Document Detail

Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children.
MedLine Citation:
PMID:  19808243     Owner:  NLM     Status:  MEDLINE    
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.
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
Related Documents :
15768793 - T-cell repertoire in the tonsils of patients with iga nephropathy.
19393133 - Influence of cox-2-induced pge2 on the initiation and progression of tail regeneration ...
2065503 - Renal tubular dysfunction in primary sjögren's syndrome: clinical studies in 27 patients.
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    
Department of Pediatric Nephrology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Absorptiometry, Photon
Bone Density
Child, Preschool
Eye Diseases / etiology
Intracellular Signaling Peptides and Proteins / genetics
Kidney / physiopathology
Membrane Proteins / genetics
Nephrosis, Lipoid / complications,  physiopathology,  therapy*
Reg. No./Substance:
0/Intracellular Signaling Peptides and Proteins; 0/Membrane Proteins; 0/NPHS2 protein; 0/nephrin
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 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Scaling of measured glomerular filtration rate in kidney donor candidates by anthropometric estimate...
Next Document:  Is maximum conservative management an equivalent treatment option to dialysis for elderly patients w...