Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hereditary haemorrhagic teleangiectasia (Rendu-Osler-Weber disease) is an inborn error of vascular structure with multiple manifestations. Its incidence is about 1-2:100 000 in the European population. The incidence of telangiectases and/or fistula formation was estimated to be 1 in 10 carriers of the Osler trait. The findings in the family reported herewith suggest a much higher incidence if angiography is more frequently performed. Apart from the skin and mucous membrane, teleangiectases and/or arteriovenous fistulas may be present in the lungs, intestinal tract, spleen, kidney, brain, and bones. The liver apparently is more involved than was orginally suspected. The vascular derangement includes teleangiectases, arteriovenous fistulas, and connective tissue formation with fibrosis and atypical cirrhosis. In intestinal bleeding laser coagulation seems to be very efficient. The pathogenesis of teleangiectases is not known but involves several factors such as special formation of venules, capillaries and arterioles, abnormal perivascular connective tissue and endothelial cells.
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PMID:The liver in hereditary haemorrhagic teleangiectasia: an inborn error of vascular structure with multiple manifestations: a reappraisal. 30 29

Indications, selection of donor and recipient, medical and surgical management and complications, problems of organ procurement. Renal transplantation has become routine therapy. Organs are predominantly obtained from cadavers, transplantations from living donors are rarely indicated. Advances in preservation methods have improved organ quality and prolonged storage time. Selection of the most suitable recipient is based on histocompatibility matching. Blood transfusions before transplantation seem to improve the results. Recognition of a rejection crisis is primarily based on clinical symptoms. Persistent rejection calls for prompt explantation and the patient has to return to dialysis. Infections, serum-hepatitis and gastro-intestinal bleeding are the most common complications. Late complicatons are diabetes mellitus, cirrhosis of the liver, osteopathy, recurring glomerulonephritis, and, rarely, malignomas. Transplantation frequency in the Federal Republic of Germany could be increased by more awareness of physicians and a better knowledge of the general public about the need for cadaver donors.
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PMID:[Kidney transplantation from a nephrological-urological viewpoint--results and problems. 2. Diagnosis and therapy after transplantation, complications, long-term results]. 33 52

Thirty patients with alcoholic cirrhosis, ascitic during 13.6 +/- 13 months (mean +/- S.D.) were cured of ascites and followed up during 2 to 9 years (4.3 +/- 2.7 years). Twenty six were compared with a same number of cirrhotics, matched for age and sex, who died during the year after the first admission. Many biological data show statistical difference. Nevertheless no valuable prognosis can be predicted in an individual case. The clinical improvement is associated with major, sometimes total biological recovery. Other complications of cirrhosis (gastro-intestinal bleeding, hepatoma) may occur (7 cases with 5 deaths) or alcoholic hepatitis if alcohol withdrawal is stopped (3 cases, 2 deaths). Some associated diseases look unexpectedly frequent: diabetes (4 cases), obesity (9), nodular lipomatosis (14 cases) whose frequency looks higher than that can be calculated for a similar group of healthy subjects.
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PMID:[Recovery after treatment for cirrhotic ascites : a study of 90 cases. Frequency of arterial hypertension (author's transl)]. 49 44

The clinical syndrome of portal-systemic encephalopathy is caused by far advanced cirrhosis of the liver in most cases; it is characterized by increasing drowsiness, disturbances of mentation, flapping tremor and hyperreflexia. An early diagnosis can be established by testing writing and drawing abilities. Increased occurrence of spider nevi, a dry, deep red tongue, and hemorrhagic lesions of skin and mucous membranes are symptoms of incipient hepatic insufficiency. The syndrome is initiated in most cases by excessive intake of protein or alcohol, by intestinal bleeding, by diuretics, or by intercurrent infections. Therapy has to include elimination of causes, reduced intake of protein, enemas with acetate buffer solution and oral medication with lactulose, bifidum milk, and certain amino acids in order to lower hyperammoniemia; in serious cases neomycin has to be given. At the same time a normalization of fluid and electrolyte balance has to be achieved; replacement of potassium is especially important, when hypokalemia and alcalosis are present. In general prognosis of portal-systemic encephalopathy however is serious, depending primarily upon the fact, whether or not sufficient functional hepatic parenchyma is present.
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PMID:[Clinical picture and therapy of portal-systemic encephalopathy (author's transl)]. 89 27

Medical emergencies involving the liver and biliary tract are common clinical problems. If it is already known that the patient has cirrhosis it may be an easy matter to identify the cause of complications such as gastro-intestinal bleeding or coma, but it must be borne in mind that oesophageal varices are not the only cause of such bleeding in cirrhotics and that hepatic encephalopathy is not the only cause of coma. Bacterial infection should always be considered as a possible cause of deterioration in the clinical picture; it may be a complication of pre-existing acute or chronic liver or biliary tract disease or a cause of hepatobiliary disease; prompt administration of appropriate antibiotics may save the patient's life. If there is any suspicion of biliary obstruction in a patient with signs of bacteraemia the biliary tree should be drained without delay. The key to the management of hepatobiliary emergencies lies in prompt and appropriate supportive therapy, and then in a correct diagnosis which may allow specific treatment to be administered. However, it is often difficult to establish the cause, and the resources of a specialist centre may be needed. Prompt referral is indicated when a patient is clearly very ill and shows no signs of rapid improvement.
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PMID:Hepatobiliary disease: medical emergencies. 176 20

