Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
 30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Modifications of Factor VIII related antigen (F, VIII R. A.) were investigated in three hemophilic patients during the course of post transfusion hepatitis. F. VIII R. A. was found to be elevated and correlated with the increase of the S.G.P.T. During the acute phase of the disease the Factor VIII related protein was tested for its ristocetin cofactor activity, its electrophoretic mobility on crossed antigen-antibody electrophoresis and its elution pattern on Sepharose 4B columns; all these properties proved to be abnormal. Liver function tests and F. VIII R. A. were measured in 36 multitransfused hemophiliacs. There was a significant increase of the F. VIII R. A. in the plasma of patients with abnormal liver function tests. Only a few of the multitransfused hemophiliacs had clinical symptoms of hepatitis, although 72% had elevated transaminase levels.
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PMID:Modifications of factor VIII related antigen in hemophiliacs with acute hepatitis and sub-clinical liver disease. 26 93

The incidence of post-transfusion hepatitis and liver dysfunction is presented in fifty-four patients with classical hemophilia who received episodic and/or prophylactic Factor VIII concentrate. 42.5% had persistent biochemical evidence of liver dysfunction with elevated SGOT and SGPT; 3.8% have persistent (HBs) antigenicity and 90% have (HBsAb) antibodies. The results are the same for those who were treated episodically and received an average of 753 units Factor VIII per week as those treated prophylactically who received an average of 686 Factor VIII units per week. The incidence of clinical and/or subclinical disease is unaffected by the transfusion regimen or the amount of concentrate used. The necessity for close follow is emphasized for determination of chronic liver disease and its further therapy.
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PMID:Liver dysfunction in patients with hemophilia. 26 1

Classical sex-linked hemophilia (Hemophilia A) has been described as due to deficiency in the synthesis of Factor VIII procoagulant activity (VIII:C). The availability of immunological techniques provided the means of identifying Factor VIII-Related Antigen(VI-IIR:Ag) detectable by rabbit antibodies to F VIII, which is distinct from VIII:C detected by human anti-F VIII available from multitransfused patients. Hemophilia A is lacking in VIII:C but not VIIIR:Ag. Recently, a third function of the F VIII "complex" was discovered with the help of ristocetin (von Willebrand's Factor, VIIIR: RCo). This activity is reduced in von Willebrand's syndrome. Estimation of the titers of VIII:C and VIIIR:Ag provides a method for more accurate detection of hemophilic carriers. Newly available chromogenic substrates perhaps will give rise to more simplified assays of VIII:C. The development of cryoprecipitates and stable lyophilized concentrates of F VIII has greatly simplified and intensified maintenance therapy, and has opened a new era in treatment. Prophylactic therapy has been shown to be very helpful in certain "high risk" cases. The impact and benefits of home care and self-administration has been tremendous. However, the varying quality of cryoprecipitates and the high cost of more purified concentrates are still stumbling blocks in treatment regimes. Other problems exist. Spontaneous bleeding, especially central nervous system bleeding, account for the majority deaths by haemorrhage. Inhibitor kinetics have been well characterized. It is clear that there exists "low" and "high" responders. For the "high" responders, plasmapheresis, immunosuppressives and the infusion of Factor IX concentrates have been utilized with varying success. The prevention of hemophilic arthropathy and its progression by maintenance therapy seems to be still inadequate. The results of trials with more vigorous regimes are awaited. The complications of therapy still remain to be solved. Apart from the well-known complications wuch as hepatitis, haemolytic disease and F VIII inhibitors, the existence of previously unnoticed complications as splenomegaly, hypertension, renal disease and paradoxal bleeding have been recently realized. The role of altered fibrinogen, fibrin degradation products (FDP) and unclassified fibrinogen derivatives (UFD) present in cryoprecipitates and F VIII concentrates in the above complications needs to be further clarified. In conclusion, tremendous progress in various aspects of hemophilia has been achieved in developed countries. Comprehensive care can now be carried out in various centers. On the other hand, developing countries still face a number of basic problems. The concept that hemophilia is a "manageable" disease and that chronic crippling and death from exsanguination can be prevented, should be disseminated widely by various means...
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PMID:Recent advances in hemophilia. 52 46

