Tubulocystic anomalies of the mesonephric duct associated with ipsilateral renal dysgenesis.

INTRODUCTION: Renal agenesis and multicystic dysplastic kidney (MCDK) are usually associated with either an absent or atretic ureter. Occasionally, these renal anomalies may be associated with a dilated tortuous ureter, ureterocele or other cystic malformation of mesonephric duct (MND) remnants. OBJECTIVES: The objective of this study was to identify and classify anatomical variants of tubulocystic remnants of the MND, with a secondary focus on natural history and management outcomes. PATIENTS AND METHODS: A retrospective cohort study of patients seen in the study institution between 2007 and 2014 with a tubulocystic abnormality of MND structures associated with either MCDK or renal agenesis was conducted. Medical imaging and progress notes were reviewed for all patients. Data collected included anatomical information, surgical intervention, histology and outcomes of both conservatively managed and surgically resected MND remnant structures. RESULTS: Nineteen patients were identified, 5 girls and 14 boys. Median age at presentation was 4.6 years. Anomalies of the MND occurred on the left in 9 patients and on the right in 10 patients. Mean follow-up was 3.4 years. Patients fell into 3 distinct anatomical groups: Type I, including orthotopic remnants corresponding to ureteric bud structures (ureter and trigone); Type II, including ectopic MND remnants of ureteric bud structures, and Type III, including complex remnants corresponding to MND structures other than those from ureteric bud (vas, epididymis and seminal vesicles). Anomalies of structures arising from urogenital sinus and paramesonephric ducts were also identified. Most patients were asymptomatic and successfully managed conservatively. Transvesical puncture of trigonal cysts provided effective decompression in 5 patients. Partial or complete MCDK regression was seen in 7 patients, whereas MND cystic anomalies did not regress spontaneously. DISCUSSION: When MND tubulocystic structures persist along with renal agenesis or MCDK, most arise from ureteric bud structures in an orthotopic position as a ureterocele with or without a blind-ending ureter-like structure. Less commonly, ureteric bud structures insert ectopically into the urogenital tract, or tubulocystic structures arising from the remainder of the MND occur. Embryogenesis of other urogenital structures may also be abnormal, and conditions such as Zinner syndrome and obstructed hemivagina and ipsilateral renal agenesis syndrome should be considered. CONCLUSIONS: Complications are uncommon, and surgical intervention should be limited to symptomatic patients. Remnants of metanephric blastema may involute, but MND remnants persist.

Nadir creatinine in posterior urethral valves: How high is low enough?

INTRODUCTION: Large retrospective studies of people with posterior urethral valves (PUV) have reported chronic renal insufficiency (CRI) in up to one third of the participants and end-stage renal failure in up to one quarter of them. Nadir creatinine (lowest creatinine during the first year following diagnosis) is the recognised prognostic indicator for renal outcome in PUV, the most commonly used cut-off being 1 mg/dl (88.4 umol/l). OBJECTIVE: To conduct a statistical analysis of nadir creatinine in PUV patients in order to identify the optimal cut-off level as a prognostic indicator for CRI. STUDY DESIGN: Patients treated by endoscopic valve ablation at the present institution between 1993 and 2004 were reviewed. Chronic renal insufficiency was defined as CKD2 or higher. Statistical methods included receiver operating characteristic (ROC) curve analysis, Fisher exact test and diagnostic utility tests. Statistical significance was defined as P < 0.05. RESULTS AND DISCUSSION: Nadir creatinine was identified in 96 patients. The median follow-up was 9.4 (IQR 7.0, 13.4) years. A total of 29 (30.2%) patients developed CRI, with nine (9.4%) reaching end-stage renal failure. On ROC analysis, Nadir creatinine was highly prognostic for future CRI, with an Area Under the Curve of 0.887 (P < 0.001). Renal insufficiency occurred in all 10 (100%) patients with nadir creatinine >88.4 umol/l compared with 19 of 86 (22.2%) patients with lower nadir creatinine (P < 0.001). As a test for future CRI, a nadir creatinine cut-off of 88.4 umol/l gave a specificity of 100%, but poor sensitivity of 34.5%. Lowering the cut-off to 75 umol/l resulted in improvement in all diagnostic utility tests (Table). All 14 (100%) patients with nadir creatinine >75 umol/l developed CRI, compared with 15 of 82 (18.3%) patients with lower nadir creatinine (P < 0.001). Sensitivity only approached 95% at 35 umol/l, at which level specificity was low (Table). Two out of 36 (5.6%) patients with nadir creatinine <35 umol/l developed CRI. Multivariate analysis found recurrent UTI (OR 4.733; CI 1.297-17.280) and nadir creatinine >75 umol/l (OR 48.988; CI 4.9-490.11) to be independent risk factors for progression to CRI. Using cut-off values of 35 umol/l and 75 umol/l, patients can be stratified into low-, intermediate- and high-risk groups, with development of CRI in 5.3%, 28.3% and 100%, respectively (P <0.001). The stage of CKD was higher in higher risk groups. CONCLUSION: Patients with nadir creatinine >75 umol/l (0.85 mg/dl) should be considered at high risk for CRI, while patients with nadir creatinine ≤35 umol/l (0.4 mg/dl) should be considered low risk. Patients with nadir creatinine between these two values have an intermediate risk of CRI.

