destructive diabetes. Diabetes mellitus in children

Type 1 diabetes mellitus is an organ-specific autoimmune disease that leads to the destruction of insulin-producing beta cells of the pancreatic islets, manifested by absolute insulin deficiency. In some cases, patients with overt type 1 diabetes mellitus lack markers of autoimmune damage to beta cells. (idiopathic diabetes mellitus type 1).

Etiology

Type 1 diabetes mellitus is a disease with a hereditary predisposition, but its contribution to the development of the disease is small (determines its development by about 1/3). The probability of developing type 1 diabetes in a child with a sick mother is 1-2%, father - 3-6%, brother or sister - 6%. One or more humoral markers of autoimmune beta cell damage, which include antibodies to pancreatic islets, antibodies to glutamate decarboxylase (GAD 65), and antibodies to tyrosine phosphatase (IA-2 and IA-2beta), are found in 85-90 % of patients. Nevertheless, the factors of cellular immunity are of primary importance in the destruction of beta cells. Type 1 diabetes mellitus is associated with HLA haplotypes such as DQA and DQB. With an increased frequency, type 1 diabetes mellitus is combined with other autoimmune endocrine (autoimmune thyroiditis, Addison's disease) and non-endocrine diseases, such as alopecia, vitiligo, Crohn's disease, rheumatic diseases.

Pathogenesis

Type 1 diabetes mellitus manifests when 80-90% of beta cells are destroyed by the autoimmune process. The speed and intensity of this process can vary significantly. Most often when typical flow diseases in children and young people, this process proceeds quite quickly, followed by a rapid manifestation of the disease, in which only a few weeks can pass from the appearance of the first clinical symptoms to the development of ketoacidosis (up to ketoacidotic coma).

In other, much rarer cases, usually in adults over 40 years of age, the disease may be latent. (latent autoimmune diabetes in adults - LADA), at the same time, at the onset of the disease, such patients are often diagnosed with type 2 diabetes mellitus, and for several years the compensation of diabetes mellitus can be achieved by prescribing sulfonylurea drugs. But in the future, usually after 3 years, there are signs of absolute insulin deficiency (weight loss, ketonuria, severe hyperglycemia, despite taking hypoglycemic tablets).

The pathogenesis of type 1 diabetes mellitus is based on an absolute deficiency of insulin. The impossibility of glucose entry into insulin-dependent tissues (fat and muscle) leads to energy insufficiency, as a result of which lipolysis and proteolysis are intensified, which are associated with weight loss. An increase in the level of glycemia causes hyperosmolarity, which is accompanied by osmotic diuresis and severe dehydration. Under conditions of insulin deficiency and energy deficiency, the production of contrainsular hormones (glucagon, cortisol, growth hormone) is inhibited, which, despite increasing glycemia, causes stimulation of gluconeogenesis. Increased lipolysis in adipose tissue leads to a significant increase in the concentration of free fatty acids. With insulin deficiency, the liposynthetic ability of the liver is suppressed, and free fatty acids begin to be included in ketogenesis. The accumulation of ketone bodies leads to the development of diabetic ketosis, and later - ketoacidosis. With a progressive increase in dehydration and acidosis, a coma develops, which, in the absence of insulin therapy and rehydration, inevitably ends in death.

Epidemiology

Type 1 diabetes accounts for 1.5-2% of all cases of diabetes. The lifetime risk of developing type 1 diabetes in a white race is about 0.4%. The age peak manifestation of type 1 diabetes corresponds to approximately 10-13 years. In the vast majority of cases, type 1 diabetes manifests itself before the age of 40.

Clinical manifestations

In typical cases, especially in children and young adults, type 1 diabetes mellitus makes its debut with a vivid clinical picture that develops over several months or even weeks. The manifestation of type 1 diabetes can be provoked by infectious and other concomitant diseases. Characteristic symptoms common to all types of diabetes associated with hyperglycemia: polydipsia, polyuria, pruritus, but in type 1 diabetes they are very pronounced. So, throughout the day, patients can drink and excrete up to 5-10 liters of fluid. specific for type 1 diabetes mellitus, a symptom that is caused by an absolute deficiency of insulin is weight loss, reaching 10-15 kg over 1-2 months. The expressed general and muscular weakness, decrease in working capacity, drowsiness is characteristic. At the beginning of the disease, some patients may experience an increase in appetite, which is replaced by anorexia as ketoacidosis develops. The latter is characterized by the appearance of the smell of acetone (or fruity smell) from the mouth, nausea, vomiting, often abdominal pain (pseudoperitonitis), severe dehydration and ends with the development of a coma. In some cases, the first manifestation of type 1 diabetes mellitus in children is a progressive impairment of consciousness up to coma against the background of concomitant diseases, usually infectious or acute surgical pathology.

