Nephrogenic Diabetes Insipidus

  title={Nephrogenic Diabetes Insipidus},
  author={Jeff M. Sands and Daniel G Bichet},
  journal={Annals of Internal Medicine},
Clinical Principles Patients with diabetes insipidus produce large quantities of dilute urine. Diabetes insipidus can result from failure of the posterior pituitary to make or secrete vasopressin (antidiuretic hormone) or end-organ (kidney) insensitivity. Patients with congenital nephrogenic diabetes insipidus now live well into adulthood and need to be followed by internists to avoid bladder dysfunction, renal dysfunction, and complications related to inaccessibility to water. Optimal therapy… 
Diabetes Insipidus: Pathogenesis, Diagnosis, and Clinical Management
Treatment of DI is dependent on the disease classification, but severe complications may arise if not tended to appropriately, and central and nephrogenic subtypes of DI share a paradoxical treatment in thiazide diuretics.
An AMPK activator as a therapeutic option for congenital nephrogenic diabetes insipidus
It is concluded that NDI-5033 can improve urine concentration in animals with NDI and holds promise as a potential therapy for patients with congenital NDI due to V2R mutations.
Congenital nephrogenic diabetes insipidus presenting as osmotic demyelination syndrome in infancy
Early blood examination or polyuria in the patient, mother, or another family member and hypernatremic dehydration with good urine output should lead to an early diagnosis and prevent central nervous system consequences, which are a critical complication of NDI that affects prognosis.
Activation of AQP2 water channels by protein kinase A: therapeutic strategies for congenital nephrogenic diabetes insipidus
  • F. Ando
  • Biology, Medicine
    Clinical and Experimental Nephrology
  • 2021
The pathophysiological roles of PKA are discussed and novel strategies to activate PKA in renal collecting ducts are proposed, including the first low-molecular-weight compound to be discovered that can phosphorylate AQP2 more effectively than preexisting drug candidates.
Gradient washout and secondary nephrogenic diabetes insipidus after brain injury in an infant: a case report
An unusual case of an infant with glioblastoma who, after tumor resection, was treated for concurrent central diabetes insipidus and cerebral salt wasting complicated by secondary nephrogenic diabetes ins ipidus is reported.
Vasopressin–aquaporin-2 pathway: recent advances in understanding water balance disorders
This review highlights some of the new insights and recent advances in therapeutic intervention targeting the dysfunctions in the vasopressin–AQP2 pathway causing diseases characterized by water balance disorders.
Thiazide diuretics and the rate of disease progression in autosomal dominant polycystic kidney disease: an observational study
  • B. Kramers, Iris W. Koorevaar, E. Meijer
  • Medicine
    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
  • 2020
Data do not show that thiazide diuretics have a detrimental effect on the rate of disease progression in ADPKD and suggest that these drugs can be prescribed as second-line antihypertensives.
The association of glucagon with disease severity and progression in patients with autosomal dominant polycystic kidney disease: an observational cohort study
Cross-sectionally, glucagon was associated with eGFR and height-adjusted total kidney volume, but in the opposite direction of the hypothesis, and these lost significance after adjustment for confounders.
A combined outpatient and inpatient overnight WDT protocol is safe and feasible when the test is performed with special care at experienced centers and newer diagnostic tools are expected to improve the accuracy of PPS diagnosis.


Acquired nephrogenic diabetes insipidus.
  • A. Khanna
  • Medicine, Biology
    Seminars in nephrology
  • 2006
Nephrogenic diabetes insipidus: absence of close linkage with Xg.
The distance from the sites of Xg and colorblindness was explored to explore the linkage relationships of NDI locus and the estimation of distance to deuteranopia failed in that none of 43 males tested using Stilling's tables (Hertel 1939) was found colorblind.
Pathogenesis of nephrogenic diabetes insipidus due to chronic administration of lithium in rats.
At least two factors play an important role in the pathogenesis of NDI consequent to chronic oral administration of Li: (a) decreased ability of MCT and PCD to generate and accumulate cAMP in response to stimulation by AVP; this defect is primarily due to diminished activity of AdC in these tubular segments caused by prolonged exposure to Li.
Clinical presentation and follow-up of 30 patients with congenital nephrogenic diabetes insipidus.
The majority of patients with congenital nephrogenic diabetes insipidus were diagnosed within the first 2.5 yr of life, and main symptoms at clinical presentation were vomiting and anorexia, failure to thrive, fever, and constipation.
Amelioration of polyuria by amiloride in patients receiving long-term lithium therapy.
It is concluded that amiloride mitigates lithium-induced polyuria, at least partly, by blunting the inhibitory effect of lithium on water transport in the renal collecting tubule and obviating the need for potassium supplementation in the treatment of this kind of polyuria.
Molecular and cellular defects in nephrogenic diabetes insipidus
By in vitro expression, it has been shown that the majority of V2R mutants and all AQP2 mutants found in recessive NDI are misfolded and retained within the endoplasmic reticulum (ER), which explains the dominant-negative effect of this mutation.
Hypokalemic salt-losing tubulopathy with chronic renal failure and sensorineural deafness.
The hypokalemic salt-losing tubulopathy with chronic renal failure and sensorineural deafness represents not only genetically but also clinically a disease entity distinct from hyperprostaglandin E syndrome/antenatal Bartter syndrome.
Segment-specific ENaC downregulation in kidney of rats with lithium-induced NDI.
A marked and highly segment-specific downregulation of beta- ENaC and gamma-ENaC is identified in the cortical and outer medullary collecting duct, chief sites for collecting duct sodium reabsorption, in rats with a lithium-induced increase in fractional excretion of sodium.
Renal Diabetes Insipidus
  • K. Cyvin
  • Medicine
    Acta paediatrica Scandinavica
  • 1967
A therapeutical trial with Hydrochlorothiazid significantly lowered the serum sodium and chloride, but the effect was of relatively short duration (some weeks); the boy has been controlled regularly in the clinic and is extremely retarded.
Molecular biology of hereditary diabetes insipidus.
Patients who have congenital NDI and bear mutations in the AVPR2 or AQP2 genes have a "pure" NDI phenotype with loss of water but normal conservation of sodium, potassium, chloride, and calcium.