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)

A new method, radio-crossed immunoelectrophoresis, demonstrates alpha-fetoprotein (AFP) in sera with a sensitivity of 1 mug/1. By this method AFP with alpha mobility was not found in sera from healthy individuals, patients with chronic active hepatitis and cirrhosis, primary biliary cirrhosis, secondary liver cancer and cystic fibrosis. In some of the sera, AFP was elevated when measured by conventional radioimmunoassay method and the sera contained an AFP-like substance with gamma mobility when analyzed by radio-crossed immunoelectrophoresis. The nature of this gamma substance is still obscure and needs further investigation.
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PMID:Alpha-fetoprotein-like activity in sera from patients with malignant and non-malignant disease and healthy individuals. 6 Oct 78

Fifty cystic fibrosis (CF) patients, of whom 9 had multilobular cirrhosis, were observed regularly for a period of 3 years and various liver function tests, indicating cytolysis, cholestasis and cellular insufficiency were performed. Immunoglobulin and prothrombin were assayed. In 9 patients with cirrhosis, the tests were generally abnormal. Two distinct biochemical patterns of cirrhosis were distinguished, one clearly cholestatic and the other of a more cellular type. The distinction was made on the basis of the IgA : Transferrin ratio and of gamma-glutamyl-transpeptidase levels. In the non-cirrhotic patients, a temporary increase of cytolysis and cholestasis was observed in 50% of the cases.
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PMID:Additional data on hepatic function tests in cystic fibrosis. 23 31

Abnormalities of Hageman factor dependent pathways have been described in a wide variety of human disease states. Congenital deficiencies of factor XII (Hageman trait) prekallikrein (Fletcher trait) and high molecular weight kininogen (Williams, Fitzgerald and Flaujeac traits) although resulting in profound in vitro changes, do not cause in vivo difficulties. In contrast, deficiency of C1 esterase inhibitor (hereditary angioedema) results in significant morbidity and mortality. Acquired diseases may exhibit decreased synthesis of these three proteins in cirrhosis and dengue fever. In vivo activation of factor XII initiated pathways occur in septic shock, disseminated or localized intravascular coagulation, typhoid fever, polycythemia vera, hyperbetalipoproteinemia, coronary artery disease, nephrotic syndrome, transfusion reactions, hemodialysis and extracorporeal bypass. Activation of both the intrinsic system and tissue mediators contribute to the vasomotor phenomena in carcinoid syndrome and postgastrectomy dumping. Roles for factor XII, prekallikrein and kininogen have been suggested in gouty arthritis, allergic disorders and cystic fibrosis but the evidence is not yet convincing in these disorders.
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PMID:Participation of Hageman factor dependent pathways in human disease states. 34 10

Report about a patient with intraoperative and histological secured multilobular biliary cirrhosis of the liver and local formed purulent bile-duct and gall inflammation as an isolated hepatical form of the cystic fibrosis of the pancreas.--The character was complicated by the development of a portal vein high pressure and esophagus varicose vein haemorrhage, who at once needed a Shunt operation. Fromout the point of our view Sulprim is a good way to the therapie of purulent bile-duct inflammation in the case of cystic fibrosis of the pancreas.
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PMID:[Multilobular biliary liver cirrhosis as a purely hepatic form of mucoviscidosis]. 54 May 64

9 out of 204 unselected cystic fibrosis (CF) patients seen at the Department of Pediatrics, University of Berne, Switzerland, over the last 20 years had clinically overt liver disease. In 7 patients liver cirrhosis was demonstrated (3.4%). Focal biliary cirrhosis was the pertinent finding in 3 cases, whereas another 3 showed unspecific nodular cirrhosis. One infant presented with prolonged obstructive jaundice due to partial extrahepatic atresia and delayed passing of meconium. Two other patients had steatosis. A review of the literature dealing with clinical and laboratory findings in CF patients with liver disease is given.
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PMID:Liver involvement in cystic fibrosis. A report of 9 cases. 71 97

