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).
|Hereditary endocrinological diseases|
|Disorder of the thyroid hormon system|
|Disorders of sex development|
|Disorders of the growth control system|
|Disorders of the parathyroid hormone system|
|Genetic adrenal disease|
|Hereditary diabetes insipidus|
|Central diabetes insipidus|
|Nephrogenic diabetes insipidus|
|Diabetes insipidus and mellitus with optic atrophy and deafness|
|Wolfram syndrome 1|
|Wolfram syndrome 2|
|Hereditary dsorders of the pituitary gland|
|Kidney disease appearing as endocrinological disorders|
|Multiple endocrine neoplasia 1|
Fujiwara TM et al. (2005) Molecular biology of hereditary diabetes insipidus.[^]