Nephrogenic Diabetes Insipidus

A genetic or acquired polyuric disorder characterized by persistent hypotonic urine and HYPOKALEMIA. This condition is due to renal tubular insensitivity to VASOPRESSIN and failure to reduce urine volume. It may be the result of mutations of genes encoding VASOPRESSIN RECEPTORS or AQUAPORIN-2; KIDNEY DISEASES; adverse drug effects; or complications from PREGNANCY.

Also Known As:

Diabetes Insipidus, Nephrogenic; ADH-Resistant Diabetes Insipidus; Acquired Nephrogenic Diabetes Insipidus; Congenital Nephrogenic Diabetes Insipidus; Diabetes Insipidus Renalis; Diabetes Insipidus, Nephrogenic, Autosomal; Diabetes Insipidus, Nephrogenic, Type 1; Diabetes Insipidus, Nephrogenic, Type I; Diabetes Insipidus, Nephrogenic, Type II; Diabetes Insipidus, Nephrogenic, X-Linked; Nephrogenic Diabetes Insipidus, Type I; Nephrogenic Diabetes Insipidus, Type II; Vasopressin-Resistant Diabetes Insipidus

Networked: 977 relevant articles (21 outcomes, 57 trials/studies)

Relationship Network

Disease Context: Research Results

Urogenital Diseases: 126

Related Diseases

1.	Polyuria
2.	Bipolar Disorder (Manic Depressive Psychosis)
3.	Depressive Disorder (Melancholia)
4.	Neurogenic Diabetes Insipidus (Central Diabetes Insipidus)
5.	Chronic Renal Insufficiency

Experts

1.	Deen, Peter M T: 29 articles (01/2021 - 02/2002)
2.	Bichet, Daniel G: 26 articles (08/2020 - 06/2000)
3.	Sasaki, Sei: 18 articles (12/2018 - 06/2000)
4.	Kwon, Tae-Hwan: 17 articles (01/2020 - 12/2003)
5.	Nielsen, Søren: 15 articles (10/2014 - 03/2003)
6.	Fenton, Robert A: 13 articles (01/2021 - 03/2008)
7.	Knepper, Mark A: 11 articles (01/2021 - 12/2003)
8.	Uchida, Shinichi: 10 articles (01/2022 - 02/2002)
9.	Rej, Soham: 10 articles (11/2021 - 03/2012)
10.	Baumgarten, Ruben: 9 articles (01/2021 - 07/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Nephrogenic Diabetes Insipidus:

