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Adipsia is a disease characterized by the absence of thirst even in the It is a rare condition that typically presents as hypernatremic dehydration. We describe two sisters with chronic hypernatremia, lack of thirst, and inappropriate osmoregulated vasopressin secretion. Only one sister, who presented with. Adipsia, also known as hypodipsia, is a symptom of inappropriately decreased or absent . Type A (essential hypernatremia syndrome) involves an increase of the level in which solvent molecules can pass through cell membranes (osmotic.

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Oxford University Press; Other findings in support of the role of dopamine in thirst regulation involved the nigrostriatal pathway.

Adipsic hypernatremia in a patient with pseudotumor cerebri and the primary empty sella syndrome.

Adipsia can tend to result from lesions to hypothalamic regions involved in thirst regulation. Report of three cases and review of the literature.

Chronic and sustained hypernatremia, absence of thirst, diabetes hypernatremi, and adrenocorticotrophin insufficiency resulting from widespread destruction of the hypothalamus. Lesions or injuries to those hypothalamic regions cause adipsia because the lesions cause defects in the thirst regulating center which can lead to adipsia. As they did not show overeating, we believe the obesity may result from a disorder of energy balance.

This type may be due to some elimination of osmoreceptors. Although minimal, there is still some secretion of Hypeenatremia. Please review our privacy policy. New strategies to prevent specific inflammatory conditions would be required to treat these patients; a trial to reduce or eliminate patient autoantibodies deserves consideration.

Support Center Support Center. People affected by adipsia lack the ability to feel thirst, thus they often must be directed to drink. All patients were regularly managed by our team of pediatric endocrinologists, adipsiic individual adjustment of fluid intake, as a function of the thirst abnormality, and with nasal or, preferably, oral once it became available DDAVP, initially administered once, twice, or three times daily.


Morbidity and mortality associated with vasopressin replacement therapy in children. Author information Article notes Copyright and License information Disclaimer. Register for email alerts with links to free full-text articles Access PDFs of free articles Manage your interests Save searches and receive search alerts. Physiol Rev ; The anterior hypothalamus is in close proximity to osmoreceptors which regulate the secretion of antidiuretic hormone ADH.

Plasma sodium concentrations were considered for patients with transient adipsic DI, until this condition resolved.

Congenital nasal adpsic aperture stenosis associated with central diabetes insipidus. Figure A is modified from Related articles in Web of Science Google Scholar. Approach to the Patient With Hypogonadotropic Hypogonadism. Ann Intern Med ; The subfornical organ is the primary locus of sodium-level sensing by Na x sodium channels for the control of salt-intake behavior. Endocrinopathies associated with midline cerebral and cranial malformations.

Adipsic Hypernatremia in Two Sisters

Infobox medical condition new. In this case, desmopressin helps the kidneys to promote reabsorption of water. Patients with a history of brain tumors, or congenital malformationsmay have hypothalamic lesions, which could be indicative of adipsia.

The results hypegnatremia this study extend our knowledge about the characteristics of CDI in infants with and without hypothalamic adipsic hypernatremia syndrome. Future Directions There are still two unresolved points related to the pathophysiology of this disorder: Int J Pept ; This may have led to cases of dysnatremia being missed.


Adipsic hypernatremia is a rare disease presenting as persistent hypernatremia with disturbance of thirst regulation and hypothalamic dysfunction. Evidence for a dual central role for angiotensin in water and sodium intake. Intracranial injection of Ang II causes increased water and salt intake Nat Neurosci ; Hyperatremia hypothalamic dysfunction with precocious puberty and adipsic hypernatremia first presenting in adolescence.

Predictors of diabetes insipidus after transsphenoidal surgery: Body mass index BMI; weight in kilograms divided by height in meters squared is expressed as a standard aidpsic score for sex and chronological age Central diabetes insipidus CDI may have two main causes. These lesions can be congenital, acquired, trauma, or even surgery.

Adipsic hypernatremia in a patient with pseudotumor cerebri and the primary empty sella syndrome.

Autoimmunity to the sodium-level sensor in the brain causes essential hypernatremia. The AVP release in this subtype occurs with normally functioning levels of osmoregulation.

These findings may be useful for hypernatreima adipsic hypernatremia caused by an autoimmune response to the SFO, and support development of new strategies for prevention and treatment. National Center for Biotechnology InformationU. Close mobile search navigation Article navigation. Recurrent hypernatremia; a proposed mechanism in a patient with absence of thirst and abnormal hypernqtremia of water.

The SFO is a unique nucleus in that its afferent and efferent projections are well placed to respond to blood-borne signals and integrate them htpernatremia neuronal signals InHiyama et al. The initial characteristics of the 12 patients are presented in Table 1.