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Diabetes Insipidus

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Pediatric Endocrinology

Part of the book series: Contemporary Endocrinology ((COE))

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Abstract

Diabetes insipidus is a syndrome of dysregulated free water balance resulting from vasopressin deficiency or insensitivity of the kidney to vasopressin action. In the absence of vasopressin-mediated urinary concentration, there is increased excretion (polyuria) of dilute urine. The loss of free water leads to increased thirst and water intake (polydipsia). If the thirst is not quenched, the progressive free water deficit leads to a hyperosmolar state characterized by plasma hypernatremia. Diabetes insipidus may be categorized as central (or neurogenic), when due to vasopressin deficiency, or nephrogenic, when the result of diminished renal responsiveness to the antidiuretic action of vasopressin. Central diabetes insipidus can be treated with vasopressin or vasopressin analogues such as desmopressin. Treatment of nephrogenic diabetes insipidus typically depends upon reversal of the underlying cause, but pharmacological treatment may be partly successful.

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References

  1. Vokes T, Robertson GL. Physiology of vasopressin secretion. In: Czernichow P, Robinson AG, editors. Diabetes insipidus in man, Frontiers of hormone research, vol. 13. Basel: Karger; 1984. p. 127–55.

    Google Scholar 

  2. Knepper MA, Verbalis JG, Nielsen S. Role of aquaporins in water balance disorders. Curr Opin Nephrol Hypertens. 1997;6:367–71.

    Article  PubMed  CAS  Google Scholar 

  3. Nielsen S, Frokiaer J, Marples D, Kwon TH, Agre P, Knepper MA. Aquaporins in the kidney: from molecules to medicine. Physiol Rev. 2002;82: 205–44.

    PubMed  CAS  Google Scholar 

  4. Schrier RW, Berl T, Anderson RJ. Osmotic and nonosmotic control of vasopressin release. Am J Physiol. 1979;236:F321–32.

    PubMed  CAS  Google Scholar 

  5. Robertson GL. Differential diagnosis of polyuria. Annu Rev Med. 1988;39:425–42.

    Article  PubMed  CAS  Google Scholar 

  6. Baylis PH, Cheetham T. Diabetes insipidus. Arch Dis Child. 1998;79:84–9.

    Article  PubMed  CAS  Google Scholar 

  7. Oster JR, Singer I, Thatte L, Grant-Taylor I, Diego JM. The polyuria of solute diuresis. Arch Intern Med. 1997;157:721–9.

    Article  PubMed  CAS  Google Scholar 

  8. Hammond DN, Moll GW, Robertson GL, Chelmicka-Schorr E. Hypodipsic hypernatremia with normal osmoregulation of vasopressin. N Engl J Med. 1986;315:433–6.

    Article  PubMed  CAS  Google Scholar 

  9. Adrogue HJ, Madias NE. Hypernatremia. N Engl J Med. 2000;342:1493–9.

    Article  PubMed  CAS  Google Scholar 

  10. Colle E, Ayoub E, Raile R. Hypertonic dehydration (hypernatremia): the role of feedings high in solutes. Pediatrics. 1958;22:5–12.

    PubMed  CAS  Google Scholar 

  11. Verbalis JG, Robinson AG, Moses AM. Post-operative and post-traumatic diabetes insipidus. In: Czernichow P, Robinson AG, editors. Diabetes insipidus in man, Frontiers of hormone research, vol. 13. Basel: Karger; 1984. p. 247–65.

    Google Scholar 

  12. Kauli R, Galatzer A, Laron Z. Treatment of diabetes insipidus in children and adolescents. In: Czernichow P, Robinson AG, editors. Diabetes insipidus in man, Frontiers of hormone research, vol. 13. Basel: Karger; 1985. p. 304–13.

    Google Scholar 

  13. Czernichow P, Pomerade R, Brauner R, Rappaport R. Neurogenic diabetes insipidus in children. In: Czernichow P, Robinson AG, editors. Diabetes insipidus in man, Frontiers of hormone research, vol. 13. Baser: Karger; 1985. p. 190–209.

