It is also known as neurohypophyseal diabetes insipidus,[2][3] referring to the posterior pituitary (neurohypophysis), which is supplied by the hypothalamus in the brain. This condition has only polyuria in common with diabetes. Although not mutually exclusive, with most typical cases, the name diabetes insipidus is misleading.[4]
Vasopressin is released by the posterior pituitary, but unlike most other pituitary hormones, vasopressin is produced in the hypothalamus. Neurogenic diabetes insipidus can be a failure of production at the hypothalamus, or a failure of release at the pituitary.[6]
Geneticedit
The most rare form of central DI is familial neurogenic diabetes insipidus. This form of DI is due to an inherited mutation of the arginine vasopressin-neurophysin II (AVP-NPII) gene, inherited in an autosomal dominant manner. At one point, only 45 families worldwide were known to possess this genetic trait.[7] It is now more widely recognized, although the precise number of people affected with this form of DI is unknown at the present time.[citation needed]
There is also an X-linked familial form. Wolfram syndrome (also called DIDMOAD) is characterised by diabetes mellitus, sensorineural deafness, and optic atrophy.[citation needed]
Diagnosisedit
In many respects, the diagnosis of central diabetes insipidus begins as a diagnosis of exclusion. Specifically, other more common causes of polyuria and polydipsia are ruled out. Common rule outs include: diabetes mellitus, chronic kidney disease, hypokalemia, hypercalcemia, and psychogenic polydipsia. Once these conditions have been ruled out a water deprivation test is employed to confirm the diagnosis of CDI.[citation needed]
Treatmentedit
The disorder is treated with vasopressin analogs such as desmopressin. Nonetheless, many times desmopressin alone is not enough to bring under control all the symptoms, and another intervention must be implemented.[citation needed]
^"Diabetes Insipidus is changing its name: Arginine Vasopressin Deficiency". Pituitary Foundation. 2022-11-17. Retrieved 2024-01-31.
^Chitturi S, Harris M, Thomsett MJ, et al. (December 2008). "Utility of AVP gene testing in familial neurohypophyseal diabetes insipidus". Clin. Endocrinol. 69 (6): 926–30. doi:10.1111/j.1365-2265.2008.03303.x. PMID 18494865. S2CID 22350358.
^Lee YW, Lee KW, Ryu JW, et al. (2008). "Mutation of Glu78 of the AVP-NPII gene impairs neurophysin as a carrier protein for arginine vasopressin in a family with neurohypophyseal diabetes insipidus". Ann. Clin. Lab. Sci. 38 (1): 12–4. PMID 18316776.
^"The Pathogenesis of Diabetes Insipidus". Journal of the American Medical Association. 49 (6): 499. 1907. doi:10.1001/jama.1907.02530060049004.
central, diabetes, insipidus, confused, with, nephrogenic, diabetes, insipidus, recently, renamed, arginine, vasopressin, deficiency, form, diabetes, insipidus, that, lack, vasopressin, production, brain, vasopressin, acts, increase, volume, blood, intravascul. Not to be confused with Nephrogenic diabetes insipidus Central diabetes insipidus recently renamed arginine vasopressin deficiency AVP D 1 is a form of diabetes insipidus that is due to a lack of vasopressin ADH production in the brain Vasopressin acts to increase the volume of blood intravascularly and decrease the volume of urine produced Therefore a lack of it causes increased urine production and volume depletion Neurogenic diabetes insipidusOther namesarginine vasopressin deficiency central diabetes insipidus pituitary diabetes insipidus neurohypophyseal diabetes insipidus cranial diabetes insipidus neurogenic diabetes insipidusSpecialtyEndocrinology SymptomsIncrease thirst polyuria and dehydration It is also known as neurohypophyseal diabetes insipidus 2 3 referring to the posterior pituitary neurohypophysis which is supplied by the hypothalamus in the brain This condition has only polyuria in common with diabetes Although not mutually exclusive with most typical cases the name diabetes insipidus is misleading 4 Contents 1 Signs and symptoms 2 Causes 2 1 Unknown 2 2 