Guidelines for Genetic Testing and Management of Alport Syndrome


Por: Savige, J, Lipska-Zietkiewicz, BS, Watson, E, Hertz, JM, Deltas, C, Mari, F, Hilbert, P, Plevova, P, Byers, P, Cerkauskaite, A, Gregory, M, Cerkauskiene, R, Ljubanovic, DG, Becherucci, F, Errichiello, C, Massella, L, Aiello, V, Lennon, R, Hopkinson, L, Koziell, A, Lungu, A, Rothe, HM, Hoefele, J, Zacchia, M, Martic, TN, Gupta, A, van Eerde, A, Gear, S, Landini, S, Palazzo, V, al-Rabadi, L, Claes, K, Corveleyn, A, Van Hoof, E, van Geel, M, Williams, M, Ashton, E, Belge, H, Ars, E, Bierzynska, A, Gangemi, C, Renieri, A, Storey, H, Flinter, F

Publicada: 1 ene 2022 Ahead of Print: 1 dic 2021
Resumen:
Genetic testing for pathogenic COL4A3?5 variants is usually undertaken to investigate the cause of persistent hematuria, especially with a family history of hematuria or kidney function impairment. Alport syndrome experts now advocate genetic testing for persistent hematuria, even when a heterozygous pathogenic COL4A3 or COL4A4 is suspected, and cascade testing of their first-degree family members because of their risk of impaired kidney function. The experts recommend too that COL4A3 or COL4A4 heterozygotes do not act as kidney donors. Testing for variants in the COL4A3?COL4A5 genes should also be performed for persistent proteinuria and steroid-resistant nephrotic syndrome due to suspected inherited FSGS and for familial IgA glomerulonephritis and kidney failure of unknown cause.

Filiaciones:
Savige, J:
 Univ Melbourne, Dept Med Melbourne Hlth & Northern Hlth, Parkville, Vic, Australia

Lipska-Zietkiewicz, BS:
 Med Univ Gdansk, Ctr Rare Dis, Gdansk, Poland

 Med Univ Gdansk, Clin Genet Unit, Gdansk, Poland

Watson, E:
 North Bristol Trust, South West Genet Lab Hub, Bristol, Avon, England

Hertz, JM:
 Odense Univ Hosp, Dept Clin Genet, Odense, Denmark

Deltas, C:
 Univ Cyprus, Ctr Excellence Biobanking & Biomed Res, Med Sch, Nicosia, Cyprus

Mari, F:
 Univ Siena, Dept Med Biotechnol, Med Genet, Siena, Italy

Hilbert, P:
 Inst Pathol & Genet, Dept Biol Mol, Gosselies, Belgium

Plevova, P:
 Univ Hosp Ostrava, Dept Med Genet, Ostrava, Czech Republic

 Univ Hosp Ostrava, Dept Biomed Sci, Ostrava, Czech Republic

Byers, P:
 Univ Washington, Dept Pathobiol, Seattle, WA 98195 USA

 Univ Washington, Dept Med Med Genet, Seattle, WA 98195 USA

Cerkauskaite, A:
 Vilnius Univ, Inst Biomed Sci, Fac Med, Vilnius, Lithuania

Gregory, M:
 Univ Utah Hlth, Dept Med, Div Nephrol, Salt Lake City, UT USA

Cerkauskiene, R:
 Vilnius Univ, Inst Clin Med, Clin Pediat, Fac Med, Vilnius, Lithuania

Ljubanovic, DG:
 Univ Zagreb, Dubrava Univ Hosp, Sch Med, Dept Pathol, Zagreb, Croatia

Becherucci, F:
 Meyer Childrens Univ Hosp, Nephrol Unit, Florence, Italy

Errichiello, C:
 Meyer Childrens Univ Hosp, Nephrol Unit, Florence, Italy

Massella, L:
 Bambino Gesu Pediat Hosp, Div Nephrol & Dialysis, Rome, Italy

Aiello, V:
 Univ Bologna, S Orsola Hosp, Dialysis & Renal Transplant Unit, Dept Expt Diagnost & Specialty Med, Bologna, Italy

