Early Prognostic Value of Serum Creatinine Levels in Children With Posterior Urethral Valves

Purpose

We evaluated the prognostic value of serum creatinine level at initial treatment for future renal function in children with posterior urethral valves.

Materials and Methods

We reviewed the records of 35 patients with posterior urethral valves presenting in the first year of life and treated initially at our institution between 1973 and 1990 with valve ablation or vesicostomy. Initial assessment included serum creatinine determination, urine culture, renal ultrasonography and voiding cystourethrography. After 4 or 5 days of catheter bladder drainage renal ultrasound and serum creatinine measurement were repeated. At the end of followup patients were divided into 2 groups according to glomerular filtration rate calculated by the Schwartz formula: group 1-69 ml. or less per minute per 1.73 m.² (median 15) and group 2-greater than 70 ml. per minute per 1.73 m.² (median 110). Median followup was 102 months (8.5 years, range 50 to 219 months).

Results

Mean serum creatinine at diagnosis plus or minus standard deviation was 3.60 +/− 2.01 and 1.3 +/− 0.7 mg./dl. in groups 1 and 2, respectively (normal 0.1 to 0.6, p <0.01). Mean serum creatinine after catheterization was 2.4 +/− 1.1 and 0.6 +/− 0.2 mg./dl. in groups 1 and 2, respectively (p <0.01). Mean nadir creatinine during the first year of life was 1.7 +/− 0.6 and 0.4 +/− 0.2 mg./dl. in groups 1 and 2, respectively (p <0.01). All differences were statistically significant. Linear regression analysis of creatinine after catheterization and glomerular filtration rate at last followup demonstrated a correlation coefficient of -0.7 (p <0.01).

Conclusions

Although it is well known that nadir creatinine in the first year of life correlates with prognosis, the correlation of long-term renal function with creatinine at valve ablation or vesicostomy is more useful to the clinician. These data indicate that serum creatinine level 4 to 5 days after the initial diagnosis correlates strongly with long-term renal function in children with posterior urethral valves.     

Successful Renal Transplantation in Small Children With a Completely Thrombosed Inferior Vena Cava

In small children with end‐stage renal disease, an adult‐sized kidney transplant is the best option. However, in the face of a completely thrombosed inferior vena cava (IVC), such transplants can be challenging, given the difficulty of achieving adequate renal venous outflow and the risk of graft thrombosis. Using a new technique to anastomose the renal vein to the right hepatic vein/IVC junction, we successfully implanted an adult‐sized graft in two small children (9.8 and 14 kg) who had end‐stage renal disease and a completely thrombosed IVC. After mobilizing the right lobe of the liver and obtaining total vascular occlusion of the liver, we used a Fogarty catheter to dilate the retrohepatic IVC. In the right hepatic vein, we made a venotomy and extended it inferiorly onto the retrohepatic IVC. To that venotomy, we anastomosed the donor left renal vein, using continuous 7‐0 Prolene sutures. Both patients attained excellent renal allograft function: One had a serum creatinine level of 0.30 mg/dL at 6 mo after transplant, and the other had a level of 0.29 mg/dL at 1 year. In these two small children with completely thrombosed IVC, our technique for transplanting an adult‐sized kidney provided adequate venous outflow.     

Predicting and Modifying Risk for Development of Renal Failure in Boys with Posterior Urethral Valves

Purpose of Review

The purpose of this review is to bring the reader up to date on the current risk factors for the development of renal deterioration in the boys with posterior urethral valves (PUV) and approaches to modify this risk.

Recent Findings

Renal bladder ultrasound (RBUS) is routinely performed in boys with PUV and recent advancements allow imaging processing that can more accurately quantify renal parenchyma and correlate this with risk for renal loss. Refinement of urine studies may improve our ability to stratify patients into renal loss categories. Use of videourodynamics (VUDS) allows refined assessment of the valve bladder to identify those who might benefit from secondary procedures and/or the addition of targeted pharmacotherapy to improve bladder emptying or dangerous storage pressures.

Summary

All boys with a history of PUV are at a significant long-term risk of renal deterioration. The literature suggests that several technical advances have improved our ability to predict this risk, although there needs to be further refinement and validation before widespread use. Utilization of close follow-up, VUDS, pharmacotherapy, and bladder drainage provide the best methods to improve care to this group of patients and if more studies confirm their utility, adoption of these as part of standard of care protocols may be warranted.

