Acetonemic syndrome is a complex of symptoms that occurs due to metabolic disorders in the body. As a result, the accumulation of ketone bodies occurs. This pathological condition, which is accompanied by an increase in acetone and acetoacetic acid in the blood.
The disease occurs mainly in childhood. It manifests itself in stereotypical and regularly repeated episodes, which alternate with periods of complete well-being.
The primary form occurs in 4-6% of children aged from one to 13 years. Girls are more susceptible to it. Middle age manifestation of vomiting is 5.2. Half of all patients require symptom relief by administering intravenous fluids.
The secondary form develops when there is concomitant diseases and after operations. It requires a clear provoking factor.
According to ICD-10, the syndrome is not identified as a separate nosological unit. But in pediatrics, doctors often encounter various disorders metabolic processes which are accompanied by the described pathological condition.
According to the classification, it is classified as acetonuria (code R82.4). With this disease, an increased content of acetone in the urine is detected.
The main reason is the absolute or relative lack of carbohydrates in the child’s diet or the predominance of fatty and ketogenic acids.
The prerequisite for acetonemic syndrome is that they must take an active part in oxidative processes.
When there is a lack of carbohydrates in the body, energy needs begin to be compensated by lipolysis. This leads to the formation large quantity fatty acids.
A large number of ketone bodies leads to imbalances in the acidic and water-electrolyte spheres. This has a toxic effect on nervous system, Gastrointestinal tract. Provoking factors may include:
Sometimes the prerequisite is starvation or overeating. When exposed to several unfavorable factors, ketosis is formed.
With a significant increase in the level of keto acids, metabolic acidosis. An excess of ketone bodies has such a strong effect on the central nervous system that there is a risk of developing a coma.
The manifestation of a classic attack can last from a day to a week. Always accompanied by seizures. Its frequency and duration depends on baseline health and diet.
Sometimes there are single episodes of vomiting, but more often it is of a recurring nature. Trying to drink also triggers it plain water. Because of this, signs of intoxication occur and form.
The child turns pale, but a bright, unhealthy blush may appear on the cheeks. The child's activity gradually decreases due to muscle weakness. It becomes difficult for the baby to raise his arms and get out of bed.
The attack is characterized by stages of neurological and clinical manifestations. With small doses of acetone, excitement occurs. The baby begins to scream, cry, and show extreme anxiety.
As toxic products accumulate, excitement gives way to drowsiness and impotence. With a sharp progression of the disease, seizures and loss of consciousness may occur.
Video about acetonemic syndrome in children from Dr. Komarovsky’s school:
Usually parents call ambulance because of incessant vomiting. In a hospital setting, urine and blood tests are taken. It is revealed that the amount of acetone in biological fluids is very high.
In the future, test strips can be used at home to determine the level of acetone in the urine to adjust therapeutic and preventive measures.
How brighter color strips after immersion in urine, the higher the level of ketone bodies. This technique is not absolutely accurate, so it allows only an approximate assessment of the severity.
In hospitals, the amount of acetone is measured in units or mol/l. When transcribed into a form, there are advantages. With one or two, treatment is carried out at home. If there are 3-4 pluses, then treatment in the hospital is prescribed, since a life-threatening condition arises.
Treatment is carried out in 3 stages:
This is a common one used for acetonemic syndrome. It is a dopamine receptor blocker and acts as an antiemetic drug. Available in ampoules for injection.
During a crisis you need to drink sweet tea, eat watermelons or melon. Possible use mineral water. The latter cannot be used if frequent rises of acetone are observed.
At the stage of precursors (, lethargy, headache, smell of acetone from the mouth) the child should not starve. When vomiting occurs, it will not be possible to feed the child.
Preference should be given to products that contain easily digestible carbohydrates. It could be bananas vegetable purees, kefir, liquid semolina porridge. In minimal quantities you can eat buckwheat, oatmeal, corn porridge, baked apples sweet varieties, biscuits.
When improving general condition vegetable soup is introduced. You will have to completely eliminate marinades and smoked foods. All foods should be steamed or boiled. The baby should be fed every 2-3 hours.
The main principle of nutrition is the exclusion from the diet of foods that contain purine compounds and fats in large quantities. During periods of remission, emphasis should be placed on dairy products, vegetables, and fruits.
Children with the syndrome must be registered with an endocrinologist, undergo annual glucose testing, etc. The prognosis is generally favorable.
As people grow older, the occurrence of acetonemic crises stops. Most often this happens in adolescence. Upon timely receipt medical care and with competent therapeutic tactics ketoacidosis is relieved.
When diagnosing recurrent acetonemic conditions, it is necessary to follow a high-carbohydrate and high-protein diet and regularly check for the presence of acetone in the urine using test strips. It is important to avoid long breaks between meals.
Pediatric ketoacidosis- a heterogeneous group of conditions accompanied by the appearance of ketone bodies in the blood and urine. Ketone bodies- the main carriers of energy from the liver to other tissues and the main source of energy obtained by brain tissue from lipids. Main reasons ketoacidosis in newborns - diabetes, glycogenosis type I (232200), glycinemia (232000, 232050), methylmalonic aciduria (251000), lactic acidosis, succinyl-CoA - acetoacetate transferase deficiency.
