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Structural-functional relationships in Alport syndrome

K H Kim1, Y Kim, M C Gubler

  • 1Department of Pediatrics, University of Minnesota, Minneapolis 55455, USA.

Journal of the American Society of Nephrology : JASN
|March 1, 1995
PubMed
Summary

Alport syndrome patients show altered glomerular basement membrane width and increased interstitial fibrosis, partially explaining reduced kidney function. Decreased glomerular capillary wall hydraulic conductivity may be a key factor in Alport syndrome renal dysfunction.

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Alport syndrome: abnormalities of type IV collagen genes and proteins.

Renal failure·2000

Area of Science:

  • Nephrology
  • Pathology
  • Renal Medicine

Background:

  • Alport syndrome is a genetic disorder affecting collagen IV, crucial for kidney structure.
  • Renal dysfunction in Alport syndrome is complex and not fully understood structurally.
  • Morphometric analysis can reveal structural changes underlying kidney disease.

Purpose of the Study:

  • To investigate the structural basis of renal dysfunction in Alport syndrome.
  • To correlate renal morphometric parameters with creatinine clearance.
  • To compare structural findings in Alport syndrome with controls and diabetic patients.

Main Methods:

  • Renal morphometric analysis was conducted on kidney biopsies from Alport syndrome patients and controls.
  • Measurements included glomerular basement membrane width, mesangial volume fraction, GBM surface density, and interstitial volume fraction.

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  • Glomerular sclerosis and glomerular number were also assessed.

    • Main Results:

    • Alport syndrome patients exhibited skewed glomerular basement membrane width distribution and increased cortical interstitial volume fraction.
    • Mesangial volume fraction and peripheral GBM surface density showed inverse correlations with creatinine clearance.
    • Cortical interstitial volume fraction and glomerular sclerosis were highly inversely correlated with creatinine clearance, but less so than in diabetic patients.

    Conclusions:

    • Structural changes like interstitial fibrosis and GBM alterations partially explain reduced kidney function in Alport syndrome.
    • The observed reductions in creatinine clearance are disproportionately greater in Alport syndrome compared to diabetes with similar structural changes.
    • Decreased glomerular capillary wall hydraulic conductivity is hypothesized as a potential explanation for the observed discrepancies.