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Ray's syndrome - Diseases of digestive organs at children

Table of contents
Diseases of digestive organs at children
Oral cavity
Diseases of teeth
Malformations of the sky and soft tissues of an oral cavity
Diseases of a mucous membrane of an oral cavity and gums
Diseases of lips and language
Sialadens
Digestive tract
Basic reasons of gastrointestinal frustration
Gullet
Atresia and esophageal and tracheal fistula
Guttural and tracheal and esophageal crevice, inborn stenosis of a gullet
Other diseases of a gullet
Stomach and intestines
Peptic ulcer
Inborn hypertrophic pyloric stenosis
Inborn impassability of intestines
Inborn impassability of a duodenum
Disturbances of turn of intestines
Inborn impassability of a small bowel
Inborn megacolon
Diverticulums and duplikatura
The acquired impassability of intestines
Intestines invagination
Foreign bodys of a stomach and intestines
Motive frustration. stomach and intestines
Anomalies of a structure of anorectal area
Infectious diseases of intestines
Nonspecific ulcer colitis
Illness Krone
Necrotic coloenteritis of newborns
The coloenteritis connected with treatment by antibiotics
Gastrointestinal symptoms of anaphylactoid purpura, gemolitiko-uraemic syndrome
Intolerance of food proteins
Eosinophilic gastroenteritis
Absorption disturbance syndromes
Malabsorption
Immunodeficiency and intestines
Syndrome of "a congestive loop"
Syndrome of a short small bowel
Gee's disease
Sprue after acute enteritis
Tropical to a spr
Whipple's illness, intestines lymphangiectasia, Uolmap's illness, idiopathic diffusion defeat of mucous
Enzymopathies and disturbances of mechanisms of transport of nutrients
Irritable colon
Acute appendicitis
Diseases of an anus, direct and large intestine
Tumors of a digestive tract at children
Hernias of a digestive tract at children
Exocrine part of a pancreas
Pancreatitis
Embryonic development of structure and function of a liver
Diagnosis of diseases of a liver
Cholestatic states at babies
Parenchymatous jaundices at children of chest age
Disturbances of a metabolism of a liver and zhelchevydelitelny system
Anomalies of a structure of bilious ways
Cysts of bilious channels
Cholestasia and diseases of a liver connected with completely parenteral food
Medicinal damage of a liver
Ray's syndrome
Chronic hepatitis
Wilson's illness — Konovalova
Indian juvenile cirrhosis
Damages of a liver at chronic colitis
Cirrhosis and chronic liver failure
Portal hypertensia and varicosity of a gullet
Fatty infiltration of a liver
Cholecystitis
Peritoneum diseases
Peritonitis
Peritoneum hernias

Ray's syndrome — the acute disease arising against a nonspecific viral upper respiratory tract infection or a digestive tract it quite often accompanies also chicken pox, flu A or B. There are messages on emergence of this syndrome at poisoning with an aflatoxin (Thailand) and warfarin (Israel). In Jamaica the disease reminding Ray's syndrome which is caused by the hypoglycine A (metilentsiklopropanuksusny acid) which is contained in unripe fruits of a tree like is known.
Epidemiology and etiology. The illness affects children of all age, but most often it occurs at children of 6 — 9 and 11 — 14 years. Boys and girls are ill equally often. Communication of a disease with reception of antipyretics is noticed. In the last decade mortality decreased from 50 to 30%.
The reason of a syndrome of Ray is unknown. The hypothesis according to which dysfunction of mitochondrions is the cornerstone of a pathogeny is most widespread. This hypothesis is based on ultrastructural changes of mitochondrions (hypostasis, a polymorphism, disappearance of intra mitochondrial dense little bodies); these changes can be primary or secondary. Viruses, toxins, drugs and internal causes have the effect against the hereditary or acquired predisposition. Consider that the main defect consists in disturbance of an intra mitochondrial stage of an exchange of urea in which 2 enzymes — a karbamil-fosfatsintetaza and ornithinetranscarboamylase participate. The first of them talizirut transformation of ammonia into carboamylphosphate, and another reacts with carboamylphosphate and ornithine therefore citrulline is formed. Disturbance of these processes conducts to a giperammoniyemiya and formation of orotovy acid. As the last slows down synthesis of lipoproteins, an adiposity in a liver at Ray's syndrome, perhaps, there is a result of deficit of these proteins.
Clinical and datas of laboratory. In typical cases of an illness the beginning is shown by heavy nausea and vomiting against an insignificant viral infection. During the first hours or days of an illness the child is hyperactive, excited. The following stages are characterized by reduced reactivity, drowsiness, a stupor, loss of deep tendon jerks, disturbance of a muscle tone of extremities, spasms and a coma. The liver is soft, it is moderately increased. It is impossible to predict coma duration, recovery or death of the patient can become its outcome. The average duration of lethal cases — 4 — 5 days. Most of patients completely recover, but at children 2 years are aged younger and at school students heavy neurologic disturbances therefore during the acute period the long intensive care is required can develop.
Characteristic manifestations of an abnormal liver function consist in fast increase of level of aminotransferases, lengthening of a prothrombin time, increase of concentration of ammonia in blood in initial stages of an illness, however the level of serumal bilirubin does not change. The maintenance of a kreatininfosfataza also considerably exceeds norm. At children of early and chest age the hypoglycemia is possible. Expressiveness of changes of these indicators has, however, no essential predictive value. The respiratory alkalosis is often followed by the metabolic acidosis caused by accumulation of lactic acid. Ray's syndrome needs to be differentiated with hereditary defects of an exchange of urea, poisoning with salicylates, a hepatic coma, deficit of a carnitine, a plumbism and some organic acidurias.
Patomorfologiya. In hepatocytes microvesicular accumulations of fat in the absence of necrotic, inflammatory or fibrous changes (fig. 12-31) are observed. The similar, but less expressed fatty deposits are available in proximal renal tubules, a myocardium and a pancreas. Sometimes there is pancreatitis, but it is usually found only at autopsy. Brain hypostasis, a degeneration of neurons are noted, but inflammatory elements are absent. Electronic microscopic examination reveals accumulation of a large number of droplets of fat in cytoplasm of hepatocytes, swelling and deformation of mitochondrions, a hyperplasia of a smooth endoplasmic reticulum, a large number of peroxisomas.

