Primary causes of kidney disease and mortality in dialysis-dependent children.

BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) is associated with a slower progression to end-stage renal disease (ESRD) in pre-dialysis patients. However, little is known about the associated mortality risks after transitioning to dialysis. METHODS: This retrospective cohort study included 0-21 year-old incident dialysis patients from the United States Renal Data System starting dialysis between 1995 and 2016. We examined the association of CAKUT vs. non-CAKUT with all-cause mortality, using Cox regression adjusted for case mix variables. We also examined the mortality risk associated with 14 non-CAKUT vs. CAKUT ESRD etiologies and under stratification by estimated glomerular filtration rate (eGFR). RESULTS: Among 25,761 patients, the median (interquartile range) age was 17 (11-19) years, and 4780 (19%) had CAKUT. CAKUT was associated with lower mortality, with an adjusted hazard ratio (aHR) of 0.72 (95%CI, 0.64-0.81) (reference: non-CAKUT). In age-stratified analyses, CAKUT vs. non-CAKUT aHRs (95%CI) were 0.66 (0.54-0.80), 0.56 (0.39-0.80), 0.66 (0.50-0.86), and 0.97 (0.80-1.18) among patients < 6, 6-< 13, 13-< 18, and ≥ 18 years at dialysis initiation, respectively. Among non-CAKUT ESRD etiologies, the risk of mortality associated with primary glomerulonephritis (aHR, 0.93; 95%CI 0.80-1.09) and focal segmental glomerulosclerosis (aHR, 0.89; 95%CI, 0.75-1.04) were comparable or slightly lower compared to CAKUT, whereas most other primary causes were associated with higher mortality risk. While the CAKUT group had lower mortality risk compared to the non-CAKUT group patients with eGFR ≥5 mL/min/1.73m2, CAKUT was associated with higher mortality in patients with eGFR < 5 mL/min/1.73 m2. CONCLUSIONS: CAKUT is associated with lower mortality among children < 18 years old, but showed comparable mortality with non-CAKUT among patients ≥ 18 years old. ESRD etiology should be considered in risk assessment for children initiating dialysis.

Colostomy in children on chronic peritoneal dialysis.

BACKGROUND: This study aimed to evaluate outcome of children on chronic peritoneal dialysis (PD) with a concurrent colostomy. METHODS: Patients were identified through the International Pediatric Peritoneal Dialysis Network (IPPN) registry. Matched controls were randomly selected from the registry. Data were collected through the IPPN database and a survey disseminated to all participating sites. RESULTS: Fifteen centers reported 20 children who received chronic PD with a co-existing colostomy. The most common cause of end stage kidney disease was congenital anomalies of the kidney and urinary tract (n = 16, 80%). The main reason for colostomy placement was anorectal malformation (n = 13, 65%). The median age at colostomy creation and PD catheter (PDC) insertion were 0.1 (IQR, 0-2.2) and 2.8 (IQR 0.2-18.8) months, respectively. The colostomies and PDCs were present together for a median 18 (IQR, 4.9-35.8) months. The median age at PDC placement in 46 controls was 3.4 (IQR, 0.2-7.4) months of age. Fourteen patients (70%) developed 39 episodes of peritonitis. The annualized peritonitis rate was significantly higher in the colostomy group (1.13 vs. 0.70 episodes per patient year; p = 0.02). Predominant causative microorganisms were Staphylococcus aureus (15%) and Pseudomonas aeruginosa (13%). There were 12 exit site infection (ESI) episodes reported exclusively in colostomy patients. Seven colostomy children (35%) died during their course of PD, in two cases due to peritonitis. CONCLUSION: Although feasible in children with a colostomy, chronic PD is associated with an increased risk of peritonitis and mortality. Continued efforts to reduce infection risk for this complex patient population are essential.

Insignificant impact of VUR on the progression of CKD in children with CAKUT.

