'Waterproofing layers' for urethrocutaneous fistula repair after hypospadias surgery: evidence synthesis with systematic review and meta-analysis.

OBJECTIVE: To summarize the available evidence and to quantitatively evaluate the global results of different waterproofing layers in substantiating the UCF repair. MATERIAL AND METHODS: After defining the study protocol, the review was conducted according to the PRISMA guidelines by a team comprising experts in hypospadiology, systematic reviews and meta-analysis, epidemiology, biostatistics and data science. Studies published from 2000 onwards, reporting on the results of UCF closure after hypospadias repair were searched for on PUBMED, Embase and Google Scholar. Study quality was assessed using Joanna Briggs Checklist (JBI) critical appraisal tool. The results with different techniques were compared with the two samples independent proportions test with the help of Microsoft Excel, MedCalc software and an online calculator. RESULTS: Seventy-three studies were shortlisted for the synthesis; the final analysis included 2886 patients (71 studies) with UCF repair failure in 539. A summary of various dimensions involved with the UCF repair has been generated including time gap after last surgery, stent-vs-no stent, supra-pubic catheterization, suture material, suturing technique, associated anomalies, complications, etc. The success rates associated with different techniques were calculated and compared: simple catheterization (100%), simple primary closure (73.2%), dartos (78.8%), double dartos flaps (81%), scrotal flaps (94.6%), tunica vaginalis (94.3%), PATIO repair (93.5%), biomaterials or dermal substitutes (92%), biocompatible adhesives (56.5%) and skin-based flaps (54.5%). Several techniques were identified as solitary publications and discussed. CONCLUSIONS: Tunica vaginalis and scrotal flaps offer the best results after UCF closure in the synthesis. However, it is not possible to label any technique as ideal or perfect. Almost all popular waterproofing layers have depicted absolute (100%) success sometimes. There are a vast number of other factors (patient's local anatomy, surgeon's expertise and technical perspectives) which influence the final outcome.

The role of Plasma Renin Activity in prenatally diagnosed non-obstructed hydronephrosis at risk for surgery-an observational study.

BACKGROUND: Patients with asymptomatic prenatally diagnosed hydronephrosis may progress to needing surgery; no biomarker supporting the identification of these cases is currently available. The aim of the study was to assess the role of Plasma Renin Activity (PRA) as a discriminatory factor to identify patients with asymptomatic prenatally diagnosed hydronephrosis who require close monitoring and are at-risk for surgery. METHODS: The study group consists of 30 children prenatally diagnosed with asymptomatic unilateral hydronephrosis, initially managed with medical follow-up. Pyeloplasty was indicated if patients became symptomatic or if there was a significant drop in split renal function (SRF) during follow-up. An association was sought between PRA and renal parameters in post-hoc analysis. RESULTS: During the mean follow-up of 55.5 ± 8.2 months, 13/30 (43.3%) patients developed delayed drainage and 8/30 (26.6%) underwent pyeloplasty. Mean PRA was higher at presentation in the group which later presented with a drop in differential kidney function ≥ 10%, while it was within the normal range for age in the other patients. Before pyeloplasty, progression to delayed drainage coincided with a drop in SRF and a rise in PRA. While PRA levels normalized after pyeloplasty, SRF improved but did not reach initial values. A mean rise of 68.9% in PRA preceded current indications for surgery by 27.5 ± 9.5 months. CONCLUSIONS: A progressive increase in PRA in children with asymptomatic prenatally diagnosed hydronephrosis reflects obstructive stress in the tubulo-interstitial compartment. This stress is relieved by pyeloplasty and is reflected by a drop in PRA after surgery. PRA can, thus, serve as the discriminatory factor to identify hydronephrosis patients 'at-risk' for surgery even before the current criteria for pyeloplasty are met.

Early Evidence on Genetic Polymorphisms in Conferring A "Two-Hit" Propensity to Renal Injury in Asian Indian Children.