Печень при синдроме Рея
Fig. 12-31, Liver at Ray's syndrome. Homogeneous microvesicles in hepatocytes, lack of signs of an inflammation and necrosis. Coloring gematoksilineoziny. X 100. The fragment of the same frozen bioptat painted across Gimza is presented on insert. In each hepatocyte droplets of neutral fat are visible. A light horizontal site — the central vein. X 200.

Treatment. All patients with suspicion on Ray's syndrome need to be hospitalized in an intensive care unit even if they are in consciousness and answer questions. Carry out a maintenance therapy and define a stage of an illness (tab. 12-18).
Table 12-18. Stages of a syndrome of Ray


Stage

Criteria

I

Drowsiness, vomiting, indifference

II

Delirium, excitement, hyperventilation

III

Dekortikatsionny poses, easy coma, hypoventilation, lack of a pupillary reflex

IV

Dekortikatsionny poses, deep coma, lack of spontaneous breath, mydriatic pupils

V

Spasms, apnoea

Install systems for maintenance of arterial and central venous pressure, enter catheters into a bladder and a stomach. At fever apply the cooling covers, intravenously enter liquids for 15% glucose solution. The prothrombin time is korrigirut by intramuscular administration of vitamin K (5 mg); for impact on intestinal microflora and reduction of amount of ammonia enter Neomycinum with an enema into blood or through a gastric catheter.
If the condition of the patient reaches the III stage, then carry out forced ventilation of the lungs, watch intracranial pressure. Intracranial pressure is registered constantly, it is supported at the level of 2 mm of mercury. or below, applying intravenous injections of osmotic diuretics, such as man-nitol. Carry out controlled hyperventilation so that the partial pressure of CO2 made 25 — 30 mm of mercury. Mannitolum is entered at the rate of 0,25 g/kg in the form of 20% of solution within 10 min. in the beginning. The dose can be raised to 0.4 g/kg gradually; if intracranial pressure remains high (more than 20 mm of mercury.), the drug is administered continuously to maintain osmolarity of blood at the level of 320 mosmmol/l. In cases, when against reduced intracranial pressure (less than 20 mm of mercury.) there are its sudden rises, they are stopped jet introduction of Mannitolum in a dose of 0,25 g/kg within 10 min. Krom of Mannitolum, solution of urea or glycerin use 30%.

At impossibility to control intracranial pressure by means of osmotherapy it is possible to try to apply phenobarbital though merits and demerits of this experimental method in relation to Ray's syndrome are not studied yet. Phenobarbital is entered intravenously, in the beginning in a dose of 5 mg/kg, then on 2,5 mg/kg by each 4 h until contents it in blood reaches 30 — 50 mg/l. When intracranial pressure decreases to normal amounts or there are complications, phenobarbital injections gradually stop. As the most serious complications of treatment by barbiturates it is possible to consider falling of arterial pressure, reduction of cordial emission and a hypoxia.



 
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