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)

Focal biliary cirrhosis is an uncommon finding in infants with cystic fibrosis, but it is present in more than a fifth of surviving children and adolescents. It was found at postmortem examination in only five of 47 infants with CF younger than 3 months, in five of 32 infants from 3 to 12 months, and in 18 of 67 children older than 1 year. In infants under 3 months, excessive mucus in intrahepatic bile ducts was seen in 11 necropsies; in 15 others there were only nonspecific periportal changes. Cholestasis was found in the livers of 18 of the 26 infants. Excessive mucus in the biliary tree was occasionally associated with periportal changes and cholestasis in older infants. The periportal changes, which are regarded as nonspecific, were never found in infants more than 1 year of age.
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PMID:Hepatic changes in young infants with cystic fibrosis: possible relation to focal biliary cirrhosis. 113 49

In 36 children with cystic fibrosis (CF) the isoenzymes of alkaline phosphatase (AP) were determined microelectrophoretically in polyacrylamide- and starch-gel. The study was done to evaluate the clinical significance of these additional data for the diagnosis of liver involvement in DF. The results led to the following conclusions: 1. Serum activity of total AP is comparatively unsensitive "masking" alterations in the isoenzyme pattern contributing to the AP serum activity. 2. In 17 children resp. 47% bile-duct phosphatase was increased indicating a secretostasis while other marker enzymes of cholestasis were normal in part. 3. The activity of bone phosphatase in the serum showed a significant correlation to the degree of growth retardation in these patients. 4. Intestinal phosphatase was present in the serum of only one child with cirrhosis of the liver being an indicator for liver insufficiency. 5. Determination of AP isoenzymes in the serum may provide additional information about the organs involved for the physician in handling CF patients.
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PMID:Isoenzymes of alkaline phosphatase in the serum of patients with cystic fibrosis. 113 40

The biliary tree of 66 patients with cystic fibrosis was examined by conventional roentgenographic methods. Forty-five per cent of the oral cholecystograms were judged abnormal by our criteria. A study was considered abnormal if there was no visualization or if there was visualization of a microgallbladder or structural abnormality including marginal irregularities, septate gallbladder or cholelithiasis. Intravenous cholangiography was used to further study the 22 patients who did not visualize on the oral study. Again, anatomic abnormalities were prevalent but six patients in this group had normal appearing gallbladders. Abdominal pain, a frequent symptom in cystic fibrosis, was not associated with roentgenographic abnormality. No correlation was seen between the external biliary tree abnormalities and multifocal biliary cirrhosis which was present in 40% of these patients. Further, no correlation was seen between serum gamma glutamyl transpeptidase levels and either of these lesions.
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PMID:Clinical observations on the biliary system in cystic fibrosis. 127 40

In an attempt to produce an animal model for the disease cystic fibrosis (CF), mice were treated chronically with the diuretics amiloride and furosemide, in order to cause chronic inhibition of transepithelial ion transport. Experiments were carried out on adult mice (2 months treatment); in addition, pregnant mice were treated with diuretics, and tissue from offspring 2 and 7 days post partum was investigated. Since biliary cirrhosis is a common occurrence in CF, hepatocytes in the treated mice were investigated by X-ray microanalysis and by light and electron microscopy. Treatment with amiloride caused a significant decrease in cellular Na concentration in adult animals and in in utero treated mice 2 days after birth. The decrease in Na was paralleled by a decrease in Cl, but K levels were not affected. Furosemide caused a slight increase of cellular Na concentrations, especially in animals aged 7 days. In the adult animals, both amiloride and furosemide caused a significant decrease of the cellular Na and Cl levels. No signs of cirrhosis could be observed. Inconsistent changes in the accumulation of lipid droplets in hepatocytes of adult animals treated with amiloride were observed by electron microscopy. It can be concluded that chronic treatment with diuretics, even though it causes some, possibly pathological, changes of the liver, is only of very limited value for generating an animal model to study liver disease in CF.
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PMID:Effect of chronic treatment with diuretics on mouse liver: a morphological and microanalytical investigation. 129 84

Increased portal pressure is the product of both increased resistance to splanchnic flow through the liver and increased blood flow in the portal circuit. Although portal hypertension in children is less common than in adults, the important clinical end results are the same, ie, esophageal variceal hemorrhage, ascites, and hypersplenism. The etiology of portal hypertension in children is very different from adults in whom cirrhosis (most commonly secondary to alcohol) is the predominant cause. In children, extrahepatic obstruction due to portal vein thrombosis is the most common cause. However, as children survive longer with biliary atresia, cystic fibrosis, and other liver diseases, the incidence of intrahepatic obstruction causing portal hypertension is increasing. The treatment has also undergone a dramatic evolution over the last decade with the near extinction of portosystemic shunt procedures and their replacement with endoscopic treatment of esophageal varices and liver transplantation.
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PMID:Portal hypertension. 134 80

Ursodeoxycholic acid (UDCA) allows symptomatic treatment of cholestatic liver diseases such as primary biliary cirrhosis, primary sclerosing cholangitis, intrahepatic biliary atresia, and cholestasis of cystic fibrosis. Patients should be treated at an early stage of the disease in order to prevent progression to cirrhosis. Since UDCA has no toxic effects longterm treatment with this substance is possible without the risk of undesired side effects. In patients with primary biliary cirrhosis and rapid progression of the disease, UDCA may be combined with an immunosuppressive substance (i.e. cyclosporin). In primary sclerosing cholangitis, biliary atresia and cholestasis of cystic fibrosis, UDCA at present seems the only treatment of which a benefit for the patients can be expected. In endstage disease liver transplantation is indicated. The role of UDCA in chronic hepatitis and alcohol induced liver disease needs to be clarified in further studies. Whether the improvement of laboratory tests in such patients indicates amelioration of the course of disease, still is unclear.
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PMID:[Treatment of cholestatic liver diseases; the role of ursodeoxycholic acid]. 144 78