Acquired 2 3 Genetic 3 Diagnosis 4 Treatment 5 See also 6 References 7 External linksSigns and symptoms editThis section is empty You can help by adding to it October 2021 Increased thirst polyuria and dehydration with metabolic encephalopathy Causes editUnknown edit In at least 25 of cases the most commonly occurring classification neurogenic diabetes insipidus is of unknown cause 5 Acquired edit The lack of vasopressin production usually results from some sort of damage to the pituitary gland It may be due to damage to the brain caused by Benign suprasellar tumors 20 of cases 5 Infections encephalitis tuberculosis etc Trauma 17 of cases 5 or neurosurgery 9 of cases 5 Non infectious granuloma sarcoidosis Langerhans cell histiocytosis etc Leukaemia Autoimmune associated with thyroiditis Other rare causes which include hemochromatosis and histiocytosis Vasopressin is released by the posterior pituitary but unlike most other pituitary hormones vasopressin is produced in the hypothalamus Neurogenic diabetes insipidus can be a failure of production at the hypothalamus or a failure of release at the pituitary 6 Genetic edit The most rare form of central DI is familial neurogenic diabetes insipidus This form of DI is due to an inherited mutation of the arginine vasopressin neurophysin II AVP NPII gene inherited in an autosomal dominant manner At one point only 45 families worldwide were known to possess this genetic trait 7 It is now more widely recognized although the precise number of people affected with this form of DI is unknown at the present time citation needed There is also an X linked familial form Wolfram syndrome also called DIDMOAD is characterised by diabetes mellitus sensorineural deafness and optic atrophy citation needed Diagnosis editIn many respects the diagnosis of central diabetes insipidus begins as a diagnosis of exclusion Specifically other more common causes of polyuria and polydipsia are ruled out Common rule outs include diabetes mellitus chronic kidney disease hypokalemia hypercalcemia and psychogenic polydipsia Once these conditions have been ruled out a water deprivation test is employed to confirm the diagnosis of CDI citation needed Treatment editThe disorder is treated with vasopressin analogs such as desmopressin Nonetheless many times desmopressin alone is not enough to bring under control all the symptoms and another intervention must be implemented citation needed See also editFluid deprivation testReferences edit Diabetes Insipidus is changing its name Arginine Vasopressin Deficiency Pituitary Foundation 2022 11 17 Retrieved 2024 01 31 Chitturi S Harris M Thomsett MJ et al December 2008 Utility of AVP gene testing in familial neurohypophyseal diabetes insipidus Clin Endocrinol 69 6 926 30 doi 10 1111 j 1365 2265 2008 03303 x PMID 18494865 S2CID 22350358 Lee YW Lee KW Ryu JW et al 2008 Mutation of Glu78 of the AVP NPII gene impairs neurophysin as a carrier protein for arginine vasopressin in a family with neurohypophyseal diabetes insipidus Ann Clin Lab Sci 38 1 12 4 PMID 18316776 The Pathogenesis of Diabetes Insipidus Journal of the American Medical Association 49 6 499 1907 doi 10 1001 jama 1907 02530060049004 a b c d Diabetes Inspidus Library of the National Medical Society 2008 a href Template Cite journal html title Template Cite journal cite journal a Cite journal requires journal help http www medical library org journals4a diabetes insipidus htm Archived 2012 02 22 at the Wayback Machine Central Diabetes Insipidus Pituitary Gland Disorders Merck Manual Home Health Handbook Retrieved 2009 04 04 Familial Neurogenic Diabetes Insipidus a disease caused by a traffic jam The Diabetes Insipidus Foundation 2006 a href Template Cite journal html title Template Cite journal cite journal a Cite journal requires journal help http diabetesinsipidus org 4di familial htm Archived 2009 04 29 at the Wayback MachineExternal links edit Retrieved from https en wikipedia org w index php title Central diabetes insipidus amp oldid 1210184224, wikipedia, wiki, book, books, library,
