Page 67 of 73
Chronic damage of a liver can be an outcome of many diseases. Chronic active hepatitis, a galactosemia, a mucoviscidosis, Wilson's illness — Konovalova, disturbances of structure of a biliary path (an atresia and a choledochal cyst) conduct to cirrhosis which can be prevented by means of special measures. Now it is not possible to avoid development of cirrhosis after various acute diseases of a liver (hepatitis and sepsis of newborns, a viral hepatitis, toxic hepatitis). At a number of patients cirrhosis arises for no apparent reason. Treatment in such cases is directed to suspending destructive process and to prevent complications, including such serious as gastrointestinal bleedings or a hepatic coma. At emergence of these life-threatening complications it is necessary to take emergency measures.
Maintaining patients. The most important aspect of maintaining patients with cirrhosis — the planned supervision consisting in physical inspection, implementation of functional tests and periodic carrying out a chreskozhpy biopsy of a liver. After each cycle of inspection of the patient has to receive the recommendations concerning food, physical activity, a work-rest schedule. It is necessary to avoid considerable exercise stresses. Parents and children of advanced age need to explain that the injuries accompanying an infection, various gepatotoksichny drugs can cause an exacerbation of an illness. Patients should not accept streptocides of the prolonged action, tetracyclines, novobiocin, laurylsulphatic salts of erythromycin, anticonvulsants (Trimethadionum and diphenylhydantoin). fenotiazina, acetphenetidiene and acetaminophenum. To apply anabolic hormones to treatment of patients who refrakterna to a dietotherapy, follows with care as they possess a potential hepatotoxic.
In all cases of far come disease of a liver, uncomplicated ascites or a coma, a main goal — to provide the diet containing enough energy and all necessary nutrients to avoid a growth inhibition? and deficit of separate nutrients. By drawing up a diet it is necessary to consider a condition of this patient, but anyway it has to contain a large amount of protein. It is usually enough to accept daily on 1,5 g of protein on 1 kg of body weight. Though disturbance of absorption of fat occurs at children infrequently unlike adult patients at whom alcoholic cirrhosis is combined with pancreatitis, it is reasonable to appoint fat-soluble vitamins, especially at an atresia of bilious ways. Such patients, and also at patients with deficit of a prothrombin have an introduction of water-soluble analog of vitamin K subcutaneously or intramusculary in a dose of 2 — 4 mg allows to normalize during 1 — 2 days a prothrombin time. At a hypofibrinogenemia injection of freshly frozen plasma is required (if a blood coagulation in a slowed-up way).
Skin itch. The severe itch is a serious problem at an atresia inside - and extrahepatic channels. It can be eliminated, having entered high doses of a holestiramin (Questranum). Unfortunately, this drug often causes diarrhea. The dose it is raised to maksimalnoperenosimy (16 g/days). As at such patients deficit of vitamin E is often noted, it is reasonable to administer the water-soluble drug in a dose to 1000 ME/days. At treatment holestiraminy administration of vitamin D' and its derivatives is also shown.
Heavy complications. Carry bleedings from expanded veins of a gullet to heavy complications of cirrhosis, ascites and a hepatic coma. Portal hypertensia is the important reason of all these complications. However shunting with the purpose to unload portal system at cirrhosis does not improve function of a liver and can even lead to encephalopathy. Besides, the small diameter of veins at the child complicates creation of a reliable anastomosis. Preventive creation of a porto-caval anastomosis does not improve the forecast, and surgical interventions according to urgent indications are followed by high mortality.
Transthoracic bandaging of expanded veins of a gullet at children — rather effective palliative operation, but at adults at such planned operations with the subsequent creation of a porto-caval anastomosis mortality is higher, than after a neostomy according to the emergency indications. Thus, at cirrhosis treatment of complications of portal hypertensia, but not attempt to recover a blood stream by means of surgical intervention is more justified (except for bleedings which cannot be stopped otherwise).
