deamino-arginine-vasopressin and Hemarthrosis

It is important to discard those practices that do not add value. As a result, several initiatives have emerged. All of them try to improve patient safety and the use of health resources.. To present a compendium of "do not do recommendations" in the context of hemophilia.. A review of the literature and current clinical guidelines has been made, based on the best evidence available to date.. The following 13 recommendations stand out: 1) Do not delay the administration of factor after trauma; 2) do not use fresh frozen plasma or cryoprecipitate; 3) do not use desmopressin in case of hematuria; 4) do not change the product in the first 50 prophylaxis exposures; 5) do not interrupt immunotolerance; 6) do not administer aspirin or NSAIDs; 7) do not administer intramuscular injections; 8) do not do routine radiographs of the joint in case of acute hemarthrosis; 9) Do not apply closed casts for fractures; 10) do not discourage the performance of physical activities; 11) do not deny surgery to a patient with an inhibitor; 12) do not perform instrumental deliveries in fetuses with hemophilia; 13) do not use factor IX (FIX) in patients with hemophilia B with inhibitor and a history of anaphylaxis after administration of FIX.. The information mentioned previously can be useful in the management of hemophilia, from different levels of care. As far as we know, this is the first initiative of this type regarding hemophilia.

Topics: Anti-Inflammatory Agents, Non-Steroidal; Aspirin; Blood Coagulation Factors; Deamino Arginine Vasopressin; Disease Management; Exercise; Factor VIII; Fibrinogen; Hemarthrosis; Hemophilia A; Hemostatics; Humans; Practice Guidelines as Topic

Treatment of mucosal bleeding (epistaxis, gastrointestinal bleeding, and menorrhagia) and joint bleeding remains problematic in clinically severe von Willebrand disease (VWD). Patients are often unresponsive to treatment (e.g. desmopressin or antifibrinolytic therapy) and may require von Willebrand factor (VWF) replacement therapy. There are little data on the use of prophylaxis in VWD, and none have been applied in a prospective, treatment escalation design.. Evaluate the effect of escalating dose prophylaxis in severe VWD.. Patients eligible for enrollment in this prospective study included those with type 1 VWD with VW factor activity-ristocetin cofactor ratio ≤ 20% and unresponsive to desmopressin, patients with type 2 VWD not responsive to desmopressin and all subjects with type 2B and type 3 VWD. Entry criteria were strictly defined, as were therapy escalation parameters and clinical data collection.. Eleven subjects completed the study. Six had type 2A, and five had type 3 VWD. Six patients presented with epistaxis, three with GI bleeding, and two with joint bleeding. Seven had dose escalation above the first level. Among the 10 subjects with evaluable bleeding log data, use of prophylaxis decreased the median annualized bleeding rate from 25 to 6.1 (95% confidence interval of the rate difference: -51.6 to -1.7), and the median annualized bleeding rate was even lower (4.0; 95% confidence interval: -57.5 to -5.3) when the subjects reached their final dosing level.. This is the first prospective study to demonstrate that prophylaxis with VW factor concentrates is highly effective in reducing mucosal and joint bleeding rates in clinically severe VWD.

Topics: Blood Loss, Surgical; Blood Transfusion; Clinical Trials as Topic; Deamino Arginine Vasopressin; Drug Administration Schedule; Factor VIII; Female; Hemarthrosis; Hemorrhage; Hospitalization; Humans; Male; Menorrhagia; Multicenter Studies as Topic; Postoperative Hemorrhage; Prospective Studies; Recombinant Proteins; Retrospective Studies; von Willebrand Diseases; von Willebrand Factor

