deamino-arginine-vasopressin and Acquired-Immunodeficiency-Syndrome

Although the nature of haemophilia has been understood for thousands of years, knowledge of its molecular genetics is recent. These X-linked bleeding disorders have diverse underlying DNA defects and, in 1992, DNA inversion within the X chromosome was found to explain half of the most serious cases of haemophilia A. The life-span and quality-of-life for patients with haemophilia had improved steadily throughout the early 1980s but the principal cause of death remained intracranial haemorrhage until the epidemic of HIV infection due to contaminated factor concentrates. Infection with hepatitis C virus is almost universal for patients treated with clotting factors before 1985. No curative treatment is available for hepatitis C at present. Knowledge of the transmission of viruses in concentrates has led to important developments in processing techniques to eliminate them. Recombinant technology has produced factor VIII and, more recently, factor IX concentrate which is likely to be very safe. Development of inhibitors to factor concentrates (especially factor VIII) remains one of the most serious complications of haemophilia. The variety of treatments available testifies to the lack of a single universally efficacious one. The use of prophylactic treatment has been conclusively demonstrated to result in a preservation of joint function in severely affected patients who might otherwise develop significant joint problems. The many facets of the care of patients with severe haemophilia, ranging from dental care to genetic counselling, can be advantageously co-ordinated in a haemophilia comprehensive care centre.

Topics: Acquired Immunodeficiency Syndrome; Cause of Death; Cerebral Hemorrhage; Contraindications; Deamino Arginine Vasopressin; Drug Contamination; Factor IX; Factor VIII; Female; Hemophilia A; Hepatitis C; Humans; Hypoglycemic Agents; Male; Recombinant Proteins; United Kingdom

The emergence of the acquired immunodeficiency syndrome (AIDS) has fueled concerns of both physicians and their patients about safety of blood transfusions. Although AIDS has generated the most fear, the risk today is extremely remote (1/60.000 units of blood). The risk of transmitting infectious disease by homologous transfusion is decreasing, as more donor screening and testing measures are implemented. The blood supply is safer that at any time, but small transfusion risks exist. The most common problems associated with transfusions are temporary: one in 100-300 recipients will experience fever or rash. The biggest problem is a mismatch of the well-known ABO blood groups and once in every 100-400.000 transfusions the hemolytic reaction is fatal. Viral hepatitis is another serious and important risk. At present hepatitis seems to strike between 1 and 3 percent of transfusion recipients. Most, if not all, of transfusion-associated hepatitis cases are caused by hepatitis C virus. Cytomegalovirus can cause primary infection, reactivation or reinfection by transfusion. Immunosuppressed patients are more likely to develop more severe disease. Epstein-Barr virus does not seem to cause significant post-transfusion disease. Bacterial or protozoal infections are an infrequently encountered adverse effect of transfusion. However, some clinical cases document the potential hazard of blood components as a vector for bacteria or protozoa. Homologous blood transfusion down-regulates some immune functions. Host defences against malignancy and infection may in some instances be severely compromised by transfusions of homologous blood.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Acquired Immunodeficiency Syndrome; Adult; Blood Donors; Blood Transfusion, Autologous; Deamino Arginine Vasopressin; Epoprostenol; Erythropoietin; Female; Graft vs Host Disease; Hemodilution; Hepatitis, Viral, Human; Humans; Infant, Newborn; Infections; Male; Pregnancy; Prognosis; Protozoan Infections; Risk Factors; Transfusion Reaction

Topics: Acquired Immunodeficiency Syndrome; Analgesics; Anti-Inflammatory Agents; Antifibrinolytic Agents; Blood Transfusion; Child; Child, Preschool; Danazol; Deamino Arginine Vasopressin; Factor IX; Factor VIII; Fibrinogen; Hemarthrosis; Hemophilia A; Hemorrhage; Humans; Infant; Infant, Newborn; Male; Plasma; von Willebrand Diseases

Treatment of hemophilia and von Willebrand's disease has become easier in recent years because of the development of more effective factor replacement products. The median age and the life expectancy of patients with hemophilia have risen markedly, as has the median age at death.

Topics: Acquired Immunodeficiency Syndrome; Antigens; Blood Coagulation Factors; Cerebral Hemorrhage; Danazol; Deamino Arginine Vasopressin; Factor VIII; Female; Gastrointestinal Hemorrhage; Hemophilia A; Hemorrhage; Hepatitis B; Hepatitis C; Humans; Pregnancy; Surgical Procedures, Operative; Thrombosis; Tooth Extraction; von Willebrand Diseases

Topics: Acquired Immunodeficiency Syndrome; Adult; AIDS-Related Opportunistic Infections; Antiviral Agents; Cytomegalovirus Retinitis; Deamino Arginine Vasopressin; Diabetes Insipidus, Nephrogenic; Diarrhea; Foscarnet; Ganciclovir; Humans; Male; Osmolar Concentration; Polyuria; Reverse Transcriptase Inhibitors; Urine

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. Surgic

Topics: Acquired Immunodeficiency Syndrome; Adult; Deamino Arginine Vasopressin; Factor VIII; Hemarthrosis; Hemophilia A; Hepatitis, Viral, Human; Humans; Knee Prosthesis; Male

Topics: Acquired Immunodeficiency Syndrome; Blood Coagulation Factors; Blood Transfusion; Deamino Arginine Vasopressin; Genetic Carrier Screening; Hemophilia A; Humans; Patient Care Team; Prenatal Diagnosis

Topics: Acquired Immunodeficiency Syndrome; Antigens; Blood Coagulation Tests; Blood Transfusion; Child; Deamino Arginine Vasopressin; Factor IX; Factor VIII; Female; Genetic Carrier Screening; Hemophilia A; Hepatitis B; Humans; Pregnancy; Prenatal Diagnosis; von Willebrand Diseases; von Willebrand Factor