Mycophenolate Mofetil Versus Prednisone for Induction Therapy in Steroid-Sensitive Idiopathic Nephrotic Syndrome in Children: An Observational Study.

Rationale & Objective: High-dose steroids are recommended for the induction of idiopathic nephrotic syndrome. The aim of this study was to compare standard induction therapy with Mycophenolate Mofetil (MMF). We hypothesized that MMF could be noninferior to steroids in maintaining steroid-induced remission. The second aim was to reduce steroid-induced side effects. Study Design: This was an observational study. Setting & Population: Patients 2-11 years with first episode of nephrotic syndrome who entered remission within 2 weeks of standard steroid treatment were eligible for enrollment. Patients in the experimental group completed 12-week induction with MMF, whereas the control group continued a standard 12-week steroid protocol. Exposures: MMF and prednisolone were used in the study. Outcomes: The primary study outcomes were relapse rate and relapse-free interval during a 52-week follow-up. Analytical Approach: Descriptive statistics were used for analysis. Results: Ten of 41 eligible patients consented to participate in the MMF group and 8 completed the study. The control group included 31 patients, with 23 patients who completed 52 weeks follow-up. During the induction phase, 3 out of 10 patients (30%) in the MMF group and 1 out of 31 (3%) in the control group (P = 0.04) developed relapse. During the 52 weeks follow-up period, 7 out of 10 patients (70%) in the MMF group and 19 out of 31 (61%) in the control group developed relapse (P = 0.72). The median relapse-free interval was 11 and 19 weeks in MMF and control groups, respectively (P = 0.60). No serious side effects were recorded in either group. Limitations: The limitations of the study were low patient numbers receiving MMF and single-center design. Conclusions: Our small cohort of patients treated with MMF reported a higher relapse rate during the induction phase. However, by 12 months of follow-up the relapse rate and relapse-free intervals were similar between both groups. All patients tolerated MMF without significant side effects, and those who relapsed remained steroid-sensitive.

Despite their known side effects steroids remain a standard induction therapy for new onset nephrotic syndrome in children. The aim of this study was to assess whether mycophenolate mofetil (MMF) can be as successful as steroids in maintaining steroid-induced remission. Patients who achieved remission within 2 weeks of steroid treatment had either continued steroids for an additional 10 weeks or switched to MMF. The results of the study showed a higher relapse and infection rate in the MMF group. By 12 months there were no differences in the relapse rate and relapse-free interval between the groups. Larger multicenter studies are underway to demonstrate noninferiority of MMF to steroids in steroid-sensitive nephrotic syndrome.

Acute kidney injury requiring kidney replacement therapy in childhood lupus nephritis: a cohort study of the Pediatric Nephrology Research Consortium and Childhood Arthritis and Rheumatology Research Alliance.

BACKGROUND: Acute kidney injury (AKI) is common in lupus nephritis (LN) and a risk factor for development of chronic kidney disease. In adults with LN, AKI severity correlates with the incidence of kidney failure and patient survival. Data on AKI outcomes in children with LN, particularly those requiring kidney replacement therapy (KRT), are limited. METHODS: A multicenter, retrospective cohort study was performed in children diagnosed between 2010 and 2019 with LN and AKI stage 3 treated with dialysis (AKI stage 3D). Descriptive statistics were used to characterize demographics, clinical data, and kidney biopsy findings; treatment data for LN were not included. Logistic regression was used to examine the association of these variables with kidney failure. RESULTS: Fifty-nine patients (mean age 14.3 years, 84.7% female) were identified. The most common KRT indications were fluid overload (86.4%) and elevated blood urea nitrogen/creatinine (74.6%). Mean follow-up duration was 3.9 ± 2.9 years. AKI recovery without progression to kidney failure occurred in 37.3% of patients. AKI recovery with later progression to kidney failure occurred in 25.4% of patients, and there was no kidney recovery from AKI in 35.6% of patients. Older age, severe (> 50%) tubular atrophy and interstitial fibrosis, and National Institutes of Health (NIH) chronicity index score > 4 on kidney biopsy were associated with kidney failure. CONCLUSIONS: Children with LN and AKI stage 3D have a high long-term risk of kidney failure. Severe tubular atrophy and interstitial fibrosis at the time of AKI, but not AKI duration, are predictive of kidney disease progression. A higher resolution version of the Graphical abstract is available as Supplementary information.

Ambulatory Blood Pressure Patterns in Children, Adolescents, and Young Adults With Childhood-Onset Systemic Lupus Erythematosus and Normal Office Blood Pressure.

OBJECTIVE: Cardiovascular disease is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). Hypertension (HTN) and nondipping are modifiable risk factors for cardiovascular disease. Limited studies are available for childhood-onset SLE (cSLE). We aimed to assess ambulatory blood pressure monitoring (ABPM) pattern in cSLE patients with normal office blood pressure. METHODS: This cross-sectional single-center study enrolled 25 patients with cSLE, normal office blood pressure and normal renal function. We performed 24-hour ABPM and echocardiography to assess end-organ damage. Descriptive statistics were obtained, and comparison of variables using Fisher exact test, t test, and Mann-Whitney U test was performed to identify potential risk factors for nondipping. RESULTS: Of the 25 patients, 22 were women; the median age was 18 years (interquartile range, 16-20 years). Median SLE duration was 4.2 years (interquartile range, 2.9-8.1 years); 18 patients had a history of lupus nephritis (LN). Four patients, 3 of whom had LN, had masked HTN. Fourteen patients (56%) were nondipping. The majority of patients without LN (71%) had a nondipping profile. Echocardiography was done for 15 patients. Left ventricular mass index, relative wall thickness, and ejection fraction were normal in all patients. Ambulatory blood pressure monitoring results led to changes in therapy in 5 patients. CONCLUSION: Our data provide evidence of high prevalence of nondipping and masked HTN in patients with cSLE, even in patients without LN. Identifying ABPM abnormalities in these patients could potentially improve outcomes.

COVID-19 in Children With Kidney Disease: A Report of 2 Cases.

The presentation of novel coronavirus disease 2019 (COVID-19) in children with kidney disease is largely unknown. We report on 2 children with kidney disease not receiving long-term immunosuppression who were hospitalized due to COVID-19. The first case is an infant with end-stage kidney disease secondary to bilateral cystic dysplastic kidneys and posterior urethral valves receiving peritoneal dialysis, with a history of prematurity previously requiring mechanical ventilation in the neonatal intensive care unit, who presented with fever, hypertension, and emesis. He had no respiratory symptoms and recovered with supportive care. His hypertension was managed well with amlodipine. The second case is a child with steroid-sensitive nephrotic syndrome who presented with a relapse of nephrotic syndrome with concurrent peritonitis and sepsis caused by Streptococcus agalactiae. He was treated with antibiotics and prophylactic anticoagulation therspy. Steroid therapy was initiated after 48 hours of antibiotic therapy. Neither child required mechanical ventilation or developed COVID-19-related multisystem inflammatory syndrome.