Family, or congenital, diabetes insipidus

Family, or congenital, diabetes insipidus is an extremely rare disease that occurs in early childhood, regardless of sex. In pathoanatomical research, the insufficiency of development of supraoptic neurons of the hypothalamus was less common than paraventricular ones; a decreased neurohypophysis was also detected. In very rare cases, diabetes insipidus can be attributed to genetic diseases with autosomal dominant heredity or to JC-linked diseases; can be observed in the framework of such a rare disease as the Lawrence-Muna-Barde-Biddle syndrome.

[1], [2], [3], [4], [5], [6]

Causes of the congenital diabetes insipidus

The causes of diabetes insipidus can also be vascular diseases. First of all, these are aneurysms of the arterial circle of the large brain (Willis circle), most often an aneurysm of the anterior communicating artery. The rupture of the aneurysm of the anterior part of the arterial circle of the large brain can lead to damage to the supraoptic nuclei of the hypothalamus and the infundibular region. Thus, a clinical picture of diabetes insipidus can be seen in ischemic postnatal necrosis of the pituitary gland within the framework of the Shykhain syndrome, when it is combined with insufficiency of the anterior pituitary hormones.

[7], [8], [9], [10], [11], [12], [13], [14], [15]

Treatment of the congenital diabetes insipidus

Treatment of congenital diabetes insipidus of moderate severity should not begin with the use of drugs containing antidiuretic hormone. The initial treatment should contain diuretic drugs of chlortiazidovoy series (hypothiazide is preferred to 25 mg 4 times a day) and a hypoglycemic drug, the sulfanylurea derivative - chlorpropamide, 100-200 mg per day.

The mechanism of action of these drugs in diabetes insipidus is not completely clear. It is believed that hypothiazide improves the concentration ability of the kidneys (inhibits the reabsorption of sodium in the ascending knee of the Henle loop, thereby preventing maximum urine dilution). By reducing the amount of sodium in the body a few times, hypothiazide reduces the extracellular volume of the fluid and increases the reabsorption of salts and water in the proximal tubules, resulting in an increase in the relative density of urine and proportionally decrease its volume. In addition, hypothiazide acts depressingly on the central mechanisms of thirst.

Hypoglycemic drugs increase the effect of antidiuretic hormone on the renal tubules and stimulate the secretion of antidiuretic hormone. There are reports of the effectiveness of small doses of finlepsin - 0.2 g 1-2 times a day. Finlepsin is able to cause hyponatraemia, thereby regulating the salt balance and improving the course of the disease. There was also a positive effect when taking clofibrate (Miscil- eron) 2 capsules (0.25 g) 3 times a day.

The mechanism of action of this drug in diabetes insipidus is not fully understood. It is believed that it is capable of releasing an endogenous antidiuretic hormone.

In the treatment of diabetes insipidus, it is necessary to influence the psychopathological syndrome by prescribing psychotropic drugs. There are indications of a decrease in symptoms of diabetes insipidus under the influence of amitriptyline and melleril. These drugs can reduce the hyperosmolarity of liquid media, cause hyponatremia. It is possible that, acting through a change in the level of catecholamines, these drugs improve the secretion of antidiuretic hormone.

In severe diabetes insipidus, it is necessary to use drugs containing an antidiuretic hormone: adiurecrin in powder, which is inhaled through the nose at 0,03-0,05 g 3 times a day (the action occurs after 15-20 minutes and lasts about 6-8 hours) or pituitrin in the form of subcutaneous or intramuscular injections of 1 ml (5 units) 2 times a day. Treatment with drugs containing antidiuretic hormone should be long. All these drugs are ineffective in the treatment of patients with nephrogenic diabetes insipidus. Along with pharmacotherapy, one should also remember about such an auxiliary therapeutic method as limiting salt intake.