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Alport IF

T. David Bourne, MD renal pathologist and neuropathologist at arkana laboratories
By David Bourne, MD

Feb 14, 2018

Alport Syndrome Detected with IF
Camera information: Calibration= 6.450 Microns per pixel; Capture format= 1392 x 1040, Full Frame HQ; Gamma= 0.99; Gain= 1.0 x; Exposure= 760.0 ms; Auto exposure= Off; Image type= Colour; Shading= (None); Sharpening= Low; Black clip= 0; White clip= 255; Microscope information:

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.

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