Nephrogenic Diabetes Insipidus (NDI) is a disease condition wherein the kidneys are unable to retain water, resulting in an excessive amount of dilute urine production, and excessive thirst. Congenital NDI is characterized by irritability, lethargy, fever, vomiting, diarrhea, seizures, and a failure to thrive in infants. Affected infants show signs of dehydration, along with a low blood pressure and rapid pulse. Complications of the condition result when the child does not drink enough water to prevent dehydration. In such a case, the highly concentrated blood plasma can cause permanent brain damage and death. Greatly increased urine output with a low osmolality, in the presence of normal or high levels of the anti-diuretic hormone are diagnostic indicators for NDI.
Management of NDI requires most of all, a regular intake of water by the patient. Certain medications may help in reducing the rate of urine formation; most notably non-steroidal anti-inflammatory drugs and thiazide diuretics. Patients may present with dehydration or shock, which requires treatment with isotonic fluid or gradual replacement of the water deficit. Patients will need life long monitoring. However, with proper management, a normal life expectancy can be attained.