These studies demonstrate that mutation of NPHS1 is not a major cause of CNS in Japanese patients, and that mutation of NPHS2 can be responsible for CNS in this population.
The aim of this study was to define the incidence and characteristics of sclerosing encapsulating peritonitis (SEP) in pediatric peritoneal dialysis (PD) patients in Japan. A questionnaire was sent to all dialysis units with at least two pediatric PD patients. Among 687 patients registered, 11 cases (1.6%) of SEP were diagnosed. The mean age of patients with SEP at the start of PD was 9.7+/-3.6 years and at SEP diagnosis, 19.1+/-3.8 years. All patients had undergone PD for more than 5 years, and the mean PD duration was 9.6+/-3.3 years. SEP was diagnosed in 6.6% and 12% of patients dialyzed for >5 years and >8 years, respectively. The incidence of peritonitis among patients with SEP was not different from that among the Japanese pediatric registry. All patients had virtually no residual urine volume and 9 had impaired peritoneal ultrafiltration. Peritoneal calcification was the most-frequent radiological finding. Peritoneal biopsy was performed in 7 patients and confirmed sclerotic peritonitis in all. Ten patients transferred to hemodialysis, and only 1 patient underwent surgery. Three patients died. We recommend that patients on PD for more than 5 years who have impaired peritoneal ultrafiltration or peritoneal calcification should be carefully managed as presumptive cases of SEP.
To obtain data on peritonitis and exit-site and/or tunnel infections (ESI/TI) in Japanese children undergoing peritoneal dialysis (PD) from January 1999 through June 2003, we surveyed 22 members of the Japanese Study Group of Pediatric Peritoneal Dialysis (JSPPD) by questionnaire. One hundred and thirty patients were eligible. Seventy episodes of bacterial peritonitis occurred in 45 patients (0.17 episodes/patient-year), and 123 ESI/TI occurred in 60 patients (0.29 episodes/patient-year). S. aureus and MRSA were found to be the causative organisms in 39% and 13% of the peritonitis episodes, and in 59% and 20% of the ESI/TI, respectively. Tunnel infection was found in 55% of the MRSA peritonitis episodes. Eleven percent of the peritonitis episodes relapsed, and 19% needed hemodialysis. One patient died due to MRSA peritonitis. The PD catheter was removed in all fungal and 78% of MRSA peritonitis. However, the type of organism did not influence the need for catheter-related surgery for ESI/TI. Neither peritonitis nor ESI/TI was prevented by the use of a swan-neck catheter, a downward-pointing exit site, povidone iodine exit-site care, bathing instruments, or nasal mupirocin. In conclusion, MRSA peritonitis was not uncommon in children in Japan, was frequently associated with tunnel infections, and had a poor outcome. No association was found between the occurrence of infection and preventive measures previously reported as effective. Alternative approaches are needed in children, especially for MRSA.
Objective Over the past 10 years, we have collected data on pediatric patients less than 16 years of age from the National Registry of CAPD (continuous ambulatory peritoneal dialysis). We present our experience with this population. Design The database details the patient number, age, weight, height, outcome, cause of death, reason for terminating CAPD therapy, peritonitis, and catheter survival. Patients Of the 434 patients (239 males, 195 females), 37 patients (8.5%) were under 1 year of age and 164 patients (37.8%) were under 6 years of age. About half of the patients were less than 20 kg in weight, clearly indicating that CAPD was the treatment of choice in young children. The duration on CAPD for these patients was less than 2 years for 233 patients (54%), and was 5 years or more in 48 patients (11%). Results The outcome of the total patient population of 434 as of M ay, 1991, is as follows 229 patients (52.8%) were being successfully treated with CAPD, 47 patients (10.8%) died, and 78 patients (18.0%) received a kidney transplantation. The patient survival rate was 85.6% at 3 years and 81.7% at 5 years. The technique survival rate was 74.9% at 3 years and 63.5% at 5 years. The rate of peritonitis was one episode over 28.6 patient-months. The mean catheter duration was 1.68 years. Peritonitis rate, catheter survival rate, and the rate of tunnel infection were worse in children less than 6 years of age than in older children. Conclusion The excellent patient and technique survival rates indicate that CAPD is an effective treatment for children with end-stage renal disease in Japan. The high infection rates in younger children indicate that extra careful management is needed for this young age group.
(1) title of the article:Pediatric live kidney transplantation is safe and available for the patients with urological anomalies compared with as well as those with primary renal diseases respectively, in the urological or renal group. The acute rejection free and overall graft survival were similar in both groups. One patient in the urological group lost his graft while six patients in the renal group lost their grafts. Thus, the post-transplant clinical outcome of pediatric transplantation in patients with urological anomalies is comparable to that of recipients with primary renal disease. Appropriate urinary tract reconstruction and management is essential to reduce the risk of graft dysfunction due to urinary problems.
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