| Literature DB >> 35585366 |
Andrew Mallett1,2,3, Hugh McCarthy4,5,6, Rachael Kermond7.
Abstract
Kidney tubules are responsible for the preservation of fluid, electrolyte and acid-base homeostasis via passive and active mechanisms. These physiological processes can be disrupted by inherited or acquired aetiologies. The net result is a tubulopathy. It is important to make a prompt and accurate diagnosis of tubulopathies in children and young adults. This allows timely and appropriate management, including disease-specific therapies, and avoids complications such as growth failure. Tubulopathies can present with a variety of non-specific clinical features which can be diagnostically challenging. In this review, we build from this common anatomical and physiological understanding to present a tangible appreciation of tubulopathies as they are likely to be clinically encountered among affected children and young adults.Entities:
Keywords: Genetics; Hypokalaemia; Magnesium; Nephrocalcinosis; Rare disease; Salt-wasting; Tubulopathy
Year: 2022 PMID: 35585366 DOI: 10.1007/s00467-022-05606-1
Source DB: PubMed Journal: Pediatr Nephrol ISSN: 0931-041X Impact factor: 3.714