The Atsetonemichesky syndrome – the simptomokompleks caused by violation of exchange processes and accumulation in blood of the child of ketone ph. the Atsetonemichesky syndrome is shown by atsetonemichesky crises: repeated vomiting, dehydration, intoxication, an acetone smell from a mouth, an abdominal syndrome, subfebrilitety. The Atsetonemichesky syndrome is diagnosed on the basis of clinical data and laboratory indicators (identification of a ketonuriya, violation of electrolytic balance, increase in level of urea, etc.). At atsetonemichesky crisis infusional therapy, statements of a cleaning enema, oral dehydration, a diet with inclusion of digestible carbohydrates is shown.
Atsetonemichesky syndrome (syndrome of cyclic atsetonemichesky vomiting, not diabetic ketoacidosis) – the pathological state which is followed by increase the content in blood of the ketone bodies (acetone, b-oxyoleic acid, acetoacetic acid) which are formed owing to violation of exchange of amino acids and fats. At children speak about an atsetonemichesky syndrome in case of the repeating atsetonemichesky crises.
In pediatrics distinguish primary (idiopathic) atsetonemichesky syndrome which is independent pathology and the secondary atsetonemichesky syndrome accompanying a current of a number of diseases. About 5% of children aged from 1 year up to 12-13 years are inclined to development of primary atsetonemichesky syndrome; the ratio of girls to boys makes 11:9.
The secondary giperketonemiya can arise at dekompensirovanny diabetes at children, an insulin hypoglycemia, a giperinsulinizm, a thyrotoxicosis, Itsenko-Cushing's disease, a glikogenovy disease, ChMT, brain tumors in the field of the Turkish saddle, toxic damages of a liver, infectious toxicosis, hemolytic anemia, leukemia, starvation, etc. states. As the current and the forecast of a secondary atsetonemichesky syndrome is defined by the main disease, further it will be a question of primary not diabetic ketoacidosis.
Reasons of an atsetonemichesky syndrome
Absolute or relative insufficiency of carbohydrates in a food allowance of the child or prevalence of fatty acids and ketogene amino acids is the cornerstone of development of an atsetonemichesky syndrome. Development of an atsetonemichesky syndrome is promoted by insufficiency of the enzymes of a liver participating in oxidizing processes. Besides, features of metabolism at children are that that decrease in a ketoliz - process of utilization of ketone bodies takes place.
At absolute or relative insufficiency of carbohydrates energy needs of an organism are compensated strengthened lipolizy with formation of excess of free fatty acids. In the conditions of normal metabolism in a liver there is a transformation of free fatty acids in a metabolite acetyl-coenzyme A which participates further in resynthesis of fatty acids and formation of cholesterol. Only a small part acetyl-coenzyme A is spent for formation of ketone bodies.
At the strengthened lipoliz the quantity acetyl-coenzyme A is superfluous, and activity of the enzymes intensifying formation of fatty acids and cholesterol is insufficient. Therefore utilization acetyl-coenzyme A happens generally by a ketoliz.
A large number of ketone bodies (acetone, b-oxyoleic acid, acetoacetic acid) causes violation of the acid and main and water and electrolytic balance, has toxic effect on TsNS and digestive tract that finds the expression in clinic of an atsetonemichesky syndrome.
As the factors provoking an atsetonemichesky syndrome psychoemotional stresses, intoxications, pain, insolation, infections can act (a SARS, a gastroenteritis, pneumonia, neuroinfections). An important role in development of an atsetonemichesky syndrome is played by almimentarny factors - starvation, an overeating, the excessive use of proteinaceous and greasy food at deficiency of carbohydrates. The Atsetonemichesky syndrome at newborns is usually connected with the late toxicosis taking place at pregnant – a nephropathy.
Symptoms of an atsetonemichesky syndrome
The Atsetonemichesky syndrome often occurs at children with anomalies of the constitution (neuro diathesis). Such children are distinguished by hypererethism and a bystry exhaustion of nervous system; they have a thin constitution, are often excessively timid, have neurosises and an uneasy dream. At the same time at the child with neuro anomaly of the constitution quicker than peers the speech, memory and other informative processes develop. Children with neuro diathesis are inclined to violation of exchange of purin and uric acid therefore they at mature age are subject to development of an urolithic disease, gout, arthritises, a glomerulonefrita, obesity, diabetes 2 types.
