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)

Cystic fibrosis (CF) is an autosomal recessive disorder that is characterized by an abnormality of exocrine gland function. Adult patients represent a rapidly growing percentage of the CF population. Pulmonary changes are seen in nearly every case and are the most serious complication of CF. In advanced lung disease, bronchiectasis, emphysematous bullae, and subpleural blebs can frequently develop. Although pulmonary disease is the most common cause of death and morbidity among CF patients, there also can be involvement of other groups, particularly in adults, which show characteristic signs on CT and spiral CT. Pancreatic abnormalities are present in 85-90% of CF patients. The degree of pancreatic involvement varies, ranging form accumulations of mucus in the small ducts to totally plugged ducts, which can cause atrophy of the exocrine glands and progressive fibrosis. Pancreatic dysfunction on CT is demonstrated as fatty replacement and fibrosis of the pancreas. However, there may be scattered foci of pancreatic calcifications that can be detectable on plain radiographs. Hepatobiliary involvement follows the same pattern as pancreatic abnormalities. Bile canaliculi are plugged by mucinous material and when this plugging is of long duration, biliary cirrhosis with diffuse hepatic nodularity may develop. Such severe hepatic involvement is see in only about 2-5% of patients, although minor hepatic alterations, such as diffuse fatty changes, are fairly common. Hepatobiliary involvement is characterized by hepatic nodularity, compatible with cirrhosis, splenomegaly, and ascites. Complete obstruction of the ileum by meconium occurs in approximately 10% of newborns with CF. Intestinal findings on CT include obstruction, although this is more common in children. These CT signs should be evaluated carefully in adult patients since they may be suggestive of CF. Computed tomography offers unique information about organ involvement (other than pulmonary) that can alter diagnosis and patient management.
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PMID:Cystic fibrosis: spectrum of thoracic and abdominal CT findings in the adult patient. 975 98

The clinical histories of 46 adult patients (24 men and 22 women, mean age 20.6 +/- 5.1 years) diagnosed of cystic fibrosis were reviewed evaluating the digestive alterations. The age at diagnosis of cystic fibrosis was 5.63 +/- 5.3 years (range: newborns-19 years). The initial diagnosis was established by ileus meconium, in four, lung disease in 15, steatorrhea in 12, lung disease and steatorrhea in 13 and following the diagnosis of cystic fibrosis in siblings in two. Four patients presented ileus meconium, nine occlusive syndrome of the distal intestine, 42 steatorrhea (20 severe, 12 moderate and 10 mild), with the severity of the steatorrhea not being associated with the severity of the respiratory insufficiency. Two patients presents rectal prolapse, five gastroesophageal reflux syndrome (four with hiatal hernia), six cholelithiasis, one recurrent pancreatitis without detection of biliary lithiasis, one neonatal cholestasis and 10 malnutrition (five severe and five moderate) fundamentally in relation to the severity of the lung disease and, to a lesser degree, liver disease. In 10 patients chronic liver disease was diagnosed corresponding to established cirrhosis in seven, indicating liver transplantation in two. In most cases, the liver disease was already manifest in adolescence even in the cirrhotic stage. Cholangiography by magnetic resonance was useful in the study of liver disease showing abnormalities which imitated primary sclerosing cholangitis. Treatment with ursodesoxicholic acid at a dosis of 20 mg/kg/day led to a significant decrease in the transaminase values and overall of gammaglutamyltranspeptidase but did not avoid complications in the cirrhotic stages. Genetic studies performed in 36 patients detected the delta F508 mutation in 69.4%, being found in almost all of the patients with ileus meconium, occlusive syndrome of the distal intestine, liver disease, cholelithiasis and malnutrition.
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PMID:[Digestive alterations in cystic fibrosis. Retrospective study of a series of 46 adult patients]. 1019 90

The hepatopulmonary syndrome is defined as the triad of liver disease, hypoxaemia and intrapulmonary vascular dilatation. This syndrome has been described in patients with liver cirrhosis, noncirrhotic portal hypertension, and fulminant hepatic failure, however, there are no previous descriptions of hepatopulmonary syndrome in patients with acute nonfulminant viral hepatitis. We report a 47-year-old, previously healthy man that presented with acute hepatitis A, and developed progressive dyspnoea, platypnoea and orthodeoxia with no evidence of parenchymal or thromboembolic lung disease. PaO2 on room air was 58 mmHg, O2 saturation was 88% and alveolar-arterial O2 gradient was 62%. During his hospitalization serum albumin level decreased to 3.1 g/dl and prothrombin time was prolonged to 16.8 s, however, he remained alert with no signs of hepatic encephalopathy. Contrast echocardiography revealed left heart chamber opacification 3-4 cardiac cycles after the opacification of the right heart chamber, consistent with hepatopulmonary syndrome. During the following days there was a gradual improvement in the patient's condition, with resolution of his dyspnoea and gradual increase of PaO2. Repeat contrast echocardiography and PaO2 determinations, 3 weeks later, were normal. On long-term follow-up the patient remained asymptomatic with normal liver function tests and normal O2 saturation. This report indicates that hepatopulmonary syndrome may be a transient manifestation of acute hepatitis A in the absence of fulminant liver failure.
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PMID:Transient hepatopulmonary syndrome in a patient with acute hepatitis A. 1115 56