Bleeding. Treatment of gastrointestinal bleedings at patients with cirrhosis is complicated not only because of their general serious condition, but also owing to disturbance of synthesis of factors of coagulation, the thrombocytopenia (caused by a hypersplenism) p circulation in blood of fibrinolysins. Situation is complicated by the fact that from the blood which streamed in intestines a large amount of ammonia reabsorbirutsya, there is an imbalance of electrolytes, in particular a hyponatremia, a hypopotassemia and a heavy metabolic alkalosis. Correction of these disturbances promotes the prevention of a coma and preservation of life of the patient; therefore, it has to be a component of fight against bleeding.
It is important to watch passability of respiratory tracts and if necessary to carry out an oxygenotherapy. Transfusion of whole fresh blood supports its constant volume, normalizes blood supply of fabrics, provides an organism with factors of coagulation and thrombocytes. If there is no fresh blood, then enter stored blood and trombotsitny weight. Liquid, electrolytes, vitamins of group B and vitamin K enter parenterally. It is necessary to measure often the venous pressure and amount of the emitted urine (for this purpose enter a constant catheter into a bladder). Measures for the prevention of a hepatic coma carry out at all gastrointestinal bleedings which arose against a heavy liver failure. Blood from a large intestine is deleted by means of enemas and purgatives to warn an ammonia reabsorption. Blood is also sucked away from a stomach through a probe. Neomycinum solution (on 2 — 4 g/days) is given inside or entered in an enema with the purpose to suppress the microflora producing ammonia.
Not only expanded veins of a gullet can be a bleeding point at cirrhosis. As the bleeding round ulcers and hemorrhagic gastritis treat not as esophageal bleeding therefore it is important to define as soon as possible localization of bleeding, however anyway begin with hemotransfusion. Endoscopic research allows to make the correct diagnosis with a big accuracy, especially if expanded veins continue to bleed.
By training the patient for endoscopy it is necessary to wash out a stomach ice water. This procedure sometimes slows down or even stops bleeding from gullet veins. In case of detection of the bleeding gullet veins intravenously enter vasopressin (10 — 20 units into 25 ml of normal saline solution within 10 min.) for pressure decrease in portal system. Increase of arterial pressure and developing of diarrhea testifies to adequacy of the entered dose. If injection of vasopressin improves a condition of the patient, then it can be repeated bucketed in 1 h.
If, despite everything the taken measures, life-threatening bleeding proceeds, then use a special probe cylinder (Sengshtagen's probe — Blekmera). The gastric cylinder is placed at the level of a diaphragm, then it is inflated up to the volume of 300 ml at children of advanced age (or smaller volume at children of younger age). Additional tractions of a probe usually are not required. If the compression of veins in the area kardioezofagealyyugo transition stops bleeding, to inflate the second, esophageal, the cylinder does not follow. Otherwise the esophageal cylinder is filled with air, bringing pressure in it to 30 mm of mercury. and controlling this level by means of the manometer. Through the same probe it is possible to enter antibiotics and purgatives, to suck away blood from a stomach. Introduction of a probe is fraught with dangerous complications, such as throwing of emetic masses in lungs, asthma owing to hit of a probe in respiratory tracts and injury of a mucous membrane of a gullet. Even in those medical institutions which have experience of carrying out such procedure mortality after it reaches 20%.
The specified measures in most cases allow to stop bleeding, however sometimes it is necessary to resort to portal shunting. If the condition of the patient does not allow to perform operation, then try to sclerose veins special drugs, injecting them directly in veins or in a liver parenchyma. This measure gives only short-term effect; the long-term results are unfavourable.
Ascites. The combination of a hypoalbuminemia, a hyper aldosteronism, a renal failure and disturbance of functions of a liver conducts to a delay in an organism of sodium and water. The main reason of accumulation of liquid in an abdominal cavity — portal hypertensia. Many origins of ascites are well studied, but is still not clear whether this state is pathological or physiological reaction. Therefore, to limit liquid accumulation, it is necessary to use easy, safe means. It is desirable to resort to a paracentesis only at development of acute respiratory insufficiency. About 50 ml of ascitic liquid can be taken for research, century of a particular with the purpose to exclude peritonitis.