Propranolol, a beta-blocking agent, has been proposed in the prevention of gastro-intestinal bleeding in cirrhotic patients, and is known for its bronchoconstrictive effects. Since hypoxemia is a frequent finding in patients with cirrhosis, this work was undertaken to study the effects of intravenous propranolol on pulmonary function and on gas exchange in these patients. The 10 patients studied had cirrhosis associated with an increase in the alveolar arterial O2 difference, (A-a)DO2, an index of arterial oxygenation impairment. Their 1-s forced expiratory volume/forced vital capacity (FEV1/FVC) was normal, but in most a reduction of the forced expiratory flow of 25-75% of vital capacity was observed (FEF 25-75), suggestive of some degree of small airway obstruction. Although propranolol induced a significant decrease of FEF 25-75 from 67.7 +/- 19.3% to 55.4 +/- 21.5% (P less than 0.01), suggesting a bronchoconstriction of the small airways, there was no significant decrease in mean arterial oxygen partial pressure (PaO2) (74.1 +/- 6.4 mmHg before and 77.0 +/- 6.5 mmHg after propranolol). Indeed, a slight but significant improvement of the (A-a)DO2 was observed, from 39.1 +/- 5.9 mmHg to 34.4 +/- 4.9 mmHg (P less than 0.02). Although the mechanism of this beneficial effect remains to be elucidated, we conclude that in spite of its bronchoconstrictive action, propranolol is not contra-indicated in cirrhotic patients with hypoxemia who have normal expiratory flow.
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PMID:Effects of propranolol on pulmonary gas exchange in patients with cirrhosis. 280 64

The value of sclerotherapy as prophylaxis against the first episode of variceal hemorrhage has not been established. Therefore, we randomly assigned 133 patients with cirrhosis of the liver (of alcoholic origin in 66 percent), esophageal varices, and no previous intestinal bleeding to either prophylactic sclerotherapy (n = 68) or no prophylaxis (n = 65). The groups were comparable in hepatic function, endoscopic findings, and the pathogenesis of cirrhosis. All patients who subsequently had a first episode of variceal hemorrhage received sclerotherapy whenever possible. During a median follow-up of 22 months, variceal hemorrhage occurred in 28 percent of the patients receiving sclerotherapy and 37 percent of the controls (P = 0.3). Thirty-five percent of the sclerotherapy group and 46 percent of the control group died. The survival curves (Kaplan-Meier) of both groups were similar (P = 0.2). However, among patients with alcoholic and moderately decompensated cirrhosis (Child-Pugh group B), survival was significantly higher in those receiving sclerotherapy, although the risk of bleeding was only marginally reduced by this procedure. We conclude that prophylactic sclerotherapy does not significantly reduce the risk of bleeding from esophageal varices, but that a subgroup of patients with esophageal varices and moderately decompensated alcoholic cirrhosis may benefit from prophylactic sclerotherapy because of factors not solely attributable to prevention of an initial episode of variceal bleeding.
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PMID:Prophylactic sclerotherapy before the first episode of variceal hemorrhage in patients with cirrhosis. 328 71

One-hundred-seventy-five patients with oesophageal variceal bleeding and liver cirrhosis who underwent long-term injection sclerotherapy were prospectively followed for 44 +/- SD 17 months. While aetiology (alcoholic vs. non-alcoholic cirrhosis) had no influence on survival, the Child status assessed at the time of initiation of sclerotherapy was of important prognostic value (mortality rate after a mean follow-up of 44 months: Child A 5%, Child B 45%, and Child C 83%). Initiation of sclerotherapy (elective vs. emergency) had no influence on survival in the A/B patients, whereas electively treated Child C patients had a somewhat better outcome than those in whom sclerotherapy was started during active bleeding. Rebleeding, especially within the first two months after starting repeat sclerotherapy, was significantly influenced by the Child status of the patients. About one-fourth of all deaths were combined with rebleeding in the Child B and C patients, but none of the A patients had intestinal bleeding prior to death. It is concluded that Child A patients have an excellent long-term prognosis after variceal haemorrhage treated by repeated endoscopic injection of the varices.
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PMID:Injection sclerotherapy of oesophageal variceal haemorrhage. A prospective long-term follow-up study. 350 37

A surgical porto-systemic shunt was carried out in 37 children and adolescents (age: 2 yrs-18 yrs) who presented with cirrhosis and portal hypertension. All patients were classified "A" or "B" according to Child's criteria. Proof of the patency of the shunt was obtained by endoscopy and/or angiography in 31 children. Two children died in the early postoperative period. Thrombosis of the shunt occurred in 4 children. With a follow-up of 6 months to 11 years, none of the 31 children operated on successfully presented with gastro-intestinal bleeding due to portal hypertension. Nine presented with one or several episodes of porto-systemic encephalopathy. Five of these were transient allowing for a normal diet to be resumed later. These results indicate that porto-systemic shunts are effective to prevent gastro-intestinal bleeding, even in young children with cirrhosis. However, in addition to Child's criteria, indications for shunt surgery must take into account the degree of hemorrhage risk and the possible need for a liver transplantation later.
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PMID:[Results of portasystemic shunts in cirrhosis in children]. 387 28

The estimation of the intraoperative fluid requirements can be particularly difficult in patients with compartmental disturbances, e.g. hepatic cirrhosis. The case history of a 41 year old female with a known history of liver cirrhosis is reported who underwent emergency surgery due to upper gastro-intestinal bleeding. In order to maintain hemodynamic stability preoperative blood loss was replaced with fresh blood. During the operation 4 liters of bank and fresh blood with 5 liters of erythrocyte-free fluids were administered. After operation reliable clinical signs of hypovolemia and a marked increase in hematocrit were noted. The severe hemoconcentration was explained by excessive fluid shifts away from the intravascular compartment since a total amount of 10 liters of ascites was collected for the next 24 hours. This overproduction can be related to portal hypertension, hypoalbuminemia and activation of the renin-angiotensin-aldosterone system.
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PMID:[Hemoconcentration caused by excessive intraoperative ascites production in ulcer bleeding]. 684 55


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