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Adipsic Diabetes Insipidus in Pediatric Patients

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Abstract

Objective

To present symptoms, complications and proposition of management protocol in children diagnosed with adipsic diabetes insipidus (aDI).

Methods

Clinical and biochemical analysis of six pediatric patients diagnosed with aDI, four boys aged 5, 13, 16, and 17 y and two girls aged 2.5 and 10 y. The etiology of aDI was germinoma (n = 2), extensive surgery due to optic glioma (n = 1) and astrocytoma (n = 1), congenital brain malformations (n = 1) and complications secondary to bacterial meningitis (n = 1). Two patients had severely impaired vision and two had hemiparesis.

Results

In all the patients, loss of thirst reflex was observed. The serum electrolytes in all patients showed sodium concentration from 159 to 176.6 mmol/L with plasma osmolality from above 297 mOsmol/kg. Polyuria was absent in three most severely dehydrated patients on admission. In two patients in whom DDAVP (1-desamino-8-D-arginine vasopressin; Desmopressin) therapy was withdrawn based on lack of polyuria deep venous thrombosis developed.

Conclusions

Lack of polydipsia and polyuria, the key symptoms of diabetes insipidus (DI), may delay the diagnosis of aDI and may lead to severe complications of chronic hyperosmolar status. The fluid intake in patients diagnosed with aDI need to be supervised daily based on calculated constant volume of oral fluids, daily measurements of fluid balance, body weight and sodium levels, especially in patients whose vision is compromised or who are physically unable to take care of themselves.

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Abbreviations

ACTH:

Adrenocorticotropic hormone

LH:

Luteinizing hormone

FSH:

Follicle stimulating hormone

GH:

Growth hormone

TSH:

Thyroid stimulating hormone

DDAVP:

1-desamino-8-D-arginine vasopressin; Desmopressin

AVP:

Arginine vasopressin

DI:

Diabetes insipidus

aDI:

Adipsic diabetes insipidus

References

  1. Di Iorgi N, Napoli F, Allegri AEM, Olivieri I, Bertelli E, Gallizia A, et al. Diabetes insipidus—diagnosis and management. Horm Res Paediatr. 2012;77:69–84.

    Article  PubMed  Google Scholar 

  2. Crowley RK, Sherlock M, Agha A, Smith D, Thompson CJ. Clinical insights into adipsic diabetes insipidus: a large case series. Clin Endocrinol. 2007;66:475–82.

    CAS  Google Scholar 

  3. Smith D, McKenna K, Moore K, Tormey W, Finucane J, Phillips J, et al. Baroregulation of vasopressin release in adipsic diabetes insipidus. J Clin Endocrinol Metab. 2002;87:4564–8.

    Article  CAS  PubMed  Google Scholar 

  4. Masera N, Grant DB, Stanhope R, Preece MA. Diabetes insipidus with impaired osmotic regulation in septo-optic dysplasia and agenesis of the corpus callosum. Arch Dis Child. 1994;70:51–3.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  5. Secco A, Allegri AE, di Iorgi N, Napoli F, Calcagno A, Bertelli E, et al. Posterior pituitary (PP) evaluation in patients with anterior pituitary defect associated with ectopic PP and septo-optic dysplasia. Eur J Endocrinol. 2011;165:411–20.

    Article  CAS  PubMed  Google Scholar 

  6. Smith D, Finucane F, Phillips J, Baylis PH, Finucane J, Tormey W, et al. Abnormal regulation of thirst and vasopressin secretion following surgery for craniopharyngioma. Clin Endocrinol. 2004;61:273–9.

    Article  CAS  Google Scholar 

  7. Bergada I, Aversa L, Heinrich JJ. Peripheral venous thrombosis in children and adolescents with adipsic hypernatraemia secondary to hypothalamic tumours. Horm Res. 2004;61:108–10.

    Article  CAS  PubMed  Google Scholar 

  8. Latcha S, Lubetzky M, Weinstein AM. Severe hyperosmolarity and hypernatraemia in an adipsic young woman. Clin Nephrol. 2011;76:407–11.

    Article  CAS  PubMed  Google Scholar 

  9. Zantut-Wittmann DE, Garmes HM, Panzan AD, Lima Mde O, Baptista MT. Severe rhabdomyolysis due to adipsic hypernatraemia after craniopharyngioma surgery. Arq Bras Endocrinol Metab. 2007;51:1175–9.

    Article  Google Scholar 

  10. Ball SG, Vaidja B, Baylis PH. Hypothalamic adipsic syndrome: diagnosis and management. Clin Endocrinol. 1997;47:405–9.

