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February 14, 2018

Alport IF

Alport Syndrome Detected with IF

In patients suspected of having Alport syndrome, immunofluorescence staining can be used to detect abnormal expression of various alpha chains of type IV collagen. This biopsy from a young girl with hematuria shows discontinuous staining along Bowman’s capsule and along the glomerular capillary loops for the alpha 5 chain of type IV collagen (green). In a female patient, this mosaic pattern of decreased or absent staining is suspicious for heterozygous X-linked Alport syndrome. Intact staining of the alpha 2 chains (red) serves as a positive internal control for tissue integrity.