A Rare Case of Central Diabetes Incipidus Due to Post Pituitary Gland Agenesis: A Case Report

Document Type : Case Report

Authors

1 Department of Pediatrics, Shahid Sadoughi University of Medical sciences, Yazd, Iran

2 Department of Radiology, Shahid Sadoughi University of Medical Science, Yazd, Iran

Abstract

Background: Diabetes insipidus is a syndrome that begins with polyuria and is often associated with polydipsia. Three significant differential diagnosis are important to consider in evaluating the causes of polyuria, including primary polydipsia, diabetes mellitus, and diabetes insipidus. In diabetes incipidus, ADH hormone is not synthesized and secreted in central DI or the hormone has lost its function in the renal tubules in nephrogenic DI type.
Case Presentation: The present case was about 5.5 year-old-boy with polyurea and polydipsia from the beginning of the infancy. The patient had the serum osmolality of 277 mOsm/kg with the 24 hours urine volume of 4500 cc and urine osmolality of 200 mOsm/kg. The patient underwent water deprivation test for 12 hours. After administration of 20 μg of desmopressin spray, the urine osmolality increased to 720 mOsm/kg. Central diabetes insipidus was diagnosed. There was posterior hypophyseal agenesis in the brain MRI.
Conclusion: The above patient seemed to be one of the rare cases of autosomal recessive central DI that became symptomatic with polyuria and polydipsia from the early days of life. This rare and interesting case had isolated posterior pituitary agenesis. Desmopressin treatment resolved the patient's complaints. The patient was asymptomatic, and had normal growth in one year follow up.

Keywords

References

  1. Nichols R. Polyuria and polydipsia. Problems associated with patient evaluation. Probl Vet Med 1990; 2(4): 610-6.
  2. Rose BD, Post TW. Clinical physiology of acid-base and electrolyte disorders. 5th ed. New York, US: McGraw-Hill Education; 2001. p. 748-67.
  3. Arima H, Azuma Y, Morishita Y, Hagiwara D. Central diabetes insipidus. Nagoya J Med Sci 2016; 78(4): 349-58.
  4. Morello JP, Bichet DG. Nephrogenic diabetes insipidus. Annu Rev Physiol 2001; 63: 607-30.
  5. Earley LE, Orloff J. The mechanism of antidiuresis associated with the administration of hydrochlorothiazide to patients with vasopressin-resistant diabetes insipidus. J Clin Invest 1962; 41(11): 1988-97.
  6. Di IN, Napoli F, Allegri AE, Olivieri I, Bertelli E, Gallizia A, et al. Diabetes insipidus--diagnosis and management. Horm Res Paediatr 2012; 77(2): 69-84.
  7. Kimmel DW, O'Neill BP. Systemic cancer presenting as diabetes insipidus. Clinical and radiographic features of 11 patients with a review of metastatic-induced diabetes insipidus. Cancer 1983; 52(12): 2355-8.
  8. De BA, Colao A, Bizzarro A, Di SF, Coronella C, Solimeno S, et al. Longitudinal study of vasopressin-cell antibodies and of hypothalamic-pituitary region on magnetic resonance imaging in patients with autoimmune and idiopathic complete central diabetes insipidus. J Clin Endocrinol Metab 2002; 87(8): 3825-9.
  9. Maghnie M, Cosi G, Genovese E, Manca-Bitti ML, Cohen A, Zecca S, et al. Central diabetes insipidus in children and young adults. N Engl J Med 2000; 343(14): 998-1007.
  10. Grois N, Fahrner B, Arceci RJ, Henter JI, McClain K, Lassmann H, et al. Central nervous system disease in Langerhans cell histiocytosis. J Pediatr 2010; 156(6): 873-81.
  11. Hoyt WF, Kaplan SL, Grumbach MM, Glaser JS. Septo-optic dysplasia and pituitary dwarfism. Lancet 1970; 1(7652): 893-4.
  12. Karaca Z, Tanriverdi F, Atmaca H, Unluhizarci K, Kelestimur F. Posterior pituitary functions are not altered after growth hormone replacement therapy in hypopituitarism due to Sheehan's syndrome. Growth Horm IGF Res 2012; 22(3-4): 146-9.
  13. Simon D, Hadjiathanasiou C, Garel C, Czernichow P, Leger J. Phenotypic variability in children with growth hormone deficiency associated with posterior pituitary ectopia. Clin Endocrinol (Oxf) 2006; 64(4): 416-22.
World Journal of Peri and Neonatology
Volume 2, Issue 2
October 2019
Pages 87-90

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History

  • Receive Date: 04 October 2020
  • Accept Date: 04 October 2020
  • First Publish Date: 04 October 2020

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