At cirrhosis ascites can suddenly arise after bleeding, an intercurrent infection, surgical intervention. It is necessary to avoid such stressful situations and to treat intensively infectious diseases. Acute ascites often passes after reduction of reception of sodium with food up to 0,3 — 0,5 g in "ducks. It is important that the caloric content of food and content of protein in it corresponded to requirements of the growing organism.
"Chronic" ascites reflects the accruing decompensation of a liver and portal hypertensia. It causes discomfort, an asthma, sharp decrease in physical activity. Except a dietotherapy, in such cases it is necessary to apply a combination of diuretics (tiazid, furosemide, Acidum etacrynicum) and the means influencing removal with urine of sodium and potassium (Spironolactonum, Triamterenum).
Treatment is begun in the conditions of a hospital. Consumption of sodium is limited to 0,5 g, or 1 — 2 mmol/kg of body weight a day. If the renal clearance of water is not broken, consumption of liquid is not limited, but nevertheless volume it should not exceed 1 l a day. The amount of protein in a diet is also reduced to g/(kg-days) at children with far come cirrhosis to minimize probability of a hepatic coma and to limit intake of the salt which is contained in the majority of products rich with protein. It is reasonable to get advice of the nutritionist with the purpose to make the menu containing electrolyte-deficient proteins, starchy substances and vitamins B such combinations which provide the sufficient caloric content of food and do it tasty. In an initiation of treatment concentration of sodium and potassium in plasma is defined daily, and in urine — in 2 days. The purpose of a hyposodium diet — to achieve excretion with urine more than 15 mmol/days of sodium and decrease in body weight by 250 g a day. The negative sodium balance within 100 — 150 mmol helps to bring about 1 l of liquid out of an organism. To support the constant level of potassium in blood, it is appointed sometimes in addition in the form of chloride (to 90 mmol/days). Such dietary measures allow to achieve sufficient diuresis in 50% of cases of the beginning ascites.
If ascites does not give in to a dietotherapy, then sick appoint diuretics, using the least toxic of them in the most effective combinations. Means of the choice is Spironolactonum (veroshpiron) in the dose of 75 — 100 mg divided into 3 receptions. Decrease in body weight and excretion of sodium with urine are registered during the subsequent 4 days. If this drug does not yield desirable result (a body degrowth less than 1 kg and excretion of sodium less than 15 mmol/days), then add diuretic of a tiazidovy row. Furosemide in a daily dose of 60 — 80 mg stimulates release of urine in volume, smaller the volume of reabsorbiruyemy astsitny liquid. As from an abdominal cavity about 700 ml of liquid a day come to blood, and with urine it is lost to 300 ml, this scheme of treatment is effective and safe for the patient who does not have hypostases.
If liquid leaves a blood channel quicker, than an abdominal cavity or edematous fabrics, then can develop hypovolemia and a renal azotemia. For fast recovery of volume of plasma it is necessary to enter intravenously albumine (1 mg/kg), and to lower a dose of furosemide so that excretion was much lower than the volume of reabsorbiruyemy astsitny liquid. It is important to watch potassium concentration in urine to prevent the hypopotassemia caused by action of tiazid on renal tubules. It is not recommended to appoint potassium chloride in capsules because this drug can cause an ulceration of a mucous membrane of intestines or its impassability. If, despite the taken measures, the hypopotassemia remains, then as diuretic use Triamterenum separately or in a combination with Spironolactonum as they strengthen kaliysberegayushchy action of each other.
It is possible to liquidate ascites and to pick up a suitable maintenance therapy in the conditions of a hospital usually for several weeks. The subsequent treatment in house conditions includes a hyposodium diet and reception of diuretics if body weight begins to increase. Children with chronic active hepatitis often need steroid therapy and additional reception of potassium during aggravations of a basic disease.
At most of patients it is possible to recover a water and salt metabolism. Sometimes the decompensation of function of a liver is complicated by a relative hyponatremia owing to expansion of a vascular bed the late liquid or a hypopotassemia which is difficult for korrigirovat potassium additives. On this background the renal failure can develop as it is spontaneous, and as a result of intestinal bleeding. In such situation diuretics are contraindicated. In blood of an urea nitrogen, oliguria and delay of liquid reduce consumption of protein and water because of increase of concentration, at emergence of a hyperpotassemia reduce also amount of food potassium. The forecast very bad, often develops a coma. Patients with ascites quite often should do shunting operations thanks to which astsitny liquid goes to a jugular vein.
