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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Intracranial injection of Ang II causes increased water and salt intake Morbidity and mortality associated with vasopressin replacement therapy in children.

High proportion of pituitary abnormalities and other congenital defects in children with congenital nasal pyriform aperture stenosis.

Clinical data for the patients were obtained from their medical records. Alobar holoprosencephaly, diabetes insipidus and coloboma without craniofacial abnormalities: Our study was thus unable to provide further insight into the mechanism underlying permanent and transient congenital DI.

Nat Neurosci ; Mechanisms of brain renin angiotensin system-induced drinking and blood pressure: These vulnerable patients have high rates of comorbidity and require careful monitoring. Despite the inclusion of all patients with neonatal DI, the number of subjects investigated was small because this complex condition is very rare. Plasma osmolality values returned to normal after treatment with forced hydration and a vasopressin analogue, desamino-D-arginine vasopressin.

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

Open arrows indicate release of peptides to the circulation. To our knowledge, this is the first report of such a disorder in two sisters.


Similar damage was noted in the SFO of the patients. Create a free personal account to download free article PDFs, sign up for alerts, and more. Create a free personal account to make a comment, download free article PDFs, sign up for alerts and more.

Saudi Med J ; Diagnosing adipsia can be adipsicc as there is no set of concrete physical signs that are adipsia specific. Adipsic hypernatremia patients with specific immune responses to SFO display common clinical features. These patients typically have Langerhans histiocytosis and teratoma in the hypothalamus.

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

Subfornical organ neurons with efferent projections to the hypothalamic paraventricular nucleus: Consistent with this view, some patients experienced episodic inflammation with infections such as influenza virus and opsoclonus myoclonus syndrome OMSa feature often associated with neuroblastic tumors.

It is associated with greater morbidity and a high risk of developing both hypernatremia and hyponatremia, due to the condition itself or secondary to treatment with vasopressin analogs or fluid administration.

In progress issue alert. Email alerts New issue alert. Angiotensin, thirst, and sodium appetite. Holoprosencephaly associated with diabetes insipidus and syndrome of inappropriate secretion of antidiuretic hormone.

C, Magnetic resonance time-of-flight angiography. Brain Res ; Get free access to newly published articles. Type C is generally the adipsia type found in patients with adipsic diabetes insipidus. Diabetes insipidus associated with symptomatic congenital cytomegalovirus infection. Molecular structure of vasopressin.


All statistical analyses were carried out with SAS software, version 9. Journal List Clin Pediatr Endocrinol v. Sodium-level-sensitive sodium channel Na x is expressed in glial laminate processes in the sensory circumventricular organs. Type D is the least commonly diagnosed and researched type of adipsia. Plasma sodium concentrations were considered for hgpernatremia with transient adipsic DI, until this condition resolved.

The patients of this group required adequate hydration and various doses of DDAVP, with lower doses generally required for patients with adipsia than for those with normodipsia. The underlying cause remains unknown at the age of 23 years for one patient with CDI and normal thirst.

Adipsic Hypernatremia in Two Sisters | JAMA Pediatrics | JAMA Network

In this review, we aim to highlight the characteristic clinical features of these patients, in addition to etiological mechanisms related to SFO function.

Endocrine diseases Nephrology Rare diseases Neurology Thirst. The final group consisted of a single patient with a unique combination of CDI and normal thirst associated with GHD diagnosed hypernatremis childhood, with no underlying etiology detectable on MRI.

Early Identification and Outcome Adel Djermane. J Pediatr ; However, this would be unlikely to result in a substantial misclassification bias, given the large number of plasma sodium determinations.

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