A clinicopathological study of Alport syndrome and detection of type Ⅳ collagen chains in Alport patients

摘要

Conclusion Alport syndrome (AS) is a hetero- geneous hereditary disease characterized by progress- sive hematuric nephritis with or without sensorineural hearing loss and ovular defects. Ultrastructural alterations of GBM are helpful to the diagnosis of AS. IIF study suggests that type Ⅳ collagen in basement membrane of AS was abnormal and IlF study of type Ⅳ collagen chains distribution is useful for confirming the diagnosis of AS. Objective To summarize the clinical and patho- logical findings of Alport syndrome (AS), detect the distribution of type Ⅳ collagen within basement mem- brane of patients with AS and evaluate the diagnostic value of indirect immunofluorescence (ilF) study of type Ⅳcollagen in AS.