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)

Guidelines have been prepared by the National Hemophilia Foundation, USA, for treating patients with haemophilia, these are: 1. General recommendations. The risks of withholding treatment far outweigh risks of treatment. Patients should however be educated to use appropriate clotting factor doses to minimize overuse and contain costs. 2. Factor VIIIC-deficient patients. DDAVP should be used whenever possible by patients with mild or moderate factor VIIIC deficiency. When feasible, an alternative to concentrates may be the use of cryo-precipitate prepared from one well-screened donor or from a small number of such donors. (a) Prevention of hepatitis. Hepatitis B vaccination is essential for uninfected patients. Preliminary data suggest that products that are pasteurized, solvent/detergent-treated or monoclonal antibody-purified are at a reduced risk of transmitting hepatitis viruses. (b) Prevention of HIV-1. Concentrates pasteurized, treated with solvent/detergent, purified with monoclonal antibody, heated in suspension with organic solvents, or dry heat-treated for long periods are preferred. These products carry a substantially reduced risk of transmitting HIV-1. 3. Factor IX deficiency. For patients with severe deficiency the use of virus-inactivated Factor IX concentrate is recommended. For mild to moderate patients when feasible an alternative would be fresh, frozen plasma prepared from one well-screened and repeatedly-tested donor or from a small number of such donors. In the past few years, significant progress has been made in understanding the nature of the defect in haemophilia both at the molecular and structural levels, such a foundation is necessary for definitive treatments in the future. For now, however, the dark side of replacement therapy must be accepted along with its benefits.
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PMID:HIV-1 infection in haemophilia. 210 39

Von Willebrand's disease is a genetic bleeding disorder characterized by either a reduced plasma concentration of von Willebrand's factor (vWF) or a qualitative deficiency in that vWF which is produced. Previous therapy consisted of injecting concentrates of vWF manufactured from the pooled plasma of multiple donors. With the increased incidence and risk of serum borne transmission of such diseases as hepatitis and AIDS, the advantages of an alternative mode of therapy was obvious. In the course of using 1-desamino-8-D-arginine (desmopressin or DDAVP, a synthetic analogue of 8-arginine vasopressin, a hormone secreted in the posterior pituitary gland) in the treatment of diabetes insipidus, it was discovered that this drug causes the release of bound vWF into the plasma. The elevation lasts for several hours and is effective in producing hemostasis in some types of mild to moderate von Willebrand's disease. In 1984, desmopressin was approved for this usage in the United States. This paper discusses the use of DDAVP in the management of von Willebrand's disease and present two case reports of patients with von Willebrand's disease and in need of periodontal surgery.
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PMID:The use of desmopressin in the management of two patients with von Willebrand's disease undergoing periodontal surgery. 2 case reports. 232 24

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

Of 120 patients presenting with mild bleeding disorders, 63 were found to have a definite coagulopathy. The commonest disorders were haemophilia, Christmas disease and von Willebrand's disease (vWd), the latter being predominant. Diagnosis led to prophylactic treatment prior to surgery in 18 patients with prevention of excessive haemorrhage. Three patients who had received blood products developed hepatitis. DDAVP (desamino-cys-1-8-D-arginine vasopressin) is the treatment of choice in suitable mildly affected patients with haemophilia A and vWd. Examination of blood group distribution suggests an excess of group O among patients with bleeding disorders, especially those with vWd.
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PMID:Mild bleeding disorders: review of 120 patients. 633 71

von Willebrand's disease (vWD) is the most frequent inherited disorder and is the result of a deficiency and/or abnormality of von Willebrand factor (vWF). As a consequence, the level of factor VIII, the presence of which reflects the ability of vWF to stabilise it, is usually low. Bleeding time, which reflects the ability of vWF to promote platelet adhesion to subendothelium, is therefore prolonged. However, from a therapeutic point of view, it appears that the correction of factor VIII and bleeding time is sufficient to prevent or treat bleeding in these patients. There are 2 main therapeutic tools to improve or normalise these major determinants of the bleeding tendency in this disorder. The majority of patients, identified by a test infusion, can be successfully treated by giving desmopressin (DDAVP), a synthetic analogue of vasopressin. Desmopressin is able to induce the increase of autologous vWF released from endothelial cells, leading to the correction of factor VIII levels and of bleeding time. In the remaining cases, blood products, namely factor/vWF concentrates, are required to accomplish haemostasis. All these concentrates are able to correct factor VIII levels, whereas their effect on bleeding time may not be consistent. The modern virucidal techniques abolish the risk of transmission of blood-borne viruses (e.g. hepatitis viruses and HIV) and make these products safer than blood-bank cryoprecipitate.
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PMID:Current management of von Willebrand's disease. 853 49

While the function of each organ is used by each transplant team to assess suitability for transplantation, little is known about the donor characteristics and clinical interventions that contribute toward overall organ transplantation potential. We conduct a retrospective review of United Network for Organ Sharing (UNOS) deceased donor registry data from January 2005 to December 2006. This registry contains all deceased donors from whom organs were recovered during this time period (n = 15,601). Ordinary least-squares (OLS) regression models using variables in the registry are estimated to predict the number of organs transplanted. Outcome is the number of organs transplanted per donor. Organ yield is found to depend significantly on donor age, anoxia as cause of death, history of myocardial infarction (MI), hypertension and/or diabetes, body mass index (BMI), B or AB blood type, cocaine and/or cigarette use and hepatitis infection (p < 0.01). In addition, the clinical interventions of steroid administration, desmopressin (DDAVP) and diuretic usage, as well as oxygenation, are associated with organ yield. Both intrinsic donor characteristics and medical management practice are observed to be highly variable across organ procurement organizations (OPOs). These findings may provide important information to explore and assess the efficacy of clinical interventions, compare OPO performance and point to best practices.
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PMID:Deceased organ donor characteristics and clinical interventions associated with organ yield. 1834 85

The evolution of comprehensive care centres for haemophilia has altered the work of those involved in the care of haemophilia patients. The role of the haemophilia sister has expanded and the concept of the haemophilia nurse specialist has emerged. We describe the implementation of a local policy which has enabled haemophilia nurse specialists to requisition and administer clotting factor concentrates, DDAVP, tranexamic acid and hepatitis vaccines independently, in hospital and community settings. Since the introduction of this policy on 2 August 1994, prescribing practice has not changed in the haemophilia centre, nor has the involvement of medical staff in the care of haemophilia patients. This approach is widely applicable in other centres and should be a logical progression of the haemophilia nurse specialist's role.
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PMID:Implementation of a nurse practitioner policy for the requisition and administration of drugs in a haemophilia comprehensive care centre. 2721 34