The diabetes insipidus (from Ancient Greek διαβαίνειν diabainein to pass through 'and Latin insipidus , without taste' "tasteless Harnruhr" - as opposed to "honeyed Harnruhr", the diabetes mellitus ), and diabetes spurius , Wasserharnruhr and (more rarely) water Ruhr called is a congenital or acquired disease that is characterized by increased urine excretion ( polyuria ) and an increased feeling of thirst ( polydipsia ) with increased drinking and is mostly caused by a disruption of the neurohumoral regulation between the posterior pituitary gland and hypothalamic nuclei. Children, adolescents and young adults are more likely to be affected than older people.
Forms of diabetes insipidus
Diabetes insipidus centralis
With central diabetes insipidus (also called diabetes insipidus neurohormonalis and pituitary-diencephalic polyuria ) the cause is a lack or insufficient production of the antidiuretic hormone ADH (syn .: vasopressin) in the hypothalamus , a lack of transport of the ADH from the hypothalamus via the pituitary stalk into the Posterior lobe of the pituitary gland or lack of storage or lack of secretion of ADH in the posterior lobe of the pituitary gland. The ADH has an antidiuretic effect on the collecting pipes - it counteracts urine excretion - and leads to the formation of more concentrated urine.
ADH deficiency can be caused by a traumatic brain injury with tearing off the pituitary stalk, a cyst, an operation, an inflammation, an infiltrative disease, a bleeding, an infarction or a tumor in the hypothalamus or in the pituitary gland . Rare can also granulomatosis , z. B. Langerhans cell histiocytosis , sarcoid or granulomatosis with polyangiitis trigger central diabetes insipidus. But familial diabetes insipidus with a congenital, autosomal dominant inherited genetic defect is also possible as a cause. In a third of all diabetes insipidus cases, the cause is unknown and an autoimmune disease with autoantibodies against the vasopressin-producing cells is suspected.
Diabetes insipidus renalis
In the rare form of renal (also: nephrogenic = originating from the kidney) diabetes insipidus (syn. ADH- or vasopressin-resistant diabetes insipidus ), the defect lies in the kidney, which cannot produce normally concentrated urine despite the presence of the hormone ADH because the aquaporin channel AQP2, which is necessary for reabsorbing the water from the primary urine, is defective or missing, or because the renal tubules are too severely damaged by chronic kidney disease or medication (e.g. lithium ).
- Laboratory examination of serum and urine molarity
- ADH administration with an increase in urine molarity in central diabetes insipidus and no increase in renal diabetes insipidus
- In the thirst test, the ADH concentration (antidiuretic hormone, vasopressin ) in the blood plasma or urine is measured after a nocturnal period of thirst ; In the case of diabetes insipidus centralis, there is no increase in ADH. Another test is to periodically take measurements of the volume, specific gravity and osmolarity of the urine collected during thirst . A constant urine volume and low osmolarity are symptomatic of diabetes insipidus. In healthy individuals, the urine weight and osmolarity increase physiologically. Copeptin is now measured instead of ADH because it is easier to measure than ADH.
- Finding the underlying disease
|Bartter syndrome||Schwartz-Bartter Syndrome||Diabetes insipidus centralis||Diabetes insipidus renalis|
|Pathophysiology||Channel defect in the kidney
(Na + / K + / 2Cl - -Symporter, ROMK or CLCKB )
|inappropriately high ADH secretion
||insufficient ADH secretion
ADH type 2 receptor defect
or aquaporin 2 defect
|etiology||hereditary (autosomal recessive)||
|clinic||Salt appetite, muscle weakness and muscle pain , cramps||CNS symptoms , muscle weakness and pain , cramps||increased amount of urine , increased amount of drink||increased amount of urine , increased amount of drink|
|laboratory||Serum: Na + ↓, K + ↓, osmolality ↓, pH value ↑||Serum: ADH ↑, Na + ↓, K + ↓, osmolality ↓, pH value ↑||Serum: ADH ↓, Na + ↑, osmolality ↑||Serum: ADH ↔, Na + ↑, osmolality ↑|
|Urine: Na + ↑, K + ↑, osmolality ↑||Urine: Na + ↑, osmolality ↑||Urine: Na + ↓, osmolality ↓||Urine: Na + ↓, osmolality ↓|
|Further diagnostics||Evidence of secondary aldosteronism|
Therapeutic approaches are the correction and avoidance of a possible water deficit as well as a reduction of the urine losses. In awake patients with an intact feeling of thirst, polyuria and polydipsia are often restrictive in everyday life and must therefore be remedied. In comatose patients, however, there is a risk of dehydration and hypernatremia.
In the case of ADH deficiency, synthetic ADH (or the ADH analogue desmopressin) is administered daily as a nasal spray , tablet or subcutaneous injection. The ADH gets into the blood and with the blood to the kidneys. Treatment is more difficult in renal diabetes insipidus . Increased fluid intake is mandatory here. Thiazide diuretics can also be helpful because they cause increased sodium excretion and more concentrated urine. Especially in the presence of a central diabetes insipidus needs to treatable underlying diseases, such as tumors of the midbrain are sought (diencephalon).
- Joachim Frey : Pituitary-diencephalic poly- and oliguria. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition, ibid. 1961, pp. 917-919.
- Child-friendly explanation among other things about diabetes insipidus
- Sandrina Balanescua, Jonas Rutishauser: Diabetes insipidus: differential diagnosis and therapy (PDF; 682 kB) Switzerland. Med. Forum, 2010
- Garabed Eknoyan: A history of diabetes insipidus: paving the road to internal water balance . In: American Journal of Kidney Diseases . 56, No. 6, December 2010, , pp. 1175-1183. doi : 10.1053 / j.ajkd.2010.08.002 . PMID 20888099 . Medical history presentation (English)
- Diabetes insipidus . On: Medizinfo.de ; accessed on February 9, 2018.
- Ludwig Weissbecker: Diseases of the pituitary-diencephalon system. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition ibid. 1961, pp. 1008-1013, here: pp. 1009 f.
- Diabetes insipidus . On: Medizinfo.de ; accessed on February 9, 2018.
- Diabetes insipidus renalis , abstract on Medizinische-genetik.de, accessed on August 23, 2019
- Jump up W. Fenske, J. Refardt, I. Chifu, I. Schnyder, B. Winzeler, J. Drummond, A. Ribeiro-Oliveira, T. Drescher, S. Bilz, DR Vogt, U. Malzahn, M. Kroiss, E. Christ, C. Henzen, S. Fischli, A. Tönjes, B. Müller, J. Schopohl, J. Flitsch, G. Brabant, M. Fassnacht, M. Christ-Crain: A Copeptin-Based Approach in the Diagnosis of Diabetes Insipidus. In: The New England Journal of Medicine . tape 379 , 2018, p. 428-39 , doi : 10.1056 / NEJMoa1803760 .
- Gerd Herold and colleagues: Internal Medicine 2020. Self-published, Cologne 2020, ISBN 978-3-9814660-9-6 , pp. 633 and 803–805.
- Sandrina Balanescua, Jonas Rutishauser: Diabetes insipidus: differential diagnosis and therapy. In: Swiss Medical Forum. 2010, No. 123.