A retrospective survey of transfusion hepatitis associated with a brand of commercial Factor VIII was carried out in 24 Haemophilia Centres from January 1974 until December 1975. Of 371 patients who were transfused with this product, and were followed up, 78 cases of hepatitis affecting 66 patients were found (17.7%). Two types of hepatitis were observed: hepatitis B and non-B hepatitis, the latter with an incubation period of between 8 and 60 days. Twelve patients contracted two types of hepatitis, non-B followed by hepatitis B. Only one patient died after contracting hepatitis B. Four of the suspect batches of concentrate were found to be positive for HBsAg by radioimmunoassay. There was evidence that the presence of hepatitis B surface antibody in a patient's serum prior to exposure was associated with immunity to hepatitis B. Evidence was presented suggesting that the non-B hepatitis observed was not due to hepatitis A. The factors affecting the incidence of transfusion hepatitis in haemophiliacs were discussed.
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PMID:Commercial factor VIII associated hepatitis, 1974-75, in the United Kingdom: a retrospective survey. 64 45

A 38-year-old man with severe factor IX and mild factor VIII deficiencies complicated by cirrhosis secondary to chronic non-A non-B hepatitis underwent orthotopic liver transplantation as treatment for both the cirrhosis and his congenital coagulopathy. Intraoperative hemostasis was obtained with factor VII-depleted prothrombin complex concentrate and fresh frozen plasma. Factor VIII and factor IX levels were assayed frequently in the perioperative period, and both returned to normal within 24 hr and remained normal postoperatively. Liver transplantation can be considered as definitive therapy for hemophilia A and/or B with transfusion-related liver disease.
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PMID:Orthotopic liver transplantation in a patient with combined hemophilia A and B. 210 34

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

The development of factor VIII concentrates has greatly facilitated hemophilia care and has made the home care of hemophilia possible. However, factor VIII concentrate that has been produced using traditional methods contains large amounts of foreign proteins and viruses. This has resulted in the development of immunologic abnormalities in many hemophiliacs and has exposed many of these patients to blood-borne viruses such as the human immunodeficiency virus (HIV) and hepatitis viruses. Factor VIII circulates in plasma in complex with the von Willebrand factor (vWF). Both factor VIII and vWF have been purified and monoclonal antibodies (mAb) have been generated to both of these proteins. When bound to a solid support, these mAb's can be used to isolate selectively the proteins of interest. Recently, two separate procedures have been used in the immunoaffinity purification of factor VIII on a commercial scale. One product (Monoclate) has been prepared using a mAb to the vWF bound to a chromatography column. The other product (Hemophil M) uses immobilized mAb to the factor VIII molecule. Factor VIII concentrate purified using either of these approaches is far more pure than traditional factor VIII concentrates. In addition, the use of both viral purification and viral inactivation procedures has greatly reduced the risk of viral contamination. Early clinical studies have demonstrated that these products are effective in treating bleeding episodes and that the risk of viral infection with HIV or hepatitis viruses is low. Factor VIII concentrate produced using mAb technology appears to be the product of choice in previously untransfused hemophiliacs. Its role in the treatment of patients who have already been infected with HIV is less clear.
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PMID:Immunoaffinity purification of factor VIII. 250 62