1.	LithiumIBA 09/01/1979 - "Lithium-induced nephrogenic diabetes insipidus: studies of tubular function and pathogenesis." 05/01/1972 - "Lithium-induced nephrogenic diabetes insipidus: in vivo and in vitro studies." 10/01/2023 - "Metabolic reprogramming of renal epithelial cells contributes to lithium-induced nephrogenic diabetes insipidus." 01/23/2023 - "BACKGROUND Nephrogenic diabetes insipidus is a well-known adverse effect of lithium use. " 01/01/2023 - "[Lithium-induced nephrogenic diabetes insipidus during acute intoxication: a case report]."
2.	Deamino Arginine Vasopressin (Desmopressin)FDA LinkGeneric 01/01/2019 - "A water deprivation test and desmopressin trial confirmed nephrogenic diabetes insipidus. " 04/28/2020 - "Incomplete response to desmopressin suggests partial nephrogenic diabetes insipidus. " 11/29/2014 - "Her water deprivation test and desmopressin test results were consistent with nephrogenic diabetes insipidus. " 11/01/2010 - "Differential diagnosis of hereditary nephrogenic diabetes insipidus with desmopressin infusion test." 04/01/2009 - "Administration of 1-deamino-8-D-arginine vasopressin (dDAVP) neither decreased urine volume nor increased urine osmolality indicating congenital nephrogenic diabetes insipidus. "
3.	Hydrochlorothiazide (Esidrix)FDA LinkGeneric 11/01/1962 - "THE MECHANISM OF ANTIDIURESIS ASSOCIATED WITH THE ADMINISTRATION OF HYDROCHLOROTHIAZIDE TO PATIENTS WITH VASOPRESSIN-RESISTANT DIABETES INSIPIDUS." 02/01/2013 - "She was diagnosed as nephrogenic diabetes insipidus due to hypercalcemia caused by hypervitaminosis D, and was treated with hydrochlorothiazide 2 mg/kg twice daily, and hydration." 12/01/2005 - "He had been diagnosed clinically with nephrogenic diabetes insipidus and treated by amiloride and hydrochlorothiazide combination without a satisfactory outcome at another center since 1 year of age. " 01/01/2004 - "Treatment of congenital nephrogenic diabetes insipidus with hydrochlorothiazide and amiloride in an adult patient." 10/01/2003 - "Treatment of congenital nephrogenic diabetes insipidus by hydrochlorothiazide and cyclooxygenase-2 inhibitor."
4.	Sodium Chloride Symporter Inhibitors (Diuretics, Thiazide)IBA 01/01/2006 - "Following oral administration of a thiazide diuretic, known to exert an antidiuretic action in nephrogenic diabetes insipidus, urine output was dramatically reduced. " 12/01/2022 - "Thiazide diuretics reduce urinary volume (UV) in patients with nephrogenic diabetes insipidus, which lacks V2 receptor function. " 01/01/2018 - "A possible strategy to ameliorate these side-effects may be addition of a thiazide diuretic, this is an established treatment in nephrogenic diabetes insipidus, a condition where vasopressin V2 receptor function is absent. " 09/01/2009 - "This report emphasizes the importance of evaluating the water balance and of a quick response with strict protocols following the initiation of indomethacin and thiazide diuretics in nephrogenic diabetes insipidus." 11/01/1987 - "Amiloride has additional advantages over conventional treatment of nephrogenic diabetes insipidus using thiazide diuretics. "
5.	Prostaglandin-Endoperoxide Synthases (Cyclooxygenase)IBA 02/01/1986 - "Thus, prostaglandin synthetase inhibitors are not uniformly effective in treatment of nephrogenic diabetes insipidus. " 01/01/1981 - "Prostaglandin synthetase inhibitors in the treatment of nephrogenic diabetes insipidus." 01/01/1985 - "It is concluded that the hydrochlorothiazide-amiloride regimen is superior to hydrochlorothiazide alone and can be a satisfactory alternative to the hydrochlorothiazide-prostaglandin synthetase inhibitor combination in the treatment of congenital nephrogenic diabetes insipidus." 02/01/1986 - "The antidiuretic effect of two prostaglandin synthetase inhibitors, ibuprofen (25 mg/kg/day) and indomethacin (2 mg/kg/day), was studied in patients aged 8 to 18 years with hereditary nephrogenic diabetes insipidus. "
6.	Vasopressin Receptors (Arginine Vasopressin Receptor)IBA 04/01/2007 - "Molecular genetic study of congenital nephrogenic diabetes insipidus and rescue of mutant vasopressin V2 receptor by chemical chaperones." 06/01/2000 - "The aim of this study was to identify loss-of-function mutations of the V2 vasopressin receptor gene (AVPR2) in Italian patients affected by X-linked nephrogenic diabetes insipidus (NDI). " 10/01/1998 - "Functional studies of twelve mutant V2 vasopressin receptors related to nephrogenic diabetes insipidus: molecular basis of a mild clinical phenotype." 09/15/1997 - "Recent studies with transfected COS-7 cells have shown that functionally inactive mutant V2 vasopressin receptors (occurring in patients with nephrogenic diabetes insipidus) can be functionally rescued by coexpression of a carboxy-terminal V2 receptor fragment (V2-tail) spanning the region where various mutations occur [Schöneberg, T., J. " 08/01/1995 - "Expression studies of two vasopressin V2 receptor gene mutations, R202C and 804insG, in nephrogenic diabetes insipidus."
7.	Aquaporin 2IBA 04/27/2023 - "KEY POINTS: Prior studies have shown in a variety of animal models of acquired nephrogenic diabetes insipidus (NDI) that loss of the aquaporin-2 (AQP2) protein is a common feature. " 07/01/2010 - "In the present study, we measured whether MI could increase expression of two human aquaporin 2 (AQP2) mutants which were recently identified as causing nephrogenic diabetes insipidus (NDI). " 10/01/2000 - "Importance of aquaporin-2 expression levels in genotype -phenotype studies in nephrogenic diabetes insipidus." 01/01/2023 - "Structural and functional analysis of aquaporin-2 mutants involved in nephrogenic diabetes insipidus." 10/01/2022 - "A Novel AQP2 Sequence Variant Causing Aquaporin-2 Retention in the Cytoplasm and Autosomal Dominant Nephrogenic Diabetes Insipidus."
8.	Proteins (Proteins, Gene)FDA Link 12/01/2022 - "Gαs and Gαq/11 protein coupling bias of two AVPR2 mutants (R68W and V162A) that cause nephrogenic diabetes insipidus." 01/01/2021 - "Integrating Population Variants and Protein Structural Analysis to Improve Clinical Genetic Diagnosis and Treatment in Nephrogenic Diabetes Insipidus." 01/01/2020 - "AQP2: Mutations Associated with Congenital Nephrogenic Diabetes Insipidus and Regulation by Post-Translational Modifications and Protein-Protein Interactions." 01/01/2016 - "Mutations in the protein induce nephrogenic diabetes insipidus (NDI), which challenges the water balance by producing large urinary volumes. " 01/01/2015 - "Congenital nephrogenic diabetes insipidus (NDI) is a conformation disease resulting from protein misfolding. "
9.	Amiloride (Midamor)FDA LinkGeneric 12/01/1986 - "They conclude that amiloride can be useful in treating lithium-induced nephrogenic diabetes insipidus and merits future randomized clinical trials." 09/01/2008 - "Lithium-induced nephrogenic diabetes insipidus: renal effects of amiloride." 04/01/2008 - "Amiloride restores renal medullary osmolytes in lithium-induced nephrogenic diabetes insipidus." 12/01/2005 - "He had been diagnosed clinically with nephrogenic diabetes insipidus and treated by amiloride and hydrochlorothiazide combination without a satisfactory outcome at another center since 1 year of age. " 01/01/2004 - "Treatment of congenital nephrogenic diabetes insipidus with hydrochlorothiazide and amiloride in an adult patient."
10.	Indomethacin (Indometacin)FDA LinkGeneric 01/01/2015 - "Two children with nephrogenic diabetes insipidus (NDI) were treated with oral indomethacin (0.75-1.2 mg/kg/day) three times a day for a mean duration of 3 yrs. " 01/01/2015 - "Response to low dose indomethacin in two children with nephrogenic diabetes insipidus." 07/01/1990 - "Six months later nephrogenic diabetes insipidus was diagnosed and indomethacin was again effectively used. " 03/01/1986 - "Indomethacin for nephrogenic diabetes insipidus in a four-week-old infant." 01/01/1997 - "Indomethacin (and controlled volume reduction if continued high urine output), while observing renal function, appears the emergency treatment of choice for serious complications of nephrogenic diabetes insipidus."