    Google Scholar 

  14. Rogers DG. Morbid obesity in a young child. Clin Pediatr. 2000;39:169–71.

    Article  CAS  Google Scholar 

  15. Maghnie M, Cosi G, Genovese E, et al. Central diabetes insipidus in children and young adults. N Engl J Med. 2000;343:998–1007.

    Article  PubMed  CAS  Google Scholar 

  16. Lees MM, Hodgkins P, Reardon W, et al. Frontonasal dysplasia with optic disc anomalies and other midline craniofacial defects: a report of six cases. Clin Dysmorphol. 1998;7:157–62.

    Article  PubMed  CAS  Google Scholar 

  17. Strom TM, Hortnagel K, Hofmann S, et al. Diabetes insipidus, diabetes mellitus, optic atrophy and deafness (DIDMOAD) caused by mutations in a novel gene (wolframin) coding for a predicted transmembrane protein. Hum Mol Genet. 1998;7:2021–8.

    Article  PubMed  CAS  Google Scholar 

  18. Pedersen EB, Lamm LU, Albertsen K, et al. Familial cranial diabetes insipidus: a report of five families. Genetic, diagnostic and therapeutic aspects. Q J Med. 1985;57:883–96.

    PubMed  CAS  Google Scholar 

  19. Bergeron C, Kovacs K, Ezrin C, Mizzen C. Hereditary diabetes insipidus: an immunohistochemical study of the hypothalamus and pituitary gland. Acta Neuropathol. 1991;81:345–8.

    Article  PubMed  CAS  Google Scholar 

  20. Rittig S, Robertson GL, Siggaard C, et al. Identification of 13 new mutations in the vasopressin-neurophysin II gene in 17 kindreds with familial autosomal dominant neurohypophyseal diabetes insipidus. Am J Hum Genet. 1996;58:107–17.

    PubMed  CAS  Google Scholar 

  21. Grant FD, Ahmadi A, Hosley CM, Majzoub JA. Two novel mutations of the vasopressin gene associated with familial diabetes insipidus and identification of an asymptomatic carrier infant. J Clin Endocrinol Metab. 1998;83:3958–64.

    Article  PubMed  CAS  Google Scholar 

  22. Willcutts MD, Felner E, White PC. Autosomal recessive familial neurohypophyseal diabetes insipidus with continued secretion of mutant weakly active vasopressin. Hum Mol Genet. 1999;8:1303–7.

    Article  PubMed  CAS  Google Scholar 

  23. Greger NG, Kirkland RT, Clayton GW, Kirkland JL. Central diabetes insipidus. 22 years’ experience. Am J Dis Child. 1986;140:551–4.

    PubMed  CAS  Google Scholar 

  24. Charmandari E, Brook CG. 20 years of experience in idiopathic central diabetes insipidus. Lancet. 1999; 353:2212–3.

    Article  PubMed  CAS  Google Scholar 

  25. Moses AM. Clinical and laboratory observations in the adult with diabetes insipidus and related syndromes. In: Czernichow P, Robinson AG, editors. Diabetes insipidus in man, Frontiers of hormone research, vol. 13. Basel: Karger; 1985. p. 156–75.

    Google Scholar 

  26. Mootha SL, Barkovich AJ, Grumbach MM, et al. Idiopathic hypothalamic diabetes insipidus, pituitary stalk thickening, and the occult intracranial germinoma in children and adolescents. J Clin Endocrinol Metab. 1997;82(5):1362–7.

    Article  PubMed  CAS  Google Scholar 

  27. Heinze HJ, Bercu BB. Acquired hypophysitis in adolescence. J Pediatr Endocrinol Metab. 1997;10:315–21.

    Article  PubMed  CAS  Google Scholar 

  28. Honegger J, Fahlbusch R, Bornemann A, et al. Lymphocytic and granulomatous hypophysitis: experience with nine cases. Neurosurgery. 1997;40:713–22.

