Related to this topic: Support | Patient+ | Weblinks | Equipment | Books | Your Experience | Other resources | Glossaries
Print options: Printer friendly version of this leaflet (html)     Other options:  AddThis Social Bookmark Button (what's this?)

PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people.

Diabetes Insipidus (DI)

This is caused by hyposecretion of, or hyporesponsiveness to Anti Diuretic Hormone (ADH), i.e .arginine vasopressin.1 ADH is synthesised in the hypothalamus and transported as neurosecretory vesicles to the posterior pituitary where it is released into the circulation, governed by plasma osmolality. Its deficiency or failure to act causes an inability to concentrate urine in the distal renal tubules; leading to the passage of copious volumes of dilute urine. Usually the sufferer passes >3litres/24 hours of low osmolality (<300mosmol/kg) urine.2

There are two types:

Cranial Diabetes Insipidus (CDI)3

This is caused by a deficiency of circulation-osmoregulated ADH. It is usually due to disease of the hypothalamus or surrounding tissues. Posterior pituitary disease tends not to cause diabetes insipidus, as secretion continues in the hypothalamus, unless pituitary tumour extends above the sella, putting pressure on the hypothalamus.

Causes

Acquired

Inherited

  • DIDMOAD (Wolfram Syndrome) - autosomal recessive (combination of diabetes insipidus, diabetes mellitus, optic atrophy, deafness)4
  • Autosomal dominant mutations of vasopressin gene
Nephrogenic Diabetes Insipidus (NDI)5

This is caused by renal hyporesponsiveness to adequate vasopressin levels. Causes include:

Presentation2

Symptoms

The predominant symptoms in older patients are polyuria (large output of urine with micturition up to every 30 minutes), polydypsia, nocturia, and, in children, enuresis. Infants tend to suffer failure to thrive, irritability, protracted crying, anorexia and fatiguability.

Signs

Bladder enlargement may occur. Signs of dehydration may be present. Confirm that the urine volume output >3 l/24 hours.

Differential diagnosis2
  • Psychogenic polydypsia (PP)
  • Osmotic diuresis (esp. diabetes mellitus)
  • Diuretic abuse
Investigations2
  • Biochemistry - plasma glucose, urea and electrolytes, and plasma and urine osmolality should all be measured.
  • Fluid deprivation test with response to desmopressin - this should be used if glucose and electrolyte levels are normal. The patient is deprived of fluids for up to 8 hours or 5% loss of body weight, following which desmopressin (DDAVP) 2g IM is given. See Table I for interpretation of the results.
  • Plasma vasopressin levels and osmolality in response to infusion of 5% hypertonic saline at 0.05 ml/kg/min for 2 hours may be measured in cases of diagnostic difficulty.
  • A therapeutic trial of low dose desmopressin is another option, with careful monitoring of plasma osmolality or serum sodium. CDI patients improve, and those with NDI are unaffected. Those with PP develop hyponatraemia and may stop drinking.
  • MRI of the pituitary, hypothalamus and surrounding tissues, including pineal gland may be contributory in helping to determine the underlying cause.

Table 1 - Classification of causes of diabetes insipidus on basis of water deprivation and DDAVP response

Urine osmolarity after fluid deprivation mOsm/kg
Urine osmolarity after DDAVP mOsm/kg
Likely diagnosis
<300
>800
CDI
<300
<300
NDI
>800
>800
Primary/psychogenic polydipsia
<300
>800
Partial CDI or NDI or PP or diuretic abuse

Management2,12

Non-drug

If the urine output is < 4 litres/24 hour specific therapy may not be required. However, patients should be advised that they must drink enough to satisfy their thirst. Metabolic abnormalities should be corrected. Medication regimes should be reviewed to identify any drugs likely to cause NDI.