Lennon, R:
 Univ Manchester, Wellcome Ctr Cell Matrix Res, Fac Biol Med & Hlth, Div Cell Matrix Biol & Regenerat Med,Sch Biol Sci, Manchester, Lancs, England

Hopkinson, L:
 Univ Manchester, Wellcome Ctr Cell Matrix Res, Fac Biol Med & Hlth, Div Cell Matrix Biol & Regenerat Med,Sch Biol Sci, Manchester, Lancs, England

Koziell, A:
 Kings Coll London, Sch Immunol & Microbial Sci, Fac Life Sci, London, England

Lungu, A:
 Fundeni Clin Inst, Pediat Nephrol Dept, Bucharest, Romania

Rothe, HM:
 Ctr Nephrol & Metab Disorders, Weisswasser, Germany

Hoefele, J:
 Tech Univ Munich, Inst Human Genet, Munich, Germany

Zacchia, M:
 Univ Campania, Nephrol Unit, Naples, Italy

Martic, TN:
 Univ Zagreb, Dept Biol, Sch Med, Zagreb, Croatia

Gupta, A:
 Birmingham Childrens Hosp, Birmingham, W Midlands, England

van Eerde, A:
 Univ Med Ctr, Dept Genet, Utrecht, Netherlands

Gear, S:
 Alport UK, Cirencester, England

Landini, S:
 Univ Florence, Dept Clin & Expt Biomed Sci Mario Serio, Med Genet Unit, Florence, Italy

Palazzo, V:
 Meyer Childrens Univ Hosp, Med Genet Unit, Florence, Italy

al-Rabadi, L:
 Univ Utah, Hlth Sci Ctr, Salt Lake City, UT USA

Claes, K:
 Katholieke Univ Leuven, Univ Hosp Leuven, Dept Nephrol & Renal Transplantat, Leuven, Belgium

Corveleyn, A:
 Univ Hosp, Ctr Human Genet, Leuven, Belgium

 Katholieke Univ Leuven, Leuven, Belgium

Van Hoof, E:
 Univ Hosp, Ctr Human Genet, Leuven, Belgium

 Katholieke Univ Leuven, Leuven, Belgium

van Geel, M:
 Maastricht Univ Ctr, Dept Clin Genet, Maastricht, Netherlands

Williams, M:
 Southmead Hosp, Bristol Genet Lab Pathol Sci, Southmead, England

Ashton, E:
 Great Ormond St Hosp Sick Children, North East Thames Reg Genet Lab, London, England

Belge, H:
 Ctr Human Genet, Inst Pathol Genet, Gosselies, Belgium

Ars, E:
 Univ Autonoma Barcelona, Inst Invest Biomed Sant Pau, Inst Invest Carlos III, Fundacio Puigvert,Mol Biol Lab, Barcelona, Spain

Bierzynska, A:
 Univ Bristol, Bristol Med Sch, Bristol Renal Unit, Bristol, Avon, England

Gangemi, C:
 Univ Hosp Verona, Div Nephrol & Dialysis, Verona, Italy

Renieri, A:
 Univ Siena, Dept Med Biotechnol, Med Genet, Siena, Italy

Storey, H:
 Guys Hosp, Viapath Labs, Mol Genet, London, England

Flinter, F:
 Guys & St Thomas Natl Hlth Serv Fdn Trust, Dept Clin Genet, London, England
ISSN: 15559041





Clinical Journal of the American Society of Nephrology
Editorial
AMER SOC NEPHROLOGY, 1725 I ST, NW STE 510, WASHINGTON, DC 20006 USA, Estados Unidos America
Tipo de documento: Article
Volumen: 17 Número: 1
Páginas: 143-154
WOS Id: 000732619400001
ID de PubMed: 34930753
imagen Green Published, All Open Access; Bronze

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