Coma. At the patient with cirrhosis to whom the accompanying infection, disturbance of water exchange, diuretics, sedative drugs and tranquilizers, surgical interventions, bleedings and proteinaceous intoxication can provoke. Medical actions include antibiotic treatment, correction hypo - and overhydratations, cancellation of diuretics and other gepatotoksichny drugs, the prevention of bleedings, refusal of surgical interventions.
When the damaging effect of ammonia became clear, treatment of hepatic encephalopathy radically changed — from the diets enriched with protein passed to its limited consumption. However the ammonia role in development of a hepatic coma is finally not found out, and results of controlled clinical tests of a dietotherapy, antibioticotherapia and potentially toxic diuretics are not published yet.
Protein restriction, on the one hand, preserves a brain, and with another — leads to a further aggravation of symptoms of a liver.
Modern approach to treatment of a hepatic coma is based on aspiration to suppress production of ammonia. For this purpose reduce amount of protein in food during the coma period and appoint the low-absorbed antibiotics (Neomycinum) to suppress the microflora producing ammonia. During the acute period appoint 2 — 4 g of Neomycinum a day. As the supporting means it is possible to use lactulose on 10 — 15 ml 3 times a day.
Lactulose — not absorbed synthetic disaccharide — suppresses the intestinal microflora which is actively producing ammonia. Contents it in blood under the influence of lactulose decreases also because drug promotes transition of the ionized ammonia to not ionized ammonium (NH3-> of NH4). The last is not soaked up in intestines and can be utilized by fekalpy bacteria as a nitrogen source. The dose of lactulose stated above allows to avoid the diarrhea connected with lactification and increase of osmotic pressure in intestines. Do to the patients who is in a coma enemas with lactulose in addition to introduction of Neomycinum; it allows to suppress quicker the microflora producing ammonia and other absorbed metabolites. Maintenance of normal level of potassium and cancellation of diuretics are also means of regulation of excretion of ammonia kidneys.
The patients who are in a pryokomatozny state are transferred to protein-free food. During the coma period it is important to avoid overhydratation and a hyponatremia. On the content of sodium in urine judge digestion of the sodium entered intravenously, and simultaneous measurement of osmolarity of serum and urine allows to estimate extent of hydration. Injections of 10% of solution of glucose supplement with introduction of carefully calculated doses of drugs of sodium and potassium. Disturbance of a blood coagulation is korrigirut infusions of freshly frozen plasma or fresh whole blood, especially if the prothrombin time does not change, despite administration of vitamin K (10 mg). Antibacterial protection is provided with antibiotics of a broad spectrum of activity (for example, ampicillin); if specific microbes are sowed, then to them select the corresponding antibacterial drugs.
Energy demands of an organism satisfy only at the expense of glucose which is entered in the maximum tolerable doses that it is the most effective to fight against disintegration of muscular tissue and products of ammonia. With the purpose to improve utilization of glucose and for fight against a hyperglycemia in addition appoint insulin. In an exit phase from a coma enter protein again, gradually increasing its quantity by 0,5 g/kg of body weight, under control of concentration of ammonia in blood. The task is in that within several days to bring consumption of protein to 1,5 g/(kg-days).
Exchange hemotransfusions and corticosteroids do not give effect at a hepatic coma. Among experimental methods 253 treatments it is necessary to call extracorporal hemoperfusion through absorbent carbon and a dialysis through porous acrylonitrile membranes. Ketoanalogues of irreplaceable amino acids, apparently, can be useful to treatment of some patients with rather safe function of a liver.
Patients with an uncomplicated chronic illness of a liver will respond to treatment the means mentioned above, but when progressing process, the renal failure or encephalopathy which arose after creation of a porto-caval anastomosis, the forecast usually bad.
Liver tumors see in appropriate section.