Hereditary diabetes insipidus
Hereditary diabetes insipidus is a disorder caused by mutation of genes that impaire either vasopressin production or action. It is clinically characterized by an abnormally large volume of diluted urine.
Less than 10% of diabetes insipidus can be attributed to hereditary forms.[Error: Macro 'ref' doesn't exist]
As most patients with diabetes insipidus have aquired forms, this has to be excluded primarily.
Polyuria is most prominent in diabetes insipidus. Urine osmolality is usually below 300 mosmol/kg and total solute excretion normal (600 to 900 mosmol/day).
Fujiwara TM et al. (2005) Molecular biology of hereditary diabetes insipidus.[^]