GB90.4A
Nephrogenic diabetes insipidus
Diabetes insípido nefrogênico
CategoryDefinition
Nephrogenic diabetes insipidus is a condition in which the kidney tubules respond poorly to pituitary secreted anti-diuretic hormone, resulting in a failure to concentrate the urine, and water loss. Polyuria with dilute urine and polydypsia (excessive thirst) are present. It can be congenital or acquired with many causes. The congenital forms may be attributed to vasopressin receptor or aquaporin-2 defects. They are characterised by polyuria with polydipsia, recurrent bouts of fever, constipation, and acute hypernatraemic dehydration after birth that may cause neurological sequelae.
Exclusions
- Central diabetes insipidus
Index Terms
Nephrogenic diabetes insipidusrenal diabetes insipidusfamilial nephrogenic diabetesantidiuretic-hormone-resistant diabetes insipidusadiuretin-resistant diabetes insipidusNDI - [nephrogenic diabetes insipidus]diabetes tenuifluusADH - [antidiuretic-hormone] resistant diabetes insipiduscongenital nephrogenic diabetes insipidushereditary nephrogenic diabetes insipidusfamilial nephrogenic diabetes insipidusprimary nephrogenic diabetes insipidusNephrogenic diabetes insipidus - intracranial calcificationNephrogenic Diabetes Insipidus associated with familial or genetic disorders elsewhere classifiedAQP2 mutationX-linked AVPR2 mutationAcquired nephrogenic diabetes insipidus due to causes classified elsewhereVasopressin-resistant hyposthenuriavasopressin-resistant diabetes insipidusVasopressin-unresponsiveness hyposthenuria