    Article  PubMed  CAS  Google Scholar 

  29. Iwasaki Y, Oiso Y, Kondo K, et al. Aggravation of subclinical diabetes insipidus during pregnancy. N Engl J Med. 1991;324:522–6.

    Article  PubMed  CAS  Google Scholar 

  30. Fujiwara TM, Morgan K, Bichet DG. Molecular biology of diabetes insipidus. Annu Rev Med. 1995;46: 331–43.

    Article  PubMed  CAS  Google Scholar 

  31. Schoneberg T, Schulz A, Biebermann H, et al. V2 vasopressin receptor dysfunction in nephrogenic diabetes insipidus caused by different molecular mechanisms. Hum Mutat. 1998;12:196–205.

    Article  PubMed  CAS  Google Scholar 

  32. Wildin RS, Cogdell DE, Valadez V. AVPR2 variants and V2 vasopressin receptor function in nephrogenic diabetes insipidus. Kidney Int. 1998;54:1909–22.

    Article  PubMed  CAS  Google Scholar 

  33. Feldman BJ, Rosenthal SM, Vargas GA, et al. Nephrogenic syndrome of inappropriate antidiuresis. N Engl J Med. 2005;352:1884–90.

    Article  PubMed  CAS  Google Scholar 

  34. Kamsteeg EJ, Deen PM, van Os CH. Defective processing and trafficking of water channels in nephrogenic diabetes insipidus. Exp Nephrol. 2000;8: 326–31.

    Article  PubMed  CAS  Google Scholar 

  35. Tamarappoo BK, Yang B, Verkman AS. Misfolding of mutant aquaporin-2 water channels in nephrogenic diabetes insipidus. J Biol Chem. 1999;274:34825–31.

    Article  PubMed  CAS  Google Scholar 

  36. Goji K, Kuwahara M, Gu Y, Matsuo M, Marumo F, Sasaki S. Novel mutations in aquaporin-2 gene in female siblings with nephrogenic diabetes insipidus: evidence of disrupted water channel function. J Clin Endocrinol Metab. 1998;83:3205–9.

    Article  PubMed  CAS  Google Scholar 

  37. Kamsteeg EJ, Wormhoudt TA, Rijss JP, van Os CH, Deen PM. An impaired routing of wild-type aquaporin-2 after tetramerization with an aquaporin-2 mutant explains dominant nephrogenic diabetes insipidus. EMBO J. 1999;18:2394–400.

    Article  PubMed  CAS  Google Scholar 

  38. Leung AK, Robson WL, Halperin ML. Polyuria in childhood. Clin Pediatr. 1991;30:634–40.

    Article  CAS  Google Scholar 

  39. Bendz H, Aurell M. Drug-induced diabetes insipidus: incidence, prevention and management. Drug Saf. 1999;21:449–56.

    Article  PubMed  CAS  Google Scholar 

  40. Timmer RT, Sands JM. Lithium intoxication. J Am Soc Nephrol. 1999;10:666–74.

    PubMed  CAS  Google Scholar 

  41. Robertson GL. Diagnosis of diabetes insipidus. In: Czernichow P, Robinson AG, editors. Diabetes insipidus in man, Frontiers of hormone research, vol. 13. Basel: Karger; 1985. p. 176–89.

    Google Scholar 

  42. Dunger DB, Seckl JR, Grant DB, Yeoman L, Lightman SL. A short water deprivation test incorporating urinary arginine vasopressin estimations for the investigation of posterior pituitary function in children. Acta Endocrinol (Copenh). 1988;117:13–8.

    CAS  Google Scholar 

  43. Mohn A, Acerini CL, Cheetham TD, Lightman SL, Dunger DB. Hypertonic saline test for the investigation of posterior pituitary function. Arch Dis Child. 1998;79:431–4.

    Article  PubMed  CAS  Google Scholar 

  44. Kluge M, Riedl S, Erhart-Hofmann B, Hartmann J, Waldhauser F. Improved extraction procedure and RIA for determination of arginine8-vasopressin in plasma: role of premeasurement sample treatment and reference values in children. Clin Chem. 1999;45:98–103.

