In a healthy person, AVP acts as a signal to the nephrons to reabsorb water into the body. In nephrogenic diabetes insipidus, the nephrons in the kidneys are not able to respond to this signal, leading to excessive water loss in large amounts of urine. The thirst increases to try to balance this loss from the body.
Nephrogenic diabetes insipidus can be either acquired or hereditary. The acquired form is brought on by certain drugs and chronic diseases and can occur at any time during life. Certain drugs, such as lithium or antiviral medications such as foscarnet (Foscavir), also can cause nephrogenic diabetes insipidus. Nephrogenic diabetes insipidus may also be a temporary complication associated with pregnancy.
The most common inherited nephrogenic diabetes insipidus is an X-linked trait with variable penetrance in heterozygous females that affects the arginine vasopressin (AVP) receptor 2 gene. Heterozygous females may have no symptoms or a variable degree of polyuria and polydipsia, or they may be as severely affected as males.
The two main symptoms of nephrogenic diabetes insipidus are chronic excessive thirst (polydipsia) and excessive urine production (polyuria). Excessive urination at night (nocturia) also occurs. Infants with hereditary nephrogenic diabetes insipidus may eat poorly and fail to gain weight and grow at the expected rate (failure to thrive). They may also be irritable and experience fevers, diarrhea, and vomiting.
Nephrogenic diabetes insipidus
