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Inappropriate antidiuretic hormone secretion syndrome

ORPHA83449
Synonym(s) SIADH
Prevalence >1 / 1000
Inheritance X-linked recessive
Age of onset Infancy
Neonatal
ICD-10
  • E22.2
OMIM -
UMLS
  • C0021141
MeSH
  • D007177
MedDRA
  • 10053198

Summary

Syndrome of inappropriate antidiuretic hormone (ADH) secretion (SIADH) is characterized by continued ADH secretion, leading to hyponatremia, hypoosmolality and natriuresis. Exact prevalence is unknown. The disease has been described in all age groups. SIADH is often associated with tumors, pulmonary disease, central nervous system disorders or exposure to drugs. Occasionally, it is found in patients with adrenal, thyroid or pituitary insufficiency. The disorder is caused by gain-of-function mutations in the gene encoding the vasopression V2 receptor. Fluid restriction is the most common treatment. The outcome is related to the underlying and associated disorders.


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