There have been striking advances in the management of complications of cirrhosis in the 50 years since biliary cirrhosis was recognised as a feature occurring in a minority of patients with cystic fibrosis. Many questions remain unanswered with regard to its pathogenesis, its effect on other systems, morbidity and mortality and to its optimum management. Factors which may initiate liver disease and/or cause progressive biliary fibrosis leading to biliary cirrhosis in some cystic fibrosis patients are being defined but much is still poorly understood and further research is required. Clinical and pathological studies confirm that cirrhosis is frequently asymptomatic and only slowly progressive, with the prevalence rising with age. In a recent large epidemiological study, however, it was found that the age related prevalence fell in patients over the age of 20 years although deaths from liver disease were rare. Does the presence of liver disease cause premature respiratory death in teenagers? Current treatments control variceal bleeding, the important sequel for most patients with biliary cirrhosis, with less morbidity and mortality than in the past but require reappraisal as newer techniques become available. Endoscopic radiologically controlled methods are emerging as an important adjunct to the surgical control of biliary complications which cause symptoms in up to 4%. Ursodeoxycholic acid improves liver function tests but its effect on hepatic fibrosis and portal hypertension will only be demonstrated by large scale prospective controlled trials. Should liver transplantation have a larger role in management? In this chapter we have attempted to summarise the current state of knowledge and to assess the efficacy of present management.
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PMID:Liver and biliary problems in cystic fibrosis. 145 6

Liver disease associated with cystic fibrosis (CF) is considered a secondary effect of the basic defect of the disease, leading to obstruction of bile ductules by abnormal mucoid secretions; additional factors have been involved in the pathogenesis, such as abnormalities in bile acid metabolism, nutritional deficiencies, drug hepatotoxicity, stenosis of the common bile duct by the fibrotic pancreas. Clinical presentation of liver disease in CF is rare during the first few years of life, although neonatal cholestasis can be occasionally the first manifestation of the disease. Isolated massive steatosis has been reported in less than 5% of cases as a consequence of malnutrition. Focal biliary cirrhosis is the pathognomonic hepatic lesion and is present in 25-30% of CF patients, most of whom are asymptomatic. The focally distributed lesions can extend leading to multi-lobular biliary cirrhosis with occurrence of signs and symptoms of cirrhosis and portal hypertension. Early diagnosis of CF-associated liver disease is difficult since liver function tests may be normal even in cases of overt cirrhosis: no test has proved to be sufficiently sensitive and specific and even liver biopsy is of questionable relevance due to the focal distribution of hepatic lesions. Clinical examination is of major importance, since the presence of hepatomegaly seems to correlate well with the histologic finding of fibrosis. The rationale for the use of the choleretic non-toxic bile acid ursodeoxycholic acid in CF-associated liver disease is to reduce the viscosity of bile and to replace toxic bile acids which accumulate in the hepatocyte.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Liver disease in cystic fibrosis. 147 Feb 80

We retrospectively studied the clinical use of MR angiography, a gradient-echo technique susceptible to motion, in the preoperative evaluation of the hepatic vasculature in children with liver disease. Twenty imaging examinations were performed in 18 patients 3 months to 10 years old (mean, 2.3 years). Eleven patients had liver tumors and seven had cirrhosis associated with biliary atresia, cystic fibrosis, and short-bowel syndrome. Respiratory motion artifacts were present in most patients, but all MR angiographic studies were technically adequate. Comparing MR angiograms with spin-echo images, we found that vascular visualization was better with MR angiography in 12 cases (60%), equal in five (25%), and worse in three (15%). In 10 of 20 cases, MR angiography provided additional vascular information not present on spin-echo images. MR angiography is a useful adjunct to spin-echo imaging for assessing hepatic vascular anatomy in children with liver disease.
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PMID:Diagnosis of liver disease in children: value of MR angiography. 150 37

Cystic fibrosis (CF) is the commonest, fatal, autosomal recessive disorder and is associated with lung sepsis, pancreatic failure and elevated sweat electrolytes. The CF gene on chromosome 7 encodes a protein identified as CF transmembrane conductance regulator (CFTR) which regulates chloride ion transport in epithelial cell membranes. Almost 100 mutations have been identified in this gene which cause defective chloride-channel control. Recently, this abnormality has been reversed in affected CF cells in vitro by retrovirus-mediated transfer of a normal gene. Fifty years ago, most cases died in childhood, but now up to 80% reach adulthood. Chronic lung sepsis is the principal cause of death, and intensive antibiotic therapy with chest physiotherapy is used to control this. Advanced lung disease can be successfully treated by heart-lung transplantation. Nebulised recombinant DNase and antineutrophil elastase agents such as alpha-1-antitrypsin and secretory leucoprotease inhibitor are potentially promising new therapies. Pancreatic insufficiency is managed by high-calorie diets and enteric coated enzyme supplements. Other prominent gastrointestinal complications include meconium ileus equivalent, biliary cirrhosis and cholelithiasis. Specially dedicated CF centres have led to improved survival rates and allow experienced staff to treat the many complications of CF while promoting research in this multisystem disorder.
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PMID:Cystic fibrosis in adolescents and adults. The coming of age of cystic fibrosis. 155 Dec 44


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