| Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children. | |
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MedLine Citation:
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PMID: 19808243 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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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Authors:
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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:
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Type: Journal Article Date: 2009-09-24 |
Journal Detail:
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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:
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Created Date: 2009-10-07 Completed Date: 2010-01-07 Revised Date: 2010-10-04 |
Medline Journal Info:
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Nlm Unique ID: 101271570 Medline TA: Clin J Am Soc Nephrol Country: United States |
Other Details:
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Languages: eng Pagination: 1593-600 Citation Subset: IM |
Affiliation:
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Department of Pediatric Nephrology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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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:
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0/Intracellular Signaling Peptides and Proteins; 0/Membrane Proteins; 0/NPHS2 protein; 0/nephrin |
| Comments/Corrections | |
Comment In:
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Clin J Am Soc Nephrol. 2009 Oct;4(10):1547-8
[PMID:
19808239
]
Clin J Am Soc Nephrol. 2009 Oct;4(10):1549-50 [PMID: 19808240 ] |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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