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Central diabetes insipidus.

Hiroshi Arima1, Yoshinori Azuma1, Yoshiaki Morishita1

  • 1Department of Endocrinology and Diabetes, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Nagoya Journal of Medical Science
|December 24, 2016
PubMed
Summary

Central diabetes insipidus (CDI) results from arginine vasopressin (AVP) deficiency, leading to excessive urination and thirst. Treatment with desmopressin, particularly oral disintegrating tablets, improves quality of life and reduces hyponatremia risk.

Keywords:
adipsiaarginine vasopressindesmopressinpolydipsiapolyuria

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Area of Science:

  • Endocrinology
  • Nephrology
  • Genetics

Background:

  • Central diabetes insipidus (CDI) is a condition marked by polyuria and polydipsia due to arginine vasopressin (AVP) deficiency.
  • CDI subtypes include idiopathic, secondary (often caused by CNS tumors), and familial forms with numerous identified AVP gene mutations.

Approach:

  • Review of CDI classification, etiologies, and treatment strategies.
  • Focus on desmopressin therapy, comparing administration routes and formulations.
  • Discussion of challenges in managing adipsic CDI patients.

Key Points:

  • Idiopathic CDI may involve infundibulo-neurohypophysitis; secondary CDI frequently stems from tumors like craniopharyngioma.
  • Familial CDI is typically autosomal dominant, linked to over 80 mutations in the AVP gene locus.
  • Oral desmopressin, especially disintegrating tablets, enhances patient quality of life and lowers hyponatremia incidence.

Conclusions:

  • Desmopressin is the primary treatment for CDI, with oral formulations offering administration advantages.
  • Adipsic CDI presents significant management difficulties, high morbidity, and mortality due to impaired thirst sensation.
  • Further research into managing complex CDI cases, including adipsic forms, is warranted.