spieceare[bracketed].---------------------------------------------------------------------INTRODUCTIONHereditarynephritisisadisparategroupofoftenill-definedconditionsthataresimilaronlyinthattheyruninfamiliesandpresentmanydiagnosticdifficulties.Becausetheincidenceanddiversityofsuchdiseasesarenotgenerallyrecognized,ounselingarelost.monandbestknownhereditarynephritisisAlportsyndrome.Inthischapter,onimmuneglomerulonephritischaracterizedultrastructurallybyirregularthickening,thinning,andlamellationoftheglomerularbasementmembrane(GBM).ur.Theseincludehearingloss,variousoculardefects,abnormalitiesofplateletnumberandfunction,granulocyteinclusions,andesophagealandgenitalleiomyomatosis(tumors).Weregardnonrenalfeaturesashelpfuldiagnosticpointersinsomekindreds,althoughtheyarenotessentialtothediagnosis.
全文预览
