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Acquired arginine vasopressin deficiency
A subtype of central diabetes insipidus (CDI) characterized by polyuria and polydipsia, due to an idiopathic or secondary decrease in vasopressin (AVP) production.
ORPHA:95626
Classification level: Subtype of disorder
- Acquired CDI
- Acquired neurogenic diabetes insipidus
Prevalence: Unknown
Inheritance: -
Age of onset: All ages
The prevalence is unknown.
It occurs equally in both sexes and in all age groups but most often has an onset around the ages of 10-20. Symptoms include polyuria, polydipsia, usually associated with weight loss, and nocturia.
Acquired CDI is secondary to a condition damaging the central nervous system or it is idiopathic (unknown etiology). Causes that lead to damage of the hypothalamus-neurohypophyseal brain region include head trauma (resulting from an accident or surgery), congenital abnormalities (pituitary stalk interruption syndrome (PSIS), infections (meningitis, encephalitis), autoimmune disorders (primary hypophisitis) and inflammatory (sarcoidosis and Wegener's granulomatosis) or neoplastic (germinoma, meningioma, Langerhans cell histiocytosis, craniopharyngioma, Rathke cleft cysts and metastasis). Up to 50% of CDI cases are idiopathic and the cause is unknown but autoimmunity is suspected to be involved in a number of them.
Last update: July 2012
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: produced/endorsed by FSMR(s)
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