     

Successful Renal Transplantation During Pregnancy

Little is known about the implications of performing a renal transplant on a patient who is already pregnant. This case study reports a successful outcome of pregnancy, diagnosed coincidentally following renal transplantation at 13 weeks gestation. The recipient was a 23-year-old woman with chronic kidney disease who received a live-related renal transplant from her father. Pregnancy was discovered at routine ultrasound scanning of the renal allograft at 5 days posttransplant and estimated at 13 weeks gestation. She received ciclosporin monotherapy as immunosuppression throughout the pregnancy, and was given valacyclovir as prophylaxis against cytomegalovirus (CMV) infection. Renal function remained stable throughout the pregnancy, which progressed normally, resulting in the vaginal delivery of a healthy, liveborn male infant at 37 weeks gestation. This case study demonstrates that transplantation during pregnancy can have a successful outcome.     

Induction With Basiliximab in Renal Transplantation

Introduction

The use of monoclonal antibodies in renal transplantation for induction therapy has been associated with a marked reduction in acute rejection rates with an impact on graft and patient survivals.

Objective

We sought to evaluate the efficacy of renal transplant induction protocols using Basiliximab based on the rates of acute rejection episodes (ARE) and delayed graft function (DGF) of infectious complications in the first 6 months posttransplant, as well as patient and graft survivals.

Methods

We retrospectively evaluated all renal transplants performed between 2000 and 2008 that were primary grafts from cadaveric heart-beating donors, into recipients with a panel reactive antibody titer <5% and who were treated with an immunosuppression scheme based on cyclosporine, mycophenolate mofetil/mycophenolic acid plus corticosteroids, with (group 1) or without basiliximab (group 2).

Results

We enrolled 52 recipients in group 1 (induction with basiliximab) and 189 in group 2 (without basiliximab). The baseline characteristics were similar among the groups, except for time on dialysis which was longer in group 1 and the number of HLA matches, which was lower in group 1. The ARE rate was lower among group 1 (7.8% vs 27.8%; P = .001); rates of DGF and infectious complications were similar. There was no significant difference in graft and patient survivals.

Conclusion

In this study, induction with basiliximab was associated with a reduced rate rate of ARE, despite a lower number of HLA matches and a longer previous time on dialysis. The use of this induction modality was not associated with a greater rate of infectious complications.     

Graft-dependent Renal Hyperparathyroidism Despite Successful Kidney Transplantation

Introduction Only a few reports can be found on the recurrence or persistence of hyperparathyroidism after total parathyroidectomy and autotransplantation (PTX + AT) following kidney transplantation (KTX). The objective of the present study was to assess the frequency and pathophysiological mechanisms responsible for the development of graft-dependent renal hyperparathyroidism (rHPT) after KTX. Patients and methods Between 1986 and 2006, 69 patients underwent surgery for rHPT after KTX at our institution. Patients with reoperations at the parathyroid autograft (AT) were identified. Kidney graft function (KGF) was assessed by the glomerular filtration rate (GFR). Representative parts of the parathyroid gland chosen for autotransplantation during the initial parathyroidectomy and of the excised AT at reoperation were reanalyzed according to the morphologic pattern and the proliferative index. Results Eight of the 69 patients underwent reoperation of the AT. All patients had undergone initial PTX + AT before KTX. The GFR before parathyroid reoperation was 66.6 ± 9.6 ml/min per1.73 m2 (mean ± SEM). Histopathological re-examination revealed nodular hyperplasia in the parathyroid tissue for autotransplantation and in the excised parathyroid autografts. The Ki67 index was increased in the glands chosen for autotransplantation prior to KTX, but was overall low in the excised autografts. Discussion Although not reported in the literature to date, tertiary hyperparathyroidism (tHPT) may arise from parathyroid autografts even in patients with a good KGF. In these cases, graft-dependent tHPT represents the inability of autonomous, nodular parathyroid tissue to regress despite the recovery of renal function. Non-nodular tissue should be selected for parathyroid autotransplantation to decrease the incidence of graft-dependent recurrent rHPT.     

The Long-Term Outcome of Posterior Urethral Valves Treated with Primary Valve Ablation and Observation

Purpose

We believe that primary valve ablation with observation is the preferred management for posterior urethral valves. However, debate continues as to the role of high diversion. We examined the long-term outcome of a large series of patients treated with primary valve ablation, and compared it to the outcome of high diversion and vesicostomy.

Materials and Methods

We reviewed the records of 100 patients treated with primary valve ablation (74 percent), vesicostomy (13 percent) or high diversion (9 percent) before 1985. Median followup was 11.2 years.

Results

Overall 13 percent of our patients had end stage renal disease by age 15 years. Three patients initially treated with valve ablation and 3 initially treated with vesicostomy later underwent high diversion but none benefited from the secondary procedure. Four patients initially treated with valve ablation subsequently underwent vesicostomy but only 1 benefited. Bladder storage capacity was well preserved. Diurnal urinary continence developed in 46 percent of patients at age 10 years and only 1 remained incontinent after age 20 years. One patient with diversion who awaits transplantation had a small contracted bladder. Recent urodynamic studies in 10 cases of delayed urinary continence have not shown decreased bladder compliance or capacity. Kaplan-Meier analysis of outcomes of the different treatments indicated no statistical difference in patient age at end stage renal disease development. However, comparing the number of surgical procedures in the different treatment groups revealed a significant increase in the amount of surgery in infants with diversion. Our results were equivalent to those of the best published series, many of which strongly advocate high diversion.

