Diabetes. Diabetes insipidus: what is this disease and why does it appear? Idiopathic diabetes mellitus type 1

Diabetes insipidus is a fairly rare disease associated with impaired absorption of fluid by the kidneys. This disease is also called diabetes, since its development leads to the fact that urine ceases to concentrate and diluted, in large quantities, leaves the body.

A similar disease occurs in animals, most often dogs and people, and at any age. Naturally, such a serious malfunction of the kidneys negatively affects the functionality of the entire body. 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 (excretion of 6-15 liters of urine per day) and polydipsia (thirst).

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

Causes of diabetes insipidus

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

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

Medicine explains this by serious changes in the genotype, which contribute to the occurrence of disturbances in the functioning of the antidiuretic hormone. The hereditary nature of this disease is explained by a congenital defect in the structure of the diencephalon and midbrain.

When 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 when its release into the blood from the pituitary gland is impaired, presumably its causes are:

• Pathology of the hypothalamus, since it is responsible for the regulation of urine excretion and the synthesis of antidiuretic hormone, disruption of its functioning leads to this disease. The causes and provoking factors of dysfunction of the hypothalamus can be acute or chronic infectious diseases: sore throat, influenza, sexually transmitted diseases, tuberculosis.
• Surgical interventions on the brain and inflammatory pathologies of the brain.
• Concussion, traumatic brain injury.
• Autoimmune diseases.
• Cystic, degenerative, inflammatory kidney lesions that impair vasopressin perception.
• 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 with cerebral circulation in the vessels that supply the hypothalamus and pituitary gland.

2) Renal diabetes insipidus - in this case, vasopressin is produced in normal quantities, 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 the renal medulla;
• hereditary factor - congenital pathology;
• sickle cell anemia;
• increased potassium or decreased calcium levels in the blood;
• chronic renal failure;
• amyloidosis (deposition of amyloid in tissues) or polycystic disease (formation of multiple cysts) of the kidneys;
• taking medications that can have a toxic effect on kidney tissue (Demeclocilin, Amphotericin B, Lithium);
• sometimes the pathology occurs in old age or against the background of weakening by another pathology.

Sometimes, due to stress, increased thirst may occur (psychogenic polydipsia). 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 eliminated on their own after the root cause is eliminated.

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 decreased or complete absence 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 the potassium content in the blood and an increase in calcium levels, with insufficient protein intake from food - protein starvation, Sjögren's syndrome, and some birth defects. In some cases, the disease is familial.
2. Neurogenic diabetes insipidus (central). Develops as a result of pathological changes in the nervous system, in particular in the hypothalamus or posterior pituitary gland. As a rule, the cause of the disease in this case is surgery to completely or partially remove the pituitary gland, infiltrative pathology of this area (hemochromatosis, sarcoidosis), trauma or changes of an inflammatory nature. In some cases, neurogenic diabetes insipidus is idiopathic, occurring simultaneously in several members of the same family.

Symptoms of diabetes insipidus

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

• Symptoms of diabetes insipidus in men are decreased libido 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.
• The symptoms of diabetes in children are pronounced. In newborns and young children, the condition of this disease is usually severe. There is an increase in body temperature, unexplained vomiting occurs, and disorders of the nervous system develop. In older children up to adolescence, a symptom of diabetes insipidus is bedwetting, or enuresis.

Later, as it progresses, the following symptoms appear:

• Due to the consumption of large amounts of liquid, the stomach stretches and sometimes even drops;
• Signs of dehydration (lack of water in the body) appear: dry skin and mucous membranes (dry mouth), decreased body weight;
• Due to the release of urine in large volumes, the bladder is stretched;
• Due to 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, and there is a tendency to constipation;
• Blood pressure often decreases and heart rate increases;
• 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 increase.
• Sometimes bedwetting (enuresis) occurs.

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

• emotional lability (sometimes even psychosis develops) and irritability;
• insomnia and headaches;
• decreased 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
• daily urine volume is more than 3 liters per day
• plasma hyperosmolality (more than 290 mOsm/kg, depends on fluid intake)
• high sodium content
• hypoosmolality of urine (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. Magnetic resonance imaging of the brain is necessary.

How to treat diabetes insipidus?

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

Medicines 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 medicine should be taken several times a day or once every few days (long-acting drugs).

For diabetes insipidus of the central form, the drugs Carbamazepine or Chlorpropamide are most often used - these drugs stimulate the production and release of vasopressin. In order to prevent dehydration of the body against the background of copious and frequent urination, the patient is given intravenous drips of saline solutions.

