Kidney transplantation in children with posterior urethral valves

There is controversy about the outcome of renal transplantation in patients with PUV. The objective of this study was to analyze the outcome of renal transplantation in children with PUV. Fifteen patients had a history of PUV as the etiology of their ESRD. Forty-five patients comprised a control group without lower urinary tract anomalies. Mean age and follow-up duration were not significantly different between the case and the control group (p = 0.1). The immunosuppressive protocol and the year of transplantation were similar in these two groups (p = 0.2, 0.4, respectively). Among patients with PUV, 37.5 had acute rejection; and 56.2 had chronic rejection. Among the controls, 22.2 had acute rejection and 28.8 had chronic rejection. None of these differences was significant. Mean survival time was seven yr in affected patients and 6.2 yr in the control group (p = 0.9). Among patients with PUV, the rate of graft survival in the first year after transplantation was 95; and those in the third, fifth, and seventh yr, 91, 65, and 50, respectively. For the controls, the graft survival was 83 at one yr; 80 at three yr; 71 at five yr; and 60 at seven yr after transplantation (p = 0.9). Conclusively, this study showed that a history of PUV had no effect on graft function. Graft survival was not different among these patients compared with patients free of these anomalies. We also showed that urological complications were few in these patients. © 2008 Blackwell Munksgaard

The outcome of renal transplantation in Bardet-Biedl syndrome

Background: Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder characterized by obesity, polydactyly of the hands and feet, retinitis pigmentosa, hypogenitalism and various degrees of intellectual impairment and renal anomalies. Other clinical features include: speech disorder, brachydactyly, developmental delay, polyuria/polydipsia, ataxia, poor coordination/clumsiness, diabetes mellitus, left ventricular hypertrophy, hepatic fibrosis. Spasticity and mental retardation fulfill the criteria for Laurence-Moon-Bardet-Biedl syndrome (LMBBS). Methods: From March 1998 to April 2003, five patients (4 females) with LMBBS were transplanted with kidneys taken from living unrelated donors (LURD). All 5 patients had Retinitis pigmentosa and obesity with a body mass index (BMI) up to 39.33. Findings: The cause of end stage renal failure (ESRF) was reflux nephropathy in one, neuropathic bladder in one and renal hypoplasia/dysplasia in three patients. Mean age at transplantation was 11 years (Range 6 to 17 years). Immunosuppressives were prednisolone, cyclosporine (Neoral) and mycophenolate mofetile (Cellcept). All of them suffered at least one episode of acute rejection shortly after transplantation but reversed with Methyl prednisolone pulses and in the last follow-up the mean creatinine was 1.2 (range 0.6 to 2mg/dl). Mean glomerular filtration rate (GFR) before transplantation was 10ml/min/1.73m2 and in the last follow-up 79 (range 38 to 137) based on Schwartz formula.Conclusions: Renal transplantation is safe and successful and renal replacement (RR) therapy of choice in patients with LMBBS and ESRF but special attention should be paid to BMI and steroid free immunosuppression should be considered if other suitable drugs such as Rapamycin are affordable

Long-term upper and lower urinary tract functions in children with posterior urethral valves

Objective: Several factors have been identified as predictive of future renal function in children with posterior urethral valves (PUV). Our aim was to analyse upper and lower urinary tract outcome in patients with PUV, and determine any factors from the period of early management that were predictive of future renal function. Methods: The charts of 67 boys (mean age 2.4 years) diagnosed with PUV were reviewed. The most common presenting symptom was dribbling in 43.2 and UTI in 28.3. Twenty-three (34.8) patients developed end-stage renal disease aged 1-15 years. The mean time of renal survival was calculated as 7.8 (SEM = 0.73) years. Results: Incontinence in patients over 5 years old, lower urinary tract dysfunction, serum creatinine level in first year or at the time of diagnosis, and presence of vesicoureteral reflux and high-grade bilateral reflux were significant risk factors for occurrence of renal failure in the future. Lower tract dysfunction was seen in 58.6 of patients. Postnatal diagnosis and presence of high-grade reflux were significant risk factors for the future occurrence of lower urinary tract dysfunction. Conclusion: It is important to recognize that PUV have consequences not only during childhood or before treatment, but also during or after the treatment period. Long-term assessment and follow-up of upper and lower urinary tract functions after valve ablation is necessary

Outcome of renal transplantation in children: A multi-center national report from Iran

The outcome of pediatric renal transplantation was previously reported by a single-center study at the year 2006. Therefore, we aimed to evaluate and report the characteristics and outcome of renal pediatric renal transplantation in a multi-center nationwide study. In this nationwide report, medical records of 907 children (�18 yr) with renal transplantation in eight major pediatric transplant centers of Iran were recorded. These 907 patients received a total of 922 transplants. All children who failed to follow-up were excluded. Rather than baseline characteristics, graft and patient outcomes were considered for survival analysis. For further analysis, they were divided into two groups: patients who had graft survival time more than 10 yr (n = 91) and the ones with graft survival time of equal or less than 10 yr (n = 831). Of 922 recipients, 515 (55.8) were boys and 407 (44.2) were girls with the mean age of 13.10 (s.d. = 3.54) yr. DGF and AR were occurred in 10 and 39.5 of the transplanted children, respectively. Transplantation year, dialyzing status before transplantation, DGF, and AR were significant enough to predict graft survival in cox regression model (overall model: p < 0.001). Nowadays, there is a successful live donor pediatric renal transplantation in Iran. Graft survival has improved in our recipients and now the graft survival rates are near to international standards. © 2011 John Wiley & Sons A/S