Patients with severe forms of von Willebrand's disease (VWD) may have frequent haemarthroses, especially when factor VIII (FVIII) levels are below 10 U/dL, so that some of them develop target joints like patients with severe haemophilia A. Some patients have recurrent gastrointestinal bleeding, often without lesions in the gastrointestinal tract, and need treatment every day or every other day. Finally, there are children who have epistaxis frequently and severely enough to cause anaemia. In these frequent and severe bleeders, the optimal therapy may be secondary long-term prophylaxis with von Willebrand factor (VWF)/FVIII concentrates rather than on-demand treatment on the occasion of bleeding episodes. The largest experience on such prophylaxis in VWD has been in Sweden in 35 patients with severe forms of VWD. Long-term prophylaxis was also implemented in a cohort of Italian patients with VWD: prophylaxis was used in seven patients with types 3 (n = 1 ), 2A (n = 4), 2M (n = 1) and type 1 (n = 1) VWD because of recurrent gastrointestinal bleeds and in four patients with type 3 VWD because of joint bleeds. Prophylaxis prevented bleeding completely in eight patients and largely reduced hospitalisation for blood transfusions in the remaining three. The cost-effectiveness of these prophylaxis regimens versus on-demand therapy will now be investigated in one large international study

Topics: Clinical Trials as Topic; Deamino Arginine Vasopressin; Drug Administration Schedule; Drug Combinations; Epistaxis; Factor VIII; Female; Fibrinogen; Gastrointestinal Hemorrhage; Hemarthrosis; Hemorrhage; Humans; Male; Multicenter Studies as Topic; Prospective Studies; Retrospective Studies; Secondary Prevention; von Willebrand Diseases; von Willebrand Factor

Recent years have witnessed advances in the treatment of haemophilia such as the introduction of prophylaxis, continuous infusion and pharmacological treatment with desmopressin (DDAVP). Prophylactic treatment on a long-term basis appears to be effective in preventing the development of arthropathy in severe haemophilia. The largest body of experience is that from Sweden, where prophylaxis is started at the age of 1-2 years. The dosage used is 25-40 U factor VIII/IX per kilogram bodyweight given three times or twice weekly, respectively. In some cases an intravenous access device has to be used during the first years of treatment. The patients grow up like normal boys and can live virtually normal lives. The beneficial psychological impact of prophylaxis on the family cannot be overestimated. Side-effects are not more frequent with prophylaxis than with on-demand treatment. The feasibility of continuous infusion of factor VIII/IX concentrates during bleeding episodes, or as cover for surgery, has been documented. This mode of delivery increases convenience and the cost-benefit ratio of the treatment, with savings in postoperative replacement of factor concentrate of about 50-75%. Many modern concentrates are stable enough for the purpose, and several pump systems, including portable ones, are available. The haemostatic drug DDAVP can be effectively used in most cases of mild haemophilia A. Intravenous administration is to be preferred as cover for surgery or in the case of severe bleeds. There is an effective nasal spray which can also be used for home therapy in mild or moderate bleedings.

Topics: Adolescent; Catheterization, Central Venous; Catheterization, Peripheral; Child; Child, Preschool; Deamino Arginine Vasopressin; Factor IX; Factor VIII; Female; Hemarthrosis; Hemophilia A; Hemophilia B; Hemorrhage; Hemostatics; Humans; Infant; Infusions, Intravenous; Injections, Intravenous; Male; Practice Guidelines as Topic; Treatment Outcome; World Health Organization

Over the past few years considerable progress has been made in elucidating the molecular genetics of hemophilia A, in carrier detection and prenatal diagnosis, and in the production of safer clotting factor concentrates. Recombinant FVIII, shown to be safe and effective in ongoing prelicensure clinical trials that began in the spring of 1987, should soon be licensed and commercially available. There is now considerable interest in beginning prophylactic therapy regimens at 1 or 2 years of age, in an attempt to prevent chronic joint disease and other complications of serious bleeding episodes. The possibility of gene insertion therapy for persons with hemophilia now seems to be a realistic one--perhaps achievable in the 1990s. Although many problems remain--major problems resulting from HIV, HCV, and HBV infections; how to deal with existing musculoskeletal problems; how to pay for the higher-priced new technologies; high titer inhibitors; just to name a few--the many recent scientific advances and their clinical applications make this an exciting time. This is truly, as indicated in the title of the proceedings of the XIX Congress of the World Federation of Hemophilia, a new decade of hopes and challenges.

Topics: Child, Preschool; Deamino Arginine Vasopressin; Dental Care; Factor VIII; Fetal Diseases; Hemarthrosis; Hemophilia A; