8 females. The average monthly income of the families recruited was 2,946 ZAR (261 USD). This amount included the contribution of a monthly state-provided social grant of 1,300 ZAR (115 USD) in 16/19 subjects. The average monthly expenditure of the HD and PD groups made up 27.1% and 4.9% of their average income. CONCLUSION: Transport cost for our patients on dialysis significantly impacts on the overall family income, especially for patients on HD, and, without government aid, the families of our patients would have far less money available for their daily needs.

Idiopathic nephrotic syndrome in South African children.

BACKGROUND: Different histo-pathological types and treatment response patterns of Idiopathic nephrotic syndrome (INS) have been associated with differences in ethnicity and geographical location. OBJECTIVE: To provide an update on the steroid response and renal histo-pathological pattern in children treated for INS. METHOD: Medical records of children with INS treated at the Charlotte Maxeke Johannesburg Academic Hospital were reviewed. RESULTS: Mean age was 5.3 years ± 2.8. The majority (68.1%) of the 163 children were of the black racial group. The highest rate of INS was seen in the 2-6 year age group (71.2%). The black racial group had the highest rate (42/111; 37.8%) of focal segmental glomerulosclerosis (FSGS), and the white race had the highest rate (9/14; 64.3%) of minimal change disease (MCD). Ninety four (57.7%) patients were steroid sensitive (SSNS) while 69 patients (42.3%) were steroid resistant (SRNS). Minimal change disease was the most common histo-pathological type seen in SSNS (60%), while FSGS was the most common observed in patients who had SRNS (65.2%). CONCLUSION: There appears to be a higher rate of FSGS in all the racial groups, and also a higher rate of MCD in the black race group, when compared to previous reports.