Prognosis and acute complications at the first onset of idiopathic nephrotic syndrome in children: a nationwide survey in Japan (JP-SHINE study)

Abstract

Background Information on the epidemiology of idiopathic nephrotic syndrome (INS) in children, complications of INS and the side effects of steroid therapy is scarce. Methods The Japanese Pediatric Survey Holding Information of Nephrotic Syndrome, a nationwide cohort study, was conducted by the Japanese Study Group of Renal Disease in Children and enrolled 2099 children with newly diagnosed INS between 1 January 2010 and 31 December 2012. We conducted a follow-up study of the complications during the first onset and the patients’ prognosis in this cohort. Results We obtained follow-up data on 999 children (672 males) with a median age at onset of 4.5 years [interquartile range (IQR) 2.8–9.4] and a median follow-up period of 4.1 years (IQR 2.5–5.1). At the first onset, 24% of patients experienced severe acute kidney injury (AKI), defined as a serum creatinine increase to a level two or more times the baseline. On logistic regression analysis, age, hematuria, severe hypoalbuminemia (serum albumin <1.0 g/dL) and severe bacterial infection were not independent factors, but female sex {hazard ratio [HR] 1.5 [95% confidence interval (CI) 1.1–1.7]} and hypertension [HR 4.0 (95% CI 2.6–6.0)] were significantly related to AKI. During the observation period, ocular hypertension requiring treatment occurred in 17.4% of patients, among which 0.4% received surgical treatment. Progression to frequently relapsing nephrotic syndrome/steroid-dependent nephrotic syndrome in 3 years was seen in 44.2% of the patients and was shown by the Cox regression analysis to be significantly related to younger age and days until remission at the first episode, but not to sex, hematuria, the minimum serum albumin level or AKI. Two patients died during the observation period. One patient showed progression to end-stage kidney disease. Conclusion Based on the results of a multicenter questionnaire survey, the overall survival and renal survival rates were found to be excellent. However, proper management of complications, particularly in AKI and ocular hypertension, is mandatory.

Two cases of idiopathic steroid-resistant nephrotic syndrome complicated with thrombotic microangiopathy

“…The etiology of hypertension in NS is multifactorial and complex [9]. It was reported that 24% of patients with NS experienced severe AKI, and hypertension was also significantly related to AKI [11]. In our cases and the previously reported case, the patients developed severe NS with AKI and required frequent administration of albumin and diuretics.…”

“…There is a relationship between hypertension and NS [9,10]. In a Japanese nationwide observational study, 10.8% of new-onset NS patients experienced hypertension requiring treatment [11]. In particular, the patients with SRNS had a much higher prevalence of hypertension compared to the patients with steroid-sensitive NS (66.7 and 14.3%, respectively) [10].…”

Long-Term Outcome of Secondary Steroid-Resistant Nephrotic Syndrome in Chinese Children

“…2 Secondary SRNS, which is also called late steroid-resistant NS or late steroid non-responders, 3 accounted for 13.8-35.9% of SRNS according to published cohort studies of INS. [4][5][6] Although being recognized decades ago, secondary SRNS is still not fully understood. Recent studies have shown that secondary SRNS is associated with a high risk of post-transplant recurrence that is a major cause of allograft loss.…”

Influenza Virus Vaccination in Pediatric Nephrotic Syndrome Induces a Significant Reduction of Relapse and Influenza Virus Infection: A Nationwide Survey

“…The second questionnaire regarding patients who ful lled the eligibility criteria was sent to 388 institutions and recorded information including patients' basic characteristics, renal biopsy, idiopathic NS complications, steroid therapy side effects, and prognosis. We collected data for 999 patients 21 . In the present study, we recorded age when the patient received the rst inactivated subunitantigen u vaccine, the total number of u vaccinations received, total number of u infections, total number of NS relapses, rituximab (RTX) use at the rst u vaccination, and immunosuppressant use at the rst u vaccination (cyclosporine, mycophenolate mofetil, mizoribine, cyclophosphamide, tacrolimus) between 1 May 2015 and 31 April 2016.…”