Nephrogenic diabetes insipidus

Diabetes insipidus is a rare disease that causes frequent urination. The large volume of urine is diluted, mostly water.

Nephrogenic diabetes insipidus results from inability of the kidneys to concentrate urine. This due to failure of the kidneys to respond to antidiuretic hormone (ADH; and also named arginine vasopressin or AVP), resulting in increased excretion of dilute urine.

The kidneys’ ability to respond to ADH can be impaired by drugs—like lithium, for example—and by chronic disorders including polycystic kidney disease, sickle cell disease, kidney failure, partial blockage of the ureters, and inherited genetic disorders.

Polyuria, with hyposthenuria, and polydipsia are the fundamental clinical manifestations of the disease.

Nephrogenic diabetes insipidus may be caused by a defect in the vasopressin-induced permeability of the distal tubules and collecting ducts to water, an insufficient buildup of the corticopapillary interstitial osmotic gradient, or to a combination of these two factors.
Nephrogenic diabetes insipidus

Nephrogenic diabetes insipidus

Nephrogenic diabetes insipidus is characterized by renal resistance to the action of antidiuretic hormone (ADH) secretion.  The renal tube is unresponsive to ADH.

This disorder can be congenital or acquired. Genetic causes are less common but more severe than acquired forms of the disease, although genetic etiologies are more common in children than in adults.

Acquired causes of nephrogenic diabetes insipidus are more common. It may be caused by drugs such as lithium and demeclocycline, both of which are thought to interfere with vasopressin-stimulated cAMP generation or action.

In 85% of congenital diabetes insipidus, the disorder is inherited as an x-linked mutation.

Patients with nephrogenic diabetes insipidus usually have onset of symptoms in infancy, and present with recurrent episodes of dehydration, fever, failure to thrive and psychomotor retardation.

Patients with nephrogenic diabetes insipidus may develop severe dehydration of water is withheld for prolonged periods.
Nephrogenic diabetes insipidus