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)

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.
Schweiz Med Wochenschr 1978 Dec 23
PMID:[Severe alpha 1-antitrypsin deficiency: clinical observations of 21 patients]. 31 May 77

A patient with an ileal conduit diversion after cystectomy for carcinoma of the bladder is seen with cirrhosis and portal hypertension. Massive bleeding from the peristomal varices developed. Our experiences with conservative treatment is outlined.
Urology 1978 Dec
PMID:Exsanguinating hemorrhage from urinary ileal conduit in patient with portal hypertension. 31 Nov

Rapid progression of acute type B hepatitis to chronic active liver disease and cirrhosis in a young male with hypogammaglobulinemia is described. Absent circulating IgA, significantly low IgG, and normal IgM levels were detected during the acute phase of illness. Enumeration of peripheral lymphocytes revealed a decreased number of T cells and normal numbers of B cells. In vitro pokeweed stimulation of Ig synthesis correlated with the in vivo circulating levels of the three immunoglobulins. Cell-mediated immune responses were normal except for lymphocyte stimulation to hepatitis B surface antigen. It was concluded that the defective synthesis of IgG and IgA antibodies to hepatitis B surface antigen contributed to the accelerated progression to chronic active type B hepatitis in this person.
Gastroenterology 1977 Dec
PMID:Rapid progression of chronic active type B hepatitis in a patient with hypogammaglobulinemia. 33 24

Indications, selection of donor and recipient, medical and surgical management and complications, problems of organ procurement. Renal transplantation has become routine therapy. Organs are predominantly obtained from cadavers, transplantations from living donors are rarely indicated. Advances in preservation methods have improved organ quality and prolonged storage time. Selection of the most suitable recipient is based on histocompatibility matching. Blood transfusions before transplantation seem to improve the results. Recognition of a rejection crisis is primarily based on clinical symptoms. Persistent rejection calls for prompt explantation and the patient has to return to dialysis. Infections, serum-hepatitis and gastro-intestinal bleeding are the most common complications. Late complicatons are diabetes mellitus, cirrhosis of the liver, osteopathy, recurring glomerulonephritis, and, rarely, malignomas. Transplantation frequency in the Federal Republic of Germany could be increased by more awareness of physicians and a better knowledge of the general public about the need for cadaver donors.
Fortschr Med 1977 Dec 15
PMID:[Kidney transplantation from a nephrological-urological viewpoint--results and problems. 2. Diagnosis and therapy after transplantation, complications, long-term results]. 33 52

The records of 192 cadaver renal allotransplants were reviewed in order to evaluate the role of the hepatitis B antigen (HBsAg) carrier state on graft function, patient survival, and the incidence of severe hepatic dysfunction. Twenty-one allografts were placed into patients with hepatitis B antigenemia. After follow-up ranging from 1.5 to 8 years (mean 4.7), graft and patient survivals were not statistically different from antigen-negative patients. In addition, the acquisition of HBsAg after grafting did not seem to affect the rate of graft failuue or death. Neither cirrhosis not fatal hepatic failure developed in the HBsAg carrier group, whereas five HBsAg-negative recipients died of hepatic disease. Among HBsAg-positive recipients, nine with functioning renal allografts and five with graft failures, there was a low incidence of e antigen, suggesting low infectivity. This may explain the lack of correlation of the surface antigen with serious liver disease. Severe hepatic disease developing in renal graft recipients is most likely attributable to causes other than hepatitis B infection. The presence of hepatitis B antigenemia alone should not be a deterrent to renal transplantation.
Transplantation 1977 Dec
PMID:The effect of hepatitis B antigenemia on long-term success and hepatic disease in renal transplant recipients. 33 36

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.
Thromb Haemost 1977 Dec 15
PMID:Participation of Hageman factor dependent pathways in human disease states. 34 10