Short-Term Adjuvant Therapy with Terminalia arjuna Attenuates Ongoing Inflammation and Immune Imbalance in Patients with Stable Coronary Artery Disease: In Vitro and In Vivo Evidence.

The present study evaluated the cardioprotective effects of Terminalia arjuna on classical and immuno-inflammatory markers in coronary artery disease (CAD) as an adjuvant therapy. One hundred sixteen patients with stable CAD were administered placebo/T. arjuna (500 mg twice a day) along with medications in a randomized, double-blind clinical trial. To understand the specificity and efficacy of T. arjuna, we evaluated its effect through microarray and in silico analysis in few representative samples. Data was further validated via real-time PCR (n = 50) each at baseline, 3 months, and 6 months, respectively. rIL-18 cytokine was used to induce inflammation in vitro to compare its effects with atorvastatin. T. arjuna significantly down-regulated TG, VLDL-C, and immuno-inflammatory markers in stable CAD versus placebo-treated subjects. Microarray and pathway analysis of a few samples from T. arjuna/placebo-treated groups and real-time PCR validation further confirmed our observations. Our data demonstrate the anti-inflammatory and immunomodulatory effects of T. arjuna that may attenuate ongoing inflammation and immune imbalance in medicated CAD subjects.

Health-related quality of life in patients reliant upon mitrofanoff catheterisation.

OBJECTIVE: Aim of the study was determine health care outcomes from the patients' perspective in patients reliant on Mitrofanoff catheterisation for bladder emptying. SUBJECTS/PATIENTS: Patients over the age of 16 dependent on Mitrofanoff catheterisation for bladder emptying were asked to complete a health care outcome questionnaire, the SF-36 (®) Health Survey v2. Quality of life measures for 8 health concepts were compared against published data for the normal population. RESULTS: Out of a total of 25 patients who were eligible for enrolment into our study, we were able to contact 19 patients. The norm-based score for Physical Functioning (PF=50.4), Role Physical (RP=53.8), Bodily Pain (BP=55.6), Vitality (VT=56.9), Social Functioning (SF=51.5), Role Emotional (RE=52.2), and Mental Health (MH=54.6) were all higher than those reported within the normal population (normal=50.0). Physical and mental component summary measures were higher than in the normal population. When compared against age-matched norms our patient group scored higher than the normal population for all measures except Physical Functioning (50.4 vs. 53.4) and physical component summary (51.9 vs. 53.5). The self-reported scores for Vitality, Mental Health and the mental component summary were all statistically significantly better than those seen in the age-matched control population (p<0.01). CONCLUSIONS: Quality of life in patients dependent on Mitrofanoff catheterisation for bladder emptying is good. The SF-36 measures 8 major health care outcomes and in our patients these measures of health were similar to those seen in the general population, rather than the poorer outcomes reported in patients with other chronic medical conditions.

Urinomas protect renal function in posterior urethral valves--a population based study.

BACKGROUND/PURPOSE: Urinomas have been thought to protect renal function in boys with posterior urethral valves (PUVs), although recent reports have disputed this. This study tested the hypothesis that urinomas protect global renal function in boys with PUV. METHODS: A retrospective analysis of all boys with PUV presenting to a tertiary unit derived from a region with an estimated population of 5.5 million was performed. Comparisons of the initial nadir creatinine, current creatinine, and renal status score (RSS) were made between those with and without urinomas. The RSS was derived from nephrology assessment of current renal status (0 = normal to 4 = end-stage renal failure or transplantation). Results were given as median (range), except for RSS, which was given as mean +/- SEM. P < or = .05 was regarded as significant. RESULTS: During 1989-2009, 9 of 89 PUV boys were diagnosed with urinomas. Initial nadir creatinine was statistically lower in boys with urinomas (31 [18-44] vs 45 [20-574] mumol/L, P < .01). Length of follow-up was similar (5.1 [2.2-17.3] vs 5.9 [1.8-19.7] years, P = .59). Follow-up creatinine was significantly lower in urinoma boys (44 [25-77] vs 61 [29-1227] micromol/L, P < .05), as was the RSS (0.14 +/- 0.14 vs 0.91 +/- 0.14, P < .01). No urinoma boys progressed to end-stage renal failure or required transplant. CONCLUSION: This population-based study of PUV boys demonstrates that urinomas reduce nadir creatinine and significantly protect long-term global renal function.

