ACETONEMIA (acetone + grech, haima blood; synonym ketonemiya) — existence in blood of ketone bodies: acetone, acetoacetic and beta and hydroxy-butyric acids which are products of partial oxidation of fatty acids and are formed at disintegration of ketogenic amino acids. The normal maintenance of ketone bodies in blood makes from 1,0 to 2,0 mg of % (on acetone).
The main part of ketone bodies is oxidized in a tricarbonic acid cycle (see. Tricarboxylic acids cycle ) to carbonic acid and water; partially ketone bodies are used for synthesis of sterols, higher fatty acids, phosphatides, replaceable amino acids. Besides, small elimination of ketone bodies with urine and expired air normal takes place.
Increase in maintenance of ketone bodies in blood and urine (a giperketonemiya, a hyperketonuria) is promoted strengthening of a ketogenesis in a liver, by oppression of a ketoliz and resynthesis of higher fatty acids, and also partially defeat of ways of allocation of ketone bodies (e.g., kidneys at uraemia). And. it is observed at a diabetes mellitus, a renal glycosuria, an insulin hypoglycemia, glycogenoses, a thyrotoxicosis, heavy intoxications and infectious diseases, injuries of a skull, at the difficult postoperative period, atsetonemichesky vomiting, starvation, damages of a liver, bezuglevodisty food. At these diseases and states there is an excess formation of ketone bodies, braking of their oxidation, delay of their resynthesis in higher fatty acids, depletion of a liver a glycogen and excess receipt in this body of higher fatty acids. Giperketonemiya is always followed acetonuria (see) also leads to accumulation of ketone bodies in fabrics, development of metabolic acidosis (see). The most considerable giperketonemiya is observed at a diabetic (ketoatsidotichesky) coma. Ketone bodies possess action, toxic for an organism, owing to ability of acetone to dissolve fats.
The factors promoting easier emergence of a giperketonemiya at children are the lowered tolerance to fats, more intensive processes of glycolysis and a lipolysis in connection with the increased energy demands of the growing organism.
Atsetonemichesky vomiting — periodic pernicious vomiting with development And., hyper acetonemias and acetonurias. This state occurs preferential at children before the period of puberty and is characterized by emergence of crises with development of a giperketonemiya.
The pathogeny of atsetonemichesky vomiting is completely not found out. It is supposed that children after the postponed infectious diseases, injuries have skulls, organic diseases of c. the N of page in gipotalamo-diencephalic area remains for a long time the dominant center of congestive excitement inducing disturbances of a lipometabolism (strengthening of a ketogenesis, disturbance of normal use of ketone bodies in connection with exhaustion of carbohydrate stocks in an organism). In a pathogeny of atsetonemichesky vomiting anomalies of the constitution, relative insolvency of enzymatic systems of a liver, disturbance of endocrine regulation of metabolism can matter.
Emotional, infectious, toxic, traumatic, allergic and dietary factors provoke crisis.
A clinical picture
Crises can suddenly arise or after harbingers (anorexia, slackness, headaches and dizziness, abdominal pains) with intervals in several weeks or months, are characterized by pernicious vomiting with the phenomena of intoxication and an eksikoz. During attacks of vomiting temperature increases, the concern, often spasms, the consciousness sometimes darkened, a delay of a chair, colicy pains in a stomach appears. In urine and expired air acetone, in blood — the increased concentration of ketone bodies is found. On the electroencephalogram various deviations which are not disappearing completely after the termination of an attack come to light. Vomiting during the attack proceeding 2 — 4 days happens repeated. With urine of the patient during this period protein, cylinders, an indican is secreted in the increased quantity. In blood the level of sugar often decreases and quickly concentration of cholesterol and ß-lipopro-teidov increases. In hard cases the atsetonemichesky coma able develops to end letalno. On section fatty regeneration of a liver, to a lesser extent kidneys and heart is found.
Differential diagnosis it is necessary to carry out with comas of other origin (see. Coma ), and also with the diseases which are followed And. and acetonuria (diabetes mellitus, ketotichesky hypoglycemia), etc.
Forecast diseases in most cases favorable. Attacks are stopped within several hours. By the period of puberty attacks disappear completely.
in the period of an attack it is necessary to liquidate first of all acidosis — to appoint a gastric lavage and intestines of 1 — 2% solution of sodium bicarbonate, inside Ringer's solution, normal saline solution, 5% solution of glucose, 1% solution of sodium bicarbonate and mineral alkaline water. At increase of an eksikoz parenteral administration of saline solutions under laboratory control of water-salt and acid-base balance is necessary. The insulino-glyukozoterapiya is recommended: 4 — 6 PIECES of insulin a day and enteral and parenteral administration of solutions of glucose. At development of an alkalosis (e.g., at long vomiting) appoint a natural gastric juice or 0,5 — 1% solution divorced salt to - you with pepsin, 5% solution of glucose, saline solutions inside.
At a hyperthermia — antipyretics, at spasms — Chlorali hydras in an enema, phenobarbital, bromides. Purpose of lipocainum, methionine, drugs of potassium, a complex of vitamins is shown. In hard cases apply glucocorticoids.
In the mezhpristupny period — the correct mode, good nutrition with restriction of ketogenic substances (fats), drugs of calcium, vitamins. There are data on positive effect of anti-epileptic therapy.
Bibliography: Leytes S. M. and Lapteva H. H. Sketches on a pathophysiology of a metabolism and endocrine system, page 132, M., 1967, bibliogr.; Ferdman D. L. Biochemistry, page 315, M., 1966; Bremer J. Pathogenesis of ketonemia, Scand. J. clin. Lab. Invest., v. 23, p. 105, 1969.
Atsetonemichesky vomiting — Maslov M. S. Children's diseases, page 174, L., 1946; G. G's Knock. To a question of a role of a nervous factor in a pathogeny of periodic pernicious vomiting with an acetonemia (an atseto-nemichesky coma) at children, Pediatrics, No. 2, page 16, 1952; R and h e in L., - to r about in Y. and Stateva S. A metabolism at children's age, the lane with bolg., page 129, Sofia, 1967; Fankoni G. and Valgren L. The guide to children's diseases, the lane with it., page 152, M., 1960; Petermann H.D.u. Haltrich E. Elektroenzephalographische Untersuchungen im symptomfreien Intervall bei Kindern mit rezidivierendem azetonämischen Erbrechen, Z. ärztl. Fortbild., S. 833, 1971.
BB. A. Knyazev, L. L. Vakhrusheva.