Martín Cuesta1, Mark J Hannon1, Christopher J Thompson2. 1. Academic Department of Endocrinology, Beaumont Hospital/RCSI Medical School, Beaumont Road, Dublin 9, Co., Dublin, Ireland. 2. Academic Department of Endocrinology, Beaumont Hospital/RCSI Medical School, Beaumont Road, Dublin 9, Co., Dublin, Ireland. christhompson@beaumont.ie.
Abstract
INTRODUCTION: Adipsic diabetes insipidus (ADI) is a very rare disorder, characterized by hypotonic polyuria due to arginine vasopressin (AVP) deficiency and failure to generate the sensation of thirst in response to hypernatraemia. As the sensation of thirst is the key homeostatic mechanism that prevents hypernatraemic dehydration in patients with untreated diabetes insipidus (DI), adipsia leads to failure to respond to aquaresis with appropriate fluid intake. This predisposes to the development of significant hypernatraemia, which is the typical biochemical manifestation of adipsic DI. METHODS: A literature search was performed to review the background, etiology, management and associated complications of this rare condition. RESULTS: ADI has been reported to occur in association with clipping of an anterior communicating artery aneurysm following subarachnoid haemorrhage, major hypothalamic surgery, traumatic brain injury and toluene exposure among other conditions. Management is very difficult and patients are prone to marked changes in plasma sodium concentration, in particular to the development of severe hypernatraemia. Associated hypothalamic disorders, such as severe obesity, sleep apnoea and thermoregulatory disorders are often observed in patients with ADI. CONCLUSION: The management of ADI is challenging and is associated with significant morbidity and mortality. Prognosis is variable; hypothalamic complications lead to early death in some patients, but recent reports highlight the possibility of recovery of thirst.
INTRODUCTION:Adipsic diabetes insipidus (ADI) is a very rare disorder, characterized by hypotonic polyuria due to arginine vasopressin (AVP) deficiency and failure to generate the sensation of thirst in response to hypernatraemia. As the sensation of thirst is the key homeostatic mechanism that prevents hypernatraemic dehydration in patients with untreated diabetes insipidus (DI), adipsia leads to failure to respond to aquaresis with appropriate fluid intake. This predisposes to the development of significant hypernatraemia, which is the typical biochemical manifestation of adipsic DI. METHODS: A literature search was performed to review the background, etiology, management and associated complications of this rare condition. RESULTS: ADI has been reported to occur in association with clipping of an anterior communicating artery aneurysm following subarachnoid haemorrhage, major hypothalamic surgery, traumatic brain injury and toluene exposure among other conditions. Management is very difficult and patients are prone to marked changes in plasma sodium concentration, in particular to the development of severe hypernatraemia. Associated hypothalamic disorders, such as severe obesity, sleep apnoea and thermoregulatory disorders are often observed in patients with ADI. CONCLUSION: The management of ADI is challenging and is associated with significant morbidity and mortality. Prognosis is variable; hypothalamic complications lead to early death in some patients, but recent reports highlight the possibility of recovery of thirst.
Authors: D Smith; F Finucane; J Phillips; P H Baylis; J Finucane; W Tormey; C J Thompson Journal: Clin Endocrinol (Oxf) Date: 2004-08 Impact factor: 3.478
Authors: Joshua A Levine; Susan L Karam; Clare O'Connor; Smita Kumar; Malini Soundarrajan; Deepika McConnell; Abeer T Ammar; Ashley M Gale; Donald Zimmerman; Emily D Szmuilowicz Journal: AACE Clin Case Rep Date: 2018 Nov-Dec
Authors: Friso de Vries; Daniel J Lobatto; Marco J T Verstegen; Wouter R van Furth; Alberto M Pereira; Nienke R Biermasz Journal: Pituitary Date: 2020-09-29 Impact factor: 4.107