Abstract
A 6-year-old girl with recurrent episodes of hypertonic dehydration was studied. She denied thirst even with a plasma osmolality as high as 421 mosmol/kg. The hypernatremia was associated with an ability to concentrate urine (854 mosmol/kg). Volume expansion with water corrected hypernatremia (162 to 148 mEq/l) and resulted in an increased urine flow and urinary dilution (137 mosmol/kg) because of suppression of endogenousvasopressin (AVP) release (5.1 pg/ml). Hypertonic saline infusion raised the plasma AVP level (25.6 pg/ml) in response to changes in plasma osmolality (305 to 330 mosmol/kg) and led to a maximal urine osmolality of 818 mosmol/kg. With chronic forced fluid intake, the patient maintained a normal resum sodium concentration (range, 135–145 mEq/l) with a urine osmolality as low as 65 mosmol/kg. These findings are consistent with an isolated defect in the osmoregulation of thirst as the cause of the chronic hypertonic dehydration without deficiency in AVP secretion.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Robertson GL, Berl T (1986) Water metabolism. In: Brenner BM, Rector FC Jr (eds) The kidney, 3rd edn. Saunders, Philadelphia, pp 385–432
Robertson GL (1983) Thirst and vasopressin function in normal and disordered states of water balance. J Lab Clin Med 101: 351–371
Andersson G (1971) Thirst and brain control of water balance. Am Sci 59: 408–414
Robertson GL, Athar S, Shelton RL (1977) Osmotic control of vasopressin function in disturbances. In: Andreoli TE, Grantham JJ, Rector FC Jr (eds) Disturbances in body fluid osmolality. American Physiology Society, Bethesda, pp 125–148
Peck JW, Blass EM (1975) Localization of thirst and antidiuretic osmoreceptors by intracranial injections in rats. Am J Physiol 228: 1501–1509
Assadi FK, Norman ME, Parks JS, Schwartz MW (1977) Hypernatremia associated with pineal tumor. J Pediatr 90: 605–606
DeRubertis FR, Cichelis MF, Davis BB (1974) “Essential” hypernatremia: report of three cases and review of the literature. Arch Intern Med 134: 889–895
Lascelles PT, Lewis PD (1972) Hypodipsia and hypernatremia associated with hypothalamic and suprasellar lesions. Brain 95: 249–264
DeRubertis FR, Michels MF, Beck N, Field JB, Davis BB (1971) Essential hypernatremia due to ineffective osmotic and intact volume regulation of vasopressin secretion. J Clin Invest 50: 97–111
Gossain VV, Kinzel T, Strand CV, Rovner DR (1978) Essential hypernatremia. Am J Med Sci 275: 353–358
Gill G, Baylis P, Burn J (1985) A case of essential hypernatremia due to resetting of the osmostat. Clin Endocrinol 22: 545–551
Travis LB, Dodge WF, Waggner JD, Kashemsant C (1967) Defective thirst mechanism secondary to a hypothalamic lesion: studies in a child with adipsia, polyphagia, obesity, and persistent hyperosmolality. J Pediatr 70: 915–926
Spiro SG, Jenkins JS (1971) Adipsia and hypothermia after subarachnoid hemorrhage. Br Med J 3: 411–412
Golonka JE, Richardson JA (1970) Postconcussive hyperosmolality and deficient thirst. Am J Med 48: 261–267
Conley SB, Brocklebank T, Taylor IT, Robson AM (1976) Recurrent hypernatremia: a proposed mechanism in a patient with absence of thirst and abnormal excretion of water. J Pediatr 89: 898–903
Halter JB, Goldberg AP, Robertson GL, Porte D Jr (1977) Selective osmoreceptor dysfunction in the syndrome of chronic hypernatremia. J Clin Endocrinol Metab 44: 609–616
Hammond DN, Moll GW, Robertson GL, Chelmicka-Schorr E (1986) Hypodipsic hypernatremia with normal osmoregulation of vasopressin. N Engl J Med 315: 433–436
Robertson GL (1984) Abnormalities of thirst regulation. Kidney Int 25: 460–469
Hays RM, McHugh PR, Williams HE (1963) Absence of thirst in association with hydrocephalus. N Engl J Med 269: 227–231
Sagar WE (1966) Chronic hyperosmolality. Am J Dis Child 112: 318–327
Harrington AR; Valtin H (1968) Impaired urinary concentration after vasopressin and its gradual correction in hypothalamic diabetes insipidus. J Clin Invest 47: 502–510
Alexander CS, Filbin DM, Fruchtman SA (1959) Failure of vasopressin to produce normal urine concentration in patients with diabetes insipidus. J Lab Clin Med 54: 566–571
Kamm DE, Levinsky NG, Wilson C (1965) Inhibition of renal tubular sodium reabsorption by hypernatremia. J Clin Invest 44: 1144–1150
Robertson GI, Mahr EA, Athar S, Sinha T (1973) Development and clinical application of a new method for the radioimmunoassay of arginine vasopressin in human plasma. J Clin Invest 52: 2340–2352
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Assadi, F.K., Johnston, B., Dawson, M. et al. Recurrent hypertonic dehydration due to selective defect in the osmoregulation of thirst. Pediatr Nephrol 3, 438–442 (1989). https://doi.org/10.1007/BF00850223
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00850223