Treatment of diabetes insipidus in women consists of 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 sugar insipidus? In this case, the restriction will affect another product – salt. If the patient does not suffer from renal failure, then it is possible to replace the salt with a dietary supplement, for example, Sanasol.

A diet for this disease involves limiting the consumption of protein foods (no more than 70 g per day). The patient is recommended dietary table No. 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. Fermented milk products and drinks.
6. Lean types of meat.
7. Low-fat fish and seafood.

Forecast

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

Diabetes insipidus, which occurs against the background of some other disease, in some cases goes away spontaneously after the cause that caused it is eliminated.

With this form of diabetes, the causes of the development of the pathological condition are not known, the origin is unclear, and there is no pronounced dependence on other lesions. Idiopathic diabetes is classified as type 1 diabetes.

The developing destruction of pancreatic cells responsible for the production of insulin leads to insufficient amounts. At idiopathic In diabetes, insulin synthesis is disrupted without any organic changes in the gland itself.

Such people periodically need replacement therapy. Symptoms when contacting an endocrinologist often include increased thirst, fatigue, and dry mouth. This condition runs in families. It is found predominantly in Asians and Africans.

Signs acute pathological conditions are:

• hyperglycemia (14-18mmol/l);
• ketosis;
• increasing BMI;
• absence of autoimmune cell damage.

Most often, with competently carried out substitution therapy within six months a stable remission occurs, which does not require glucose-lowering drugs for one and a half to two years.

Over time, carbohydrate metabolism may be disrupted again, and signs of type 2 diabetes appear, characteristic of the older age group, when cells lose the ability to properly use insulin.

According to research average age people with idiopathic diabetes are no more than 30 years old, and the connection with HLA is not detected.

This condition requires regular monitoring by a good specialist and laboratory monitoring.

Diabetes insipidus

This type of diabetes develops due to a deficiency in the body vasopressin(a hormone produced in the hypothalamus), responsible for the correct regulation and ratio of all biological fluids inside a person: blood, intercellular fluid, lymph, water.

Brain surgeries, tumors, and unknown etiological factors can cause pathological processes in the pituitary gland, which lead to increased thirst and polyuria (increased amount of urine).

In every fifth case diabetes insipidus The cause is a failed neurosurgery. In a third of cases, the idiopathic type of diabetes develops.

In the acute course of the disease, if destructive changes in the pituitary gland are not detected, diabetes insipidus in the idiopathic form can be assumed.

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 an absolute deficiency of insulin. 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 (it determines its development by approximately 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 anti-pancreatic islet antibodies, anti-glutamate decarboxylase antibodies (GAD 65) and anti-tyrosine phosphatase antibodies (IA-2 and IA-2beta), are found in 85-90 % of patients. Nevertheless, the main importance in the destruction of beta cells is given to cellular immunity factors. Type 1 diabetes mellitus is associated with HLA haplotypes DQA and DQB. With 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 manifests itself when 80-90% of beta cells are destroyed by an autoimmune process. The speed and intensity of this process can vary significantly. Most often when typical course 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 of adults - LADA), Moreover, at the onset of the disease, such patients are often diagnosed with type 2 diabetes mellitus, and for several years, compensation for diabetes mellitus can be achieved by prescribing sulfonylurea drugs. But later, usually after 3 years, signs of absolute insulin deficiency appear (weight loss, ketonuria, severe hyperglycemia, despite taking tableted hypoglycemic drugs).

The pathogenesis of type 1 diabetes mellitus is based on absolute insulin deficiency. The inability of glucose to enter insulin-dependent tissues (fat and muscle) leads to energy deficiency, resulting in intensified lipolysis and proteolysis, which are associated with weight loss. An increase in glycemic levels 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 disinhibited, 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 capacity 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 subsequently 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 mellitus accounts for 1.5-2% of all diabetes cases. The lifetime risk of developing type 1 diabetes in a Caucasian person is about 0.4%. The peak age for the manifestation of type 1 diabetes mellitus is 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 people, type 1 diabetes mellitus debuts with a clear clinical picture that develops over several months or even weeks. The manifestation of type 1 diabetes mellitus can be triggered by infectious and other concomitant diseases. Characteristic symptoms common to all types of diabetes, associated with hyperglycemia: polydipsia, polyuria, itching, but in type 1 diabetes mellitus 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, the symptom, which is caused by an absolute deficiency of insulin, is weight loss reaching 10-15 kg over 1-2 months. Characterized by severe general and muscle weakness, decreased performance, and drowsiness. At the beginning of the disease, some patients may experience an increase in appetite, which gives way to anorexia as ketoacidosis develops. The latter is characterized by the appearance of an acetone odor (or fruity odor) from the mouth, nausea, vomiting, often abdominal pain (pseudoperitonitis), severe dehydration and ends in 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 due to concomitant diseases, usually infectious or acute surgical pathology.

