Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019163 (hepatitis B)
 38,309 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Classic hemophilia or factor VIII deficiency is a recessive, sex-linked bleeding diathesis. The primary clinical problem is hemorrhage, which can be severe and often life threatening, even in the presence of only minor trauma. In the past this inadequate hemostasis has been treated with transfusions of cryoprecipitate, fresh frozen plasma, or commercially prepared factor VIII concentrate. Unfortunately, such treatment carries with it a number of risks, including the development of hepatitis B or hemolytic anemia and the formation of anti-factor VIII antibodies. Because of hemorrhage severity and the risks of conventional treatment, elective surgery in general and oral surgery in particular have often been neglected in patients with hemophilia. This article reviews a drug, 1-desamino-8-d-arginine (DDAVP), heretofore not discussed in the dental literature, and reports on its use in conjunction with epsilon-aminocaproic acid (EACA), a synthetic antifibrinolytic agent, in the surgical dental treatment of a patient with hemophilia A. The results suggest that certain dental surgical procedures can be performed in the presence of subclinical and mild hemophilia without conventional factor VIII replacement therapy with its associated costs and risks.
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PMID:DDAVP: review of indications for its use in the treatment of factor VIII deficiency and report of a case. 622 10

To assess the immunologic status of healthy persons with hemophilia A, we performed studies of T cell immunity in 21 patients, 10 given only cryoprecipitate and 11 given factor VIII concentrate. Patients in the factor VIII group had significantly decreased helper/suppressor T cell ratios. Both groups had diminished mononuclear cell response to phytohemagglutinin and normal mixed lymphocyte culture, compared with controls. Abnormalities in T cell number or function did not correlate with the presence of antibody to cytomegalovirus, Epstein-Barr virus, or hepatitis B. Physicians caring for patients with hemophilia A should realize that asymptomatic individuals may have early evidence of immunodeficiency.
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PMID:Immunologic abnormalities in patients with hemophilia A. 641 59

Titrated stocks of hepatitis B virus and Hutchinson strain non-A, non-B hepatitis virus were diluted in normal serum to contain, respectively, greater than or equal to 10(6) and greater than or equal to 10(4) chimpanzee infectious doses (CID50) per milliliter and exposed to 1% Tween 80 and 20% ether at 4 degrees C for 18 h. After evaporation of the ether, the treated sera were each inoculated into two chimpanzees. The animals remained free of serologic and biochemical evidence of hepatitis during a 6-month follow-up period, and were then shown to be susceptible to infection by challenge with the original untreated inocula. To assess the effect of exposure to Tween 80/ether on coagulation factors, four lots of antihemophilic factor (AHF) concentrate and 2 lots of commercial factor IX concentrate were treated as above. For the AHF concentrate there was an average of 70% recovery of factor VIII procoagulant activity, 93% recovery of factor VIII-related antigen, and 73% recovery of fibronectin opsonin activity and no detectable change in ristocetin cofactor activity or in fibronectin antigen. Crossed immunoelectrophoresis revealed no change in migration rate of fibrinogen, fibronectin, and von Willebrand factor (vWF), although the quantity of fibrinogen was reduced. Factor VIII procoagulant activity and vWF activity remained associated during chromatography on BioGel A15.
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PMID:Inactivation of hepatitis B and Hutchinson strain non-A, non-B hepatitis viruses by exposure to Tween 80 and ether. 642 34

The epidemiology of viral hepatitis and liver function were studied in a retrospective survey of 69 patients with moderate and severe hemophilia A and B, and with severe von Willebrand's disease. Forty-nine patients were on prophylactic self-therapy and 20 on episodic treatment by medical personnel. Serologic markers of viral hepatitis (HBsAg, anti-HBs, anti-HBc, anti-HAV, and in some cases HBeAg and anti-HBe) and liver function tests (ASAT, ALAT, IgG) were followed for up to 12 years. There was a history of clinical hepatitis in 19%, and 96% showed some serologic evidence of exposure to hepatitis B virus. Only one patient was a HBsAg carrier. The prevalence of elevated ASAT and/or ALAT was 65% and the incidence 96%. In 68% of the patients there had been a transaminase elevation for more than 6 months. The clinical picture, serologic markers or liver function tests showed no significant difference between the types of hemophilia, amounts and modes of therapy, or age groups. The chronic hepatitis seen in our hemophiliacs seemed to be a slowly or non-progressive disease.
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PMID:Hepatitis, epidemiology and liver function in hemophiliacs in Sweden. 661 Feb 81

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

Circulating immune complexes (ICs), assayed by the L1210 enzyme-linked immunoassay, were detected in 48% of patients with hemophilia. A, 50% of patients with von Willebrand's disease, and in none of our patients with hemophilia B. Eighty-five % of the hemophilia A and B patients had mild to moderate disease with only one patient demonstrating a circulating inhibitor. No correlation was found between IC levels and hepatitis B infection, SGOT, disease severity, total quantity of factor VIII or IX infused, time interval from list infusion, or rheumatoid factor positivity. Although the nature of the ICs is not known, the similarity of IC levels between hemophilia A and von Willebrand's disease is discussed with regard to antibodies generated to non-procoagulant portions of the factor VIII molecule.
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PMID:Immune complexes in hemophilia. 679 22

A retrospective survey on clinical hepatitis in patients with bleeding disorders was performed. Nine episodes of hepatitis non-A, non-B occurred in 8 out of 20 patients (40%) with mild hemophilia A or von Willebrand's disease, who had been treated with commercial factor VIII concentrates. Only two episodes of hepatitis B occurred during the study period. The non-A, non-B attack rate after the first treatment was 40% with factor VIII concentrate obtained from large plasma pools (= 2,000 donors) including professional plasma donors as compared to 8% after treatment with factor VIII concentrate obtained from smaller (100-250 donors) plasma pools from Scandinavian donors.
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PMID:Acute hepatitis non-A, non-B following administration of factor VIII concentrates. 680 Jan 31

Recent studies in multiply transfused patients with hemophilia A and persistent liver function abnormalities have shown a high incidence of chronic active hepatitis. The purpose of the present study was to determine the severity of liver disease in multiply transfused patients with intermittent liver enzyme abnormalities. Fifteen patients with elevated enzymes on two or three out of four determinations at 6-mo intervals were studied. None had signs or symptoms of chronic liver disease. Thirteen had serologic evidence of prior exposure to the hepatitis B virus. Liver biopsy performed on these patients after replacement therapy with factor VIII showed chronic persistent hepatitis or other mild forms of liver disease in 14 of the 15 patients. Patients with chronic persistent hepatitis had significantly higher mean liver enzymes at time of biopsy than patients with milder forms of hepatic inflammation, but there was no relationship between liver histology and hepatitis B serology or the amount of factor VIII used in the 6 mo preceding biopsy. These findings support the continued use of factor VIII concentrates in patients with hemophilia.
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PMID:Chronic hepatitis in patients with hemophilia A: histologic studies in patients with intermittently abnormal liver function tests. 681 49

Chronic liver disease has become a significant complication of the therapy of hemophilia disorders. We describe two patients with hemophilia A and hepatitis B virus hepatitis who progressed to cirrhosis with bleeding esophageal varices. Each underwent distal splenorenal shunt under plasma concentrate therapy without difficulty. One patient died 19 months after operation and unsuspected hepatocellular carcinoma was found at autopsy. These cases illustrate the potential severity of liver disease in hemophilia and the ability to safely perform surgery for portal hypertension if required.
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PMID:Cirrhosis, variceal bleeding, and distal splenorenal shunt in hemophilia A. 712 25


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