A decrease in H3-dopamine uptake was demonstrated in the blood platelets of 22 hepatic encephalopathy (HE) patients when compared to that of patients with liver cirrhosis, but without HE, and controls. There was a direct correlation between the stage of HE and the decrease in H3-dopamine uptake. As blood platelets have characteristics similar to neurons which contain amines, they have been proposed as a model for the study of amine metabolism in neurological, as well as liver diseases. A defective dopamine uptake by the HE platelets suggests that a similar biochemical derangement is, also, present in the nerve cells of the dopaminergic system. This could account for the clinical evidence of extrapiramidal dysfunction and the arousal effect of levodopa in HE. Platelets from 10 cirrhosis, but HE-free, patients had a dopamine uptake which was intermediate between the HE patients and controls. When octopamine was added at the same concentrations as in serum of HE patients, the blood platelets from five controls showed a decrease in dopamine uptake proportional to the concentration of octopamine added. Octopamine may impair dopamine uptake by platelets from HE patients.
Acta Hepatogastroenterol (Stuttg) 1977 Dec
PMID:Dopamine uptake by platelets in hepatic encephalopathy; evidence for a possible depletion of brain dopamine. 34 31

The results of the controlled study covering 21 cases of decompensated hepatic cirrhosis are repoted. Nine controls received conventional therapy with diuretics and vitamin supplement. Testosteron 100 mg intramuscularly, on alternate days for four weeks, was administered to 12 others, in addition to the conventional therapy. Patients in the testosterone group responded with reduction in ascites and pedal edema together with a subjective feeling of improvement. Serum albumin rose at the end of the four weeks while globulin fell in those that received the hormone. The difference in respect of both serum albumin and globulin in the testosterone group became statistically significant at the end of four weeks.
Aust N Z J Med 1977 Dec
PMID:Testosterone in the management of cirrhosis of the liver--a controlled study. 35 Feb 13

Hypertriglyceridaemia occurring in patients with liver disease has been studied by measuring hepatic triglyceride lipase (H-TGL) and plasma lipoprotein lipase (LPL) by selective precipitation of H-TGL with specific antibodies. Lipid analysis, determination of lecithin-cholesterol-acyltransferase (LCAT) activity, and liver function tests were performed in parallel in fifty patients with acute hepatitis, twenty patients with chronic active or persistent hepatitis and fifty with cirrhosis of the liver. Total post-heparin lipolytic activity (PHLA) decreased with the severity of liver dysfunction. This decrease was due to low H-TGL and only to some degree to low LPL activity. With improvement over several weeks of hospitalization, hypertriglyceridaemia disappeared with a concomitant increase of H-TGL and LPL. It is concluded that impaired triglyceride metabolism in liver disease is at least partly caused by diminished plasma hepatic TGL activity.
Eur J Clin Invest 1977 Dec
PMID:Secondary hypertriglyceridaemia in patients with parenchymal liver disease. 41 76

Polymorphonuclear leucocyte function was investigated in twenty patients with alcoholic cirrhosis and three patients with cryptogenic cirrhosis. Bacterial ingestion, oxygen-dependent bactericidal capacity, and chemotactic response were measured. Serum dependent abnormalities were common; they included deficiencies of ingestion and of all subsequent oxygen-dependent metabolic events (three patients), all oxygen-dependent metabolic events (one patient), cytochrome c reduction and iodination deficiencies (six patients), isolated cytochrome c reduction deficiency (ten patients), and chemotactic deficiencies (fourteen out of eighteen patients). Serum-independent abnormalities were much less common; they included increased ingestion rate (four patients), decreased stimulated reduction of nitroblue tetrazolium (three patients), and decreased myeloperoxidase content (eight patients). Polymorphonuclear leucocyte abnormalities are frequent in cirrhosis and may account in part for increased susceptibility to infection in that disease.
Eur J Clin Invest 1977 Dec
PMID:Blood polymorphonuclear dysfunction in patients with alcoholic cirrhosis. 41 78


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