Silent But Giant: Giant ureteric calculus in a 7-year-old boy.

Giant ureteric calculi are extremely rare in children. We present a case of a child who was originally admitted for observation following non-accidental injury and had an episode of painless haematuria as an inpatient. Ultrasonography demonstrated left hydronephrosis and a 2cm echogenic area in the proximal ureter. A plain abdominal radiograph surprisingly revealed two left ureteric calculi, one 7cm and the other 4cm in length. Stone extraction was achieved using an open left ureterolithotomy and pyelolithotomy.

What is the effect of circumcision on risk of urinary tract infection in boys with posterior urethral valves?

PURPOSE: Boys with posterior urethral valves (PUV) have increased risks of urinary tract infection (UTI) voiding dysfunction and ongoing renal damage. Circumcision has been shown epidemiologically to reduce UTIs, but no trial has yet confirmed this in PUV. Circumcision is not routinely performed in boys with PUV in our unit, but one quarter of our patients are circumcised for religious reasons. It may be hypothesized that circumcision reduces the risk of subsequent urinary tract infection in boys with PUV. This study aims to test this hypothesis by comparing the risk of UTI, and subsequent renal outcome, in PUV in uncircumcised boys with those who were circumcised. METHODS: A retrospective cross-sectional case note review of boys with PUV was performed, and the following were documented: age at presentation, method of diagnosis, method of treatment, initial renal status, and timing of treatment; use and timing of urinary tract diversion; timing of circumcision; and UTIs-date, organism, and treatment. RESULTS: Seventy-eight patients were identified, mean age 6.7 years (range, 1-18). These boys experienced 78 UTIs in the uncircumcised state. Subsequently, 27 were circumcised, experiencing 8 UTIs. Eighteen boys were diverted. The incidence of UTI was reduced from 0.50 +/- 0.14 (mean +/- SEM) UTIs annually uncircumcised to 0.09 +/- 0.02 (mean +/- SEM) circumcised (P < .01, Student's t test). CONCLUSION: In PUV, circumcision reduces the incidence of UTI by 83%, every circumcision prevents 1 UTI on average. Early circumcision in all PUV is beneficial, but a larger randomised control trial should be considered to confirm this.

Graft-versus-host disease and phimosis.

Graft-versus-host disease rarely causes genitourinary problems. We report a case of pathological phimosis in a child secondary to chronic graft-versus-host disease.

Laparoscopic nephrectomy for xanthogranulomatous pyelonephritis in childhood: the way forward.

OBJECTIVE: Xanthogranulomatous pyelonephritis (XGP) is a severe, chronic renal parenchymal infection. Nephrectomy is curative. Open nephrectomy is a well-known operative challenge. We describe successful laparoscopic nephrectomy in three patients with XGP. MATERIALS AND METHODS: Case notes and radiology of three patients who underwent laparoscopic nephrectomy for XGP were reviewed. The preoperative presentation, operative details, postoperative recovery and complications were recorded. RESULTS: The ages of the three children were 9, 5 and 1.3 years. Retroperitoneoscopy was performed via 3 x 5-mm ports. Creation of retroperitoneal space was easier than anticipated despite the perinephric inflammation. Excellent visualization of renal pedicle was obtained. The renal vessels were divided using the ultrasonic dissector (Sonosurg, Olympus). The kidney was then mobilized and dissected off adjacent structures. It was extracted via a small extension to one of the incisions on each occasion. None of them needed conversion. Average operating time was 3h and 30 min. Histology of XGP was confirmed in all. CONCLUSIONS: Laparoscopic nephrectomy has replaced open nephrectomy for most conditions. Preoperatively, it was thought that laparoscopy might be unsuitable because of the involvement of perinephric tissues commonly seen in this condition; however, excellent visualization was achieved enhancing the ease of dissection. Dealing with the pedicle before handling the kidney also proved beneficial, allowing better visualization and safe dissection. Postoperative pain and morbidity were greatly reduced. The cosmetic result was excellent. We recommend the laparoscopic approach for nephrectomy in XGP when expertise is available.

Laparoscopic excision of Mullerian structures in a neonate with mixed gonadal dysgenesis: a case report.

Excision of Mullerian structures in children with disorders of sexual differentiation is an operative challenge. We report our experience with laparoscopic excision of Mullerian structures in a neonate with mixed gonadal dysgenesis. The salient features of the procedure were excellent visualisation and ease of dissection.

