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)

Clinical data from 246 adult Swedish individuals with severe alpha1-antitrypsin deficiency, Pi Z, diagnosed in 1963--77, were analyzed. Primary emphysema was present in 109 cases. Of 75 Pi Z patients with other types of chronic obstructive pulmonary disease (COPD), all but 7 showed signs of emphysema. Median age at onset of dyspnoea in Pi Z smokers was 40 years, compared to 53 in non-smokers (p less than 0.001). Of the Pi Z individuals over the age of 50, 19% had a diagnosis of liver cirrhosis and 15% signs of glomerular renal damage. Of 91 deceased patients, 56 died from COPD and 12 from liver disease. A greatly reduced survival was demonstrated in Pi Z individuals, regardless of sex. Smoking Pi Z individuals had a significantly lower life expectancy than Pi Z non-smokers (p less than 0.01).
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PMID:Natural history and life expectancy in severe alpha1-antitrypsin deficiency, Pi Z. 30 8

The disease course is described in 21 patients with low serum concentrations of alpha1-antitrypsin of the phenotype Z (genotype pi ZZ). 13 of these patients have long-standing disease characterized by bronchitis or dyspnea beginning before the age of 40 and progressing to emphysema (11 patients) and to corpulmonale (7 patients). The remaining 8 patients are children with hepatopathy characterized by prolonged jaundice at birth, persistent hepatomegaly and persistently elevated liver enzymes. In 2 children, the evolution to cirrhosis was ascertained by biopsy.
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PMID:[Severe alpha 1-antitrypsin deficiency: clinical observations of 21 patients]. 31 May 77

This is the definitive review of the cardiovascular effects of liver disease. Physiologic and pathologic studies accomplished primarily in the 1950s and 1960s have been applied to a case of a 16-year-old boy with cirrhosis of the liver who presented with weakness, dyspnea, cyanosis, and clubbing. The probable mechanisms for his signs and symptoms are discussed in detail. The cause for the hyperkinetic circulation is unknown. By exclusion, the cyanosis is attributed to intrapulmonary shunting. Portapulmonary shunts are not quantitatively important. The suitability of the terms micronodular and macronodular cirrhosis is highlighted. Indications for various types of surgical portal shunts are discussed. The value of preoperative hemodynamic measurements of the portal circulation to the individual patient is debated. A liver transplant is considered in this case with no promising medical therapy and a very poor prognosis. The renal disease manifested by red cell casts in the urine is thought to be caused by an immunologic reaction in the kidney somehow related to his liver disease. This discussion is led by Dr. Jack Myers, but his opinions are correlated with those of a pathologist, radiologist, surgeon, and gastroenterologist. It is a clinical tour de force, although not all the predictions are confirmed by laboratory studies such as this patient's hepatic wedge pressure, pulmonary artery pressure, and cardiac fluoroscopy.
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PMID:Multidisciplinary Conferences in Gastroenterology. Cardiovascular effects of severe liver disease. 127 11

We reported a successful pleuro-venous shunt operation (Pl-V shunt) for a patient of liver cirrhosis with secondary hydrothorax. A 78-year-old female was admitted to our hospital because of severe dyspnea and palpitation. Chest x-ray film revealed right sided massive pleural effusion. Over several weeks the chest tube drainaged about 1,500 ml of transudative fluid per day. We performed Pl-V shunting and pleural effusion subsequently decreased in amount and dyspnea disappeared. This Pl-V shunting is thought to be useful for such a patient with massive pleural effusion which failed to respond to medical therapy.
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PMID:[A case of hepatic hydrothorax treated by pleuro-venous shunt]. 133 25

The earliest written report of selenium poisoning is thought to be the description by Marco Polo of a necrotic hoof disease of horses that occurred in China in 13. century. However recognition of Se as toxic principle come in the early 1930s. Severity of Se poisoning depends on chemical forms of the element, species of animals and routes of administration. The soluble Se salts (Na2SeO3 and Na2SeO4) appear to be among the more toxic compounds; the Se inherent in grains and selenoamino acids (selenomethionine and selenocystine) appear to have relative moderate toxicity; the poorly soluble forms (e.g., elemental Se, Na2Se, SeS2 and diphenyl selenide) are among the least toxic of the Se compounds. In general, toxicity of Se compounds are substantially less when they are administered orally than when they are given parenterally. Rosenfeld and Beath described three clinical types of Se intoxication: acute selenosis, subacute selenosis (i.e., blind staggers type), and chronic selenosis (i.e., alkali disease type). Acute poisoning occurs when high Se content plants are consumed in large quantities within short period. Accidental acute poisoning occurs as consequence of errors in formulation of a Se supplemented diet. The most characteristic sign of acute selenosis is garlic breath due to the pulmonary excretion of volatile Se metabolites. Other signs include lethargy, excessive salivation, vomiting, dyspnea, muscle tremors and respiratory distress. Pathological findings are: congestion of the liver and kidney, fatty degeneration and focal necrosis of the liver, endocarditis and myocarditis. Subacute selenosis ("blind staggers") occurs as a consequence of exposure to large doses of Se over a longer period of time and manifests with neurological signs (e.g., blindness, ataxia, disorientation) and respiratory distress. This form of selenosis is most frequently observed in grazing animals that have consumed Se-accumulated plants. Chronic selenosis ("alkali disease") comes about when animals consume moderate levels of Se (more than 5 mg/kg and less than 40 mg/kg) for period of weeks or months. The usual clinical signs of chronic selenosis in horses, cattle and swine are: loss of hair (horses and cattle lose long hair from the mane and tails), emaciation, hoof lesions and lameness. In advanced cases liver cirrhosis, atrophy of the heart and anemia occur. In swine symmetrical poliomyclomalacia of cervical and lumbal/sacral spinal cord segment has been seen. Sheep seen to be more tolerant and get milder form of the disease. They lose appetite and have reduced gain. In growing chicks reduced gain and feed intake, rough feathers, and characteristics of nervousness has been observed. Reduced egg production, embryonic deformations and reduced hatchability has been observed in hens.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Selenium toxicity in domestic animals]. 134 Apr 80

