Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:2.6.1.2 (alanine aminotransferase)
 26,722 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eighty-seven asymptomatic children with either hemophilia A or B were treated before they were 21 years of age. Seventy-two received factor concentrates and 15 cryoprecipitate or fresh-frozen plasma only. Thirty-two of the 72 in the former group have persistently elevated alanine aminotransferase values compared to one of 15 in the latter group. In a subset of children treated with factor concentrates before five years of age, four of seven are chronically HBsAg positive. Liver biopsies were performed in 13 of the 32 asymptomatic patients with abnormal ALT values in the fraction group. It is recommended that until further data become available, children with mild hemophilia and all less than 5 years of age should receive only cryo or FFP.
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PMID:The high risk of chronic liver disease in multitransfused juvenile hemophiliac patients. 44 27

The prevalence of antibodies to hepatitis C virus (anti-HCV) was studied in various population subsets in the Netherlands with anti-HCV C100 enzyme linked immunosorbent assay (ELISA), and confirmed with recombinant immunoblot assay (RIBA). Anti-HCV C100 ELISA positivity and RIBA positivity were found in 39 (0.7%) and 5 (0.1%) of 5,434 blood donors from Amsterdam; 25 (5%) and 2 (0.4%) of 481 blood donors from Surinam (South America); 19 (9%) and 2 (1%) of 213 multitransfused patients; 28 (4%) and 15 (2%) of 633 hemodialysis patients; 179 (80%) and 150 (67%) of 225 hemophilia A and B patients; 8 (80%) and 4 (40%) of 10 intravenous drug abusers; 18 (15%) and 2 (2%) of 119 anti-HIV-positive homosexual men; 2 (2%) and none of 106 anti-HIV-negative homosexual men; 6 (32%) and 3 (16%) of 19 patients with acute hepatitis non-A, non-B (NANBH); 13 (65%) and 8 (40%) of 20 patients with chronic NANBH and/or cryptogenic cirrhosis; and 4 (40%) and 1 (10%) of 10 patients with idiopathic autoimmune chronic hepatitis. Among blood donors, a positive correlation between a history of jaundice after the age of 18 years and the presence of RIBA-confirmed anti-HCV antibodies was found. Among both blood donors and hemodialysis patients, a positive correlation of RIBA-confirmed anti-HCV positivity with elevated alanine aminotransferase levels, but not with the presence of anti-hepatitis B core antibodies was found.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Prevalence of anti-HCV antibodies confirmed by recombinant immunoblot in different population subsets in The Netherlands. 164 6

Alpha's Wet Heat-Treatment process is being applied to both Factor VIII (AHF) and Factor IX Complex (PTC). Twelve hemophilia A, five hemophilia B, and one von Willebrands patient have been followed for at least 6 months for evidence of non-A, non-B hepatitis. No ALT elevations were seen in the hemophilia B patients. There have been four cases of ALT elevation, three in hemophilia A patients and one in the von Willebrand's patient. A subset of these patients have been followed for over one year for anti-HTLV-III status. No patient, either hemophilia A, hemophilia B, or von Willebrand's seroconverted to anti-HTLV-III positive status. Intravenous gamma globulin was studied in 11 normal patients given a single infusion and in 23 immune deficient patients with multiple infusions and evaluations of liver enzymes over a two year period. No elevated ALT or AST values were seen in either group.
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PMID:Evaluation of human viral disease transmission through plasma products. 244 Jul 44