Code by international classification diseases ICD-10:
Insufficiency of succinyl-CoA-acetoacetate transferase (#245050, EC 2. 8. 3. 5, 5p13, defect of the SCOT, r gene) - an enzyme of the mitochondrial matrix that catalyzes the first step of the breakdown of ketone bodies.
Richards-Randle syndrome (*245100, r) is ketoaciduria with mental impairment and other symptoms.
Lactic acidosis - several types caused by mutations in various enzymes of lactic acid metabolism: . deficiency of lipoyl transacetylase E2 (245348, r, À); . deficiency of the component of the pyruvate dehydrogenase complex containing X - lipoyl (*245349, 11p13, PDX1, r gene); . congenital infantile form of lactic acidosis (*245400, r); . lactic acidosis with the release of D - lactic acid (245450, r). General symptoms- lactic acidosis, delayed psychomotor development, muscle hypotension. Some forms have specific manifestations, such as microcephaly, muscle twitching, baldness, necrotizing encephalopathy, etc.
Ketoadipic aciduria (245130, r).
ICD-10. E88. 8 Other specified metabolic disorders.
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Treatment Goals: normalization metabolic disorders(replenishment of insulin deficiency, fight against dehydration and hypovolemic shock, restoration of physiological acid-base balance, correction electrolyte disturbances, elimination of intoxication, treatment of concomitant diseases that led to the development of DKA).
Non-drug treatment
: table No. 9, isocaloric diet in accordance with the patient’s daily energy needs (calculation by equivalents is recommended).
Drug treatment
Insulin therapy for DKA
1. Short- or ultra-short-acting insulins are used (in the form of a solution: 10 units of insulin in 100 ml of 0.9% sodium chloride solution).
2. Insulin is administered only intravenously or using a lineomat at a dose of 0.1 U/kg body weight per hour.
3. When the glycemic level decreases to 13-14 mmol/l, the dose is halved (lowering glycemia below 10 mmol/l until ketoacidosis is eliminated is contraindicated).
4. If there is no effect after 2-3 hours, the dose is increased to 0.15 IU/kg body weight per hour, less often to 0.2 IU/kg body weight per hour.
After elimination of ketoacidosis until the condition stabilizes: intensified insulin therapy.
Rehydration
1. Begins immediately after diagnosis.
2. During the first hour - 1000 ml of 0.9% sodium chloride solution intravenously (in the presence of hyperosmolarity and low blood pressure - 0.45% sodium chloride solution).
3. Over the next two hours, hourly, 500 ml of 0.9% sodium chloride solution - in the following hours, no more than 300 ml per hour.
4. In case of heart failure, the volume of fluid is reduced.
5. When glycemia decreases below 14 mmol/l saline solution replaced with a 5-10% glucose solution (the solution should be warm).
6. Children are prescribed intravenous fluid administration at the rate of: from 150 ml/kg to 50 ml/kg per day, on average daily requirement in children: up to 1 year - 1000 ml, 1-5 years - 1500 ml, 5-10 years - 2000 ml, 10-15 years - 2000-3000 ml; in the first 6 hours it is necessary to administer 50% of the daily calculated dose, in the next 6 hours - 25%, in the remaining 12 hours - 25%.
Correction of potassium levels
1. Administration of potassium chloride in the presence of laboratory or ECG signs of hypokalemia and the absence of anuria is prescribed immediately.
2. When the level of potassium in the blood is below 3 mmol/l - 3 g of dry matter KCl per hour, at 3-4 mmol/l - 2 g KCl per hour, at 4-5 mmol/l - 1.5 g KCl per hour , at 5-6 mmol/l - 0.5 g KCl per hour, at 6 mmol/l or more - stop administering potassium.
Correction of acid-base status(ABC)
Recovery of acid-base balance occurs independently due to rehydration therapy and insulin administration. Sodium bicarbonate (soda) is administered only if constant monitoring of pH is possible, at pH<7,0, но даже в этом случае целесообразность его введения дискутабельна, высок риск алкалоза. При невозможности определения рН введение бикарбоната натрия запрещено.
Complementary therapy
1. In the presence of hypercoagulation - low molecular weight heparins.
2. In the presence of hypertension - antihypertensive therapy.
3. In case of hypovolemic shock - fight against shock.
4. In the presence of intercurrent diseases, heart or kidney failure, severe complications of diabetes - appropriate therapy.
