Long-Term Outcome of Biopsy-Proven, Frequently Relapsing Minimal-Change Nephrotic Syndrome in Children
Open Access
- 1 October 2009
- journal article
- Published by Ovid Technologies (Wolters Kluwer Health) in Clinical Journal of the American Society of Nephrology
- Vol. 4 (10), 1593-1600
- https://doi.org/10.2215/cjn.05691108
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.Keywords
This publication has 44 references indexed in Scilit:
- Potassium Channel and NKCC Cotransporter Involvement in Ocular Refractive Control MechanismsPLOS ONE, 2008
- Genetics of focal segmental glomerulosclerosisPediatric Nephrology, 2007
- Specific Activation of the Glucocorticoid Receptor and Modulation of Signal Transduction Pathways in Human Lens Epithelial CellsInvestigative Ophthalmology & Visual Science, 2007
- Glucocorticoid-Induced Osteoporosis in Children: Impact of the Underlying DiseasePEDIATRICS, 2007
- Ocular Findings in Japanese Children with Nephrotic Syndrome Receiving Prolonged Corticosteroid TherapyOphthalmologica, 2006
- Children with Steroid-sensitive Nephrotic Syndrome Come of Age: Long-term OutcomeThe Journal of Pediatrics, 2005
- Nephrin gene (NPHS1) in patients with minimal change nephrotic syndrome (MCNS)Kidney International, 2004
- Long-term linear growth of children with severe steroid-responsive nephrotic syndromePediatric Nephrology, 2003
- Growth in steroid-responsive nephrotic syndrome: a study of 85 pediatric patientsPediatric Nephrology, 2003
- Prediction of Creatinine Clearance from Serum CreatinineNephron, 1976