We evaluated 37 patients with moderate or severe hemophilia A and six patients with severe factor IX deficiency for clinical or laboratory evidence of immune abnormalities. Patients were assigned to one of four groups according to the type of clotting factor replacement. Twenty patients had received only cryoprecipitate during the two years preceding the evaluation (group I); 11 additional patients were treated predominantly with cryoprecipitate but had also received up to nine bottles of factor VIII concentrate (group II); six patients received factor VIII concentrate (group III); six patients received factor IX concentrate (group IV). There was no clinical or laboratory evidence of immunodeficiency among the 43 patients. The mean absolute number of Th cells was normal in all patient groups, but the mean absolute number of Ts cells was increased compared with controls, both in patients treated with cryoprecipitate and in patients treated with factor VIII or factor IX concentrate. There was no correlation between the Th/Ts ratio and patient age, alanine aminotransferase level, hepatitis serology, in vitro lymphocyte function, or amount of clotting factor administered. Our observations demonstrate that the volunteer or commercial origin of clotting factor replacement cannot fully explain the alterations in lymphocyte subset distribution previously described in patients with hemophilia A.
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PMID:Immunologic status of hemophilia patients treated with cryoprecipitate or lyophilized concentrate. 623 71

To assess the relationship of liver dysfunction and hepatitis markers in hemophilic patients treated with factor VIII or IX concentrates, we studied 103 patients with hemophilia A and B for 6-36 mo. Elevated serum alanine aminotransferase was noted in 79% of the patients, with 51% of the patients showing persistent elevation for longer than 6 mo. Thirteen patients (12%) were HBsAg-positive, with 8 patients showing persistence of HBsAg and abnormal serum alanine aminotransferase for more than 6 mo. Overall, anti-HBs was detected in 77% of patients. Twelve episodes of acute hepatitis were documented in 10 patients during 36 mo. Six episodes were due to hepatitis B virus. The remaining 6 episodes were due to non-A, non-B hepatitis with negative HBsAg and absence of seroconversion to hepatitis B virus, hepatitis A virus, cytomegalovirus, and Epstein-Barr virus. In the six episodes of non-A, non-B hepatitis, the incubation period was less than 10 days in 3 patients and 30 days in 2 patients. In all cases with non-A, non-B hepatitis, the illness was symptomatic, but mild. Serum alanine aminotransferase returned to normal within 4 mo in 2 patients, but in 3 patients serum alanine aminotransferase persisted longer than 6 mo. One patient developed an acute B hepatitis 40 wk after non-A, non-B hepatitis. Thus, infection with the hepatitis B virus still remains prevalent as a cause of acute hepatitis in hemophiliacs receiving commercial factor concentrates, and accounts for chronic liver dysfunction in patients with persistent HBs antigenemia. In addition, acute non-A, non-B hepatitis, appears to be relatively common in hemophiliacs, and non-A, non-B virus may account for many cases of persistent liver dysfunction in these patients.
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PMID:Prevalence of type B and non-A, non-B hepatitis in hemophilia: relationship to chronic liver disease. 677 32

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

Low plasma haptoglobin values have been observed in hemophilia A patients on regular prophylactic treatment with factor VIII concentrates. Two of 3 patients treated with fraction I-0 (Kabi) and 7 of 11 patients treated with high-purity concentrates (Hyland) had low haptoglobin values. Four of 8 patients who were treated with high-purity concentrates prescreened for a low content of anti-A and anti-B immunoglobulins still showed low haptoglobin levels. Unexpectedly, 2 patients of blood group 0 showed low haptoglobin values. The presence of irregular erythrocyte alloantibodies and/or other contaminants of the concentrates might thus also be a cause of hemolysis resulting in an increased consumption of haptoglobin. Elevated lactate dehydrogenase levels were also frequent. No correlations were found between albumin, aspartate or alanine aminotransferase levels and haptoglobin levels.
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PMID:High frequency of low plasma haptoglobin values found in hemophilia A patients on prophylactic treatment with factor VIII concentrates - a sign of hemolysis? 679 43

