title: Tubular proteinuria defined by a study of Dent's (CLCN5 mutation) and other tubular diseases.
abstract: Tubular proteinuria defined by a study of Dent's ( CLCN5 mutation) and other tubular diseases.
  
  
  BACKGROUND
  The term "tubular proteinuria" is often used interchangeably with "low molecular weight proteinuria" (LMWP), although the former implies a definite etiology. A specific quantitative definition of tubular proteinuria is needed, and we address this by studying five different renal disorders.
  
  
  METHODS
  Tubular proteinuria was assessed by measuring urinary retinol-binding protein (RBP), beta2-microglobulin (beta2M), alpha1-microglobulin (alpha1M), and albumin in 138 patients: 26 affected males and 24 female carriers of the X-linked syndrome "Dent's disease," 6 patients with other Fanconi syndromes, 17 with distal renal tubular acidosis (dRTA), 39 with glomerulonephritis (GN), and 26 with Chinese herbs nephropathy (CHN).
  
  
  RESULTS
  RBP was better than beta2M or alpha1M in identifying the tubular proteinuria of Dent's disease. Median urinary RBP levels in mg/mmol creatinine were: affected male Dent's, 18.2, N = 26; carrier female Dent's, 0. 30, N = 24; dRTA, 0.027, N = 17; GN, 0.077, N = 39; and normal adults, 0.0079, N = 61. Elevated urinary RBP (>0.017) and albumin < (10 x RBP) + 2 identified all patients with the LMWP of Dent's disease and clearly distinguished their LMWP from that of dRTA and GN. This is a quantitative definition of tubular proteinuria. Consistent with this definition, 80% of those patients with CHN who had an elevated RBP had tubular proteinuria. Urinary RBP and albumin in carriers of Dent's disease were strikingly correlated over a 100-fold range (R = 0.933).
  
  
  CONCLUSIONS
  The combination of elevated urinary RBP (>0.017) and albumin < (10 x RBP) + 2 (mg protein/mmol creatinine) is a quantitative definition of tubular proteinuria. Furthermore, our findings suggest that a shared defect in tubular RBP and albumin reuptake causes this form of proteinuria.
authors.affiliation: Department of Chemical Pathology, Chase Farm Hospitals NHS Trust, Enfield, Middlesex, England. agwnorden@compuserve.com
authors.forename: A G
authors.initials: AG
authors.lastname: Norden
authors.forename: S J
authors.initials: SJ
authors.lastname: Scheinman
authors.forename: M M
authors.initials: MM
authors.lastname: Deschodt-Lanckman
authors.forename: M
authors.initials: M
authors.lastname: Lapsley
authors.forename: J L
authors.initials: JL
authors.lastname: Nortier
authors.forename: R V
authors.initials: RV
authors.lastname: Thakker
authors.forename: R J
authors.initials: RJ
authors.lastname: Unwin
authors.forename: O
authors.initials: O
authors.lastname: Wrong
mesh_terms: D000293:Adolescent; D000328:Adult; D018118:Chloride Channels / Q000235:genetics*; D004365:Drugs, Chinese Herbal; D005260:Female; D005921:Glomerulonephritis / Q000235:genetics; D006417:Hematuria; D006801:Humans; D007674:Kidney Diseases / Q000139:chemically induced; D007684:Kidney Tubules / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D008970:Molecular Weight; D009154:Mutation; D011507:Proteinuria / Q000235:genetics*
chemical_list: C097503:CLC-5 chloride channel; D018118:Chloride Channels; D004365:Drugs, Chinese Herbal