Vitamin A absorption tests using vitamin A palmitate and alcohol separately in oil and oil-water emulsions were done on 43 patients with cystic fibrosis. Patients were given 7,000 units of vitamin A per kilogram of body weight with a fatty breakfast. Pancreatic enzymes were not given with the test meal and were withheld for five hours from start of test. Blood was drawn before administration of the vitamin and at three and five hours after administration. Serum vitamin A levels were estimated using the Carr-Price technique. The percentages of patients with normal vitamin A absorption were 85 with vitamin A alcohol in oil-water emulsion, 61 with vitamin A alcohol dissolved in oil, 64 with vitamin A palmitate in oil-water emulsion, and 19 with vitamin A palmitate in oil. The number of stools per day is an inverse indicator of retention time in the intestine. Absorption of fat soluble vitamins is always abnormal when a patient has four or more stools a day. The observations that cystic fibrosis patients with abnormal liver biopsies have poor absorption of vitamin A were not statistically significant. The question of the effect of cirrhosis in cystic fibrosis on vitamin A absorption remains unresolved.
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PMID:Absorption of vitamin A in patients with cystic fibrosis. Absorption is best with emulsified vitamin A alcohol. 78 64

A case (including autopsy findings) is reported of multilobular biliary cirrhosis with a strictly nodular liver developing in a 20-year-old male with cystic fibrosis (mucoviscidosis). The pathogenesis of biliary cirrhosis in cystic fibrosis is briefly discussed.
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PMID:Multilobular biliary cirrhosis of the liver associated with cystic fibrosis (mucoviscidosis). 84 8

In 25 cases of autoptically proven mucoviscidosis 3 cases were detected with hepatic infestation. In two cases there was a focal biliary fibrosis (one male baby of 3 months, one female baby of 8 months), the third case was a 7 1/2 years old boy with a multilobulary biliary cirrhosis. The morphologie picture of these lesions is described, and their frequency, etiology and pathogenesis are discussed. Secondary alterations of the liver are for more frequent in this disorder than real liver infestations of mucoviscidosis, possible implications of this fact are shortly mentioned, too.
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PMID:[Hepatic infestation in mucoviscidosis (author's transl)]. 84 76

Cystic fibrosis was diagnosed after age 13 in 25 patients. All had an elevated sweat chloride and either a sibling with cystic fibrosis or typical pulmonary infection or digestive symptoms caused by exocrine pancreatic deficiency. Fourteen had long-standing pulmonary or digestive symptoms. In contrast, four of eight patients whose symptoms began after age 13 presented with biliary cirrhosis. Three male patients were asymptomatic at diagnosis. Opacification of all paranasal sinuses was found in all patients examined radiologically. At diagnosis, pulmonary-function testing showed obstructive changes in 19 patients and sputum cultures showed Pseudomonas aeruginosa in 15 patients. Delayed menarche in five of seven female patients and infertility in the asymptomatic male patient (two of whom were found to have aspermia) could have led to earlier diagnosis. Teenagers and young adults with long-standing pulmonary or digestive symptoms, unexplained cirrhosis, aspermia, or a sibling with cystic fibrosis should be sweat-tested by pilocarpine iontophoresis.
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PMID:Cystic fibrosis diagnosed after age 13. Twenty-five teenage and adult patients including three asymptomatic men. 88

In an infant with galactosemia high levels of galactose-1-phosphate in red blood cells and of blood galactose were observed under a "galactose-free'' diet. The child did not thrive and developed a liver cirrhosis. At the age of 5 months he died unexpectedly. Post mortem examination revealed in the pancreas and the small intestine changes suggestive of a cystic fibrosis. Since the exogenous administration of galactose by diet could be excluded the endogenous production of significant amounts of galactose-1-phosphate has to be considered.
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PMID:Galactosemia with endogenous production of galactose-1-phosphate and with cystic fibrosis-like appearance at autopsy. 93 2


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