Thin Basement Membrane Syndromeの臨床病理学的検討

Abstract

Clinical and pathologic findings are described in 10 children whose main abnormality was thin glomerular basement membrane (GBM). The subjects were aged 4-16 years, 5 males and 5 females. Six cases had family members with asymptomatic hematuria and the other 4 cases were sporadic. All the patients showed persistent microscopic hematuria with dysmorphic RBC in the urinary sediment, while renal functional impairment and deafness were not found in any of them. Six patients had episodes of macroscopic hematuria. Light and immunofluorescent microscopy indicated no characteristic glomerular changes, but electron microscopy revealed diffuse thinning of GBM. The measurements of GBM thickness were in the range of 141-211 nm. There were no electron dense deposits nor lamination of GBM, but located splitting of lamina densa was observed in 2 cases. It is suggested that for children with dysmorphic hematuria in the urinary sediment, ultrastructual examination is significant in the diagnosis of thin basement membrane syndrome.