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

In-vitro and animal studies have shown that viral agents can be removed from or inactivated in clotting factor concentrates by physical or chemical treatment. However, clinical data have as yet not substantiated the results of these studies. 13 haemophilia A patients who had not been treated previously with blood or blood products were given a dry-heated factor VIII concentrate and were tested serologically over the next 12 months. Hepatitis developed in 11 patients (84%) and was invariably of type non-A, non-B. Morbidity was not related to the lot of the therapeutic material or to the number of infusions. The incubation period was either 5 or 8-11 weeks, and only 1 patient had symptoms. Aminotransferase elevation showed both monophasic and biphasic patterns. During the follow-up period signs of the disease disappeared in 10 patients (90%). These findings contrast with the absence of non-A, non-B hepatitis in chimpanzees given the same heated concentrate. Thus, clinical studies in first-exposure haemophiliacs are essential for the true evaluation of the safety of new "treated" concentrates.
 ...
PMID:Transmission of non-A, non-B hepatitis by heat-treated factor VIII concentrate. 286 54

The presence of human immunodeficiency virus (HIV) antibody in hemophilia patients was correlated with the loss of existing antibody to hepatitis B surface antigen (HBsAb) or the inability to develop an antibody to hepatitis B after receiving commercially available hepatitis B vaccine. Of the 137 patients studied 66 were HIV-positive and 71 were HIV-negative. Evidence of HBsAb (n = 44) or exposure to hepatitis vaccine (n = 12) was found in 85% of HIV positive patients at some time during their care in our clinic. However, 20% demonstrated subsequent antibody loss and/or did not respond to hepatitis vaccine. Loss of HBsAb or vaccine nonresponse was restricted to patients less than 21 years of age (72% of all patients). This result contrasted to only a 3% loss of HBsAb or vaccine nonresponse in the HIV-negative patients who had acquired the HBsAb (n = 23) or were given the hepatitis vaccine (n = 29). This result suggests that loss or alterations of hepatitis B immunity occur in association with HIV infection or exposure.
 ...
PMID:Loss of hepatitis B antibody in human immunodeficiency virus-positive hemophilia patients. 296 61

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.
 ...
PMID:[Hemophilic patients. Treatment protocol in the dental office]. 297 76

The clinical, immunological, and serological status of 28 patients with hemophilia A and of 13 patients with hemophilia B was investigated. Thirty-four patients were treated regularly by clotting factor concentrates and 7 patients had been substituted only 1 to 4 times. Almost all patients with severe hemophilia suffered from hepatopathy. No patient had clinical evidence of the acquired immunodeficiency syndrome (AIDS). Asymptomatic hemophiliacs showed a decreased number of T-helper (OKT 4) cells and an increased number of T-suppressor (OKT 8) cells, which resulted in an inversed OKT 4/OKT 8 cell ratio. Natural killer cell activity of all patients was decreased compared to controls. After culture there was no significant difference of NK cell activity between hemophiliacs and controls. This phenomena was interpreted as a possible maturation defect of NK-cells in vivo. No relationship between immunological alterations and hepatopathy, hepatitis markers, CMV antibodies, amount and source of required factor concentrates, and the kind of hemophilia was observed. IgG immunoglobulins were higher and the OKT 4/OKT 8 ratio lower in the eight patients with lymphadenopathy than in patients without lymphadenopathy. The prevalence of antibodies to human T-lymphotropic virus (HTLVIII) was measured in 35 hemophiliacs and in 25 polytransfused patients, most of whom were suffering from acute leukemia. In 8 of 35 hemophiliacs antibodies to HTLVIII virus were detected by an enzyme linked immunosorbent assay (ELISA) and confirmatory tests. All seropositive patients were treated by blood products from the United States. Eight hemophiliacs treated by factor concentrates from German donors only were seronegative. In comparison 2 of 25 examined non-hemophilia patients receiving multiple blood products from local donors were seropositive for HTLVIII. The results show that hemophilia patients treated by imported clotting factor concentrates have a high risk of HTLVIII positivity. Hemophiliacs substituted by blood products obtained by local donor pools have only a small risk of infection. Because non-hemophiliac polytransfused patients had HTLVIII antibodies, there must be asymptomatic virus carriers in the local donor pool. The HTLVIII antibody screening of all donors and the heat treating of factor concentrates will give better therapeutic safety.
 ...
PMID:HTLV III antibodies and immunological alterations in hemophilia patients. 300 59

