Thin basement membrane disease with membranoproliferative glomerulonephritis in a 13-year-old girl

Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima, Japan.
Pediatrics International (Impact Factor: 0.73). 07/2002; 44(3):321-3. DOI: 10.1046/j.1442-200X.2002.01540.x
Source: PubMed
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Thin basement membrane nephropathy (TBMN) is a glomerular disorder characterized clinically by isolated hematuria and pathologically by diffuse thinning of the glomerular basement membrane (GBM) on ultrastructural examination. The pathologic diagnosis of TBMN is problematic, in part because of the wide range of GBM thicknesses in the normal population. GBM thickness varies with age, sex, and the different methods of tissue preparation and measurement. In addition, there are no standardized diagnostic criteria defining the degree or extent of GBM thinning required for the diagnosis of TBMN. GBM thinning is often seen in other glomerulopathies, where it may represent an overlap with TBMN or may be secondary to GBM damage and remodeling. Importantly, TBMN must be differentiated from the GBM thinning seen in some renal biopsy specimens from boys and female heterozygotes with X-linked Alport syndrome because of the very different prognoses of these two conditions.
    Seminars in Nephrology 06/2005; 25(3):149-58. · 2.94 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Many reports confirm that thin basement membrane nephropathy (TBMN) commonly occurs together with other glomerular diseases such as minimal change glomerulonephritis, diabetes, membranous nephropathy, immunoglobulin (Ig)A glomerulonephritis, and focal segmental glomerulosclerosis. We postulate 3 explanations for these observations. The association of minimal change glomerulonephritis with TBMN probably is artifactual whereas the association with diabetes and membranous glomerulonephritis probably is coincidental. However, the link between TBMN and IgA disease and focal segmental glomerulosclerosis may be pathogenetic. Clinical evidence indicates that the presence of an associated glomerulopathy significantly worsens the prognosis of TBMN. Thus, patients with TBMN and another glomerular lesion usually have more marked proteinuria, and are more likely to have hypertension and renal insufficiency. The frequency of another glomerulopathy in patients with TBMN means that all patients in whom TBMN is suspected but who have heavy proteinuria or renal insufficiency should undergo a renal biopsy examination. However, there is no evidence that TBMN alters the prognosis of another glomerulopathy, and, in particular, patients with TBMN and IgA disease do not have different clinical features or a worse prognosis than those with IgA disease alone.
    Seminars in Nephrology 06/2005; 25(3):176-9. · 2.94 Impact Factor