In rare cases, the development of type 1 diabetes in people older than 35-40 years (latent autoimmune diabetes in adults) the disease may not manifest so clearly (moderate polydipsia and polyuria, no weight loss) and even be detected incidentally during routine determination of the level of glycemia. In these cases, the patient is often initially diagnosed with type 2 diabetes mellitus and prescribed tableted antidiabetic drugs, which for some time provide acceptable compensation for diabetes. However, over the course of several years (often within a year), the patient develops symptoms due to an increasing absolute deficiency of insulin: weight loss, the inability to maintain normal glycemia against the background of tableted hypoglycemic drugs, ketosis, ketoacidosis.

Diagnostics

Considering that type 1 diabetes mellitus has a vivid clinical picture and is also a relatively rare disease, screening determination of the level of glycemia for the purpose of diagnosing type 1 diabetes mellitus is not indicated. The probability of developing the disease in the closest relatives of patients is low, which, together with the lack of effective methods for the primary prevention of type 1 diabetes, determines the inappropriateness of studying immunogenetic markers of the disease in them. Diagnosis of type 1 diabetes mellitus in the vast majority of cases is based on the detection of significant hyperglycemia in patients with severe clinical manifestations of absolute insulin deficiency. Oral glucose tolerance test for the purpose of diagnosing diabetes mellitus type 1, it is necessary to carry out very rarely.

Differential Diagnosis

In doubtful cases (detection of moderate hyperglycemia in the absence of obvious clinical manifestations, manifestation at a relatively middle age), as well as for the purpose of differential diagnosis with other types of diabetes mellitus, determination of the level is used C-peptide(basal and 2 hours after a meal). Indirect diagnostic value in doubtful cases may have a definition immunological markers type 1 diabetes mellitus - antibodies to pancreatic islets, to glutamate decarboxylase (GAD65) and tyrosine phosphatase (IA-2 and IA-2P).

Treatment of any type of diabetes mellitus is based on three main principles: hypoglycemic therapy (for type 1 diabetes mellitus - insulin therapy), diet and patient education. Insulin therapy for type 1 diabetes is substitution and its goal is to maximally imitate the physiological production of the hormone in order to achieve the accepted compensation criteria. Intensive insulin therapy is closest to the physiological secretion of insulin. The need for insulin corresponding to its basal secretion, provided with two injections of intermediate-acting insulin (morning and evening) or one injection of long-acting insulin (glargine). The total dose of basal insulin should not exceed half of the total daily requirement for the drug.

Meal or bolus secretion of insulin is replaced by injections of short-acting or ultra-short-acting insulin before each meal, while its dose is calculated based on the amount of carbohydrates that are expected to be taken during the upcoming meal, and the existing level of glycemia, determined by the patient with a glucometer before each injection of insulin.

After the manifestation of type 1 diabetes mellitus and the start of insulin therapy for a sufficiently long time, the need for insulin may be small and be less than 0.3-0.4 U / kg. This period is referred to as the remission phase, or "Honeymoon". After a period of hyperglycemia and ketoacidosis, which suppress the secretion of insulin by 10-15% of the remaining beta cells, compensation for hormonal and metabolic disorders by insulin administration restores the function of these cells, which then take over providing the body with insulin at a minimum level. This period can last from a few weeks to several years, but eventually, due to autoimmune destruction of the remaining beta cells, the "honeymoon" ends.

The term "diabetes" comes from the Greek "I flow", in ancient times it was believed that the fluid that enters the body, with this disease, passes through the body without being absorbed. Diabetes insipidus is a rare chronic pathology that fully corresponds to the ancient definition. Its cause is a lack of a hormone that regulates the excretion of water by the kidneys. As a result, the excretion of urine increases many times, practically depriving a person of a normal life.

