Linked Life Data

18000687

Source:http://linkedlifedata.com/resource/pubmed/id/18000687

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2008-1-14
pubmed:abstractText
A small fraction of patients with initial steroid-sensitive nephrotic syndrome (SSNS) develops late steroid resistance, i.e. a lack of remission after 4 weeks of relapse treatment despite previous response to steroids. The pathophysiological basis of late resistance and the long-term prognosis remain obscure. Fourteen out of 360 patients with SSNS who were seen in our department between 1954 and 2005 developed late resistance. Median age at onset of NS was 4 years and median duration of development of late resistance 4.6 months. Histology showed minimal-change (MC) nephropathy in six patients and focal segmental glomerulosclerosis (FSGS) in three patients on initial biopsy and four patients on repeat biopsies. Late resistance was treated with cyclophosphamide in five patients, cyclosporine A in three, and both drugs in one. Eight of these nine patients went into remission. All 14 patients maintained a stable kidney function during their period of observation. NPHS2 mutation analysis in eight patients revealed no pathogenic mutations, suggesting that late resistance is not typically associated with mutations in the NPHS2 gene. With respect to the clinical course, late resistance appears to resemble SSNS and is characterized by a favorable outcome.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0931-041X
pubmed:author
pubmed:issnType
Print
pubmed:volume
23
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
251-6
pubmed:meshHeading
pubmed:year
2008
pubmed:articleTitle
Clinical course and NPHS2 analysis in patients with late steroid-resistant nephrotic syndrome.
pubmed:affiliation
Division of Pediatric Nephrology, University Children's Hospital Heidelberg, Im Neuenheimer Feld 150, 69120, Heidelberg, Germany. schwaderer@freenet.de
pubmed:publicationType
Journal Article