Heat treatment at 60 degrees C for 10 h in solution (pasteurization) was introduced into the manufacturing process of factor VIII concentrate (Haemate P) in order to considerably reduce the risk of transmission of human pathogenic viruses to haemophiliacs. The results of experimental and clinical studies with regard to hepatitis B, non-A, non-B hepatitis, acquired immune deficiency syndrome (AIDS) and herpes virus infections are reviewed. From this data it is concluded that pasteurization of factor VIII results in a product which is safe with regard to these viral infections. Furthermore, it was shown that pasteurization does not form new antigenic determinants on the factor VIII molecule and compared with the native product does not alter the physiological properties of this protein in patients. In comparison to these advantageous properties of the pasteurized product a slight loss of coagulant activity seems to be acceptable. This loss of yield, however, does not influence the quality or the amount of factor VIII in the final container used for the therapy of haemophilia A patients.
 ...
PMID:Pasteurization as an efficient method to inactivate blood borne viruses in factor VIII concentrates. 301 13

Post-transfusion hepatitis is frequent among patients with hemophilia who are treated with concentrated factor VIII prepared from pooled plasma, especially if it is obtained from paid donors. In 26 patients with hemophilia A or von Willebrand's disease who had not been treated with blood or any blood product and hence were highly susceptible to the development of post-transfusion hepatitis, we infused 32 batches of a factor VIII concentrate that had been produced from large pools of human plasma (collected from paid plasmapheresis donors) and then heated in solution at 60 degrees C for 10 hours before final lyophilization. Patients were examined clinically and serologically over a period of 12 months after the first infusion of the pasteurized concentrate. Neither hepatitis nor serologic signs of other viral infections were observed. The hemostatic effectiveness of the concentrate appeared to be satisfactory relative to untreated concentrates.
 ...
PMID:Absence of hepatitis after treatment with a pasteurized factor VIII concentrate in patients with hemophilia and no previous transfusions. 310 63

At a time when the acquired immunodeficiency syndrome as well as hepatitis and other blood-borne diseases are a threat to patients with bleeding disorders who need treatment with blood products, it is rewarding to realize that a number of these patients can be safely and effectively treated with their own desmopressin-stimulated F.VIII:C and vWF. Desmopressin is clinically useful for treatment of patients with moderate and mild hemophilia. The limits of the clinical indications are established by the nature of the bleeding episode, the resting factor level, the level that must be achieved, and the length of time the level must be maintained to manage any given bleeding episode. In von Willebrand disease, desmopressin can be used more extensively to raise F.VIII:C levels than in classic hemophilia, because fewer of the patients have the severe form of the disease that is unresponsive to desmopressin. Increases in the level of F.VIII:C of about four times the resting value can be expected both in hemophilia and von Willebrand disease, but it must be borne in mind that the range of individual responses is large. Even though it is not easy to correct the prolonged bleeding time, particularly in patients with dysfunctional vWF, this drawback is of clinical relevance only in a minority of cases. A role for the use of desmopressin in acquired diseases of primary hemostasis has been proposed more recently, and experience is more limited than in congenital bleeding disorders. Uremia is probably the most firmly established indication because it has been shown that the bleeding time is often dramatically shortened by desmopressin, and hemorrhages can be stopped or prevented before surgical procedures. The indications for use of the compound in liver cirrhosis and congenital and acquired platelet dysfunctions are promising but much less established from a clinical standpoint. The bulk of available clinical experience is based on intravenous administration. Intranasal and subcutaneous administration have been successfully attempted and might be more convenient in selected circumstances, such as home treatment and the stimulation of blood donors to provide more abundant supplies of F.VIII:C and vWF. However, the responses after intranasal administration are less predictable and consistent than after intravenous administration. Desmopressin has few troublesome side-effects. Mild facial flushing, a small increase in heart rate, and, more rarely, mild headache can occur transiently during infusion. Signs of hyponatremia or cerebral edema are extremely rare, providing that excessive fluid intake is avoided.(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:Desmopressin (DDAVP) for treatment of disorders of hemostasis. 310 87