The patient constantly feels thirsty and is forced to drink liters of fluid to prevent dehydration. Unlike diabetes, diabetes insipidus does not lead to an increase in blood glucose, is not associated with the performance of the pancreas, and does not cause typical diabetic complications. These two diseases are related only by a common symptom - pronounced polyuria.

Diabetes insipidus - what is it?

Not all of the fluid that enters our kidneys becomes urine. Almost all of the primary urine after filtration is reabsorbed back into the blood through the renal tubules, a process called reabsorption. Of the 150 liters that the kidneys pass through, only 1% is excreted as concentrated secondary urine. Reabsorption is possible due to aquaporins - protein substances that make up the pores in cell membranes. One of the types of aquaporins located in the kidneys performs its functions only in the presence of vasopressin.

Vasopressin is a hormone that is synthesized in the hypothalamus (a part of the brain) and stored in the pituitary gland (a special gland located in the lower part of the brain). Its main function is to regulate water exchange. If the density of the blood increases, or there is not enough fluid in the body, the release of vasopressin increases.

If for some reason the synthesis of the hormone has decreased, or the kidney cells have ceased to perceive vasopressin, diabetes insipidus develops. Its first sign is polyuria, excess urine. The kidneys can excrete up to 20 liters of fluid per day. The patient constantly drinks water and urinates. Such a rhythm of life exhausts a person, significantly worsens his quality of life. Another name for the disease is diabetes insipidus. Patients with diabetes insipidus receive a 3rd disability group, the opportunity to be treated free of charge and receive prescribed drugs.

The disease is rare, out of 1 million people suffer from it 2-3 people. Most often, the disease begins in adulthood, from 25 to 40 years old - 6 people per 1 million population. Much less often, diabetes insipidus develops in children.

What are the forms and types of ND

Depending on the cause of polyuria, diabetes insipidus is divided into forms:

1. Central diabetes insipidus- begins with brain damage and the cessation of the release of vasopressin into the bloodstream. This form can develop after neurosurgical operations, injuries, tumors, meningitis and other inflammations of the brain. In children, the central form is more often the result of an acute or chronic infection, genetic disorders. Severe symptoms in patients appear when about 80% of the nuclei of the hypothalamus cease to function, before that, the synthesis of the hormone is taken over by intact areas.
2. Nephrogenic diabetes insipidus- develops when the receptors of the renal tubules stop responding to vasopressin. With this form of diabetes, urine is usually less than with central. Such disorders in the kidneys can be caused by stagnation of urine in them, cystic formations and tumors, and a prolonged inflammatory process. There is also a congenital form of renal diabetes insipidus, caused by malformations of the kidneys in the fetus.
3. Idiopathic diabetes insipidus- the diagnosis is more often made when vasopressin is not enough, but the cause of its shortage cannot be identified at present. It is usually a small tumor. As it grows, education is located using modern visual methods: MRI or CT. Idiopathic diabetes insipidus can also be diagnosed when the hormone level is high, but changes in the kidneys cannot be detected. It is usually due to a gene mutation. Symptoms are observed only in men. Women are carriers of the damaged gene, signs of the disease can be detected in them only by laboratory methods, there is no pronounced polyuria.
4. Gestational diabetes insipidus- possible only in pregnant women, since its cause is the hormone vasopressinase synthesized by the placenta, which destroys vasopressin. This form of the disease disappears immediately after childbirth - our article is about.

In addition to the presence of vasopressin in the blood, diabetes insipidus is classified according to other criteria:

Reasons for the development of ND

The central form of diabetes can develop in the following situations:

