title: Pathological similarities between low birth weight-related nephropathy and nephropathy associated with mitochondrial cytopathy.
abstract: BACKGROUND
  Individuals born with a low birth weight (LBW) have a higher risk of developing kidney dysfunction during their lifetime and sometimes exhibit focal segmental glomerulosclerosis (FSGS) lesions in their glomeruli. We herein try to obtain other pathological characteristics of LBW-related nephropathy.
  
  
  METHODS
  We retrospectively evaluated the renal pathology of four patients demonstrating FSGS with a history of LBW. Two mitochondrial cytopathy patients were also analyzed. DNA mutations were surveyed using a PCR-Luminex assay.
  
  
  RESULTS
  In all four FSGS patients with a history of LBW, focal segmental glomerulosclerosis were detected. Interestingly, granular swollen epithelial cells (GSECs), which have previously been reported exclusively in patients with mitochondrial cytopathy, were also observed in the distal tubules and/or collecting ducts of all four patients with a history of low birth weight in this study. Electron microscopy revealed that these granular swollen epithelial cells included an increased number of enlarged mitochondria. Furthermore, cytochrome c oxidase subunit IV staining of patients with a history of low birth weight and patients with mitochondrial DNA mutations showed unbalanced expression patterns in glomeruli and a part of tubular cells. However, no mitochondrial gene mutations were detected in any of our four patients with low birth weight-related nephropathy.
  
  
  CONCLUSIONS
  This is the first report to show the pathological similarities not only in glomeruli but also tubuli between nephropathy with a LBW history and nephropathy with mitochondrial cytopathy.
  The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_181.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Imasawa
authors.forename: Masashi
authors.initials: M
authors.lastname: Tanaka
authors.forename: Naoki
authors.initials: N
authors.lastname: Maruyama
authors.forename: Takehiko
authors.initials: T
authors.lastname: Kawaguchi
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Yamaguchi
authors.forename: Rodrigue
authors.initials: R
authors.lastname: Rossignol
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kitamura
authors.forename: Motonobu
authors.initials: M
authors.lastname: Nishimura
mesh_terms: D000328:Adult; D004272:DNA, Mitochondrial / Q000737:chemistry / Q000235:genetics; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000473:pathology*; D006801:Humans; D007230:Infant, Low Birth Weight; D007231:Infant, Newborn; D007625:Kearns-Sayre Syndrome / Q000473:pathology*; D007678:Kidney Glomerulus / Q000473:pathology; D008297:Male; D008875:Middle Aged; D017240:Mitochondrial Myopathies / Q000473:pathology*; D009154:Mutation; D011507:Proteinuria; D012189:Retrospective Studies; D017422:Sequence Analysis, DNA
chemical_list: D004272:DNA, Mitochondrial