The introduction of factor VIII and IX concentrates in the early 1960s brought a significant change in the hemophiliac's life. In consequence hemophilia treatment has been improving rapidly since, and today most life-threatening hemorrhages are controlled by replacement therapy. Hemophilic arthropathy through recurrent joint and muscle bleedings occurs later in life and is often limited to one joint only. Major surgery in hemophiliacs involves little more risk than in non-hemophilic patients, provided of course there is close teamwork between surgeon and hematologist. The most frequent causes of death are no longer hemorrhages but blood-product-associated AIDS and hepatic failure. Fortunately these side effects have been overcome by the use of virus-inactivated concentrates which in Switzerland have been generally administered since 1986. Factor VIII and IX concentrates must contain a precisely declared quantity of factor VIII and IX activity respectively, with a high specific activity. High-purity concentrates should be preferred because of the hazardous effect of foreign proteins administered intravenously in large quantities over a long period. Activation of fibrinolysis with consequent failure of hemostasis or even worsening of hemorrhage may be a clinically relevant side-effect of DDAVP therapy. When DDAVP is used for prophylactic treatment before surgery, an interval of one hour between the intravenous administration of DDAVP and surgery ensures the latter is performed at the time of highest factor VIII and von Willebrand factor level but with already decreased t-PA and fibrinolytic activity. If DDAVP is used in case of hemorrhage or postoperatively, however, the whole fibrinolytic potential must be taken into account. In these cases subcutaneous administration is advantageous due to more protracted t-PA release and the subsequent lower fibrinolytic activity, which can more easily be neutralized by tranexamic acid. To prevent hemophilic arthropathy, correct replacement therapy in hemarthroses is essential: it should be performed as early as possible, preferably in a home therapy program; adequate levels of factor VIII or IX should be achieved and maintained over a sufficient length of time. Hemophiliacs who did not receive replacement therapy during childhood often need major surgery because of severely destructed joints. Joint replacement by total knee and hip prostheses has proved very successful if certain special conditions are fulfilled. Surgical indications should, however, be carefully considered and the possibilities and limits of replacement therapy should be well known. Blood-product-associated hepatitis will be of prognostic relevance in many hemophiliacs treated formerly with non-virus-inactivated concentrates.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Current clinical aspects in hemophilia treatment]. 250 19

Diluted plasma samples containing 10(2), 10(3), 10(4), and 10(5) chimpanzee infectious doses (CID) of a human non-A, non-B hepatitis virus (NANBV) were treated with a combination of two psoralen compounds, 4'-aminomethyl-4,5',8-trimethylpsoralen and 4,5',8-trimethylpsoralen, and exposed to long wavelength ultraviolet. Each dilution was then transfused into one of four chimpanzees. In a second experiment, three samples containing 10(4.5) CID of hepatitis B virus (HBV) and two samples containing 10(4) CID of NANBV were treated with 8-methoxypsoralen (8-MOP) and ultraviolet irradiation. Two chimpanzees were each transfused with both a treated HBV and a treated NANBV sample. The third chimpanzee was inoculated with a treated HBV sample alone. In the six months after inoculation none of the animals showed biochemical or histological evidence of hepatitis. In experiments involving NANBV inocula, the susceptibility of the animals was confirmed by subsequent challenge with untreated NANBV. Factor VIII concentrate containing virus and photochemically treated as in the first experiment retained an average of 91% of its activity while that in the second experiment retained 94% of its activity.
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PMID:Photochemical decontamination of blood components containing hepatitis B and non-A, non-B virus. 290 70

Hemophilia is an inherited hemorrhagic disease which is due to the insufficiency of Factor VIII, or Factor IX, or Factor XI. Hemophilia patients are regarded as special patients with increased dental problems. The present paper consists of two parts. In the first part the types of hemophilia, ways of transmission, severity forms, and clinical characteristics are described. In the second part a protocol concerning the dental treatment of hemophilia patients is presented. There are four basic types of hemophilia: hemophilia A or classical hemophilia or Factor VIII deficiency, hemophilia B or Christmas disease, hemophilia C and von Willebrand's disease. Hemophilia is transmitted either as a sex-linked recessive or as an autosomal dominant trait, depending on the type of the disease. The severity of hemophilia depends on the amount of the coagulation factor present. According to this amount, there are four scales of severity. The clinical characteristics of the disease also depend on the amount of the factor present and vary, from occasional bleedings to serious and even life-threatening bleeding episodes. In the second part of the paper the special psychological and physiological problems of the hemophiliacs are discussed. In addition, there is reference to the hematologic coverage these patients need, as well as to the protection measures for the dental personnel against hepatitis and AIDS. The dental treatment plan at the office is presented in detail, including a discussion of the advantages and disadvantages of the treatment of hemophilia patients in the operating room under general anesthesia.
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PMID:[Hemophilic patients. Treatment protocol in the dental office]. 297 76


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