BACKGROUND: Vesicoureteral reflux (VUR) is associated with an increased risk of kidney disorders. It is unclear whether VUR is associated with progression from chronic kidney disease (CKD) to end-stage kidney disease (ESKD) in children with congenital anomalies of the kidney and urinary tract (CAKUT). METHODS: We conducted a 3-year follow-up survey of a cohort of 447 children with CKD (stage 3-5). Rates of and risk factors for progression to ESKD were determined using the Kaplan-Meier method and Cox regression respectively. RESULTS: Congenital anomaly of the kidney and urinary tract was the primary etiology in 278 out of 447 children; 118 (42.4 %) had a history of VUR at the start of the cohort study. There were significantly more boys than girls with VUR, whereas the proportions were similar in children without VUR. The types of urinary anomalies/complications of the two groups were significantly different. Three-year renal survival rates of the groups were not significantly different, irrespective of CKD stage. Age < 2 years and age after puberty, stage 4 or 5 CKD, and heavy proteinuria, but not history of VUR, were significantly associated with progression to ESKD. CONCLUSIONS: History of VUR at the start of follow-up was not associated with the progression of stage 3-5 CKD in children with CAKUT.

Primary vesicoureteral reflux in Sudanese children.

Vesicoureteral reflux (VUR) is a common congenital renal tract anomaly in children. Reports from Sudan are scanty. We report the characteristics, presentation and outcome of primary VUR in a tertiary care hospital. The records of 30 patients (16 males; 53%) followed-up between January 2004 and December 2010 were reviewed. The mean age at the time of diagnosis was 4 ± 3.9 years and 47% were <2 years of age. Renal ultrasound scan (USS) failed to predict VUR in 17% of the patients. On voiding cysturethrogram (VCUG), VUR was bilateral in 57% and severe grade in 64%. Grades were not significantly associated with age, gender or site of VUR. Initial dimercaptosuccinic acid radionuclide scan showed renal damage in 61.5% of the patients. Renal damage was significantly associated with female gender and severe VUR, but not with age of onset or history of urinary tract infection (UTI). Thirteen patients (43.3%) presented with acute UTI, eight (26.6%) with non-specific urinary tract symptoms and nine (30%) with persistently elevated serum creatinine. Urine cultures were positive in 73% of patients, and E. coli was the most common pathogen. Renal impairment at presentation was significantly associated with bilateral severe VUR and history of UTI but not age or gender. After a mean follow-up period of 1.78 years (6 months to 5 years), 70% of patients remained with normal renal function and 30% progressed to chronic kidney disease; two of them died. In conclusion, our data is different from many studies. Features of primary VUR in Sudanese children are late age of onset, equal gender affection and predominance of severe grade. Presentation is associated with a high rate of UTI, renal damage and advanced renal impairment. Measures to improve early detection and treatment of VUR may reduce the risk of kidney damage.

Decrease of the incidence of renin-angiotensin-system inhibitor induced oligohydramnios-sequence in Germany in 2011.

AIM: to assess whether the incidence of angiotensin II-receptor type 1 antagonist (AT1-antagonist)­ or ACE-inhibitor induced cases of oligohydramnios sequence (OHS) in 2011 was reduced after intensive alerts as to the causal association between AT1-antagonist /ACE-inhibitor and OHS in the German medical literature. METHOD: 3 sources of information were used: A nationwide active surveillance of OHS in German paediatric hospitals (ESPED); Embryotox, (Berlin Institute for Clinical Teratology and Drug Risk Assessment in Pregnancy) and screening of pubmed (AT1-antagonist/ACE-inhibitor induced OHS). RESULTS: 45 cases of OHS were identified, no case due to maternal AT1-antagonist/ACE-inhibitor treatment. Causes for OHS were: premature rupture of membranes (PPROM) (n = 28), congenital anomalies of fetal kidneys and urinary tract(CAKUT (n = 15), placental insufficiency (n = 1),unknown cause (n = 1). Mortality until discharge was 37.8 % (32.1 % PPROM, 57.1 % CAKUT). Embryotox identified 3 exposures to AT1-antagonists in pregnancy, no case was associated with OHS. The pubmed search did not identify any case of OHS related to AT1-antagonist/ACE-inhibitor in pregnancy in Germany in 2011. CONCLUSION: Treatment of pregnant women with ACE inhibitors or AT1-antagonists still occurs but no cases of AT1-antagonist- or ACE-inhibitor induced OHS were reported in 2011 in Germany most likely due to repeated published alerts underlining the importance of consequent education. OHS remains a serious condition with high mortality despite modern intensive care.

Part 1: Vesicoureteral reflux treatment: the past, present, and future.

