Nephroprotective treatment exemplified by Alport syndome
Gross O, Rascher W (2019)
Publication Type: Journal article
Publication year: 2019
Journal
Book Volume: 167
Pages Range: 522-529
Journal Issue: 6
DOI: 10.1007/s00112-018-0641-9
Abstract
In children with the hereditary kidney disease Alport syndrome, the extreme importance of clarification of (micro)hematuria in childhood in order to enable an early nephroprotective treatment can be shown. This not only decisively delays the kidney disease but also improves life expectancy. Children with Alport syndrome develop progressive renal fibrosis due to genetic variants in type IV collagen genes. In the past the symptomatic hematuria symptoms in heterozygous carrier patients was misclassified as familial benign hematuria; however, the disease process by these heterozygous carrier patients often does not have a benign course of the disease. Therefore, all homozygous and heterozygous patients are now summarized under the diagnosis Alport syndrome. If diagnosed early Alport syndrome can be well treated. Renal failure can be delayed by many years and life expectancy can be improved. Because of the available treatment options, the diagnosis should be made as soon as possible in every affected child by molecular genetic testing. Unfortunately, the diagnosis is often made too late at the age of early adolescence when the renal function is already severely impaired.
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Germany (DE)How to cite
APA:
Gross, O., & Rascher, W. (2019). Nephroprotective treatment exemplified by Alport syndome. Monatsschrift Kinderheilkunde, 167(6), 522-529. https://doi.org/10.1007/s00112-018-0641-9
MLA:
Gross, O., and Wolfgang Rascher. "Nephroprotective treatment exemplified by Alport syndome." Monatsschrift Kinderheilkunde 167.6 (2019): 522-529.
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