A 57-year-old man had suffered from poorly controlled diabetes mellitus and liver cirrhosis due to alcohol and hepatitis C for about 10 years. He developed fever and swelling of the right cheek and neck due to periodontal infection. The symptoms worsened in spite of antibiotic therapy and were accompanied by dyspnea. He was therefore referred to our hospital. Chest radiographs and computed tomographs revealed widening of the superior mediastinum, pulmonary infiltrates and right pleural effusion. He was diagnosed as having mediastinitis, right pyothorax and pneumonia caused by periodontal infection. Tracheotomy and mechanical ventilation were performed. Antibiotic therapy resulted in improvement of the mediastinitis and pyothorax. However, renal and liver dysfunction developed and the patient died of multiorgan failure after 35 days of hospitalization. Death due to periodontal infection is rare. We give a review of the literature.
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PMID:[A fatal case of acute mediastinitis caused by periodontal infection]. 146 87

We admitted a 60 year old patient with severe right hydrothorax. He was known to suffer from decompensated non-alcoholic cirrhosis. His invaliding dyspnea was only temporarily improved by repeated thoracocentesis, due to the very rapid refilling of the thorax from the ascites; until the development of a "tension hydrothorax" with dangerous mediastinal deviation. By insertion a peritoneo-venous shunt described by LeVeen the clinical picture was improved, and the patient no longe required further pleural aspiration. Six per cent of cases of ascites complicating hepatic cirrhosis may be associated with hydrothorax of the same origin. The diaphragm is normally perforated by a multitude of tiny virtual holes, closed by the peritoneum and the thoracic pleura. The high intra-abdominal pressure in ascites tears these fragile membranes, and allows the peritoneal fluid to overflow into the pleural cavity. Usually treated by fluid restriction and diuretics, when this condition becomes intractable or when a tension hydrothorax appears, interfering with respiratory function a LeVeen peritoneo-venous shunt should be inserted.
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PMID:[Hydrothorax of hepatic origin. Description of a clinical case, pathophysiology]. 160 61

In a prospective study 53 patients with alcohol-induced liver disease (fatty liver in 27, cirrhosis in 26) were studied clinically and with non-invasive techniques (electrocardiogram, systolic time intervals, M-mode echocardiography, upright bicycle stress test) to detect a possible cardiac involvement. Mean daily alcohol consumption was comparable in both groups (136 g/day over 16 years vs 124 g/day over 14 years). 15 to 41% of patients (more patients with fatty liver) complaint of angina pectoris and dyspnea at exercise or had palpitations. Echocardiography and systolic time intervals demonstrated in both groups (in patients with cirrhosis despite of a more intensive therapy with digitalis and diuretics) a marked enlargement of left ventricular dimensions with a significant (p less than 0.05) degree of dysfunction (PEP/LVET). Electrocardiography showed abnormalities in 26 to 44% of patients: signs of right ventricular enlargement in 26% of patients with fatty liver, and a prolongation of myocardial repolarisation (QTc) in 44% of patients with cirrhosis. Patients with alcohol-induced liver disease deserve more attention of their cardiac complaints, clinical and functional findings.
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PMID:[Cardiac findings in alcoholic liver disease]. 226 11

Pulmonary perfusion imaging with Tc-99m MAA revealed significant uptake in the lungs, brain, spleen, and both kidneys of a 48-year-old woman with liver cirrhosis and pulmonary telangiectasia associated with marked hypoxemia and cyanosis. Dynamic pulmonary perfusion imaging revealed a gradual reduction after peak uptake in both lungs. Several weeks after albumin replacement, the hypoxia and dyspnea disappeared with no change in hepatocellular function. At that time, dynamic pulmonary perfusion imaging revealed a plateau-like time-activity curve of uptake in the lungs, as compared with the findings obtained during the state of severe hypoxemia. These observations suggest that pulmonary telangiectasia in a patient with liver cirrhosis may be due to functional vasodilatation. Serial dynamic pulmonary perfusion imaging indicates the passage of the MAA particles through the widened lumen of the pulmonary alveolar capillaries.
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PMID:Reversibility of pulmonary telangiectasia in liver cirrhosis evidenced by serial dynamic pulmonary perfusion imaging. 260 48

A 54-year-old man, who had the history of a blood transfusion 29 years ago, was admitted to our hospital because of dyspnea and abdominal fullness. Physical examination revealed jaundice and massive ascites and laboratory data suggested liver cirrhosis. The high level of AFP and a CT scan indicated the association of hepatocellular carcinoma and its metastasis to the right adrenal gland. On the 21st hospital day, he suddenly complained of severe pain in the right upper quadrant and the right flank, and fell into hemorrhagic shock. Blood transfusion was given, but he died on the 24th hospital day. Autopsy revealed liver cirrhosis, accompanied by hepatocellular carcinoma with the metastasis to the right adrenal gland and multiple pulmonary tumor thrombi. Massive hemorrhaging due to rupture of the right adrenal metastasis was seen in the retroperitoneal space.
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PMID:[A case of hepatocellular carcinoma associated with multiple pulmonary tumor thrombi and rupture of its right adrenal metastasis]. 303 33


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