Single-system ectopic ureter: a 15-year review.

Ectopic ureters present in childhood with symptoms related to an abnormal site or structure (refluxing, obstructed) of the ureteric orifice. The majority drain duplex kidneys. The diagnosis is relatively easy if the poles are functioning or hydronephrotic. Associated malformations are rarely seen and the results of surgery are gratifying. If an ectopic ureter drains a single kidney, it is called a single-system ectopic ureter (SSEU). We reviewed a 15-year experience (1980-1995) with 127 ectopic ureters from our hospital:11 SSEUs in ten consecutive children were managed during this period. Our data lead us to believe that SSEUs are a special subset of ectopic ureters. Diagnosis is often delayed because the ectopic ureter may be associated with a single small, dysplastic, poorly-functioning, non-visualised kidney and the child may be thought to have a contralateral normal "solitary kidney". Associated systemic malformations are common. Residual symptoms of wetting may persist in the early postoperative period. A high degree of suspicion must be maintained for this entity when a child presents with urinary symptoms of wetting or recurrent infection and a "solitary kidney". Early endoscopic examination of the genitourinary tract will clinch the diagnosis in the majority of cases. Residual symptoms of wetting in the postoperative period generally resolve with passage of time.

Central-venous access through the peripheral route in surgical neonates: an audit of 125 consecutive lines from a regional neonatal centre.

A long Percutaneous silastic IV line is frequently used in surgical neonates for infusion of hyperosmolar parenteral nutrition fluid into a central vein for several days without the need for operative insertion of a Broviac catheter or risks of direct puncture of a central vein. Our study was aimed at auditing the performance of 125 consecutive lines over a 2-year period. During this period, insertion was attempted in 125 babies; in 13 cases the line could not be inserted because of technical problems. The gestational ages varied between 25 and 41 weeks and weights between 630 g and 4.2 kg. Success did not appear to be related to the age or weight of the baby. The mean duration of complication-free performance was 22.4 days. There was a significant difference between the complication rate of lines inserted in the operating theatre versus those on the ward (P < 0.05). There was no significant increase in complications in lines used for over 4 weeks. The technique adopted by us for inserting these lines is likely to succeed in the majority of cases, including premature and small-for-dates babies. In our experience, lines inserted in the controlled theatre environment either before or after abdominal surgery performed better. The manufacturer's recommendation to electively change the line every 4 weeks needs further prospective evaluation.

Central venous access in neonates through the peripheral route.

Central venous access is frequently used for monitoring and administration of drugs in the intensive care unit, and for administration of parenteral nutrition. The improving results of neonatal surgery have closely followed the evolution of neonatal intensive care and parenteral nutrition. Nutritional support by the parenteral route is required in the majority of cases for only a few weeks. Percutaneous sialastic long line introduced into a central vein through a peripheral venous puncture has emerged as one of the most popular techniques for delivery of parenteral nutrition in neonates.

A study on p53 protein and anti-p53 antibodies in the sera of patients with oesophageal cancer.

p53 protein levels were examined in sera of 15 oesophageal cancer patients using the p53 monoclonal antibody Pab 1801. Antibodies specific for p53 were also analyzed in 20 patients (including 15 patients used for p53 protein level study) and 20 healthy controls. Anti-p53 antibodies were present in 30% (6/20) patients while no antibodies were detected in sera of healthy controls. Comparative analysis of the two parameters, i.e., p53 protein levels and the corresponding antibodies in the patients revealed no correlation.

Duodenal atresia: a comparison of three modes of treatment.

To determine the most successful mode of treatment, 33 consecutive cases of duodenal atresia treated by duodenoduodenostomy and not associated with other gastro-intestinal anomalies were analysed retrospectively. These patients have been placed in a nonrandomised fashion into one of three groups: Group A: Duodenostomy (side to side) with gastrostomy and transanastomotic feeding tube (n = 12); Group B: Duodenoduodenostomy (diamond shape) with jejunostomy feeding tube (n = 12); Group C: Duodenoduodenostomy (diamond shape) only (n = 9). A nasogastric tube was used in all cases. There was no difference between the groups for gestational age, birthweight, and age at operation. The outcome measures used to compare these groups were the time taken to achieve full preanastomotic feeds and the duration of hospital stay. There was no difference in time taken to achieve full pre-anastomotic feeds between Group A and Group B. Patients in Group C took significantly less time to achieve full pre-anastomotic feeds than either of the other two groups (p < 0.05, Mann-Whitney U). The duration of hospital stay was also significantly shorter for patients in Group C (median = 12 days) than for patients in either Group A or B (median = 24, 20 days respectively) (p < 0.05, Mann-Whitney U).