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)

Twenty-six patients, mean age 20.5 years (range 11-33 years) at last assessment or death, attended an adult cystic fibrosis clinic between 1975 and 1983. Twenty-one presented in infancy, and 5 later (3-17 years). Most morbidity was due to recurrent respiratory infection and 5 of the 7 deaths were from respiratory failure. Cor pulmonale occurred in 4 patients, pneumothorax in 3 and severe haemoptysis necessitating lobectomy in 2. Declining spirometric values and persistent isolation of Pseudomonas aeruginosa from sputum samples were associated with a poor prognosis. Minor gastrointestinal symptoms were common (19 patients). Four patients developed intestinal obstruction. Six patients had abnormal liver function tests and one patient died from hepatic cirrhosis. Diabetes was diagnosed in 3 patients and 9 patients experienced joint pains. The prognosis and quality of life for patients with cystic fibrosis appear to be improving, and all but 2 of the patients attending the clinic are at school, university or are employed.
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PMID:Cystic fibrosis--a review of 26 adolescent and adult patients. 393 89

We have reported a case of acute oliguric renal failure with hyperkalemia in a patient with cirrhosis, ascites, and cor pulmonale after indomethacin therapy. Prompt restoration of renal function followed drug withdrawal, while re-exposure to a single dose of indomethacin caused recurrence of acute reversible oliguria. Our case supports the hypothesis that endogenous renal prostaglandins play a role in the maintenance of renal blood flow when circulating plasma volume is diminished. Since nonsteroidal anti-inflammatory agents interfere with this compensatory mechanism and may cause acute renal failure, they should be used with caution in such patients.
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PMID:Indomethacin-induced renal insufficiency: recurrence on rechallenge. 407 Nov 54

Abnormal lung perfusion scans using radioactive particles were found in five out of six cases of hepatic cirrhosis with arterial hypoxaemia. None had clinical evidence of cardiopulmonary disease or signs of pulmonary embolism on arteriography. The scan defects are probably caused by a disorder of the pulmonary microvasculature, which may show regional variation in severity.
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PMID:Lung perfusion scanning in hepatic cirrhosis. 464 96

74 patients with cystic fibrosis aged 1-19 years were assessed prospectively for 1-7 years for evidence of liver involvement. 20 of these patients were referred primarily because of hepatic problems. 3 of 4 with neonatal hepatitis recovered. Chronic active hepatitis developed in a further child but resolved spontaneously. 6 patients had abnormal liver-function tests without clinical evidence of liver disease. In 18 cirrhosis was detected at age 4-13 years. Liver disease was stable in these except terminally in 3 with cor pulmonale. The principal hepatic problem was variceal bleeding, which occurred in 6 patients. 50% of bleeds followed aspirin ingestion. This drug therefore should be avoided in such patients. 13 had hypersplenism. 2 had severe splenic pain necessitating splenectomy with lienorenal shunt, which was performed also in 2 patients who had bled. 3 remain well up to 5 years later. In 3 patients seen in the past 3 years injection sclerotherapy has controlled bleeding. This technique was well tolerated without the pain associated with, or intensive physiotherapy necessary after, shunt surgery; and this may be the method of choice for controlling variceal bleeding in cystic fibrosis.
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PMID:Hepatic complications of cystic fibrosis. 611 50

Cystic fibrosis is the most common fatal inherited disease of Caucasians. At present, cystic fibrosis accounts for most cases of chronic progressive pulmonary disease and for many other clinical features in the first three decades of life. Thus, it is a challenge to both pediatricians and internists, particularly chest physicians. The diagnosis is based on the triad of chronic obstructive pulmonary disease, pancreatic insufficiency, and increased levels of electrolytes in the sweat. The cardinal test for confirmation of the diagnosis is the "sweat test," which is an excellent discriminant for cystic fibrosis, even in adults. Ancillary features of cystic fibrosis may be of diagnostic assistance (eg, nasal polyposis, Pseudomonas aeruginosa in sputum, azoospermia, and others). Treatment of the pulmonary disease must be emphasized. Choice of antibiotics should be based on the results of sputum culture, but P aeruginosa is the most common pathogen. Removal of secretions by regular postural drainage and percussion is an integral part of the program. Pneumothorax, massive hemoptysis, cor pulmonale, and other complications may be encountered. Sinusitis is almost universal, and nasal polyposis is frequently present. Pancreatic insufficiency occurs in over 80 percent of the patients with cystic fibrosis and may result in intestinal malabsorption. Massive salt loss through the sweat in hot weather, a distinctive type of biliary cirrhosis without jaundice, gallbladder abnormalities, cholelithiasis, and diabetes mellitus also may be found. Of special importance are intestinal obstructive complications (meconium ileus in newborn infants with cystic fibrosis and intestinal obstruction due to fecal accumulation or intussusception in adults). Azoospermia is present in 95 percent of men and there is reduced fertility in women; however, pregnancy does occur in cystic fibrosis. This chronic and ultimately fatal disease produces a predictable set of psychosocial complications.
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PMID:Diagnosis and treatment of cystic fibrosis. An update. 637 70