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

Diagnostics

Considering that type 1 diabetes mellitus has a clear clinical picture and is also a relatively rare disease, screening determination of glycemic levels to diagnose type 1 diabetes mellitus is not indicated. The likelihood of developing the disease in close relatives of patients is low, which, together with the lack of effective methods for 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 identification of significant hyperglycemia in patients with severe clinical manifestations of absolute insulin deficiency. Oral glucose tolerance test for the purpose of diagnosing type 1 diabetes mellitus 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 advanced age), as well as for the purpose of differential diagnosis with other types of diabetes mellitus, level determination is used C-peptide(basal and 2 hours after meals). In indirect diagnostic value in doubtful cases, the definition may have immunological markers type 1 diabetes mellitus - antibodies to pancreatic islets, glutamate decarboxylase (GAD65) and tyrosine phosphatase (IA-2 and IA-2P).

Treatment 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 mellitus is substitutive nature and its goal is to mimic physiological hormone production as much as possible in order to achieve accepted compensation criteria. Intensive insulin therapy is the closest to physiological insulin secretion. The need for insulin corresponding to its basal secretion is provided by 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.

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

After the manifestation of type 1 diabetes mellitus and the initiation of insulin therapy for quite a 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 insulin secretion by the 10-15% remaining beta cells, compensation for hormonal-metabolic disorders by administering insulin restores the function of these cells, which then take over providing the body with insulin at a minimum level. This period can last from several weeks to several years, but eventually, due to autoimmune destruction of the remaining beta cells, the honeymoon ends.

A serious illness, type 1 diabetes, leaves the patient no choice: in order to survive, he must accept his illness and learn to live with it. The Roman physician Aretaeus, who lived in the 1st century AD, first made a clinical description of type 1 diabetes. By his definition, a person with diabetes “goes on water and sugar” and lives a short and painful life. Nowadays, a person diagnosed with stage 1 diabetes has the opportunity to live a long and fulfilling life. Medicine is developing, and perhaps in the very near future it will be possible to defeat this still incurable disease.

A severe hormonal disease associated with a complete deficiency of insulin in the body is provoked by disorders in the immune system. Specific cells of the pancreas stop producing insulin. At risk are children with a certain genetic status, in whom any viral infection can cause an autoimmune reaction.

According to the latest data, type 1 diabetes is possible if:

• The baby was exposed to infection in the womb;
• The child is growing quickly;
• The baby was weaned early;
• A 3-5 year old child had little contact with other children and adults.

Stage 1 diabetes mellitus often manifests itself in cases of severe emotional stress.

Classification

Currently, there are two types of insulin-dependent diabetes:

• Immune dependent;
• Idiopathic.

Immune-dependent diabetes is diagnosed in 98% of patients diagnosed with insulin-dependent diabetes. Insulin deficiency in this case is associated with the destruction of specific pancreatic cells due to the body's autoimmune reaction. In this case, autoantibodies to insulin are detected in the patient’s blood. Antibodies disappear after complete destruction of target cells.

In idiopathic diabetes, autoantibodies are not observed, and the cause of abnormal pancreatic function is still unknown. In people with this form of diabetes, which is usually seen in people of Asian and African descent, pancreatic function may periodically recover.

Symptoms

Type 1 diabetes mellitus is characterized by the following clinical manifestations:

• Constant strong thirst;
• Constant need to urinate;
• Nocturnal enuresis (in children);
• Constant feeling of hunger;
• Sudden weight loss (up to 15 kg in a few months);
• Fatigue.

You should be wary of such phenomena as:

• Dry skin;
• Red spots above the eyebrows, on the chin;
• Non-healing wounds;
• Fungal skin diseases;
• Brittle nails.

Already in ancient times, doctors noticed that the urine of some patients attracted insects. They called it "sweet piss." This symptom is typical for diabetic patients.

