Diabetes insipidus

Presse Med. 2021 Dec;50(4):104093. doi: 10.1016/j.lpm.2021.104093. Epub 2021 Oct 27.

Abstract

Diabetes insipidus (DI) is a disorder characterized by a high hypotonic urinary output of more than 50ml per kg body weight per 24 hours, with associated polydipsia of more than 3 liters a day [1,2]. Central DI results from inadequate secretion and usually deficient synthesis of Arginine vasopressin (AVP) in the hypothalamus or pituitary gland. Besides central DI further underlying etiologies of DI can be due to other primary forms (renal origin) or secondary forms of polyuria (resulting from primary polydipsia). All these forms belong to the Polyuria Polydipsia Syndrom (PPS). In most cases central and nephrogenic DI are acquired, but there are also congenital forms caused by genetic mutations of the AVP gene (central DI) [3] or by mutations in the gene for the AVP V2R or the AQP2 water channel (nephrogenic DI) [4]. Primary polydipsia (PP) as secondary form of polyuria includes an excessive intake of large amounts of fluid leading to polyuria in the presence of intact AVP secretion and appropriate antidiuretic renal response. Differentiation between the three mentioned entities is difficult [5], especially in patients with Primary polydipsia or partial, mild forms of DI [1,6], but different tests for differential diagnosis, most recently based on measurement of copeptin, and a thorough medical history mostly lead to the correct diagnosis. This is important since treatment strategies vary and application of the wrong treatment can be dangerous [7]. Treatment of central DI consists of fluid management and drug therapy with the synthetic AVP analogue Desmopressin (DDAVP), that is used as nasal or oral preparation in most cases. Main side effect can be dilutional hyponatremia [8]. In this review we will focus on central diabetes insipidus and describe the prevalence, the clinical manifestations, the etiology as well as the differential diagnosis and management of central diabetes insipidus in the out- and inpatient setting.

Publication types

  • Review

MeSH terms

  • Adult
  • Antidiuretic Agents / therapeutic use
  • Aquaporin 2 / genetics
  • Child
  • Deamino Arginine Vasopressin / therapeutic use
  • Diabetes Insipidus* / diagnosis
  • Diabetes Insipidus* / etiology
  • Diabetes Insipidus* / therapy
  • Diagnosis, Differential
  • Glycopeptides / analysis
  • Humans
  • Mutation
  • Neurophysins / genetics
  • Neurophysins / metabolism
  • Pituitary Gland / diagnostic imaging
  • Pituitary Gland / metabolism
  • Polydipsia* / classification
  • Polydipsia* / diagnosis
  • Polydipsia* / etiology
  • Polyuria* / diagnosis
  • Polyuria* / etiology
  • Protein Precursors / genetics
  • Protein Precursors / metabolism
  • Vasopressins / genetics
  • Vasopressins / metabolism

Substances

  • AQP2 protein, human
  • AVP protein, human
  • Antidiuretic Agents
  • Aquaporin 2
  • Glycopeptides
  • Neurophysins
  • Protein Precursors
  • copeptins
  • Vasopressins
  • Deamino Arginine Vasopressin