• injuries of the hypothalamus and pituitary gland - damage to these areas, swelling in the nearby area, squeezing by other tissues;
• tumors and metastases in the brain;
• as a result of surgical or radiotherapeutic intervention in the structures of the brain adjacent to the hypothalamus and pituitary gland. Such operations save the patient's life, but in rare cases (20% of the total incidence of diabetes insipidus) affect the production of the hormone. There are cases of self-healing diabetes, which begins immediately after surgery and disappears after a few days;
• radiation therapy, prescribed for the treatment of brain tumors;
• violation of blood circulation in the vessels of the head as a result of thrombosis, aneurysm or stroke;
• neuroinfectious diseases - encephalitis, meningitis;
• acute infections - whooping cough, influenza, chickenpox. In children, infectious diseases lead to diabetes insipidus more often than in adults. This is due to the peculiarities of the anatomy of the brain in childhood: the rapid growth of new vessels, the permeability of the existing, incompletely formed blood-brain barrier;
• pulmonary granulomatosis, tuberculosis;
• taking clonidine;
• congenital malformations - microcephaly, underdevelopment of parts of the brain;
• damage to the hypothalamus by intrauterine infection. Symptoms of diabetes in this case may appear years later, under the influence of stress, injury or hormonal changes.
• a gene defect that makes it impossible to synthesize vasopressin;
• Wolfram syndrome is a complex hereditary disorder that includes diabetes and diabetes insipidus, poor vision and hearing.

Possible causes of nephrogenic diabetes:

• development of renal failure due to chronic kidney disease, polycystosis, urolithiasis;
• violation of protein metabolism with the deposition of amyloid in the tissues of the kidneys;
• myeloma or sarcoma of the kidney;
• genetic inferiority of vasopressin receptors in the kidneys;
• toxic effects on the kidneys of certain drugs:

Symptoms of diabetes insipidus

The first sign of diabetes insipidus of any form is a sharply increased urination (from 4 liters), which does not stop even at night. The patient loses normal sleep, gradually he develops nervous exhaustion. Children begin nocturnal, and then daytime enuresis. Urine is transparent, almost without salts, its portions are large, from half a liter. Without treatment, due to this volume of urine, the renal pelvis and bladder gradually expand.

In response to the removal of fluid from the body, a strong thirst begins, patients drink liters of water. Preference is usually given to very cold drinks, as warm ones quench their thirst worse. Digestion worsens, the stomach stretches and drops, irritation occurs in the intestines.

At first, the water consumed is enough to satisfy its deficiency in the body, then dehydration gradually begins. Its symptoms are fatigue, headaches and dizziness, low blood pressure, and arrhythmias. In a patient with diabetes insipidus, the amount of saliva decreases, the skin dries, and lacrimal fluid is not released.

Symptoms in men -, in women - the absence of menstruation, in children - a delay in physical and intellectual development.

Diagnosis and examination

All patients with polyuria should be screened for diabetes insipidus. Diagnosis procedure:

1. History taking - asking the patient about the duration of the disease, the amount of urine excreted, other symptoms, cases of diabetes insipidus in close relatives, surgeries or brain injuries. Clarification of the nature of thirst: if it is absent at night or when the patient is busy with an interesting business, the cause of polyuria may not be diabetes insipidus, but psychogenic polydipsia.
2. Determining blood glucose to rule out diabetes - and how to do it right.
3. Urine analysis with calculation of its density and osmolarity. In favor of diabetes insipidus, the density is less than 1005, the osmolarity is less than 300.
4. Water deprivation test - the patient is deprived of any drink and liquid food for 8 hours. All this time he is under the supervision of doctors. If severe dehydration occurs, the test is terminated prematurely. Diabetes insipidus is considered confirmed if the patient's weight during this time has decreased by 5% or more, and the osmolarity and density of urine have not increased.
5. An analysis of the amount of vasopressin in the blood immediately after the test to determine the form of the disease. With central diabetes, its level remains low, with a nephrogenic form it increases greatly.
6. MRI for suspected central diabetes to detect neoplasms in the brain.
7. Ultrasound of the kidneys with a high probability of a nephrogenic form.
8. Genetic screening for suspected hereditary diabetes.

Treatment of diabetes insipidus

After identifying the cause of the disease, all the efforts of doctors are aimed at eliminating it: they remove neoplasms, relieve inflammation in the kidneys. If the central form is identified, and diabetes does not stop after treatment of the probable cause, replacement therapy is prescribed. It consists in the introduction into the blood of a synthetic analogue of the hormone absent in the patient - despopressin (tablets Minirin, Nourem, Nativa). The dosage is selected individually depending on the presence of the synthesis of its own vasopressin and the need for it. The dose is considered adequate if the symptoms of diabetes insipidus disappear.