If you miss the first symptoms indicating insulin-dependent diabetes mellitus, the patient may develop ketoacidosis and a state of diabetic coma in a short period of time. The rate of destruction of pancreatic cells varies from person to person. In some patients, delayed insulin secretion may persist for several years. Severe emotional shock, infections, operations and injuries can provoke a sharp deterioration in the patient’s condition in the initial stage of the disease.

In children and adolescents, the process of destruction is always very rapid. In approximately half of the cases, type 1 diabetes is detected in children already with severe ketoacidosis. In young children (under 4 years old), the disease can immediately become complicated by a coma.

Diagnosis

A general practitioner, pediatrician, therapist, or endocrinologist can identify insulin-dependent diabetes mellitus. The basis for suspicion of the disease is usually the results of tests for hyperglycemia:

• Blood sugar level (before and after meals);
• Sugar level in urine;
• The value of glycated hemoglobin.

To determine the type of diabetes, additional studies are carried out:

• Glucose intolerance test. Allows you to identify the stage of prediabetes, at which the cells of the pancreas begin to destroy and insulin production sharply decreases.
• An immunological test for the presence of antibodies associated with damage to pancreatic cells.

Immune-dependent diabetes mellitus type 1 has a number of features that make it possible to diagnose it even before the manifestation of classical clinical symptoms. To do this, a study of genetic markers of the disease is carried out. Scientists have identified a certain group of antigens that increase the risk of type 1 diabetes.

Complications

This disease is dangerous due to acute complications:

• Hypoglycemic coma caused by a sharp drop in blood sugar levels;
• Ketoacidotic coma caused by a sharp increase in blood sugar.

The development of glycemic coma is promoted by:

• Excessive dose of insulin;
• Drinking alcohol;
• Malnutrition.

Ketoacidotic coma can be caused by an insufficient dose of insulin or refusal of insulin. An increased need for the hormone can be observed in infectious diseases.

A comatose state requires immediate medical intervention and sending the patient to a hospital.

If the treatment is chosen incorrectly, type 1 diabetes will provoke the same complications that arise with indolent type 2 diabetes:

• Blindness;
• Limb amputations
• Stroke, heart attack;
• Kidney disease.

Diabetes type 1 and 2 - how they differ

Diabetes in general is characterized by high, constant levels of sugar in the blood. It is necessary to distinguish between the two types of diabetes in order to properly organize treatment.

The term “diabetes” comes from the Greek “I am leaking”; in ancient times it was believed that the liquid that enters the body during 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, urine output increases many times over, 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 mellitus, 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 have only one common symptom in common - pronounced polyuria.

Diabetes insipidus - what is it?

Not all the liquid that enters our kidneys becomes urine. Almost the entire volume of primary urine, after filtration, is absorbed back into the blood through the renal tubules, a process called reabsorption. Of the 150 liters that the kidneys pass through themselves, only 1% is excreted in the form of concentrated secondary urine. Reabsorption is possible thanks to aquaporins - protein substances that make up the pores in cell membranes. One type of aquaporin, 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 at the bottom of the brain). Its main function is to regulate water metabolism. If blood density increases or there is not enough fluid in the body, the release of vasopressin increases.

If for some reason the synthesis of the hormone decreases, or the kidney cells no longer 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. This rhythm of life exhausts a person and significantly worsens his quality of life. Another name for the disease is diabetes insipidus. Those with diabetes insipidus receive disability group 3, the opportunity to receive free treatment and prescribed medications.

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

What are the different forms and types of ND?

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

1. Central diabetes insipidus- begins when the brain is damaged and the release of vasopressin into the bloodstream stops. This form can develop after neurosurgeries, injuries, tumors, meningitis and other inflammations of the brain. In children, the central form is often the result of acute or chronic infection or genetic disorders. Severe symptoms in patients appear when about 80% of the hypothalamic nuclei cease to function; before this, hormone synthesis is taken over by undamaged areas.
2. Nephrogenic diabetes insipidus- develops when the receptors in the kidney tubules stop responding to vasopressin. With this form of diabetes, urine is usually produced less than with central diabetes. Such disorders in the kidneys can be caused by stagnation of urine, cystic formations and tumors, and a long-term 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 often made when there is insufficient vasopressin, but the cause of its deficiency cannot be identified at this time. This is usually a small tumor. As it grows, the formation is located using modern visual methods: MRI or CT. Idiopathic diabetes insipidus can also be diagnosed when the level of the hormone is high, but changes in the kidneys cannot be detected. It is usually explained by a gene mutation. Symptoms are observed only in men. Women are carriers of a damaged gene; signs of the disease in them can only be detected by laboratory methods; there is no pronounced polyuria.
4. Gestational diabetes insipidus- is 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 about.

