CONTEXT: Diffuse thinning of the glomerular basement membrane (GBM) is the ultrastructural diagnostic criterion of thin basement membrane nephropathy (TBMN). However, there is no consensus regarding what diagnosis should be made if the attenuation is segmental. OBJECTIVE: To develop a diagnostic approach to TBMN in cases with segmental GBM thinning. DESIGN: We compared the diagnostic sensitivities of 2 methods used for the quantitative expression of GBM width in a consecutive series of renal biopsies from 26 patients (median age, 36 years; range, 15 to 59 years) with dysmorphic hematuria (a variable degree of proteinuria in 19 patients), a thin GBM, and absence of other renal disease. The harmonic GBM width was determined from orthogonal intercepts, and the actual width in the thinnest loops was obtained by direct measurement. The GBM attenuation was categorized into diffuse or segmental types by conventional inspection. RESULTS: Segmental TBMN accounted for one third of the series. In neither type did the male patients have a higher harmonic mean GBM width than the female patients. Focal-global glomerulosclerosis was more common in diffuse TBMN. The laborious orthogonal intercept method proved insensitive for the verification of segmental TBMN, whereas the much simpler direct measurement technique captured all the cases. CONCLUSIONS: A considerable number of patients with TBMN display segmental GBM attenuation. Because the routine criterion excludes these cases from the diagnosis, we propose to define TBMN as a clinicopathologic entity of dysmorphic hematuria and a diffusely or segmentally thinned GBM confirmed by the direct measurement technique.
CONTEXT: Diffuse thinning of the glomerular basement membrane (GBM) is the ultrastructural diagnostic criterion of thin basement membrane nephropathy (TBMN). However, there is no consensus regarding what diagnosis should be made if the attenuation is segmental. OBJECTIVE: To develop a diagnostic approach to TBMN in cases with segmental GBM thinning. DESIGN: We compared the diagnostic sensitivities of 2 methods used for the quantitative expression of GBM width in a consecutive series of renal biopsies from 26 patients (median age, 36 years; range, 15 to 59 years) with dysmorphic hematuria (a variable degree of proteinuria in 19 patients), a thin GBM, and absence of other renal disease. The harmonic GBM width was determined from orthogonal intercepts, and the actual width in the thinnest loops was obtained by direct measurement. The GBM attenuation was categorized into diffuse or segmental types by conventional inspection. RESULTS: Segmental TBMN accounted for one third of the series. In neither type did the male patients have a higher harmonic mean GBM width than the female patients. Focal-global glomerulosclerosis was more common in diffuse TBMN. The laborious orthogonal intercept method proved insensitive for the verification of segmental TBMN, whereas the much simpler direct measurement technique captured all the cases. CONCLUSIONS: A considerable number of patients with TBMN display segmental GBM attenuation. Because the routine criterion excludes these cases from the diagnosis, we propose to define TBMN as a clinicopathologic entity of dysmorphic hematuria and a diffusely or segmentally thinned GBM confirmed by the direct measurement technique.
Authors: Youli Wang; Kathleen O Heilig; Andrew W Minto; Shenglin Chen; Minghui Xiang; David A Dean; Richard C Geiger; Anthony Chang; Dimitrina D Pravtcheva; Martin Schlimme; Dilip K Deb; Ying Wang; Charles W Heilig Journal: Lab Invest Date: 2009-11-16 Impact factor: 5.662