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Diabetes insipidus

Abstract

Diabetes insipidus (DI) is a disorder characterized by excretion of large amounts of hypotonic urine. Central DI results from a deficiency of the hormone arginine vasopressin (AVP) in the pituitary gland or the hypothalamus, whereas nephrogenic DI results from resistance to AVP in the kidneys. Central and nephrogenic DI are usually acquired, but genetic causes must be evaluated, especially if symptoms occur in early childhood. Central or nephrogenic DI must be differentiated from primary polydipsia, which involves excessive intake of large amounts of water despite normal AVP secretion and action. Primary polydipsia is most common in psychiatric patients and health enthusiasts but the polydipsia in a small subgroup of patients seems to be due to an abnormally low thirst threshold, a condition termed dipsogenic DI. Distinguishing between the different types of DI can be challenging and is done either by a water deprivation test or by hypertonic saline stimulation together with copeptin (or AVP) measurement. Furthermore, a detailed medical history, physical examination and imaging studies are needed to ensure an accurate DI diagnosis. Treatment of DI or primary polydipsia depends on the underlying aetiology and differs in central DI, nephrogenic DI and primary polydipsia.

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Fig. 1: Pathophysiology of DI.
Fig. 2: Pathogenetic mechanisms in acquired central DI.
Fig. 3: Pathogenetic mechanisms in nephrogenic DI.
Fig. 4: Models of pathogenesis in primary polydipsia in schizophrenia and gestational DI.
Fig. 5: Modified algorithm for differential diagnosis of polyuria–polydipsia syndrome.

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Acknowledgements

The authors thank N. Salvisberg for help with referencing.

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Introduction (M.C.-C.); Epidemiology (W.K.F.); Mechanisms/pathophysiology (D.G.B., M.B.G., S.R., J.G.V. and A.S.V.); Diagnosis, screening and prevention (M.C.-C and W.K.F.); Management (D.G.B., M.B.G. and J.G.V.); Quality of life (M.B.G.); Outlook (M.C.-C., D.G.B., W.K.F., M.B.G., S.R, J.G.V. and A.S.V.).

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Correspondence to Mirjam Christ-Crain.

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M.C.-C. and W.K.F. received speaking honoraria from Thermo Fisher AG, the manufacturer of the copeptin assay. All the other authors declare no competing interests.

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Nature Reviews Disease Primers thanks H. Arima, M. Magnie, S. Sasaki and Y. Sugimura for their contribution to the peer review of this work.

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Christ-Crain, M., Bichet, D.G., Fenske, W.K. et al. Diabetes insipidus. Nat Rev Dis Primers 5, 54 (2019). https://doi.org/10.1038/s41572-019-0103-2

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