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Familial, or congenital, non-sugar diabetes
Last reviewed: 04.07.2025
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Familial, or congenital, diabetes insipidus is an extremely rare disease that occurs in early childhood regardless of gender. In postmortem examination, underdevelopment of the supraoptic neurons of the hypothalamus was less common than that of the paraventricular neurons; a reduced neurohypophysis was also found. In very rare cases, diabetes insipidus can be attributed to genetic diseases with autosomal dominant inheritance or to JC-linked diseases; it can be observed within the framework of such a rare disease as Lawrence-Moon-Bardet-Biedl syndrome.
Causes congenital non-sugar diabetes.
The development of diabetes insipidus can also be caused by vascular diseases. First of all, these are aneurysms of the arterial circle of the brain (Willis circle), most often - aneurysms of the anterior communicating artery. Rupture of an aneurysm of the anterior part of the arterial circle of the brain can lead to damage to the supraoptic nuclei of the hypothalamus and infundibular region. This is how the clinical picture of diabetes insipidus can manifest itself in ischemic postpartum necrosis of the pituitary gland within the framework of Sheehan's syndrome, when it is combined with deficiency of hormones of the anterior pituitary gland.
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Treatment congenital non-sugar diabetes.
Treatment of moderate congenital diabetes insipidus should not be started with drugs containing antidiuretic hormone. Initial treatment should include diuretics of the chlorothiazide series (preferably hypothiazide at 25 mg 4 times a day) and a hypoglycemic drug, a sulfonylurea derivative - chlorpropamide at 100-200 mg per day.
The mechanism of action of these drugs in diabetes insipidus is currently not fully understood. It is believed that hypothiazide improves the concentration capacity of the kidneys (inhibits sodium reabsorption in the ascending limb of the loop of Henle, thereby preventing maximum dilution of urine). By slightly reducing the sodium content in the body, hypothiazide reduces the extracellular volume of fluid and increases the reabsorption of salts and water in the proximal tubules, resulting in an increase in the relative density of urine and a proportional decrease in its volume. In addition, hypothiazide has a depressing effect on the central thirst mechanisms.
Hypoglycemic drugs enhance the effect of antidiuretic hormone on the renal tubules and somewhat 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 can cause hyponatremia, thereby regulating the salt balance and improving the course of the disease. A positive effect has also been noted when taking clofibreit (miscleron) 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 to be able to release 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 reduction in the symptoms of diabetes insipidus under the influence of amitriptyline and melleril. These drugs are capable of reducing the hyperosmolarity of liquid media and causing hyponatremia. It is possible that, acting through a change in the level of catecholamines, these drugs improve the secretion of antidiuretic hormone.
In severe cases of diabetes insipidus, it is necessary to use drugs containing antidiuretic hormone: adiurecrin powder, which is inhaled through the nose at 0.03-0.05 g 3 times a day (the effect occurs in 15-20 minutes and lasts about 6-8 hours) or pituitrin in the form of subcutaneous or intramuscular injections of 1 ml (5 U) 2 times a day. Treatment with drugs containing antidiuretic hormone should be long-term. All of these drugs are ineffective in treating patients with nephrogenic diabetes insipidus. Along with pharmacotherapy, one should also remember such an auxiliary treatment method as limiting salt intake.
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