19 (51%) of 37 hemophiliac children and adolescents regularly treated with factor VIII and IX concentrates were positive for HTLV III antibodies. The prevalence of HTLV III antibodies was higher in patients with severe hemophilia (64%) requiring frequent administration of concentrates than in patients with mild hemophilia (17%). No patient showed signs of AIDS or AIDS related complex. Immunologic alterations (inverse ratio of helper- and suppressor lymphocytes, elevated immunoglobulins, and elevated total serum proteins) were more often observed in patients requiring frequent administration of concentrates than in patients requiring relatively infrequent administration of concentrates. Since in patients frequently treated with concentrates the prevalence of HTLV III antibodies was also higher, it was not possible to draw any conclusions whether the observed immunologic alterations are due to the HTLV III infection alone or are also induced by the frequent administration of coagulation factor concentrates. No HTLV III positive person was detected in 45 relatives of 18 HTLV III positive hemophiliac children living together in 16 households and actively participating in the care of those children. In contrast, 6 (11%) of 55 relatives living in 21 households with 23 hepatitis-B-positive hemophiliac children were positive for hepatitis B. Our results support the general impression, that the risk to contract hepatitis B seems to be greater than to contract HTLV III from seropositive patients, and should help to facilitate the social integration of HTLV III positive hemophiliac children.
 ...
PMID:[HTLV-III antibodies and immunologic changes in hemophilic children. Prevalence of HTLV-III antibodies in hemophilic children and their relatives living in the same household]. 310 27

At this time, when the acquired immunodeficiency syndrome, hepatitis, and other blood-borne diseases threaten patients, with bleeding disorders, who need treatment with blood products, it is rewarding to realize that a number of them can be safely and effectively treated through the stimulation of their own VIII:C and vWF production with desmopressin. Desmopressin is clinically useful for treatment of patients with moderate and mild hemophilia. The limits of the clinical indications are the nature of the bleeding episode, the resting factor level, the level that must be achieved, and the length of time the level must be maintained to manage any given bleeding episode. Desmopressin can be used more extensively to raise VIII:C in von Willebrand's disease, than in classic hemophilia, because fewer of the patients have the severe form of the disease that is unresponsive to desmopressin. VIII:C increases to about four times the resting values that can be expected in both hemophilia and von Willebrand's disease, but it must be kept in mind that the range of individual responses is large. Even though it is not easy to correct the prolonged bleeding time, particularly in patients with dysfunctional vWF, this drawback is of clinical importance for only a minority of cases. Use of desmopressin in acquired diseases of primary hemostasis has been proposed more recently, and our experience is more limited than for congenital bleeding disorders. Uremia is probably the most firmly established indication, because the bleeding time is often dramatically shortened by desmopressin, and hemorrhages can be stopped or prevented. The indications for the compound in liver cirrhosis and congenital and acquired platelet dysfunctions are promising but much less well-established. The mechanism of action of desmopressin is not well-known, and more work must be done to fill this important gap. This problem is not only of theoretical importance, because understanding of the mechanism of action of the compound should open up new perspectives into understanding the physiological mechanisms that regulate hemostasis. Many unclarified aspects of the mechanism of desmopressin action might be elucidated by using specific antagonists and also by using appropriate animal models. (Dogs and primates respond partially to desmopressin, but rats and rabbits do not respond at all).(ABSTRACT TRUNCATED AT 400 WORDS)
 ...
PMID:Vasopressin analogues. Their role in disorders of hemostasis. 312 21

The efficiency of heat treatment procedures of factor VIII and factor IX concentrates, prepared from voluntary, non-paid donors by three French Blood Transfusion Centres, on the inactivation of HIV and non-A, non-B hepatitis (NANB) viruses was assessed. Some 43 patients (26 haemophilia A, 17 haemophilia B) were followed for at least 1 year by testing for HIV antibodies and alanine aminotransferase (ALT). No HIV seroconversion was observed indicating that heat treatment was completely efficient. Among 26 haemophiliacs, 6 (4 haemophilia A, 2 haemophilia B) presented an elevation in ALT, indicating only a 75% reduction of NANB viral contamination.
 ...
PMID:Clinical and biological survey of haemophilia A and B patients infused with French heat-treated concentrates. 314 99


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>