Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Practice Guideline
. 2013 Jan;28(1):5-11.
doi: 10.1007/s00467-012-2138-4. Epub 2012 Mar 30.

Clinical practice recommendations for the treatment of Alport syndrome: a statement of the Alport Syndrome Research Collaborative

Affiliations
Practice Guideline

Clinical practice recommendations for the treatment of Alport syndrome: a statement of the Alport Syndrome Research Collaborative

Clifford E Kashtan et al. Pediatr Nephrol. 2013 Jan.

Abstract

We present clinical practice recommendations for the treatment of children with Alport syndrome who are not enrolled in clinical trials. Our goal is to promote early initiation of a standard therapeutic approach that will facilitate assessment of the safety and efficacy of the protocol. The treatment protocol is based on the reduction of proteinuria, intraglomerular pressure, and renal fibrosis via interference with the renin-angiotensin-aldosterone system.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Algorithm for identifying children with familial hematuria (Alport syndrome or hematuria with thin glomerular basement membranes) who are candidates for intervention. IHC immunohistochemistry; EM electron microscopy; GBM glomerular basement membrane *Depending upon availability and local practice

Similar articles

Cited by

References

    1. Jais JP, Knebelmann B, Giatras I, De Marchi M, Rizzoni G, Renieri A, Weber M, Gross O, Netzer K-O, Flinter F, Pirson Y, Verellen C, Wieslander J, Persson U, Tryggvason K, Martin P, Hertz JM, Schroder C, Sanak M, Krejcova S, Carvalho MF, Saus J, Antignac C, Smeets H, Gubler MC. X-linked Alport syndrome: natural history in 195 families and genotype-phenotype correlations in males. J Am Soc Nephrol. 2000;11:649–657. - PubMed
    1. Gross O, Netzer KO, Lambrecht R, Seibold S, Weber M. Meta-analysis of genotype-phenotype correlation in X-linked Alport syndrome: impact on clinical counseling. Nephrol Dial Transpl. 2002;17:1218–1227. doi: 10.1093/ndt/17.7.1218. - DOI - PubMed
    1. Bekheirnia MR, Reed B, Gregory MC, McFann K, Shamshirsaz AA, Masoumi A, Schrier RW. Genotype-phenotype correlation in X-linked Alport syndrome. J Am Soc Nephrol. 2010;21:876–883. doi: 10.1681/ASN.2009070784. - DOI - PMC - PubMed
    1. Rheault MN, Kren SM, Hartich LA, Wall M, Thomas W, Mesa HA, Avner P, Lees GE, Kashtan CE, Segal Y. X-inactivation modifies disease severity in female carriers of murine X-linked Alport syndrome. Nephrol Dial Transplant. 2010;25:764–769. doi: 10.1093/ndt/gfp551. - DOI - PMC - PubMed
    1. Grunfeld J-P, Noel LH, Hafez S, Droz D. Renal prognosis in women with hereditary nephritis. Clin Nephrol. 1985;23:267–271. - PubMed

Publication types

LinkOut - more resources