Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:2.6.1.1 (aspartate aminotransferase)
21,665 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In 100 adult patients with severe haemophilia A (78 patients) and B (22 patients) sera were screened for the presence of serological markers of hepatitis B virus (HBV) and of cytomegalovirus (CMV) and liver function tests were performed which included measurement of serum aminotransferase AST and ALT activities, total bilirubin concentration and plasma levels of factor VII and X. In all the patients at least one out of five determined HBV markers (HBsAg. HBeAg, anti-HBs, anti-HBc and anti-HBe) was detected. HBsAg was found in 10% of the patients, and its prevalence in haemophiliacs B was higher than than observed in haemophiliacs A (22.7% and 6.4%, respectively). HBsAg appeared more frequently in patients receiving factor VIII concentrates (16.7%) than in those treated with cryoprecipitate (4.5%). Anti-CMV antibody was detected in sera of 98% of the patients. In 1/3 samples of cryoprecipitate anti-HBc or anti-HBs were present, and in the half of samples anti-CMV occurred. Abnormal liver function tests indicating chronic hepatitis or liver cirrhosis were obtained in 8 patients. Raised ALT activity which could suggest chronic infection with non-A, non-B virus occurred in 6 cases. The present study indicates that haemophiliacs frequently transfused with plasma products are at high risk for viral infections leading to liver dysfunction.
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PMID:[Serological markers of hepatitis B virus and cytomegalovirus in patients with hemophilia]. 217 33

Seventeen previously untreated boys with haemophilia A were treated with high purity heat treated factor VIII concentrate (8Y) for up to 36 months. Liver function tests were assessed monthly. No boy's serum has been shown to contain HIV antibodies and no increases in alanine transaminase activity have been detected. In only one patient was a single rise in aspartate transaminase activity noted, and this was without a corresponding rise in alanine transaminase. A second patient's serum contained hepatitis B core antibody transiently. It was thought likely in both cases that the abnormalities reflected intercurrent infections rather than disease associated with transfusion. The physical treatments used in the production of 8Y seem to inactivate the agent(s) responsible for non-A, non-B hepatitis and HIV transmission by transfusion of factor VIII has been abolished. There are, however, problems associated with conducting safety trials in young haemophiliac patients.
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PMID:Safety trial of heated factor VIII concentrate (8Y). 251 Jun 7

Coagulation factor concentrates prepared in England are all subjected to heating in solution or in the lyophilised state. Concentrates of factor VIII, factor IX (II and X), factor VII and factor XI are terminally heated in the lyophilised state at 80 degrees C for 72 h but the current fibrinogen concentrate withstands only 70 degrees C for 24 h. 33 patients receiving factor VIII concentrate (code 8Y) and factor IX concentrate (code 9A) for the first time have had regular liver function tests (LFTs) and we have at least some data on 26 of them exposed for greater than 3 months. Of these 26 patients, four had received no blood products before 8Y, 15 had received only cryoprecipitate before 8Y, and seven had received no blood products before 9A. Nine have missed only one or none of their two-weekly tests, four have missed two or three tests and on the remaining 13, the LFT follow-up has been unsatisfactory, although in some cases clinical examination has been helpful. 12 batches of 8Y from greater than 70,000 donations and seven batches of 9A from greater than 40,000 donations have been used. In 13 patients who have had regular prospective LFTs, none has had an ALT or AST level above twice normal. One patient followed only irregularly has shown an isolated ALT rise at eight weeks, unconfirmed at nine or at 17 weeks.
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PMID:Interim results of surveillance for NANBH in patients receiving heated concentrates produced in England. 311 10

Thirty-eight children with severe hemophilia A, 11 years of age and under, were evaluated by initial and follow-up liver function tests (LFTs) in relation to age of onset of transfusion therapy. Each child had at least two complete evaluations within one year for a follow-up period of at least one year. The mean number of exposure days was 36 with a mean of 275 units of factor VIII per exposure day prior to initial LFTs. At initial testing, 30% of patients demonstrated antibody to HBsAg and 39--51% at least one abnormal serum enzyme level (AST, ALT, LDH). During an average follow-up period of 34.8 months, two children developed HBsAg-positive icteric hepatitis. Of those initially serologically negative for HBsAg or antibody, 44% became antibody-positive. Intermittent abnormalities of at least one serum enzyme were observed in 79% of the patient group, with 13% and 8% being persistently normal and abnormal. Eleven children born after January 1976, receiving only third-generation RIA-tested products for HBsAg, constituted a subgroup. Although only one child at first assessment had evidence of hepatitis B virus exposure, 55% had elevated ALTs, indicating considerable frequency of non-A, non-B hepatitis in this very young group.
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PMID:Hepatocellular enzyme patterns and hepatitis B virus exposure in multitransfused young and very young hemophilia patients. 679 95

Twenty-four children and adolescents who have been receiving home treatment for haemophilia A and B, and were followed up for a median period of five years, have been assessed for physical activity, social adjustment, range of joint movement and infection with hepatitis viruses. They were treated with cryoprecipitate from 1972 to 1977, and since then with factor-VIII concentrates. The average dose of factor VIII was 20 units/kg body mass. It was found that there was near normal range of physical activity and school performance, and, in virtually all families, near normal family function could be preserved. Approximately one-third of the patients showed impairment of the normal range of joint movement in flexion and extension. Although there was no clinical evidence of liver disease, elevated aspartate aminotransferase (AST) levels were found in 14 patients. Evidence of past, or present, infection with hepatitis B was found in 19 patients, and of infection with hepatitis A in seven patients. Home treatment is associated with a reduced level of disability from haemophilia, but transfusion therapy continues to be associated with a high rate of liver function abnormalities, probably of infectious origin.
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PMID:Home treatment of haemophilia. A follow-up study. 679 76