Pulmonary function tests were studied in 105 patients with cirrhosis of the liver who had no clinical or radiographical evidence of cardiopulmonary disease. Spirometric data such as VC, FRC, RV, TLC, RV/TLC and FEV1.0% were within normal limits in all subjects. However, flow-volume curve and closing volume curve were abnormal in the majority. In flow-volume curve, V25, was decreased and the V50/V25 value was increased; on the other hand, closing volume was markedly increased and FRC-CC value was reduced in the patients with hepatic cirrhosis. The closing capacity tended to exceed the FRC in the patients with arterial hypoxemia, indicating the presence of airway closure and gas trapping during resting ventilation. Therefore, ventilation-perfusion imbalance may be an important cause of arterial hypoxemia in patients with cirrhosis of the liver.
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PMID:Arterial hypoxemia in patients with hepatic cirrhosis. 642 27

One hundred consecutive patients (predominantly men, aged 60 years and older), who were admitted to the Hsinhua Hospital, Shanghai, with acute bleeding from the gastrointestinal tract during a three-year period, were reviewed. The most common causes of bleeding were peptic ulcers, chronic gastritis, prolapse of gastric mucosa, and other benign conditions. In 22 patients, bleeding was either due to oesophageal varices, or was a complication of cor pulmonale. The incidence of malignant disease was 23% (higher than that reported from the United States or the United Kingdom). The mortality rate in patients with benign causes of gastrointestinal bleeding (after exclusion of patients with malignant disease, cor pulmonale, and hepatic cirrhosis) was 4.4%--much lower than the British rate, but slightly higher than that reported in an Australian study. It is hoped that, with the increasing interest in geriatrics, and closer attention to acute bleeding from the gastrointestinal tract in the aged, the mortality rate from this condition will decline.
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PMID:Acute upper gastrointestinal bleeding in the aged. A retrospective analysis of 100 cases. 660 Dec 27

Several features of Gaucher disease have been described related to an experience gained from a study of 275 patients. These include splenic nodules, a nodular form of cirrhosis affecting the liver of patients who have severe disease as well as unsuspected involvement of the brain. The anatomic distribution of Gaucher cells in the brain may be an age related phenomenon. Various types of involvement of the lungs can produce severe cyanosis or cor pulmonale according to the anatomic distribution of the Gaucher cells. The belief that bone disease can be produced by prior splenectomy was dispelled by a study of 239 patients with type I and type III disease. The reasons for the association of malignant tumors with older patients who have type I disease is not understood but should encourage careful follow-up of these patients. As we enter the second century of our knowledge of this disease we should keep Dr. Gaucher's talent of making careful clinicopathological observations in mind as we search for clues to the better understanding of the various clinical forms of the disease.
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PMID:The pathology of Gaucher disease. 712 34

The weights of the individual carotid bodies and cardiac ventricles were obtained at necropsy in five series of subjects. The first comprised 10 cases free of cardiopulmonary disease to act as controls. The second consisted of 10 cases of pulmonary emphysema. The third was composed of 8 cases characterized by sustained alveolar hypoxia due to causes other than pulmonary emphysema. The fourth comprised 10 cases of systemic hypertension or severe left ventricular failure. The fifth was made up of 10 cases of diseases of the liver or alimentary canal. The study confirmed that enlargement of the carotid bodies is common in cases of pulmonary emphysema or sustained alveolar hypoxia with right ventricular hypertrophy. It is also common in cases of systemic hypertension with left ventricular hypertrophy. It was also revealed that enlargement of the carotid bodies may occur in cirrhosis of the liver. We believe this to be the first report of that association.
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PMID:The carotid bodies enlarge in some cases of cirrhosis of the liver. 820 96

Laparoscopic cholecystectomy may be performed safely in most patients with symptomatic cholelithiasis. There are few absolute contraindications to laparoscopic cholecystectomy. Many relative contraindications exist, which relate to the surgeon's experience and the ability of the operating team to manage potential complications. Preoperative evaluation should assess the potential nonbiliary problems that affect the performance of laparoscopic cholecystectomy, including severe cardiopulmonary disease, coagulopathy, cirrhosis, and pregnancy. Since most therapeutic laparoscopic procedures are currently performed with a carbon dioxide (CO2) pneumoperitoneum, the physiologic effects of the elevated abdominal pressure and absorbed CO2 must be understood by the surgeon. Specific nonbiliary problems addressed in this review are cardiopulmonary disease, hypercortisolism, cirrhosis and portal hypertension, morbid obesity, previous abdominal surgery, and pregnancy.
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PMID:Effect of nonbiliary problems on laparoscopic cholecystectomy. 848 Aug 95


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