Drugs

  • CDI If a large urinary volume is produced use desmopressin orally, intranasally or injected. Monitor serum sodium or plasma osmolality to prevent hyponatraemia or hypo-osmolality from chronic overdose.
  • Mild to moderate NDI Use high dose desmopressin
  • Severe NDI13 Options include desmopressin plus:
Prognosis2

Once diagnosed the condition should respond well to appropriate therapy. This should be initiated and supervised by a physician with appropriate experience.

Complications

Patients with diabetes insipidus need careful monitoring of fluid balance and therapy following surgery, with multidisciplinary care.15,16,


Document References
  1. Fujiwara TM, Bichet DG; Molecular biology of hereditary diabetes insipidus. J Am Soc Nephrol. 2005 Oct;16(10):2836-46. Epub 2005 Aug 10. [abstract]
  2. Cooperman M, Diabetes Insipidus, eMedicine 2006.; Overview of CDI and NDI.
  3. Cranial diabetes insipidus (GPN)
  4. OMIM - WOLFRAM SYNDROME
  5. Nephrogenic diabetes insipidus (GPN)
  6. Garofeanu CG, Weir M, Rosas-Arellano MP, et al; Causes of reversible nephrogenic diabetes insipidus: a systematic review. Am J Kidney Dis. 2005 Apr;45(4):626-37. [abstract]
  7. Bendz H, Aurell M; Drug-induced diabetes insipidus: incidence, prevention and management.; Drug Saf. 1999 Dec;21(6):449-56. [abstract]
  8. Lindheimer MD, Davison JM; Osmoregulation, the secretion of arginine vasopressin and its metabolism during pregnancy. Eur J Endocrinol. 1995 Feb;132(2):133-43. [abstract]
  9. Sainz Bueno JA, Villarejo Ortiz P, Hidalgo Amat J, et al; Transient diabetes insipidus during pregnancy: a clinical case and a review of the syndrome.; Eur J Obstet Gynecol Reprod Biol. 2005 Feb 1;118(2):251-4.
  10. OMIM; On-line Mendelian Inheritance In Man. Diabetes Insipidus, Nephrogenic, X-linked.; Detail on this rare genetic cause of NDI.
  11. OMIM; On-line Mendelian Inheritance In Man. Diabetes Insipidus, Nephrogenic, Autosomal.; Detail of this rare genetic cause of NDI.
  12. Pituitary Foundation (UK); Diabetes Insipidus; Resources Page
  13. Central Diabetes Insipidus; Merck Manuals 2007
  14. Magaldi AJ; New insights into the paradoxical effect of thiazides in diabetes insipidus therapy. Nephrol Dial Transplant. 2000 Dec;15(12):1903-5.
  15. Moug SJ, McKee RF, O'Reilly DS, et al; The perioperative challenge of nephrogenic diabetes insipidus: a multidisciplinary approach.; Surgeon. 2005 Apr;3(2):89-94. [abstract]
  16. Dumont AS, Nemergut EC 2nd, Jane JA Jr, et al; Postoperative care following pituitary surgery. J Intensive Care Med. 2005 May-Jun;20(3):127-40. [abstract]

Internet and Further Reading Acknowledgements EMIS is grateful to Dr Laurence Knott for writing this article. The final copy has passed scrutiny by the independent Mentor GP reviewing team. ©EMIS 2007.
DocID: 2047
Document Version: 21
DocRef: bgp977
Last Updated: 6 Jul 2007
Review Date: 5 Jul 2009






















Disclaimer: Patient UK has no control of the content of the above links. Inclusion does not imply endorsement by Patient UK.

Advertise on this site














Disclaimer: Patient UK has no control of the content of the above links. Inclusion does not imply endorsement by Patient UK.

Advertise on this site


PS - Health and Poverty

Perhaps the biggest cause of ill health in the world is poverty. Help to Make Poverty History. For example, why not lend some of your money to disadvantaged communities to enable them to trade their way out of poverty through schemes such as Shared Interest.

See also MAKEPOVERTYHISTORY North East for details and links to campaigns against poverty.

^ Top of Page