As typical manifestations of an atsetonemichesky syndrome serve atsetonemichesky crises. Similar crises at an atsetonemichesky syndrome can suddenly develop or after harbingers (so-called aura): slackness or excitement, lack of appetite, nausea, migrenepodobny headache, etc.
The typical clinic of atsetonemichesky crisis is characterized by repeated or unrestrained vomiting which arises in attempt to feed or give to drink the child. Against the background of vomiting at an atsetonemichesky syndrome symptoms of intoxication and dehydration quickly develop (muscular hypotonia, an adinamiya, pallor of skin with a flush).
Motive excitement and concern of the child are replaced by drowsiness and weakness; at a heavy current of an atsetonemichesky syndrome meningealny symptoms and spasms are possible. Fever (37,5-38,5 °C), spastic belly-aches, diarrhea or a delay of a chair are characteristic. From the child's mouth, from integuments, urine and emetic masses the acetone smell proceeds.
The first attacks of an atsetonemichesky syndrome usually appear at the age of 2-3 years, become frequent by 7 years and completely disappear to 12-13-year age.
Diagnostics of an atsetonemichesky syndrome
Recognition of an atsetonemichesky syndrome is promoted by studying of the anamnesis and complaints, clinical symptomatology, results of laboratory researches. Surely it is necessary to distinguish primary and secondary atsetonemichesky syndrome.
At objective inspection of the child with an atsetonemichesky syndrome during crisis easing of tones of heart, tachycardia, arrhythmia come to light; dryness of skin and mucous, decrease in turgor of skin, decrease in a slezovydeleniye; , gepatomegaliya, reduction of a diuresis.
Clinical blood test at an atsetonemichesky syndrome is characterized leykotsitozy, neytrofilezy, by acceleration of SOE; the general analysis of urine – ketonuriy various degree (from + to ++++). In biochemical blood test the giponatriyemiya (can be observed at loss of extracellular liquid) or a gipernatriyemiya (at loss of intracellular liquid), hyper - or a gipokaliyemiya, increase in level of urea and uric acid, normo-or a moderate hypoglycemia.
Differential diagnostics of primary atsetonemichesky syndrome is carried out with secondary ketoacidosis, a sharp stomach (appendicitis at children, peritonitis), neurosurgical pathology (meningitis, encephalitis, brain hypostasis), poisonings and intestinal infections. In this regard the child has to be in addition consulted by the children's endocrinologist, the children's surgeon, the children's neurologist, the children's infectiologist, the children's gastroenterologist.
Treatment of an atsetonemichesky syndrome
As the main directions of treatment of an atsetonemichesky syndrome serve knocking over of crises and the supporting therapy during the mezhpristupny periods directed to decrease in quantity of aggravations.
At atsetonemichesky crises hospitalization of the child is shown. Dietary correction is made: fats are strictly limited, digestible carbohydrates and plentiful fractional drink are recommended. Statement of a cleaning enema with the sodium hydrocarbonate solution neutralizing a part of the ketone bodies which got into intestines is expedient. The oral regidratation at an atsetonemichesky syndrome is made by alkaline mineral water and the combined solutions. At the expressed dehydration infusional therapy – intravenous drop administration of 5% of glucose, salt solutions is carried out. Symptomatic therapy includes introduction of antiemetic medicines, spazmolitik, sedative medicines. At the correct treatment symptoms of atsetonemichesky crisis abate for 2-5 day.
During the mezhpristupny periods observation of the child with an atsetonemichesky syndrome is carried out by the pediatrician. The organization of healthy nutrition (a vegetable milk diet, restriction of the products rich with fats), prevention of infectious diseases and psychoemotional overloads, holding the water and tempering procedures (bathtubs, a contrast shower, douches, rubdowns), a sufficient dream and stay in the fresh air is necessary.
Preventive rates of polyvitamins, gepatoprotektor, enzymes, sedative therapy, massage are shown to the child with an atsetonemichesky syndrome; control of a koprogramma. For control of acetone of urine it is recommended to investigate independently urine on the maintenance of ketone bodies by means of diagnostic test strips.
Children with an atsetonemichesky syndrome have to stay on the dispensary registry at the children's endocrinologist, annually pass a research of glucose of blood, ultrasonography of kidneys and ultrasonography of an abdominal cavity.