IPNA clinical practice recommendations for the diagnosis and management of children with steroid-resistant nephrotic syndrome.

Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given.

Adrenal Insufficiency in Children With Nephrotic Syndrome on Corticosteroid Treatment.

Background: Adrenal insufficiency can result from impaired functions at all levels of hypothalamic-pituitary-adrenal (HPA) axis. We here studied risk factors associated with adrenal insufficiency in children receiving prolonged exogenous steroid treatment for nephrotic syndrome. Method:We performed low-dose Synacthen tests (LDSTs, 0.5 µg/m2) in children with steroid-sensitive nephrotic syndrome 4-6 weeks after discontinuation of the corticosteroid therapy. We measured early morning serum cortisol levels at baseline and at intervals of 10, 20, 30, and 60 min following the stimulation test. We defined normal HPA axis stimulation responses as those with peak cortisol cut-off values >550 nmol/L. Result:We enrolled 37 children for this study research. All children enrolled had normal early morning cortisol levels. However, 13 (35.1%) demonstrated HPA axis suppression (by LDST) 4-+6 weeks after discontinuation of oral prednisolone. Nephrotic syndrome diagnosed before 5 years of age (OR, 0.75; 95% CI, 0.57-0.99; p = 0.043), and steroid-dependence [OR, 5.58; 95% confidence interval (CI), 1.06-29.34; p = 0.042] were associated with increased risk of developing adrenal suppression after steroid discontinuation. Conclusion:HPA axis suppression, may go unnoticed without proper screening. A normal early morning cortisol level (275-555 nmol/L) does not exclude adrenal insufficiency in children with steroid-sensitive nephrotic syndrome. Further screening with LDSTs, particularly in children younger than 5 years at diagnosis, may be warranted.

How public and private reforms dramatically improved access to dialysis therapy in Malaysia.

Between 1990 and 2005, dialysis treatment rates in Malaysia increased more than eightfold. Dialysis treatment reached a level comparable to rates in developed countries. This remarkable transformation was brought about in large part by the Malaysian government's large-scale purchase of dialysis services from the highly competitive private sector. This paper traces a series of public- and private-sector reforms that dramatically increased access to dialysis for patients with kidney failure from 13 per million people in the population in 1990 to 119 per million in 2005. Not all developing countries have had uniformly positive experiences with private-sector participation in health care. However, our data suggest that strong participation by the private sector in Malaysia has helped make for a stronger health care system as well as healthier patients. Yet the policy decisions that enabled the private sector to participate fully in providing dialysis have not been repeated with other medical services.

Review article: Use of renal registry data for research, health-care planning and quality improvement: what can we learn from registry data in the Asia-Pacific region?

We review renal registry data from the Asia-Pacific region with an emphasis on their uses in health care and in dialysis care in particular. The review aims to demonstrate the information value of registry data. While renal registry provides a useful data resource for epidemiological research, there are severe methodological limitations in its application for analytical or therapeutic research. However, it is the use of renal registry data for public health and health-care management purposes that registry really comes into its own, and it is primarily for these that governments have invested in national patient and disease registries. We apply data from several renal registries in the Asia-Pacific region to illustrate its wide application for planning dialysis services, for evaluating dialysis practices and health outcomes, with a view to improving the quality of dialysis care. In the course of preparing the review, we have found that the quality and accessibility of renal registry data were highly variable across the region. Given the value of renal registry, every country in the Asia-Pacific region should establish one or should ensure that their current registries are better resourced and developed. Greater data sharing and collaboration among registries in the region could help advance the nephrology to serve our patients better.

A randomized, multicenter, open-label trial to determine peritonitis rate, product defect, and technique survival between ANDY-Disc and UltraBag in patients on CAPD.