Six cases of hepatic sarcoma are reported: leiomyosarcoma in two, malignant fibrous histiocytoma in two malignant hemagiopericytoma in one and fibrosarcoma in one. In addition to the routine paraffin section and HE stain, immuno-histochemical studies with antibodies against vimentin, EMA, CK, S100, ACT, AAT, desmin, AFP, lysozyme and factor VIII and Masson trichrome staining and argyrophilia staining were done. AFP was negative in all 6 patients and the primary sarcoma was characterized by the absence of accompanying liver cirrhosis. The diagnosis, histogenesis and prognosis of primary liver sarcoma are discussed.
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PMID:[Primary sarcoma of the liver]. 795 5

Clinical and morphological findings in 91 patients with infantile hemangioendothelioma of the liver are reported. The study comprised 56 girls and 35 boys ranging in age from premature infant to 3 yr; one outlier patient was 18 yr old. Most patients with infantile hemangioendothelioma (87%) were first seen before the age of 6 mo. Congestive heart failure was evident in 15%. Skin hemangiomas were noted in 11%. Anemia, hyperbilirubinemia and increased AST level were present. Solitary lesions were more common than multiple ones (3:2). Immunohistochemical staining of tumor cells for factor VIII was positive in 20 of 21 cases tested; testing for blood group antigen was positive in 8 of 28 cases. Cytokeratin staining verified the presence of bile ducts, some of which appeared to be the result of transformation of injured liver. No pericytes were identified on electron microscopy. The 6-mo survival rate, based on 71 cases, was 70%. Average time of follow-up for the survivors was 7.7 yr. All deaths occurred during the initial presentation/hospitalization of infants, with the exception of two patients who died 3 mo and 7 mo after diagnosis. More recent analytic methods, including immunohistochemical stains and flow cytometric studies, do not contribute to the practical assessment of this tumor. Covariates with significant value in predicting death 6 mo after diagnosis included presence of congestive heart failure, jaundice, multiple tumor nodules and absence of cavernous differentiation.
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PMID:Infantile hemangioendothelioma of the liver. 802 Sep 3

In order to clarify the pathogenesis of porcine serum (PS)-induced rat liver fibrosis, three experiments differing in dose of PS or duration of treatment were performed on male Fischer 344 rats. The rats were given an intraperitoneal injection of PS twice a week for 3 to 16 weeks and euthanized 7 days after the last injection for each treatment group. Liver tissues from these animals were subjected to detailed morphological and immunohistochemical examinations. Biochemical tests on treated rat serum revealed an increase in globulin concentration but no elevation in AST, ALT and ALP activities. There were no relationships among the dose of PS, the extent of fibrosis, and the anti-PS antibody titer. A number of alpha-smooth muscle actin-positive non-myofibroblastic cells, desmin-positive cells, and lipofuscin-laden Kupffer cells were found around the central veins and in the fibrous septa. In advanced stages of fibrosis, a proliferation of elastic fibers were observed in the septa. These findings were considered to indicate gradually occurred hepatocellular necrosis. The vascular endothelial cells in the fibrous septa expressed factor VIII-related antigen, exhibited fenestration accompanied by basement membrane formation, and were surrounded by Ito cells. Most of the portal vein branches showed hypertrophic thickening of the smooth muscle layer, resulting in narrowing of the lumen. These vascular changes suggested that hemodynamic alterations of the intrahepatic circulation induced hepatocellular necrosis/apoptosis and played an important role in the pathogenesis of porcine serum-induced liver fibrosis in rats.
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PMID:Morphological and immunohistochemical studies on porcine serum-induced rat liver fibrosis. 910 74

The aim of our study was to compare the hFVIII mRNA expression in different organs, pathological changes and selected haematological and biochemical blood parameters between transgenic and non-transgenic rabbits from F3 generation. Selected physiological parameters of 3- to 4-month-old transgenic rabbits from F3 generation carrying human factor VIII gene (hFVIII) were analysed and compared with those of non-transgenic ones. Before slaughtering, the blood for haematological and biochemical analysis was taken from the central ear artery. Pathological and histological examination of vital organs and RT-PCR analysis of several tissue organs of transgenic and non-transgenic animals were performed after slaughtering. Except for the mammary gland tissue, slight hfVIII mRNA expression in the spleen, lung and brain and none expression in the liver, kidney, skeletal muscle and heart of rabbits were recorded. pathological examination of vital organs showed some pathological changes in both transgenic and non-transgenic rabbits which were confirmed by histological qualitative evaluations. Statistically significant lower values of blood haemoglobin in blood of transgenic (11.86+/-0.86) animals compared with non-transgenic (12.41+/-1.02, P<0.05) ones and lower parameters of HCT (39.22+/-2.44 versus 40.89+/-2.26, P<0.01) in blood of transgenic rabbits were observed. Parameters of WBC, RBC and PLT showed no significant differences between the analysed groups. All biochemical serum parameters of transgenic rabbits were higher in comparison with non-transgenic ones. Significant differences were found in the concentration of the urea, AST and GMT between transgenic and non-transgenic animals (P<0.001) and in the total protein content, the difference was significant at P<0.05. In conclusion, our results showed that no considerable impact on the general health was found in transgenic rabbits.
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PMID:Evaluation of haematological, biochemical and histopathological parameters of transgenic rabbits. 1793 Dec 30

Prior to the availability of test for hepatitis 'C' in 1989, any elevated AST in a pooled blood product recipient was presumed to be from non-A-non-B hepatitis. We report a patient who received pooled factor VIII in 1984 and had persistently elevated AST which proved to be of nonhepatic origin.
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PMID:A lesson from persistently elevated aspartate transaminases (AST) in a patient with severe haemophilia A. 2721 19


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