Acetonemic syndrome

Acetonemic syndrome (cyclic acetonemic vomiting syndrome, nondiabetic ketoacidosis) is a pathological condition that is accompanied by an increase in the blood content of ketone bodies (acetone, b-hydroxybutyric acid, acetoacetic acid) resulting from an amino acid metabolism and fat breakdown.

 About acetonemic syndrome in children speak in case of repeated acetonemic crises.

In pediatrics , there is a primary (idiopathic) acetonemic syndrome, which is an independent pathology, and a secondary acetonemic syndrome accompanying the course of a number of diseases. About 5% of children aged from 1 year to 12-13 years are prone to the development of primary acetonemic syndrome; The ratio of girls to boys is 11: 9.

Secondary giperketonemiya may occur in decompensated diabetes in children , insulin-induced hypoglycemia, hyperinsulinism , hyperthyroidism , Cushing’s disease , glycogen disease, head trauma , brain tumors in sella, toxic liver damage, infectious toxicosis, hemolytic anemia , leukemia , starvation, etc. states. Since the course and prognosis of secondary acetonemic syndrome is determined by the underlying disease, in the future we will discuss primary nondiabetic ketoacidosis.

The reasons

The basis of the development of acetonemic syndrome is absolute or relative deficiency of carbohydrates in the diet of the child or the predominance of fatty acids and ketogenic amino acids. The development of acetonemic syndrome contributes to the failure of liver enzymes involved in oxidative processes. In addition, features of metabolism in children are such that there is a decrease in ketolysis – the process of utilization of ketone bodies.

With absolute or relative deficiency of carbohydrates, the energy needs of the organism are compensated by enhanced lipolysis with the formation of an excess of free fatty acids. Under conditions of normal metabolism, the transformation of free fatty acids into the acetyl-coenzyme A metabolite occurs, which further participates in the resynthesis of fatty acids and the formation of cholesterol. Only a small part of acetyl-coenzyme A is spent on the formation of ketone bodies.

With enhanced lipolysis, the amount of acetyl-coenzyme A is excessive, and the activity of enzymes that activate the formation of fatty acids and cholesterol is insufficient. Therefore, the utilization of acetyl-coenzyme A occurs mainly by ketolysis.

A large number of ketone bodies (acetone, b-hydroxybutyric acid, acetoacetic acid) causes a violation of acid-base and water-electrolyte balance, has a toxic effect on the central nervous system and gastrointestinal tract, which is expressed in the clinic of acetonemic syndrome.

Psycho-emotional stress, intoxication, pain, insolation, infections ( ARVI , gastroenteritis , pneumonia , neuroinfection) can act as factors provoking acetonemic syndrome . An important role in the development of acetonemic syndrome is played by the alimentary factors – starvation, overeating, excessive consumption of protein and fatty foods with carbohydrate deficiency. Acetonemic syndrome in newborns is usually associated with late toxicosis in a pregnant woman – nephropathy .

Symptoms of acetonemic syndrome

Acetonemic syndrome often occurs in children with anomalies of the constitution ( neuro-arthritic diathesis ). Such children are distinguished by increased excitability and rapid exhaustion of the nervous system; they have a thin build, often too shy, suffer from neurosis and restless sleep. At the same time, a child with a neuro-arthritic abnormality of the constitution develops speech, memory and other cognitive processes faster than peers. Children with neuro-arthritic diathesis are prone to impaired exchange of purines and uric acid, therefore in adulthood they are prone to the development of urolithiasis, gout, arthritis, glomerulonephritis, obesity, and type 2 diabetes.

• Typical manifestations of acetonemic syndrome are acetonemic crises. Such crises in acetonemic syndrome may develop suddenly or after the precursors (the so-called aura): lethargy or agitation, lack of appetite, nausea, migraine-like headache , etc.

A typical acetonemic crisis is characterized by repeated or indomitable vomiting , which occurs when trying to feed or water a child. On the background of vomiting with acetonemic syndrome, signs of intoxication and dehydration quickly develop (muscular hypotonia, weakness, pallor of the skin with a blush).