BACKGROUND: With the various twin-bag systems available on the market, we decided to conduct a therapeutic equivalence study comparing ANDY-Disc (Fresenius Medical Care, Bad Homburg, Germany) with UltraBag (Baxter, Deerfield, IL) in patients on continuous ambulatory peritoneal dialysis (CAPD) therapy. METHODS: This multicenter, open-label, parallel-group, randomized trial is designed to show the therapeutic equivalence of ANDY-Disc with UltraBag. All CAPD patients from the 6 participating centers who met inclusion/exclusion criteria were enrolled into the trial. They were randomly assigned and converted from the Y-disconnect system (Ultraset; Baxter) to the twin-bag systems. The primary outcome variable is peritonitis, and secondary outcome parameters are technique failure or product defect. RESULTS: From April 2002 to May 2003, a total of 270 patients were recruited for this study. Overall peritonitis rates were 22.9 patient-months/episode for ANDY-Disc and 35.0 patient-months/episode for UltraBag. The overall peritonitis rate for ANDY-Disc was 53% greater compared with UltraBag, but the 95% confidence interval overlaps the prespecified margin of equivalence. There were more product defects reported with ANDY-Disc; 236 product defects compared with 17 with UltraBag. The time series of the number of product defects and peritonitis count on the ANDY-Disc arm suggests a possible cause-and-effect relationship. CONCLUSION: Therapeutic equivalence of ANDY-Disc to UltraBag could not be established with respect to peritonitis. There is a trend toward greater risk for peritonitis on the Andy-Disc arm. There also is a suggestive cause-and-effect relation between the occurrence of product defect and peritonitis on the ANDY-Disc arm during the early part of the trial.

A randomized, multicenter, open-label trial to establish therapeutic equivalence between the Carex and ultra disconnect systems in patients on continuous ambulatory peritoneal dialysis.

OBJECTIVE: In the present study, we undertook to establish therapeutic equivalence with respect to peritonitis and technique failure between the Carex disconnect system (B. Braun Carex, Mirandola, Italy) and the standard Ultra system (Baxter Healthcare, Tokyo, Japan) in patients on continuous ambulatory peritoneal dialysis (CAPD). DESIGN: This multicenter, parallel group, randomized controlled trial involved 363 prevalent CAPD patients from 8 centers. The primary endpoint was peritonitis rate; secondary endpoints were technique failure and technical problems encountered. The duration of the evaluation was 1 year. RESULTS: The risk of peritonitis on Carex varied between the centers. We found a significant treatment-center interaction effect (likelihood ratio test: p = 0.03). The incidence rate ratio (IRR) of peritonitis on Carex as compared with Ultra ranged from 0.4 to 7.2. In two centers, Carex was inferior to Ultra with regard to peritonitis; but, in five centers, the results were inconclusive. Equivalence was not demonstrated in any center. The overall rate of peritonitis in the Carex group was twice that in the Ultra group [IRR: 2.18; 95% confidence interval (CI): 1.51 to 3.14]. Technique failure and technical problems were more common with the Carex system. Technique failure rate at 1 year was 44% in the Carex group and 22% in the Ultra group. CONCLUSIONS: Equivalence between the Carex disconnect system and the Ultra disconnect system could not be demonstrated. The risk of peritonitis on Carex varied significantly between centers.

Anthropometric and growth assessment of children receiving renal replacement therapy in Malaysia.

OBJECTIVE: To report on the anthropometric and growth status of children receiving renal replacement therapy (RRT) in Malaysia. DESIGN: Cross-sectional study with some retrospective elements. SETTING: Outpatients' clinics in a major referral hospital in Malaysia. PATIENTS: Fifteen renal transplants (mean age, 13.3 +/- 2.8 years) and 35 continuous ambulatory peritoneal dialysis (CAPD) patients (mean age, 12.6 +/- 3.0 years) participated. The RRT period ranged between 3 months to 6 years for both groups. INTERVENTION: None. MAIN OUTCOME MEASURES: Body mass index (BMI), percent body fat (% BF), triceps skinfold (TSF), mid upper-arm circumference (MUAC), arm muscle area (AMA), and z scores for height and weight. RESULTS: BMI for age indicated 7% of transplant subjects (TS) versus 40% of CAPD subjects were below the 5th percentile, whereas only 7% of TS were above the 95th percentile. % BF was higher for TS compared with the CAPD group for both sexes (boys, 19.5% and girls, 29.8% v boys, 12.4% and girls, 21.5%). Despite higher percentiles for TSF, transplant subjects still showed depleted muscle mass based on MUAC and AMA. z scores for height showed 93% of transplant subjects and 62% of CAPD subjects below -2 standard deviation (SD), whereas for weight these were 27% and 54%, respectively. Sixty-four percent of TS experienced catch-up growth for height and 86% for weight. These percentages were approximately halved for the CAPD group (32% and 43%, respectively). CONCLUSION: Patterns of growth and body composition appear distinctly different with the different RRT modalities. Serial anthropometric evaluation appears vital in pre- and post-RRT monitoring in pediatric renal populations.