Long-Term Outcome of Biopsy-Proven, Frequently Relapsing Minimal-Change Nephrotic Syndrome in Children

Abstract

Background and objectives: Frequently relapsing and steroid-dependent minimal-change nephrotic syndrome (MCNS) that originates in childhood can persist after puberty in >20% of patients. These patients require immunosuppressive treatment during several decades of their life. We examined long-term adverse effects of persistent nephrotic syndrome and immunosuppressive medications, focusing on renal function, growth, obesity, osteoporosis, hypertension, ocular complications, and fertility in adult patients with biopsy-proven childhood-onset MCNS. Molecular analysis was performed to evaluate a possible association of a complicated course of MCNS with podocyte gene mutations.Design, setting, participants, & measurements: We performed a prospective clinical examination of 15 adult patients that included serum and urine analysis; dual-energy x-ray absorptiometry; ophthalmologic examination; semen examination; and molecular analysis of NPHS1, NPHS2, CD2AP, and ACTN4 genes.Results: All patients had normal GFR. Most frequent long-term complications were hypertension (in seven of 15 patients) and osteoporosis in one third of patients. Oligozoospermia was found in one patient, reduced sperm motility in four of eight patients, and teratozoospermia in six of eight patients. Ophthalmologic examination revealed myopia in 10 of 15 patients and cataract in three of 15 patients.Conclusions: Children with MCNS that persists after puberty are at risk for complications such as osteoporosis, hypertension, cataract, and sperm abnormalities. Our study underscores a need for more effective and less toxic therapies for relapsing MCNS.

Long-Term Outcome of Children with Steroid-Sensitive Idiopathic Nephrotic Syndrome

“…The occurrence of growth retardation or excess weight, despite the administration of corticosteroids on an alternateday basis, are indications for the use of steroid-sparing agents. This may explain why nowadays such complications are fairly rare, as noted by Kyrieleis et al (6). Osteoporosis was observed in four of 11 evaluated patients.…”

“…The high incidence of hypertension found in the series of Kyrieleis et al (6) may be explained by the fact that most of them were still receiving steroid therapy and/or calcineurin inhibitors. Knowing the long-term effects of corticosteroids on the cardiovascular system, it will be important to know whether these patients' hypertension remains after stopping the treatments for the nephrotic syndrome.…”

“…The patients described by Kyrieleis et al (6) received cyclosporine during 7 to 174 mo. The authors found that all patients had normal creatinine clearance as measured by the Cockcroft-Gault formula.…”

Long-term results of children diagnosed with idiopathic nephrotic syndrome; single center experience

“…In adult studies, it has been reported that corticosteroids lead to bone loss and increase fracture risk in a dose-dependent fashion (35). Some studies have reported that osteoporosis develops with a rate of 1/3 in children diagnosed with frequently recurring NS (36). However, the rate of usage of calcium is considerably low in these studies (20%).…”

Efficacy and Safety of Treatment with Rituximab for Difficult Steroid-Resistant and -Dependent Nephrotic Syndrome

“…A lthough many patients with idiopathic nephrotic syndrome have a satisfactory long-term course, 40% show steroid dependence (SDNS) and 10 to 15% are steroid resistant (SRNS) (1,2). The former are at risk of steroid toxicity, whereas the latter show a complicated course and may progress to end-stage renal disease (3,4).…”