A Clinicopathological Comparison Between Thin Basement Membrane Nephropathy And Alport Syndrome In Children

Objective: To analyze the difference in clinicopathological features between thin basement membrane nephropathy(TBMN) and Alport syndrome(AS) in children,To provide bases for differential diagnosis.Methods: The medical data of 16 children with TBMN and 26 children with AS were retrospectively studied,The outcome of TBMN and AS were followed.Results:(1)The gender difference is shown in TBMN and AS, The rate of male :female of TBMN was 1:1.29,but AS was 7.67:1.(2) The dominant clinicalmanifestation of TBMN was simple hematuria,but AS was hematuria accompanied proteinuria,which has significant difference(p<0.05).(3)1 case(11.11%) of TBMN and 11 cases(85.71%) of AS had poor vision,which has significant difference(p<0.05).(4)AS had higher levels of proteinuria than TBMN,which has significant difference(p<0.05).(5)6 cases(37.50%) of TBMN and 2 cases of AS(7.70%) combined with left renal veincompression,which has significant difference(p<0.05).(6)The most common pathological types of TBMN was glomerular minor lesion(53.33.00%),but AS was mesangial proliferative glomerulonephritis( 90.91%), which has significant difference(p <0.05).(7)No TBMN but 10 cases(45.45%)of AS showed infiltration of foamy cells in the interstitial, which has significant difference( p <0.05).(8)Under the electron microscope,16 cases(100.00%) of TBMN showed diffuse thin basement membrane,3 cases(15.79%)of AS showed thin basement membrane, 14 cases( 73.68%) of AS showed uneven thickness of the basement membrane,13 cases(68.42%)of AS showed lamination 、 laceration and reticular changes of basement membrane.(9) Indirect immunofluorescence of α3 、 α5 chains staining showed that 14 cases of( 100.00%) the TBMN and 3 cases(13.64%)of AS had positive reaction;19 cases(86.36%) of AS had negative or partial positive reaction。(10)1 case of TBMN and 4 cases of AS suffered renal insufficiency.Conclusions : Renal manifestations abnormal hearing 、pathological types 、 infiltration of foamy cells in the interstitial 、morphology of glomerular basement membrane can be used as the reference for identification of TBMN and AS. Thin Glomerular basement membrane and the positive reaction of indirect immunofluorescence detection can not rule out the diagnosis of Alport syndrome.