Serum samples from 316 patients visiting the Dutch National Hemophilia Center were collected from 1979 to 1993 and stored at -30 degrees C. Patients were placed into three different groups: 1) patients ever treated with large pool non-hepatitis C virus (HCV)-safe concentrate (n = 179); 2) patients treated with cryoprecipitate (n = 125); and 3) patients treated exclusively with HCV-save concentrate (n = 12). In order to examine the prevalence of HCV infection in the different treatment groups serum samples were tested retrospectively for anti-HCV antibody using second generation enzyme-linked immunosorbent assay (ELISA) and recombinant immunoblot assay (RIBA-2). Significant differences in the prevalence of HCV infection were found between these 3 groups (group 1: 99%, group 2: 66%, group 3: 0%). The safety of currently administered clotting products is demonstrated in 57 patients who remained without HCV markers between 1989 and 1993. To examine the natural course of HCV infection fresh-frozen plasma samples were obtained recently from a subgroup of 277 hemophilia patients for HCV-RNA detection by a well-validated cDNA-PCR assay. In contrast to other reports, no evidence was found for seronegative HCV carriers. None of 52 patients without anti-HCV had detectable HCV-RNA. Of 225 patients with anti-HCV, 182 (81%) were HCV-RNA positive. None of 39 anti-HCV positive patients with a negative HCV-RNA reaction had serum alanine aminotransferase (ALT) levels above 50 U/l, whereas 44% of HCV-RNA positive patients had persistently elevated ATL levels above 50 U/l.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Hepatitis C infection and viremia in Dutch hemophilia patients. 753 31

Twenty-one HIV negative Japanese patients with chronic hepatitis C who had congenital bleeding disorders, 15 hemophilia A, 3 hemophilia B, 1 von Willebrand's disease, 1 afibrinogenemia and 1 thrombasthenia, were treated with 9 million units 3 times a week of natural interferon (IFN)-alpha for 6 months. They were followed, biochemically and virologically, for at least 18 months after therapy discontinuation to evaluate the long-term results. Liver biopsy, hepatitis C virus (HCV) genotyping and quantification of viral load by polymerase chain reaction (PCR) were performed to identify the predictors of a favorable response to IFN treatment. One male patient with hemophilia A dropped out because of general fatigue and was excluded from evaluation. Ten (50.0%) patients continued to be HCV RNA negative in serum together with normal ALT levels throughout the study. Subtype 1b and a high level of viremia significantly associated with an unfavorable outcome on the response to IFN although liver histology was not definitive for predicting the response. We concluded that a 6-month treatment with high doses of natural IFN-alpha was effective in inducing a long-term response without relapse of viremia in 50% of chronic hepatitis C patients with congenital bleeding disorders and that HCV subtype and pretreatment level of viremia were useful predictors of the response to IFN in treating such patients.
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PMID:A long-term follow-up study of interferon treatment for chronic hepatitis C in Japanese patients with congenital bleeding disorders. 885 94

We report the case of a young HIV seropositive patient with severe hemophilia A who presented rapid liver failure related to his chronic C hepatitis. The patient had been receiving factor VIII:C clotting factor concentrates (mean 60,000 U/year) since 1975. In 1984 alanine aminotransferase presented abnormal levels. The CD4 lymphocyte count in 1991 was normal and ultrasonographic scan showed normal liver morphology. In 1991 the patient were found to be seropositive for HCV antibodies as detected by the ELISA method and confirmed by the RIBA method. One year later, a progressive increase in policlonal gamma-globulin and a decrease in the CD4+ lymphocyte count to below 500/muL were detected in concomitance with ultrasonographic evidence of a progressive increase in the longitudinal diameters of the liver and spleen and signs of liver inhomogeneity. A significant inverse correlation was observed between the increase in the longitudinal diameter of the liver and the decline in albumin levels, and between the increase in the longitudinal diameter of the liver and the drop in platelet count. Elevated levels of ammonemia, gamma-glutamyl transpeptidase, alkaline phosphatase and IgA were detected. Moreover, decreased levels of the C4 and C3 complement fractions were documented. At this time (1994), esophagogram and esophagogastroscopy evidenced varicosities in the lower esophageal section (stage F1). The patient died in 1995 March at the age of 29 years of sudden septic shock related to Pseudomonas aeruginosa infection.
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PMID:Rapid liver failure related to chronic C hepatitis in an HIV seropositive hemophilic patient with severe immunodepression. 887 Mar 78


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