Renal Diabetes Insipidus

Renal Diabetes Insipidus: causes, symptoms and treatment

Renal diabetes insipidus (RDI) is a hereditary disease that leads to impaired renal function associated with maintaining water-salt balance. In NDI, the kidneys are unable to retain enough water in the body, leading to excessive diuresis and dehydration.

Causes and pathogenesis

NDI is an inherited disease that is associated with loss of sensitivity of the renal tubules to antidiuretic hormone (ADH). This causes the kidneys to be unable to retain enough water in the body, leading to dehydration and other symptoms.

Symptoms

The disease manifests itself at 3-6 months of life with profuse diuresis, vomiting, a tendency to constipation and a feverish state. There may be no feeling of thirst. The volume of daily urine in infants can reach 2 liters, and in older children - 5-10 liters. “Salt fever” is observed and convulsive states are possible.

Persistent disturbances in water-salt balance can lead to the development of delayed physical and mental development. This is not observed with sufficient fluid administration.

Diagnostics

Renal biopsy may be required to diagnose LND. Microdissection reveals significant shortening of the proximal nephron tubules. Histological examination requires distinguishing this condition from nephronophthisis, chronic hypokalemia, idiopathic hypercalciuria, and cystinosis.

Functional studies can also help clarify the diagnosis of the disease. The test for urine concentration is based on excluding water intake for 12 hours (in children, it is better to use a night break for this purpose, for example, from 7 pm to 7 am). Carrying out this test is permissible only in doubtful cases, since in cases of obvious diabetes insipidus it is unsafe.

Treatment

Treatment of NND is aimed at eliminating dehydration and restoring water-salt balance. To do this, it is necessary to introduce a sufficient amount of fluid and electrolytes into the body. Severe dehydration may require hospitalization and intravenous fluids and electrolytes.

In addition, it is important to monitor the level of sodium and chlorine in the blood plasma and maintain it within normal limits. In some cases, it may be necessary to use drugs that increase the sensitivity of the kidneys to ADH.

Forecast

The prognosis of NND varies depending on the severity of the disease and the timeliness of treatment. With timely and adequate treatment, the prognosis is usually favorable, and children can achieve normal physical and mental development. However, if treatment is left untreated or treatment is delayed, serious complications may develop, such as delayed physical and mental development, impaired kidney function, and other health problems.

In general, for successful treatment of NDD, it is important to consult a doctor in a timely manner and follow his recommendations for treatment and monitoring of health status.