When your own hormone is produced but not enough, clofibrate, carbamazepine, or chlorpropamide may be prescribed. In some patients, they can cause an increase in the synthesis of vasopressin. Only chlorpropamide is allowed for children from these drugs, but when using it, it is necessary to control blood glucose, since it has a hypoglycemic effect.

The nephrogenic form of diabetes lacks therapies with proven efficacy. Diuretics from the thiazide group can reduce fluid loss by 25-50%. In diabetes insipidus, they do not stimulate the excretion of urine, as in healthy people, but, on the contrary, increase its reabsorption.

In addition to drugs, patients are prescribed a diet with a limited amount of proteins, so as not to overload the kidneys. To prevent dehydration, you need to drink enough fluids, preferably juices or compotes, to restore washed-out vitamins and minerals.

If the treatment helped to reach the stage of compensation for diabetes insipidus, the patient can lead a normal life with the ability to work. Full recovery is possible if the cause of the disease has been eliminated. Most often, diabetes disappears if it was caused by injuries, tumors, and surgical interventions. In other cases, patients require lifelong treatment.

Many people know that intense thirst and profuse frequent urination are one of the clinical signs of diabetes. With this insidious disease, the metabolism of glucose in the body is disturbed and the syndrome of hyperglycemia develops.

A less common pathology - diabetes insipidus - has similar symptoms, but completely different causes of development and pathogenesis. Without timely treatment, it quickly leads to unwanted complications. Why diabetes insipidus is dangerous: let's try to figure it out.

Diabetes insipidus - ICD code 10 E23.2, N25.1 - a rare disease associated with impaired functioning of the hypothalamic-pituitary system and characterized by polyuria syndromes (up to 10-15 liters of urine per day) and polydipsia (unbearable thirst). Its development is based on a lack of production of vasopressin (otherwise, antidiuretic hormone) and the excretion of a large amount of low-concentration urine by the kidneys.

It is interesting. In Latin, pathology sounds like diabetes insipidus.

The prevalence of this chronic disease is low - 3 cases per 100,000 population. It occurs in men, women and children of any age, but most often in young people. In medicine, there have been cases of diagnosing diabetes insipidus in a child under one year old.

Causes and mechanism of development

Among the likely etiological factors in the development of pathology:

• tumor formations of the hypothalamus / pituitary gland;
• metastasis of tumors to the brain;
• violation of the blood supply to the brain tissue;
• primary tubulopathies, in which there is a violation of the perception of antidiuretic hormone by target cells.

Sometimes the cause remains unexplained.

Is this pathology inherited? According to recent studies, there are genetically determined, or family forms of the disease.

The pathogenesis of disorders has been sufficiently studied by endocrinologists. Diabetes insipidus occurs when there is a deficiency of the hormone vasopressin (ADH). Normally, this substance is secreted in the hypothalamus, and then accumulates in the neurohypophysis.

It is responsible for the reabsorption of fluid from primary urine in the distal tubules of the kidneys. In case of its shortage (or in case of improper interaction with receptors in the renal nephron), the reabsorption of the liquid part of the blood after primary filtration does not occur, and polyuria syndrome develops.

It is in the causes and mechanism of the development of the pathological syndrome that the main difference between diabetes mellitus and diabetes insipidus lies:

1. The first is associated with an absolute or relative lack of pancreatic hormone insulin, the second - with a violation of the production, secretion and biological action of ADH.
2. The main laboratory symptom of DM is hyperglycemia. In diabetes insipidus, the blood sugar level remains normal.

Classification

The syndrome of diabetes insipidus occurs in various disorders of both the central nervous system and the target organ (kidneys). In modern endocrinology, it is customary to separate the forms of the disease, in contrast to the level of pathological changes.

Table: Disease classification:

Clinical manifestations

How does diabetes insipidus manifest itself?

The main clinical signs of pathology are presented below:

• polydipsia - unbearable, unnatural thirst, which temporarily disappears only after taking a very large volume of liquid;
• polyuria - profuse urination over 3-4 l / day;
• discoloration of urine - it becomes transparent and almost odorless;
• dry skin;
• decrease in sweating.

The patient can drink up to 8-10 liters of fluid per day.