Background: Congenital anomalies of the kidney and urinary tract (CAKUT) are a common cause of end-stage renal disease in children. While certain nephrogenic genes have been incriminated in these malformations, data to identify the frequency of gene polymorphisms in Asian Indian children with CAKUT are scarce. This study was done to identify the effect of polymorphisms in paired-box gene 2 (PAX2), bone morphogenetic protein (BMP)-4, angiotensin-converting enzyme (ACE), and angiotensin II receptor Type 2 (AGTR2) nephrogenic genes on the development of CAKUT. Materials and Methods: In this prospective cohort study, 158 children <12 years old (86 cases with CAKUT and 72 age-matched controls) were analyzed. DNA from both sets was extracted from peripheral blood using the Keygen DNA extraction kit, and single-nucleotide gene polymorphisms (SNPs) in PAX2, BMP-4, ACE, and AGTR2 nephrogenic genes were detected by polymerase chain reaction (PCR) using previously published primers and PCR conditions. Results: The presence of A allele SNP for AGTR2 gene at rs3736556 was found to be significantly correlated with the development of ureteropelvic junction obstruction and vesicoureteral reflux (VUR) with the TT allelic genotype having a lower incidence of pelviureteric junction obstruction (odds ratio [OR] 0.18 [95% confidence interval [CI], 0.06-0.55], P = 0.01) and VUR (OR 0.31 [95% CI, 0.11-0.91], P = 0.03). Furthermore, on substratification of the patients with the presence of the A allele of AGTR2, 24 out of 27 patients with scarring were found to harbor the D allele of the ACE gene, thus predisposing them to further renal damage. Conclusion: This study points to early evidence in the implication of nephrogenic genes in development as well as predisposition to renal injury in Asian Indian patients with CAKUT.

Role of Glutathione S Transferase M1 and T1 Gene Polymorphism in Hepatitis B Related Liver Diseases and Cryptogenic Cirrhosis.

BACKGROUND AND AIM: Progression of hepatitis B virus infection (HBV) might be affected by host genetic factors. The present study was undertaken to study the role of glutathione S-transferases (GST)-M1 and T1 gene polymorphisms in different stages of HBV infection: HBV inactive carrier, chronic hepatitis B and cirrhosis, and cryptogenic cirrhosis. METHODS: The study population comprised of 170 subjects; 120 cases (HBV inactive carrier, n = 30; HBV related chronic hepatitis, n = 30; HBV related cirrhosis, n = 30; cryptogenic cirrhosis, n = 30) and 50 unrelated healthy adults without liver disease as controls. Analysis of GSTM1 and GSTT1 gene polymorphisms was done by multiplex polymerase chain reaction. RESULTS: The GSTM1 null genotype was seen more commonly in hepatitis B cirrhosis (n = 21; 70%), chronic hepatitis B (n = 19; 63.33%) and cryptogenic cirrhosis (n = 17; 56.67%) as compared with inactive carrier (n = 9; 30%) and controls (n = 13; 26%). The GSTT1 null genotype was seen less frequently in all the groups, the observed frequencies were controls (n = 7; 14%), inactive carrier (n = 5; 16.67%), chronic hepatitis B (n = 8; 26.67%) and hepatitis B cirrhosis (n = 7; 23.33%). The difference of GSTM1 null genotype frequencies was statistically significant for hepatitis B cirrhosis vs. controls (P = 0.0002), chronic hepatitis B vs. controls (P = 0.002) and cryptogenic cirrhosis vs. controls (P = 0.01). The GSTT1 null genotype was not found to vary significantly between the groups. CONCLUSION: The patients with GSTM1 null genotype are at risk of progression of liver disease as the frequency of GSTM1 null genotype was found to be significantly higher in chronic hepatitis B, hepatitis B cirrhosis and cryptogenic cirrhosis as compared with controls.

Hepatic myelopathy in a patient with decompensated alcoholic cirrhosis and portal colopathy.

Cirrhotic or hepatic myelopathy is a rare neurological complication of chronic liver disease usually seen in adults and presents as a progressive pure motor spastic paraparesis which is usually associated with overt liver failure and a surgical or spontaneous systemic portocaval shunt. We describe the development of progressive spastic paraparesis, in a patient with alcoholic cirrhosis with portal hypertension and portal colopathy who presented with the first episode of hepatic encephalopathy. The patient had not undergone any shunt procedure.