GIPERAMMONIYEMIYA (hyperammoniaemia; grech, hyper-+ an ammoniyemiya) — the increased maintenance of free ions of ammonium (NH 4 + ) in blood, being a consequence of some patol, states. Not recommended synonym — an ammoniyemiya. At the healthy person concentration of free ions of ammonium in blood usually does not exceed 0,05 mg of %, in erythrocytes they are 3 times more, than in plasma.
Ammonium connections are formed of ammonia, the main sources to-rogo in an organism are the processes of deamination happening in all fabrics, but is preferential in muscles, a brain, a liver and kidneys. Besides, a source of ammonia are the ammonium salts which are soaking up from intestines formed at disintegration of proteins and their catabolits under the influence of an indestinal flora. The formed ammonia quickly is removed from an organism or utilized in three ways: 1) enters interaction of e amino acids (glutaminic, asparaginic, etc.); 2) it is allocated with kidneys; 3) turns into urea.
It can be observed during attacks eclampsias (see) due to the increased formation of ions of ammonium in muscles and at a decompensation of cordial activity (insufficient removal of ions of ammonium kidneys). She is also at inherited disorders of exchange — arginine - amber aminoacidurias (see), tsitrullinuriya, family G. Odnako are the most frequent reason of G. diseases of a liver, especially cirrhoses. Arises or from decline in the ability to a mochevinoobrazovaniye, or because of existence of porto-caval shunting of blood. The maintenance of free ions of ammonium in these conditions can increase by 5 — 10 times. Considerably the amount of ammonium at patients increases cirrhosis (see) later bleedings from went. - kish. path. It can be used for establishment of the reason of bleeding: G.'s absence indicates small probability of communication of bleeding with cirrhosis.
Diagnostic value also test from the provoked G. by intake has 5 g of acetic or 1 g of ammonium muriate; at the same time at healthy people the content in blood of free ions of ammonium does not change, and at patients with cirrhosis considerably increases, and in an arterial blood it is more, than in venous. High G. is noted at a considerable liver failure and plays a pathogenetic role in development of a hepatic coma (see. Hepatargy , Coma ).
Methods of definition of a giperammoniyemiya come down to department of ammonia and determination of its quantity (see. Ammonia ). The research needs to be made right after capture of blood since at its standing the content of ammonia quickly increases (twice in 2 hours).
Therapeutic actions at G. come down to restriction of intake of ammonia from intestines (purpose of food poor in proteins, reduction of putrefactive processes in intestines by its regular clarification and use of antibiotics) and to binding of ammonia of blood (appointment glutaminic to - you, arginine, apple to - you). Also administration of hypertensive solution of glucose with insulin is recommended.
Giperammoniyemiya family — the hereditary disease transmitted on autosomal recessively type, connected with defect in a tricarbonic acid cycle — Genzeleyta owing to decrease of the activity ornithine-transcarboamylase of a liver, edge is responsible for formation of citrulline from ornithine and carboamylphosphate. It is characterized by permanent increase in content in blood of ammonia. It is described in 1962 by A. Russel. The main the wedge, symptoms are connected most often with defeat of c. N of page. At the same time high degree of the mental retardation caused by toxic action of the increased concentration in blood of ammonia is noted. The disease is shown in the first weeks of life by vomiting, development of dehydration, acidosis and further a coma. The disease can be shown later (there are observations over 3-and 9-year-old children). The squirrel leads a temporary restriction to improvement of a state, but vomiting periodically renews. Strengthening in blood of ammonia most heavier affects in the early childhood, during formation of functions of a brain. In blood the urea nitrogen is sharply lowered.
Differential diagnosis carry out with atsetonemichesky vomiting (see. Acetonemias ) and various inherited disorders of synthesis of urea.
Treatment: apply intravenous drop injections of solutions of glucose, sodium bicarbonate and sodium chloride, glutaminic to - that, and also L-keto-glutaric and lemon to - you.
Bibliography: Badalyan L. O., Tabolin V. A. and Veltishch ev Yu. E. Hereditary diseases at children, M., 1971; Makkyyusik V. A. Ancestral features of the person, the lane with English, page 1970, M., 1976; Harris G. Fundamentals of biochemical genetics of the person, the lane with English, M., 1973, bibliogr.
N. D. Mikhaylova; O. K. Botvinyev (ped.).