S4 Ep80: NephMadness 2023: CKD and Transitions of Care
The Cribsiders
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The Pathophysiology of Al Port Syndrome
A normal creatinine does not exclude a diagnosis of hereditary nephritis or Al Port syndrome. The glomerular basement membrane is composed of collagen and specifically type 4 collagen. It can inappropriately let red blood cells through when normally these shouldn't be able to filter through the matureglomerular basement membranes. Or you can have abnormalities where the protein is produced but it doesn't fold properly or form the trimer properly.
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