Conclusions

By avoiding diversion in most cases bladder function is preserved and the need for bladder augmentation is decreased.     

Successful Renal Transplantation From a Deceased Donor With Pesticide Intoxication: A Case Report

The number of individuals awaiting organ transplantation exceeds the number of organs. Patients who die from intoxication are rarely accepted as potential organ donors. Herein we have presented the results of kidney transplantations performed from a deceased 20-year-old female donor with suicidal ingestion of a pesticide (carbamate). The procured kidneys were successfully transplanted. Patients and grafts are doing well at 4 months following transplantation. There are few reports of successful transplantation of organs obtained from patients who die from various intoxications. Poisoned patients represent another pool of organ donors for transplantation services.     

Twenty Years of Renal Transplantation in Children

Two hundred and sixty–eight renal transplant operations were done in 244 children over the past 20 years. The donors were parents in 229 cases, living relatives in another 1 3 eases, and cadavers in the remaining 26 cases. There were 242 first grafts, 22 second grafts, and 4 third grafts. The initial 130 grafts were carried out with conventional immunosuppressive regimens and the subsequent 1 38 were done using immunosuppression including cyclosporin–A. In lanuary 19′)5, 186 recipients (76.2%) were alive with functioning grafts, 33 (1 3.5%) were alive on dialyses, and 25 (10.2%) were dead. The management results in terms of patient and graft survival, as well as the causes of graft failure and patient death are described.     

Successful Renal Transplantation in MYH9-Related Disorder With Severe Macrothrombocytopenia: First Report in Korea

MYH9-related disorders (MYH9 RD) are genetic disorders by the variation of MYH9 gene that encodes for the nonmuscle myosin heavy chain IIA. The clinical and laboratory findings of Fechtner syndrome, an MYH9 RD, are macrothrombocytopenia, basophilic cytoplasmic inclusion bodies in leukocytes, glomerulopathy, sensorineural deafness, and cataracts. Fechtner syndrome is a rare cause of chronic kidney disease. To our knowledge, this is first report of successful renal transplant in MYH9 RD in Korea. We report the two cases with a brief review of literatures since we experienced successful living donor kidney transplantation in Fechtner syndrome with end-stage renal disease, showing very serious thrombocytopenia due to MYH9 mutation.     

Successful Renal Transplantation in Patients with Chronic Granulomatous Disease

Chronic granulomatous disease (CGD) is a genetic disease caused by structural mutations in the enzyme NADPH oxidase that results in severe immunodeficiency. End-stage renal disease occurs in this patient population, and is often attributed to the necessary use of nephrotoxic anti-infectives. In this report, we present the experiences of two centers in transplantation of three patients with CGD: one transplanted with CGD, one cured of his CGD with bone marrow transplantation who subsequently underwent kidney transplantation and one that received a kidney transplant prior to being cured of CGD via a sequential peripheral blood stem cell transplant (SCT). All three recipients have enjoyed excellent outcomes. Their courses demonstrate the absolute requirements for a multidisciplinary and compulsive approach before, during and after transplantation. These case reports also highlight the unexpectedly benign effects of immunosuppressive therapy in this patient population.     

Successful Liver-Kidney Transplantation in Two Children With aHUS Caused by a Mutation in Complement Factor H

A 12-month-old boy and his 16-year-old aunt became acutely ill 6 months apart and were diagnosed to have atypical hemolytic uremic syndrome (aHUS). Genetic analysis revealed heterozygous R1215Q mutation in complement factor H (CFH) in both patients. The same mutation was found in five healthy adult relatives indicating incomplete penetrance of the disease. The patients developed terminal renal failure and experienced reversible neurological symptoms in spite of plasma exchange (PE) therapy. In both cases, liver-kidney transplantation was successfully performed 6 months after the onset of the disease. To minimize complement activation and prevent thrombotic microangiopathy or overt thrombotic events due to the malfunctioning CFH, extensive PE with fresh frozen plasma was performed pre- and perioperatively and anticoagulation was started a few hours after the operation. No circulatory complications appeared and all four grafts started to function immediately. Also, no recurrence or other major clinical setbacks have appeared during the postoperative follow-up (15 and 9 months) and the grafts show excellent function. While more experience is needed, it seems that liver-kidney transplantation combined with pre- and perioperative PE is a rational option in the management of patients with aHUS caused by CFH mutation.