Linked Life Data

12089392

Source:http://linkedlifedata.com/resource/pubmed/id/12089392

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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
7
pubmed:dateCreated
2002-6-28
pubmed:abstractText
A plasma factor displaying permeability activity in vitro and possibly determining proteinuria has been hypothesized in idiopathic focal segmental glomerulosclerosis (FSGS). In vitro permeability activity (P(alb)) was determined in sera of five patients with autosomal recessive steroid-resistant nephrotic syndrome (NPHS2), an inherited condition indistinguishable from idiopathic FSGS on clinical grounds, but in which proteinuria is determined by homozygous mutations of podocin, a key component of the glomerular podocyte. All patients had presented intractable proteinuria with nephrotic syndrome; four developed renal failure and received a renal allograft. For comparison, sera from 31 children with nephrotic syndrome were tested. Pretransplant P(alb) was high in all cases (mean 0.81 +/- 0.06), equivalent to levels observed in idiopathic FSGS. Overall, P(alb) did not correlate with proteinuria. The posttransplant outcome was complicated in two patients by recurrence of proteinuria after 10 and 300 d, respectively, that responded to plasmapheresis plus cyclophosphamide. P(alb) levels were high at the time of the recurrence episodes and steadily decreased after plasmapheresis, to reach normal levels in the absence of proteinuria after the seventh cycle. In an attempt to explain high P(alb) in these patients, putative inhibitors of the permeability activity were studied. Coincubation of serum with homologous nephrotic urine reduced P(alb) to 0, whereas normal urine did not determine any change, which suggests loss of inhibitory substances in nephrotic urine. The urinary levels of the serum P(alb) inhibitors apo J and apo E were negligible in all cases, thus suggesting that other urinary inhibitors were responsible for the neutralizing effect. These data indicate that P(alb) is high in NPHS2, probably resulting from loss of inhibitors in urine. Lack of correlation of P(alb) with proteinuria suggests a selective loss of inhibitors. As in idiopathic FSGS, proteinuria may also recur after renal transplantation in NPHS2 patients, and post-transplant proteinuria is associated with high P(alb). The relationship between elevated P(alb) and proteinuria in NPHS2 remains to be determined.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
1046-6673
pubmed:author
pubmed:issnType
Print
pubmed:volume
13
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
1946-52
pubmed:dateRevised
2008-11-21
pubmed:meshHeading
pubmed-meshheading:12089392-Adolescent, pubmed-meshheading:12089392-Adult, pubmed-meshheading:12089392-Aged, pubmed-meshheading:12089392-Blood Physiological Phenomena, pubmed-meshheading:12089392-Child, pubmed-meshheading:12089392-Child, Preschool, pubmed-meshheading:12089392-Cohort Studies, pubmed-meshheading:12089392-Female, pubmed-meshheading:12089392-Humans, pubmed-meshheading:12089392-Intracellular Signaling Peptides and Proteins, pubmed-meshheading:12089392-Kidney Glomerulus, pubmed-meshheading:12089392-Kidney Transplantation, pubmed-meshheading:12089392-Male, pubmed-meshheading:12089392-Membrane Proteins, pubmed-meshheading:12089392-Middle Aged, pubmed-meshheading:12089392-Mutation, pubmed-meshheading:12089392-Nephrotic Syndrome, pubmed-meshheading:12089392-Permeability, pubmed-meshheading:12089392-Serum Albumin, pubmed-meshheading:12089392-Steroids, pubmed-meshheading:12089392-Treatment Outcome, pubmed-meshheading:12089392-Urine
pubmed:year
2002
pubmed:articleTitle
Serum glomerular permeability activity in patients with podocin mutations (NPHS2) and steroid-resistant nephrotic syndrome.
pubmed:affiliation
Laboratorio di Fisiopatologia dell'Uremia, Istituto G. Gaslini, Largo G. Gaslini 5, 16148 Genoa, Italy.
pubmed:publicationType
Journal Article, Research Support, Non-U.S. Gov't