Therapies and Procedures

1.	Therapeutics 12/01/2016 - "Physiological insights into novel therapies for nephrogenic diabetes insipidus." 04/01/2013 - "Nephrogenic diabetes insipidus: essential insights into the molecular background and potential therapies for treatment." 01/01/2009 - "With long-term therapy, this may result in nephrogenic diabetes insipidus. " 05/01/2008 - "Recent advances in each topic are summarized and are presented in the context of their potential to serve as a basis for the development of novel therapies that may ultimately improve life quality of nephrogenic diabetes insipidus patients." 02/01/2006 - "[Nephrogenic diabetes insipidus: Pathogenesis, pathophysiology, and therapy]."
2.	Oral Administration 01/01/2006 - "Following oral administration of a thiazide diuretic, known to exert an antidiuretic action in nephrogenic diabetes insipidus, urine output was dramatically reduced. "
3.	Drug Therapy (Chemotherapy) 05/01/2008 - "Recently created transgenic mouse models of nephrogenic diabetes insipidus produced by AQP2 gene mutation offer exciting possibilities to test new drug therapies. " 02/01/1987 - "Transient nephrogenic diabetes insipidus following high-dose cyclophosphamide chemotherapy and autologous bone marrow transplantation." 01/01/1995 - "Nephrogenic diabetes insipidus following high dose epirubicin chemotherapy for metastatic soft tissue sarcoma."
4.	Intraperitoneal Injections 11/01/2000 - "Intraperitoneal injection of the V2 agonist, deamino-Cys(1), D-Arg(8) vasopressin, induced an increase in urine osmolarity in Clcnk1+/- and Clcnk1+/+ mice from approximately 1,000 to approximately 3,000 mosm/kg H(2)O, whereas the increase in Clcnk1-/- mice was only from approximately 600 to approximately 840 mosm/kg H(2)O, indicating nephrogenic diabetes insipidus in Clcnk1-/- mice. " 01/01/1999 - "Intraperitoneal injection of the V2 agonist 1-deamino-8-D-arginine vasopressin (dDAVP) induced a threefold increase in urine osmolarity in Clcnk1+/- and Clcnk1+/+ mice, whereas only a minimal increase was seen in Clcnk1-/- mice, indicating nephrogenic diabetes insipidus. "
5.	Fluid Therapy (Oral Rehydration Therapy) 09/01/2017 - "Early diagnosis and treatment of congenital nephrogenic diabetes insipidus (CNDI) are essential due to the risk of intellectual disability caused by repeated episodes of dehydration and rapid rehydration. " 07/01/2020 - "Patients affected by nephrogenic diabetes insipidus (NDI) can present with hypernatremic dehydration, and first-line rehydration schemes are completely different from those largely applied in usual conditions determining a mild to severe hypovolemic dehydration/shock. "