Insulin preparations
Characteristic insulin preparations |
Names drugs insulin |
Notes | ||
Ultra-short-acting (analogs of human insulin) |
Lispro, Aspart, Glulisine |
Used for treatment ketoacidosis and after it liquidation |
||
Short acting |
Used for treatment ketoacidosis and after it liquidation |
|||
Average duration actions |
Apply only after elimination of ketoacidosis |
|||
Two-phase analog insulin |
Apply only after elimination of ketoacidosis |
|||
Ready-made insulin mixtures |
Short acting/ long-term actions: 30/70, 15/85, 25/75, 50/50 |
Apply only after elimination of ketoacidosis |
||
Long-term analogue peakless action |
Glargin, Levomir |
Apply only after elimination of ketoacidosis |
List of essential medications:
1. Ultra-short-acting insulin preparations (analogues of human insulin) lispro, aspart, glulisine
2. Short-acting insulin preparations
3. *Intermediate-acting insulin preparations
4. Biphasic insulin analogue
5. *Ready-made insulin mixtures (short-acting/long-acting 30/70, 15/85, 25/75, 50/50)
6. Long-term peak-free analogue (glargine, levomir)
Diabetes mellitus is dangerous due to its complications, one of which is ketoacidosis.
This is an acute insulin deficiency condition that, in the absence of medical correction measures, can lead to death.
So, what are the symptoms characteristic of this condition and how to prevent the worst outcome.
Diabetic ketoacidosis is a pathological condition associated with improper carbohydrate metabolism due to insulin deficiency, resulting in the amount of glucose and acetone in the blood significantly exceeding normal physiological indicators.
It is also called. It falls into the category of life-threatening conditions.
When the situation with impaired carbohydrate metabolism is not stopped in time by medical methods, ketoacidotic coma develops.
The development of ketoacidosis can be noticed by characteristic symptoms, which will be discussed a little later.
Clinical diagnosis of the condition is based on biochemical tests of blood and urine, and treatment is based on:
The occurrence of ketoacidosis in different types of diabetes has its own characteristics.
also called juvenile.
It is an autoimmune pathology in which a person constantly needs insulin because the body does not produce it.
The disorders are congenital.
The cause of ketoacidosis in this case is called absolute insulin deficiency. If type 1 diabetes mellitus was not diagnosed in a timely manner, then a ketoacidotic state may be a manifest manifestation of the underlying pathology in those who did not know about their diagnosis and therefore did not receive therapy.
is an acquired pathology in which insulin is synthesized by the body.
At the initial stage, its amount may even be normal.
The problem is the reduced sensitivity of tissues to the action of this protein hormone (which is called insulin resistance) due to destructive changes in the beta cells of the pancreas.
Relative insulin deficiency occurs. Over time, as the pathology develops, the production of your own insulin decreases and sometimes is completely blocked. This often leads to the development of ketoacidosis if the person does not receive adequate drug support.
There are also indirect reasons that can provoke a ketoacidotic state caused by an acute lack of insulin:
Based on the severity of the condition, ketoacidosis is divided into 3 degrees, each of which differs in its manifestations.
Mild degree characterized by the fact that:
Average the degree is expressed by a deterioration of the condition and is manifested by the fact that:
Heavy degree is characterized by:
The most common cause of ketoacidosis is type 1 diabetes.
Diabetic ketoacidosis, as mentioned earlier, occurs due to a deficiency (absolute or relative) of insulin.
It is caused by:
In all these cases, the increased need for insulin is caused by increased secretion of hormones that inhibit its functionality, as well as insufficient sensitivity of tissues to its action.
In 25% of diabetics, the cause of ketoacidosis cannot be determined.
The symptoms of ketoacidosis were mentioned in detail above when discussing the severity of this condition. The symptoms of the initial period increase over time. Later, other signs of developing disorders and progressive severity of the condition are added to it.
If we single out a set of “talking” symptoms of ketoacidosis, they will be:
Often, the diagnosis of ketoacidosis is complicated by the similarity of individual symptoms with other conditions.
Thus, the presence of nausea, vomiting and pain in the epigastrium is taken as signs of peritonitis, and the person ends up in the surgical department instead of the endocrinology department.
To identify ketoacidosis of diabetes mellitus, the following measures are needed:
Treatment is prescribed by a doctor based on the results of the examination and clinical diagnosis.
This takes into account such parameters as:
Therapy consists of:
All treatment measures are carried out in a hospital setting, with placement in the intensive care unit. Therefore, refusing hospitalization can cost your life.
The period of development of ketoacidosis can range from several hours to several days, sometimes longer. If left untreated, it can cause a number of complications, including:
When acute problems of carbohydrate metabolism caused by ketoacidosis are not resolved in a timely manner, a life-threatening complication develops - ketoacidotic coma.
It occurs in four cases out of a hundred, while the mortality rate in people under 60 years of age is up to 15%, and in older diabetics - 20%.
The following circumstances can cause the development of coma:
The symptoms of ketoacidotic coma largely depend on its form:
Ketoacidotic coma is a severe condition. Despite this, the likelihood of a favorable prognosis is quite high if emergency medical care is started no later than 6 hours from the appearance of the first signs of a complication.
The combination of ketoacidotic coma with a heart attack or cerebral circulatory problems, as well as the lack of treatment, unfortunately, produces a fatal result.
To reduce the risk of the condition discussed in this article, you must follow preventive measures:
Regular visits to your doctor and full compliance with his recommendations, as well as careful attention to your own health, will help you avoid such severe and dangerous conditions as ketoacidosis and its complications.