With insufficient fluid intake in the body of a patient with diabetes insipidus, complications such as nervous excitement, persistent hyperthermia and impaired consciousness up to coma and death occur. These symptoms indicate an extreme degree of violation of water-salt metabolism and dehydration of the body.

Note! Despite manifestations of dehydration, patients with diabetes insipidus maintain a high level of fluid excretion in the urine. Therefore, it is important to take the problem seriously and treat diabetes insipidus in a timely manner - the consequences of the disease can be fatal.

Diagnostic methods

Diagnosis of the syndrome is not particularly difficult. Examination of a patient with suspected ADH deficiency is carried out in order to:

• determining the source of damage (general laboratory tests, MRI, ultrasound of the kidneys, functional studies, genetics consultation);
• exclusion of incipient syndrome (test with dry eating).

Principles of therapy

Can diabetes insipidus be cured? Modern medicine and pharmacology allow you to cope with almost any variant of the development of the syndrome. However, before treating the pathology, it is important to understand the causes that caused it.

With neurogenic diabetes insipidus, lifelong hormone replacement therapy is indicated. DDAVP is a widely used drug for the treatment of pathology, the main active ingredient of which is Desmopressin, a synthetic analogue of ADH.

The nephrogenic form of the disease requires the appointment of thiazide diuretics and representatives of the NSAID group.

With transient diabetes insipidus complicating the course of pregnancy, specific therapy is usually not required. If its course is accompanied by the development of dehydration, the question of the use of agents based on desmopressin is decided.

How to treat incipidary syndrome? Most patients with this pathology benefit from individual or group sessions with a psychotherapist/psychologist.

The prognosis of the disease depends on its form. Most patients can count on successful compensation of the condition while taking pills. Unfortunately, it is not yet possible to talk about a complete recovery from this disease.

A disease such as diabetes, accompanied by an increase in the concentration of glucose in the blood, is common and familiar to many. A less common hormonal disorder treated by endocrinologists is diabetes insipidus.

In our review, we tried to consider what diabetes insipidus is, why this disease develops, what symptoms it has, how it is diagnosed and treated. Timely seeking medical help and complex therapy can avoid the development of serious complications and negative health consequences.

Questions to the doctor

Differential Diagnosis

Hello! Male, 27 years old, height 180 cm, weight 98 kg. A year and a half ago, I started having problems with urination: I began to go to the toilet often (up to 10 times) at night. After a couple of months things got worse and I started running small and during the day. Approximately 25-35 urinations with a volume of 200-400 ml came out per day. Accordingly, I drank a lot - up to 4-8 l / day.

Now it has calmed down a bit, and daily diuresis averages 3-5 liters, and the amount of fluid drunk is 3.5-6 liters.

I noticed a strange pattern: frequent urination disappears with extreme heat (especially when I sunbathe) and with colds. I turned to the therapist, she says, it looks like diabetes insipidus, she wrote out a bunch of tests. What examination to pass in the first place?

Hello! We need to differentiate diabetes insipidus from psychogenic polydipsia. In addition to standard tests, I recommend that you definitely pass a dry diet test, a concentration test and an MRI GM with an emphasis on the pituitary gland.

Treatment failure

I am 41. I have been suffering from diabetes insipidus since the age of 23. Now I accept Minirin. But on the background of treatment, my condition deteriorated sharply. According to the latest measurements, I drink 18 liters per day. And there was also a wild hunger, I really want to eat already 20-30 minutes after a hearty lunch. Than it can be caused?

Hello! 18 liters of water per day indicate that Minirin is not helping you. Please consult with your physician immediately. Perhaps the dose of the drug is low for you.

A similar disease occurs in animals, most often dogs and humans, and at any age. Naturally, such a serious failure in the functioning of the kidneys negatively affects the functionality of the whole organism. How does the disease manifest itself and how is it cured?

What it is?

Diabetes insipidus is a rare disease (approximately 3 per 100,000) associated with dysfunction of the hypothalamus or pituitary gland, which is characterized by polyuria (6-15 liters of urine per day) and polydipsia (thirst).

It occurs in both sexes in both adults and children. Most often, young people get sick - from 18 to 25 years. Cases of illness in children of the first year of life are known (A. D. Arbuzov, 1959, Sharapov V. S., 1992).

