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Pathophysiology, diagnosis and management of nephrogenic diabetes insipidus

Bockenhauer, D; Bichet, DG; (2015) Pathophysiology, diagnosis and management of nephrogenic diabetes insipidus. Nature Reviews Nephrology , 11 (10) pp. 576-588. 10.1038/nrneph.2015.89. Green open access

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Abstract

Healthy kidneys maintain fluid and electrolyte homoeostasis by adjusting urine volume and composition according to physiological needs. The final urine composition is determined in the last tubular segment: the collecting duct. Water permeability in the collecting duct is regulated by arginine vasopressin (AVP). Secretion of AVP from the neurohypophysis is regulated by a complex signalling network that involves osmosensors, barosensors and volume sensors. AVP facilitates aquaporin (AQP)-mediated water reabsorption via activation of the vasopressin V2 receptor (AVPR2) in the collecting duct, thus enabling concentration of urine. In nephrogenic diabetes insipidus (NDI), inability of the kidneys to respond to AVP results in functional AQP deficiency. Consequently, affected patients have constant diuresis, resulting in large volumes of dilute urine. Primary forms of NDI result from mutations in the genes that encode the key proteins AVPR2 and AQP2, whereas secondary forms are associated with biochemical abnormalities, obstructive uropathy or the use of certain medications, particularly lithium. Treatment of the disease is informed by identification of the underlying cause. Here we review the clinical aspects and diagnosis of NDI, the various aetiologies, current treatment options and potential future developments.

Type: Article
Title: Pathophysiology, diagnosis and management of nephrogenic diabetes insipidus
Location: England
Open access status: An open access version is available from UCL Discovery
DOI: 10.1038/nrneph.2015.89
Publisher version: http://dx.doi.org/10.1038/nrneph.2015.89
Language: English
Additional information: © 2015 Macmillan Publishers Limited. All rights reserved.
Keywords: Acid, base, fluid, electrolyte disorders Diabetes insipidus Mechanisms of disease Molecular neuroscience Therapeutics
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Renal Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/1471901
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