Document Detail


Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children.
MedLine Citation:
PMID:  19808243     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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; Elena N Levtchenko
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Publication Detail:
Type:  Journal Article     Date:  2009-09-24
Journal Detail:
Title:  Clinical journal of the American Society of Nephrology : CJASN     Volume:  4     ISSN:  1555-905X     ISO Abbreviation:  Clin J Am Soc Nephrol     Publication Date:  2009 Oct 
Date Detail:
Created Date:  2009-10-07     Completed Date:  2010-01-07     Revised Date:  2013-05-31    
Medline Journal Info:
Nlm Unique ID:  101271570     Medline TA:  Clin J Am Soc Nephrol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1593-600     Citation Subset:  IM    
Affiliation:
Department of Pediatric Nephrology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
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MeSH Terms
Descriptor/Qualifier:
Absorptiometry, Photon
Adolescent
Adult
Biopsy
Bone Density
Child
Child, Preschool
Eye Diseases / etiology
Female
Fertility
Humans
Infant
Intracellular Signaling Peptides and Proteins / genetics
Kidney / physiopathology
Male
Membrane Proteins / genetics
Nephrosis, Lipoid / complications,  physiopathology,  therapy*
Recurrence
Chemical
Reg. No./Substance:
0/Intracellular Signaling Peptides and Proteins; 0/Membrane Proteins; 0/NPHS2 protein; 0/nephrin
Comments/Corrections
Comment In:
Clin J Am Soc Nephrol. 2009 Oct;4(10):1549-50   [PMID:  19808240 ]
Clin J Am Soc Nephrol. 2009 Oct;4(10):1547-8   [PMID:  19808239 ]

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