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This Article Full Text Full Text (PDF) Press Release All Versions of this Article: CJN.05691108v1 4/10/1593    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Google Scholar Articles by Kyrieleis, H. A.C. Articles by Levtchenko, E. N. PubMed PubMed Citation Articles by Kyrieleis, H. A.C. Articles by Levtchenko, E. N. Related Collections Related Articles

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

Henriette A.C. Kyrieleis^*, Marije M. Löwik, Ilse Pronk, Hans R.M. Cruysberg, Jan A.M. Kremer, Wim J.G. Oyen^||, Bert L.P. van den Heuvel^*,,¶, Jack F.M. Wetzels^**, and Elena N. Levtchenko^*,¶

^* Department of Pediatric Nephrology, Laboratory of Pediatrics and Neurology, Department of Ophthalmology, Department of Gynecology and Obstetrics, ^|| Department of Nuclear Medicine, and ^** Department of Nephrology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands; and ^¶ Department of Pediatric Nephrology, University Hospitals Leuven, Leuven, Belgium

Correspondence: Dr. Elena N. Levtchenko,Department of Pediatric Nephrology, University Hospital Leuven, Herestraat 49, 3000 Leuven, Belgium. Phone: +32-16-343827; Fax: +32-16-343842; E-mail: elena.levtchenko{at}uzleuven.be

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.

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