Chapter 69
Hereditary Nephritis in Children
Judy Savige,
Judy Savige
Department of Medicine (Melbourne Health) and Northern Health, The University of Melbourne, Australia
Search for more papers by this authorJudy Savige,
Judy Savige
Department of Medicine (Melbourne Health) and Northern Health, The University of Melbourne, Australia
Search for more papers by this authorBook Editor(s):Jonathan C. Craig MBChB, DipCH, MMed(Clin Epi), PhD, FAHMS,
Donald A. Molony MD,
Giovanni F.M. Strippoli MD, PhD, MPH, MM (Epi),
Jonathan C. Craig MBChB, DipCH, MMed(Clin Epi), PhD, FAHMS
Matthew Flinders Distinguished Professor Vice President and Executive Dean
College of Medicine and Public Health, Flinders University, Adelaide, Australia
Search for more papers by this authorDonald A. Molony MD
Professor of Medicine Distinguished Teaching Professor of the University of Texas System
Division of Renal Diseases and Hypertension AND Center for Clinical Research and Evidence-based Medicine, McGovern Medical School University of Texas, Houston, TX, USA
Search for more papers by this authorGiovanni F.M. Strippoli MD, PhD, MPH, MM (Epi)
Professor of Nephrology Adjunct Professor of Epidemiology
Department of Emergency and Organ Transplantation – University of Bari, Bari, Italy
School of Public Health, University of Sydney, Sydney, NSW, Australia
Search for more papers by this authorFirst published: 18 November 2022
Summary
Hereditary nephritis usually refers to Alport syndrome and thin basement membrane nephropathy. Hematuria occurs in about 6% of the pediatric population and is mainly transient and nonglomerular, from, for example, cystitis or familial hypercalciuria. The most common cause of persistent glomerular hematuria is thin basement membrane nephropathy, and Alport syndrome is much less frequent. In children, Alport syndrome is one of the commonest causes of end-stage kidney failure after focal and segmental glomerulosclerosis and structural renal disease. Genetic testing has the advantage of not only confirming the diagnosis but also identifying the mode of inheritance. Newer agents undergoing evaluation for the treatment of Alport syndrome include some that target the interstitial fibrosis that correlates best with renal impairment, and bardoxolone with its effects on antioxidant and anti inflammatory activity. The prognosis of thin membrane nephropathy for children is excellent.
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