The purpose of this manuscript is to provide clinicians with highlights of key findings pertaining to our current understanding and treatment of the condition of vesicoureteral reflux (VUR). This includes a review of the disease, patient characteristics, current treatment options, challenges for managed care and patients, and opportunities for improvements in care. This is not intended as a comprehensive review of VUR. This manuscript does, however, serve to introduce three additional manuscripts contained within this supplement. The first article in this series is designed to provide the clinician with real-world data pertaining to treatment patterns and outcomes in patients with VUR (Examining pediatric vesicoureteral reflux: a real-world evaluation of treatment patterns and outcomes: Hensle TW, Hyun G, Grogg AL, Eaddy M). The second article considers the efficacy of prophylactic antibiotics in reducing the likelihood of urinary tract infections (UTIs) when compared with endoscopic injection with dextranomer/hyaluronic acid (Endoscopic injection versus antibiotic prophylaxis in the reduction of urinary tract infection in patients with vesicoureteral reflux: Elder JS, Shah MB, Batiste LR, et al.). The third article explores the role medication noncompliance plays in contributing to antibiotic resistance, the consequences associated with resistance (longer lasting illness and costs), and the difficulties with resistance specific to UTI pathogens in children (Considerations regarding the medical management of VUR: what have we really learned?: Koyle MA, Caldamone A). This supplement is intended to provide the clinician with valuable information regarding the treatment patterns, the role of compliance, and the efficacy of treatments for pediatric patients with VUR.

Boys with posterior urethral valves: outcome concerning renal function, bladder function and paternity at ages 31 to 44 years.

PURPOSE: The short-term prognosis for boys with posterior urethral valves (PUV) has improved in recent decades, but the long-term prognosis in terms of renal and bladder function and fertility is still a matter of great concern. This study is a followup of boys with PUV and dilated upper urinary tract treated in 1956 to 1970 at the Children's Hospital in Göteborg, Sweden. MATERIALS AND METHODS: The records of 54 boys treated for PUV were reviewed. Of 27 boys with PUV and upper urinary tract dilation, 5 boys (18%) died at an early age and 3 boys were lost to followup during adolescence, leaving 19 to be included in the followup. They all answered a questionnaire about renal and bladder function and paternity. RESULTS: Of the 19 men 32% were uremic, 21% had moderate renal failure and 47% had not been checked since adolescence. There were signs of bladder dysfunction in 40% and all these subjects had bladder symptoms suggesting detrusor weakness as the cause. All the men were continent. The ability to father children was dependent on whether or not the man was uremic. CONCLUSIONS: This long-term followup study emphasizes the importance of checking renal and bladder function throughout life in men born with PUV. Increasing attention to bladder dysfunction and its early treatment could probably improve the long-term prognosis.

Chronic peritoneal dialysis in Turkish children: a multicenter study.

Chronic peritoneal dialysis (CPD) has been utilized in the treatment of children since 1989 in Turkey. The aims of this study were to summarize our experience with CPD in children and to establish a pediatric registry data system in Turkey. Standard questionnaires were sent to all pediatric CPD centers. 514 patients treated between 1989 and 2002 in 12 pediatric centers were enrolled in the study. Reflux nephropathy was the most common (18.1%) cause of renal failure. Mean age at dialysis initiation was 10.1+/-4.6 years. Mean duration of dialysis was 24.1+/-20.5 months. Continuous ambulatory peritoneal dialysis (CAPD) was the first CPD modality for 476 (92.6%) patients, 142 of whom switched to automated peritoneal dialysis (APD) during follow-up. Currently, 47.3% of the patients are still on CPD, 15.4% were transplanted, 13.2% switched to hemodialysis, 16.7% died. The patient and technique survivals were 90% and 95% at one year and 70% and 69% at five years, respectively. The survival was significantly shorter in the youngest age group (0-24 months) compared to those in older age groups (p=0.000). We herein report the first results of the TUPEPD study providing information on demographic data and survival of pediatric CPD patients. As opposed to clear recommendations in favor of APD, there is a clear preponderance of CAPD in our pediatric CPD population. That vesicoureteral reflux (VUR) is still the leading cause of renal failure is a distressing finding. Remarkably lower survival rates and transplantation ratios are as striking and distressing as the high incidence of VUR among the causes of ESRD. We conclude that we must make a great effort to achieve better results and to change these undesirable events.