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

Classification criteria	Types of diabetes	Characteristic
Start time	congenital	Rarely observed, usually nephrogenic.
	acquired	Occurred during life due to other diseases or injuries.
Severity of diabetes at diagnosis	easy	Polyuria up to 8 liters per day.
	average	8-14 l
	heavy	> 14 l
The patient's condition after the start of treatment	compensation	There is no polyuria.
	subcompensation	Urine output and thirst increase several times a day.
	decompensation	Persistence of polyuria after administration of therapy.

Reasons for the development of ND

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

• injuries to the hypothalamus and pituitary gland - damage to these areas, swelling in the nearby area, compression by other tissues;
• tumors and metastases in the brain;
• as a result of surgical or radiotherapeutic intervention in brain structures close 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) they affect the production of the hormone. There are cases of self-healing diabetes, which begins immediately after surgery and goes away within a few days;
• radiation therapy given to treat brain tumors;
• circulatory disorders 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 existing ones, and an incompletely formed blood-brain barrier;
• pulmonary granulomatosis, tuberculosis;
• taking clonidine;
• congenital defects - microcephaly, underdevelopment of parts of the brain;
• damage to the hypothalamus by intrauterine infection. Symptoms of diabetes in this case may appear years later, due to stress, injury or hormonal changes.
• a gene defect that makes vasopressin synthesis impossible;
• Wolfram syndrome is a complex hereditary disorder that includes diabetes mellitus and diabetes insipidus, poor vision and hearing.

Possible causes of nephrogenic diabetes:

• development of renal failure due to chronic kidney disease, polycystic disease, urolithiasis;
• disturbance of protein metabolism with deposition of amyloid in the kidney tissues;
• myeloma or sarcoma of the kidney;
• genetic inferiority of receptors for vasopressin 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 at night. The patient is deprived of normal sleep, and gradually develops nervous exhaustion. In children, nocturnal and then daytime enuresis begins. The urine is clear, almost without salts, its portions are large, from half a liter. Without treatment, due to such a volume of urine, the renal pelvis and bladder gradually expand.

In response to the removal of fluid from the body, severe thirst begins; patients drink liters of water. Preference is usually given to very cold drinks, since warm ones quench thirst worse. Digestion worsens, the stomach stretches and sinks, and 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, arrhythmias. In a patient with diabetes insipidus, the amount of saliva decreases, the skin dries out, and tear fluid does not secrete.

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

Diagnostics and examination

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

1. Taking an anamnesis - asking the patient about the duration of the disease, the amount of urine excreted, other symptoms, cases of diabetes insipidus in close relatives, previous operations or brain injuries. Determining the nature of thirst: if it is absent at night or when the patient is busy with an interesting activity, the cause of polyuria may not be diabetes insipidus, but psychogenic polydipsia.
2. Determining blood glucose to exclude diabetes mellitus - and how to do it correctly.
3. Urine analysis with calculation of its density and osmolarity. Density less than 1005 and osmolarity less than 300 speak in favor of diabetes insipidus.
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 dangerous dehydration occurs, the test is stopped early. Diabetes insipidus is considered confirmed if the patient's weight has decreased by 5% or more during this time, and urine osmolarity and density have not increased.
5. Analysis of the amount of vasopressin in the blood immediately after the test to determine the form of the disease. In central diabetes, its level remains low, in the nephrogenic form it increases greatly.
6. MRI if central diabetes is suspected to detect a tumor 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 efforts of doctors are aimed at eliminating it: removing tumors, relieving 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 of introducing into the blood a synthetic analogue of a hormone missing in the patient - despopressin (tablets Minirin, Nourem, Nativa). The dosage is selected individually depending on the presence of synthesis of 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 increased vasopressin synthesis. For children, only chlorpropamide is allowed from these drugs, but when using it, it is necessary to control blood glucose, as it has a hypoglycemic effect.

There are no proven effective treatments for nephrogenic diabetes. Diuretics from the thiazides group can reduce fluid loss by 25-50%. In diabetes insipidus, they do not stimulate urine excretion, as in healthy people, but, on the contrary, enhance its reabsorption.

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

If treatment has helped to reach the stage of compensation for diabetes insipidus, the patient can lead a normal life while maintaining 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 injury, tumors and surgery. In other cases, patients require lifelong treatment.