A 27-year-old Indian woman at 23 weeks' gestation presented with decompensated liver cirrhosis, coagulopathy, restrictive lung disease with cor pulmonale and preeclampsia. She was diagnosed to have sea-blue histiocyte syndrome (SBHS) at the age of 13 years and was treated conservatively. There was worsening liver, respiratory and bone marrow function as the pregnancy progressed. She underwent a successful pregnancy despite her poor medical condition and advanced disease state. We described the first case of familial SBHS in a pregnant patient from Asia.
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PMID:Successful pregnancy in a patient with familial sea-blue histiocyte syndrome. 1122 Nov 66

Palliative care in the elderly appropriately takes place within a spectrum of curative, rehabilitative, preventive, and chronic disease management interventions, and seeks to optimize quality of life. Setting priorities among numerous legitimate treatment goals is the central task in the care of chronically ill frail individuals. Decision-making can be challenging when the goal of providing comfort comes into conflict with the goal of prolonging life, and should be guided whenever possible by consistently expressed preferences of the patient. The assessment and relief of distressing physical and psychological symptoms should receive active attention at all stages in the care of the frail elderly, both in the context of acute medical and surgical interventions and during the terminal phase of life. Pain and dyspnea are frequently reported by significant proportions of elderly individuals hospitalized for chronic lung disease, heart failure, and cirrhosis as well as for malignancies. In the treatment of dementia, the types of interventions that improve quality of life will differ in the early and late phases of the illness.
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PMID:[Role of palliation in the care of the elderly]. 1150 13

Invasive carcinoma originates from the epithelial cells lining the lumen of an organ. It is often preceded by metaplasia, dysplasia or carcinoma in situ. The purpose of this review is to suggest that this disease of the epithelium may be, in part, the result of underlying tissue-based disorganization. Human cancer is frequently associated with pre-existing tissue disease. For example, hepatocellular carcinoma usually occurs in patients with a macronodular cirrhotic liver. Most lung cancers arise among patients with chronic lung disease (bronchitis, emphysema, and chronic infection). Mechanical forces appear to play a major role in regulating normal and cancer cell growth. The loss of cell polarity by neoplastic cells, coupled to an otherwise normal growth rate is enough to explain the cancer star-shaped pattern. By changing the plane of cell division, tumor cells may escape physical constraints from surrounding cells and divide. Loss of cell polarity and the resulting cell proliferation appears to be a consequence of either tissue-based disorganization (chronic inflammation, fibrosis) or of direct carcinogenic insult. The multiple mutations frequently described in cancer may be, in part, secondary to physical stress and not primary events. Several animal and clinical trials have shown that tissue disruption (i.e. radiation-induced fibrosis or liver cirrhosis) can be successfully treated. It is possible that treatment targeted at tissue disruption would delay or reduce cancer incidence regardless of the precise biological mechanism of carcinogenesis.
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PMID:Cancer: the role of extracellular disease. 1202 30

Accumulated experimental and clinical data suggest that adrenocorticosteroids and/or endogenous ouabain-like substances may play an important role in the mechanism of furosemide diuretic action. It was reported that the drug is highly bound in the adrenals, lungs, kidney, spleen, and liver. In patients with liver cirrhosis, furosemide exerted a markedly decreased natriuretic effect compared with normal subjects, and the plasma levels of circulating endothelin and atrial natriuretic factor (ANF) were significantly elevated. In neonates, after administration of furosemide, the urinary excretion of endothelin-1 and aldosterone increased markedly, and it is known that endothelin may release ANF and aldosterone in a dose-dependent manner. Furosemide was used to stimulate zona glomerulosa, whereas ANF decreased the production of steroids in zona glomerulosa and fasciculata cell culture owing to stimulation by various factors. Because the concomitant use of ANF and furosemide appeared to be diuretically effective in newborns after cardiac surgery, one may suggest that furosemide competes with ANF for its effects on the adrenals. Furosemide administered by inhalation exerted a protective effect on allergic and perennial nonallergic rhinitis and was effective in preventing the postsurgical recurrence of nasal polyposis. The drug can also be used as an antiasthmatic agent. In preterm ventilator-dependent infants with chronic lung disease, aerosolized furosemide improved pulmonary function with no marked effect on diuresis. In adults and children with asthma, furosemide exerted a protective effect against bronchoconstriction induced by several indirect stimuli similar to that of disodium cromoglycate or nedocromil. Aerosolized furosemide had a preventive effect also on bronchoconstriction induced by inhaled lysine acetylsalicylate in patients with aspirin-sensitive asthma. In high-dose beclomethasone-dependent asthma, inhaled lysine acetylsalicylate and furosemide exerted a mutually potentiating antiasthmatic activity, allowing considerable sparing of the inhaled steroid. It is proposed that this effect may be explained by the corticosteroid-sparing action of lysine released from the lysine acetylsalicylate molecule because similar beneficial effects were also obtained after the concomitant use of epsilon-aminocaproic acid (whose chemical structure is almost the same as that of lysine) and prednisone. Furosemide exhibited an anti-inflammatory effect through inhibition of production and release of cytokines interleukin (IL)-6, IL-8, and tumor necrosis factor-alpha from peripheral mononuclear cells, which may have a beneficial effect on local inflamed tissue imbalance in the ratio of different cytokines, thus improving the sensitivity of target cells to endogenous glucocorticosteroids.
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PMID:Furosemide: progress in understanding its diuretic, anti-inflammatory, and bronchodilating mechanism of action, and use in the treatment of respiratory tract diseases. 1211 21