    Article  CAS  Google Scholar 

  11. Bircan Z, Mutlu H, Cheong HI. Differential diagnosis of hereditary nephrogenic diabetes insipidus with desmopressin infusion test. Indian J Pediatr. 2010;77:1329–31.

    Article  PubMed  Google Scholar 

  12. Czernichow P, Polak M. Testing water regulation. In: Ranke MB, Mullis PE, editors. Diagnostics of endocrine function in children and adolescents. 4th ed. Switzerland: Karger; 2011. p. 194–209.

    Chapter  Google Scholar 

  13. Wade M, Baid S, Calis K, Raff H, Sinaii N, Nieman L. Technical details influence the diagnostic accuracy of the 1 mcg ACTH stimulation test. Eur J Endocrinol. 2010;162:109–13.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  14. O’Grady MJ, Hensey C, Fallon M, Hoey H, Murphy N, Costigan C, et al. Lack of sensitivity of the 1-lg low-dose ACTH stimulation test in a paediatric population with suboptimal cortisol responses to insulin-induced hypoglycaemia. Clin Endocrinol. 2013;78:73–8.

    Article  Google Scholar 

  15. Maghnie M, Uga E, Temporini F, Di Iorgi N, Secco A, Tinelli C, et al. Evaluation of adrenal function in patients with growth hormone deficiency and hypothalamic-pituitary disorders: comparison between insulin-induced hypoglycemia, low-dose ACTH, standard ACTH and CRH stimulation tests. Eur J Endocrinol. 2005;152:735–41.

    Article  CAS  PubMed  Google Scholar 

  16. Weintrob N, Sprecher E, Josefsberg Z, Weininger C, Aurbach-Klipper Y, Lazard D, et al. Standard and low-dose short adrenocorticotropin test compared with insulin-induced hypoglycemia for assessment of the hypothalamic-pituitary-adrenal axis in children with idiopathic multiple pituitary hormone deficiencies. J Clin Endocrinol Metab. 1998;83:88–92.

    Article  CAS  PubMed  Google Scholar 

  17. Johnston RJ, Wallace WHB. Normal ovarian function and assessment of ovarian reserve in children and young people. In: Ranke MB, Mullis P-E, editors. Diagnostics of endocrine function in children and adolescents. 4th ed. Switzerland: Karger; 2011. p. 331–49.

    Chapter  Google Scholar 

  18. Aron DC, Findling MW, Tyrrell JB. Hypothalamus and pituitary gland. In: Gardner GD, Shoback D, editors. Greenspan’s basic and clinical endocrinology. 8th ed. USA: McGrawHill; 2007. p. 101–56.

    Google Scholar 

  19. Repaske DR. Disorders of water balance. In: Brook CGD, Clayton PE, Brown RS, editors. Brook’s clinical pediatric endocrinology. 6th ed. Oxford: Wiley Blackwell; 2009. p. 343–73.

    Google Scholar 

  20. Carmody D, Hannon MJ, Thompson C. Vasopressin, diabetes insipidus, and the syndrome of inappropriate antidiuretic hormone secretion. In: Jameson JL, De Groot LJ, editors. Endocrinology adult and pediatric, vol. 1. 6th ed. Philadelphia: Saunders. Elsevier; 2010. p. 386–99.

    Google Scholar 

  21. Hannon MJ, Finucane FM, Sherlock M, Agha A, Thompson CJ. Disorders of water homeostasis in neurosurgical patients. J Clin Endocrinol Metab. 2012;97:1423–33.

    Article  CAS  PubMed  Google Scholar 

  22. Sinha A, Ball S, Jenkins A, Hale J, Cheetham T. Objective assessment of thirst recovery in patients with adipsic diabetes insipidus. Pituitary. 2011;14:307–11.

    Article  CAS  PubMed  Google Scholar 

  23. Cetinkaya S, Alikasifoglu A. Central diabetes insipidus associated with Caroli syndrome. Indian J Pediatr. 2007;74:419–20.

    Article  PubMed  Google Scholar 

  24. Gardner GD. Endocrine emergences. In: Gardner GD, Shoback D, editors. Greenspan’s basic and clinical endocrinology. 8th ed. USA: McGrawHill; 2007. p. 868–93.

    Google Scholar 

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Correspondence to Dominika Malgorzata Janus.

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Janus, D.M., Wojcik, M., Zygmunt-Górska, A. et al. Adipsic Diabetes Insipidus in Pediatric Patients. Indian J Pediatr 81, 1307–1314 (2014). https://doi.org/10.1007/s12098-014-1421-8

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