Reasons for the development of diabetes insipidus

Diabetes insipidus is a pathology that is caused by a lack of vasopressin, its absolute or relative deficiency. Vasopressin (antidiuretic hormone) is secreted in the hypothalamus and, among other functions, is responsible for the normalization of the process of urination. According to the causes of origin, it is customary to distinguish three types of this disease: genetic, acquired, idiopathic.

In most patients with this rare disease, the cause is still unknown. Such diabetes is called idiopathic, it affects up to 70 percent of patients. Genetic is a hereditary factor. In this case, diabetes insipidus sometimes occurs in several family members and in several generations in a row.

Medicine explains this by serious changes in the genotype that contribute to the occurrence of disorders in the work of the antidiuretic hormone. The hereditary location of this disease is explained by a congenital defect in the structure of the diencephalon and midbrain.

Considering the causes of diabetes insipidus, the mechanisms of its development should be taken into account:

1) Central diabetes insipidus - occurs when there is insufficient secretion of vasopressin in the hypothalamus or in violation of its release into the blood from the pituitary gland, presumably its causes are:

• The pathology of the hypothalamus, since it is responsible for the regulation of urine output and the synthesis of antidiuretic hormone, a violation of its work leads to this disease. The causes and provoking factors for the occurrence of dysfunctions of the hypothalamus can be acute or chronic infectious diseases: tonsillitis, influenza, venereal diseases, tuberculosis.
• Surgical interventions on the brain and inflammatory pathologies of the brain.
• Concussion, traumatic brain injury.
• Autoimmune diseases.
• Cystic, degenerative, inflammatory lesions of the kidneys that impair the perception of vasopressin.
• Tumor processes of the hypothalamus and pituitary gland.
• Also, the presence of hypertension is one of the aggravating factors during diabetes insipidus.
• Vascular lesions of the hypothalamic-pituitary system, leading to problems of cerebral circulation in the vessels that feed the hypothalamus and pituitary gland.

2) Renal diabetes insipidus - in this case, vasopressin is produced in a normal amount, but the kidney tissue does not respond to it properly. The reasons may be as follows:

• damage to the urinary tubules of the nephron or medulla of the kidney;
• hereditary factor - congenital pathology;
• sickle cell anemia;
• an increase in potassium or a drop in the level of calcium in the blood;
• chronic renal failure;
• amyloidosis (deposition of amyloid in tissues) or polycystic (formation of multiple cysts) of the kidneys;
• taking medications that can have a toxic effect on the kidney tissue (Demeklotsilin, Amphotericin B, Lithium);
• sometimes pathology occurs in old age or against the background of weakening by another pathology.

Sometimes, against the background of stress, increased thirst (psychogenic polydipsia) can occur. Or diabetes insipidus during pregnancy, which develops in the 3rd trimester due to the destruction of vasopressin by enzymes produced by the placenta. Both types of violations are independently eliminated after the elimination of the root cause.

Classification

It is customary to distinguish 2 clinical forms of this disease:

1. Nephrogenic diabetes insipidus (peripheral). This form of the disease is a consequence of a decrease or complete lack of sensitivity of the distal renal tubules to the biological effects of vasopressin. As a rule, this is observed in the case of chronic kidney pathology (with pyelonephritis or against the background of polycystic kidney disease), a long-term decrease in potassium content in the blood and an increase in calcium levels, with insufficient intake of protein from food - protein starvation, Sjögren's syndrome, some birth defects. In some cases, the disease is familial.
2. Neurogenic diabetes insipidus (central). It develops as a result of pathological changes in the nervous system, in particular, in the hypothalamus or the posterior pituitary gland. As a rule, the cause of the disease in this case is surgery for the complete or partial removal of the pituitary gland, infiltrative pathology of this area (hemochromatosis, sarcoidosis), trauma or changes in the inflammatory nature. In some cases, neurogenic diabetes insipidus is idiopathic, being determined simultaneously in several members of the same family.

Symptoms of diabetes insipidus

The very first signs of diabetes insipidus are severe, agonizing thirst (polydipsia) and frequent profuse urination (polyuria), which disturb patients even at night. From 3 to 15 liters of urine can be excreted per day, and sometimes its amount reaches up to 20 liters per day. Therefore, the patient is tormented by intense thirst.