Motor excitement and anxiety of the child are replaced by drowsiness and weakness; in severe acetonemic syndrome, meningeal symptoms and convulsions are possible . Characterized by fever (37.5-38.5 ° C), abdominal cramping, diarrhea, or delayed stool. From the mouth of the child, from the skin, urine and vomit emanates the smell of acetone.

The first attacks of acetonemic syndrome usually appear at the age of 2-3 years, increase in frequency by the age of 7 and disappear completely by the age of 12-13.

Diagnosis of acetonemic syndrome

Recognition of acetonemic syndrome contributes to the study of history and complaints, clinical symptoms, laboratory results. Be sure to distinguish between primary and secondary acetonemic syndrome.

An objective examination of a child with acetonemic syndrome during a crisis reveals a weakening of heart sounds, tachycardia , arrhythmia ; dry skin and mucous membranes, reduced skin turgor, reduced tearing; tachypnea, hepatomegaly , decrease in diuresis.

A blood test for acetonemic syndrome is characterized by leukocytosis, neutrophilia, accelerated erythrocyte sedimentation rate; urinalysis – ketonuria of varying degrees (from + to ++++). In the biochemical analysis of blood, hyponatremia (with the loss of extracellular fluid) or hypernatremia (with loss of intracellular fluid), hyper- or hypokalemia, elevated urea and uric acid, and normal or moderate hypoglycemia may occur.

Differential diagnosis of primary acetonemic syndrome is carried out with secondary ketoacidosis, acute abdomen ( appendicitis in children , peritonitis ), neurosurgical pathology ( meningitis , encephalitis , brain edema ), poisoning and intestinal infections . In this regard, the child should be additionally consulted by the pediatric endocrinologist, pediatric infectious disease specialist, pediatric gastroenterologist .

Treatment for acetonemic syndrome

The main areas of treatment for acetonemic syndrome are the relief of crises and supportive therapy in interictal periods, aimed at reducing the number of exacerbations.

At acetonemic crises hospitalization of the child is shown. Dietary correction is carried out: fats are strictly limited, highly digestible carbohydrates and plenty of fractional drinking are recommended. It is advisable to set up a cleansing enema with a solution of sodium bicarbonate, neutralizing a portion of ketone bodies trapped in the intestine. Oral rehydration in acetonemic syndrome is produced with alkaline mineral water and combined solutions. In case of severe dehydration, infusion therapy is performed – intravenous drip administration of 5% glucose, saline solutions. Symptomatic therapy includes the introduction of antiemetic drugs, antispasmodics, sedatives. With proper treatment, the symptoms of an acetonemic crisis subside by 2-5 days.

• During interictal periods, a child with acetonemic syndrome is monitored by a pediatrician . It requires the organization of proper nutrition (plant-milk diet, restriction of foods rich in fat), the prevention of infectious diseases and psycho-emotional overload, conducting water and tempering procedures (baths, contrast showers, douches, rubdowns), adequate sleep and staying in the fresh air.

Preventive courses of multivitamins, hepatoprotectors, enzymes, sedative therapy, massage are shown to a child with acetonemic syndrome . coprogram control. To control urine acetone, it is recommended to independently examine the urine for the content of ketone bodies using diagnostic test strips.

Children with acetonemic syndrome should be at the dispensary at the children’s endocrinologist, annually undergo a study of blood glucose, ultrasound of the kidneys and ultrasound of the abdominal cavity .

Acetonemic syndrome is a symptom complex caused by metabolic disturbances and the accumulation of ketone bodies in the child’s blood. Acetonemic syndrome is manifested by acetonemic crises: repeated vomiting, dehydration, intoxication, the smell of acetone from the mouth, abdominal syndrome, low-grade fever. Acetonemic syndrome is diagnosed on the basis of clinical data and laboratory parameters (detection of ketonuria, electrolyte imbalance, increased urea levels, etc.). In case of acetonemic crisis, infusion therapy, cleansing enema, oral dehydration, diet with the inclusion of easily digestible carbohydrates are indicated.