    PubMed  CAS  Google Scholar 

  45. Elster AD. Imaging of the sella: anatomy and pathology. Semin Ultrasound CT MR. 1993;14:182–94.

    Article  PubMed  CAS  Google Scholar 

  46. Halimi P, Sigal R, Doyon D, Delivet S, Bouchard P, Pigeau I. Post-traumatic diabetes insipidus: MR demonstration of pituitary stalk rupture. J Comput Assist Tomogr. 1988;12(1):135–7.

    Article  PubMed  CAS  Google Scholar 

  47. Maghnie M, Villa A, Arico M, et al. Correlation between magnetic resonance imaging of posterior pituitary and neurohypophyseal function in children with diabetes insipidus. J Clin Endocrinol Metab. 1992;74:795–800.

    Article  PubMed  CAS  Google Scholar 

  48. Moses AM, Clayton B, Hochhauser L. Use of T1-weighted MR imaging to differentiate between primary polydipsia and central diabetes insipidus. AJNR Am J Neuroradiol. 1992;13:1273–7.

    PubMed  CAS  Google Scholar 

  49. Uribarri J, Kaskas M. Hereditary nephrogenic diabetes insipidus and bilateral nonobstructive hydronephrosis. Nephron. 1993;65:346–9.

    Article  PubMed  CAS  Google Scholar 

  50. Robinson AG, Verbalis JG. Treatment of central diabetes mellitus. In: Czernichow P, Robinson AG, editors. Diabetes insipidus in man, Frontiers of hormone research, vol. 13. Basel: Karger; 1985. p. 292–303.

    Google Scholar 

  51. Richardson DW, Robinson AG. Desmopressin. Ann Intern Med. 1985;103:228–39.

    PubMed  CAS  Google Scholar 

  52. Cobb WE, Spare S, Reichlin S. Neurogenic diabetes insipidus: management with dDAVP (1-desamino-8-D arginine vasopressin). Ann Intern Med. 1978; 88:183–8.

    PubMed  CAS  Google Scholar 

  53. Bryant WP, O’Marcaigh AS, Ledger GA, Zimmerman D. Aqueous vasopressin infusion during chemotherapy in patients with diabetes insipidus. Cancer. 1994; 74:2589–92.

    Article  PubMed  CAS  Google Scholar 

  54. Rivkees SA, Dunbar N, Wilson TA. The management of central diabetes insipidus in infancy: desmopressin, low renal solute load formula, thiazide diuretics. J Pediatr Endocrinol Metab. 2007;20:459–69.

    PubMed  CAS  Google Scholar 

  55. Blanco EJ, Lane AH, Aijaz N, Blumberg D, Wilson TA. Use of subcutaneous DDAVP in infants with central diabetes insipidus. J Pediatr Endocrinol Metab. 2006;19:919–25.

    Article  PubMed  CAS  Google Scholar 

  56. Libber S, Harrison H, Spector D. Treatment of nephrogenic diabetes insipidus with prostaglandin synthesis inhibitors. J Pediatr. 1986;108:305–11.

    Article  PubMed  CAS  Google Scholar 

  57. Kirchlechner V, Koller DY, Seidl R, Waldhauser F. Treatment of nephrogenic diabetes insipidus with hydrochlorothiazide and amiloride. Arch Dis Child. 1999;80:548–52.

    Article  PubMed  CAS  Google Scholar 

  58. Hoekstra JA, van Lieburg AF, Monnens LA, Hulstijn-Dirkmaat GM, Knoers VV. Cognitive and psychosocial functioning of patients with congenital nephrogenic diabetes insipidus. Am J Med Genet. 1996;61:81–8.

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Frederick D. Grant M.D. .

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Srivatsa, A., Grant, F.D. (2013). Diabetes Insipidus. In: Radovick, S., MacGillivray, M. (eds) Pediatric Endocrinology. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-395-4_9

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  • DOI: https://doi.org/10.1007/978-1-60761-395-4_9

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