• Symptoms of diabetes insipidus in men are a decrease in sexual desire and potency.
• Symptoms of diabetes insipidus in women: menstrual irregularities up to amenorrhea, associated infertility, and if pregnancy does occur, an increased risk of spontaneous abortion.
• Symptoms of diabetes in children are pronounced. In newborns and young children, the condition with this disease is usually severe. An increase in body temperature is noted, inexplicable vomiting occurs, and disorders of the nervous system develop. In older children up to adolescence, a symptom of diabetes insipidus is bedwetting, or enuresis.

In the future, as it progresses, the following symptoms join:

• Due to the consumption of a large amount of liquid, the stomach is stretched, and sometimes even lowered;
• There are signs of dehydration (lack of water in the body): dry skin and mucous membranes (dry mouth), weight loss;
• Due to the release of urine in large volumes, the bladder is stretched;
• Due to the lack of water in the body, the production of digestive enzymes in the stomach and intestines is disrupted. Therefore, the patient's appetite decreases, gastritis or colitis develops, there is a tendency to constipation;
• Blood pressure often goes down and heart rate quickens;
• Since there is not enough water in the body, sweating decreases;
• The patient gets tired quickly;
• Sometimes there is unexplained nausea and vomiting;
• Body temperature may rise.
• Sometimes there is bedwetting (enuresis).

Since thirst and copious urination continue at night, the patient develops mental and emotional disorders:

• emotional lability (sometimes even psychoses develop) and irritability;
• insomnia and headaches;
• decrease in mental activity.

These are the signs of diabetes insipidus in typical cases. However, the manifestations of the disease may differ slightly in men and women, as well as children.

Diagnostics

In typical cases, the diagnosis of diabetes insipidus is not difficult and is based on:

• pronounced thirst
• the volume of daily urine is more than 3 liters per day
• plasma hyperosmolality (greater than 290 mosm/kg, dependent on fluid intake)
• high sodium content
• urine hypoosmolality (100–200 mosm/kg)
• low relative density of urine (<1010)

To determine the cause of the disease, the results of X-ray, ophthalmological and neuropsychiatric examinations are carefully analyzed. It is necessary to carry out magnetic resonance imaging of the brain.

How to treat diabetes insipidus?

The successful outcome of therapy in this pathology is to accurately determine and eliminate the main cause that causes failures in the production of vasopressin, for example, a tumor or brain metastases in the central form of diabetes insipidus.

Drugs for diabetes insipidus are selected by the attending physician, all of them are a synthetic analogue of antidiuretic hormone. Depending on the duration of action of the drug, the drug should be taken several times a day or 1 time in several days (prolonged-release drugs).

In diabetes insipidus type of the central form, the most commonly used drugs are Carbamazepine or Chlorpropamide - these drugs stimulate the production and release of vasopressin. In order to prevent dehydration of the body against the background of abundant and frequent urination, saline solutions are administered intravenously to the patient.

Treatment of diabetes insipidus in women consists in an additional consultation with a gynecologist and correction of the menstrual cycle.

Nutrition rules

Everyone knows that patients with diabetes have a “special” relationship with sugars. But what can be said about nutrition if the disease is not sugar? In this case, the restriction will affect another product - salt. If the patient does not suffer from renal insufficiency, then it is possible to replace the salt with a dietary supplement, for example, Sanasol.

The diet for this disease involves limiting the consumption of protein foods (no more than 70 g per day). The patient is recommended dietary table number 7.

The diet includes the following foods and drinks:

1. Berries and fruits with a sweet and sour taste.
2. Fresh vegetables.
3. Freshly squeezed juices, kvass, teas - herbal and green.
4. Water with lemon juice.
5. Dairy products and drinks.
6. Lean types of meat.
7. Low-fat varieties of fish, seafood.

Forecast

Idiopathic diabetes insipidus, subject to adequate replacement therapy, does not pose a danger to the life of the patient, however, recovery from this form is also impossible.

Diabetes insipidus, which arose against the background of any other diseases, in some cases disappears spontaneously after the elimination of the cause that caused it.