Most cases of infections described after steroid treatment for severe acute alcoholic hepatitis are of bacterial origin. However, the rate of bacterial infections in these patients is not higher than in those who are not treated by steroids. The opportunistic infections are even more rare. We report two cases of patients with cirrhosis and human immunodeficiency virus, treated for alcoholic hepatitis with steroids and who subsequently developed severe pneumopathy due to Pneumocystis carinii. One patient had a concommitant cytomegalovirus infection and both of them died. Pneumocystis carinii infections usually occur in patients a decreased immune cellular response. Steroid treatments and also alcohol may be responsible for these opportunistic infections. Alcohol may have an immunosuppressive effect by decreasing recruitment of CD4 and CD8 lymphocytes to the lungs. In conclusion, Pneumocystis carinii pneumonia is a potential complication of steroid treatments for acute alcoholic hepatitis and should be suspected in case of unexplained pulmonary infection.
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PMID:[Pneumocystis carinii and cytomegalovirus pneumonia after corticosteroid therapy in acute severe alcoholic hepatitis: 2 case reports]. 1212 70

Chronic lung disease in humans is frequently complicated by the development of secondary pulmonary hypertension, which is associated with increased morbidity and mortality. Hypoxia, inflammation and increased shear stress are the primary stimuli although the exact pathways through which these initiating events lead to pulmonary hypertension remain to be completely elucidated. The increase in pulmonary vascular resistance is attributed, in part, to remodelling of the walls of resistance vessels. This consists of intimal, medial and adventitial hypertrophy, which can lead to encroachment into and reduction of the vascular lumen. In addition, it has been reported that there is a reduction in the number of blood vessels in the hypertensive lung, which could also contribute to increased vascular resistance. The pulmonary endothelium plays a key role in mediating and modulating these changes. These structural alterations in the pulmonary vasculature contrast sharply with the responses of the systemic vasculature to the same stimuli. In systemic organs, both hypoxia and inflammation cause angiogenesis. Furthermore, remodelling of the walls of resistance vessels is not observed in these conditions. Thus it has been generally stated that, in the adult pulmonary circulation, angiogenesis does not occur. Prompted by previous observations that chronic airway inflammation can lead to pulmonary vascular remodelling without hypertension, we have recently shown, using quantitative stereological techniques, that angiogenesis can occur in the adult pulmonary circulation. Pulmonary angiogenesis has also been reported in some other conditions including post-pneumonectomy lung growth, metastatic disease of the lung and in biliary cirrhosis. Such angiogenesis may serve to prevent or attenuate increased vascular resistance in lung disease. In view of these more recent data, the role of structural alterations in the pulmonary vasculature in the development of pulmonary hypertension should be carefully reconsidered.
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PMID:The structural basis of pulmonary hypertension in chronic lung disease: remodelling, rarefaction or angiogenesis? 1243 Sep 58

Various types of non-tuberculous mycobacteria can be the aetiologic factors of chronic lung infections especially in patients with underlying chronic lung diseases. The aim of this study is to present the cases of pulmonary mycobacterioses observed in Institute of Tuberculosis and Lung Diseases in the years 1995-2001. There were 23 patients, 12 men and 11 women in the age between 35-77 years, mean 56 years. 16 out of 23 patients had underlying respiratory problems, mainly healed tuberculosis (7) and COPD (6). Two additional patients suffered from other diseases with potential immunosuppression (leukopenia). In 5 patients no disease other than mycobacteriosis was found, but they were chronic smokers. In 19 cases cough and expectoration of purulent sputum lasting from several months to several years was observed. In 5 patients onset of disease was acute or subacute with high fever. Eight patients had haemoptysis. In chest X-ray pathological lesions including (18 cases) lung cirrhosis (10) and cavities (15) were found. In 4 cases disseminated bronchiectases with small nodules were the main radiologic feature. Mycobacteriosis was caused by M. kansasii in 11 cases, by M. intracellularae in 6, by M. xenopi in 5 and by M. scrofulaceum in 1 case.
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PMID:[Pulmonary mycobacterioses--frequency of occurrence, clinical spectrum and predisposing factors]. 1288 64


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