Alport syndrome natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Natural History

Recognition of Alport’s syndrome based on the clinical presentation and distinguishing it from other causes of hematuria or extra renal manifestations is important because Alport’s syndrome usually carries a poor prognosis with inevitable progression to end-stage renal disease (ESRD).[1]

Complications

Prognosis

Onset of symptoms often starts during infancy in X-linked Alport’s syndrome; patients reach end-stage renal disease (ESRD) during adolescence.[1] All patients with Alport's syndrome eventually progress to ESRD.

Patients with autosomal recessive Alport’s syndrome sometimes progress less rapidly. Although they may still progress to ESRD early, they may reach ESRD during adult life.[1]

References

  1. 1.0 1.1 1.2 Savige J, Gregory M, Gross O, Kashtan C, Ding J, Flinter F (2013). "Expert guidelines for the management of Alport syndrome and thin basement membrane nephropathy". J Am Soc Nephrol. 24 (3): 364–75. doi:10.1681/ASN.2012020148. PMID 23349312.

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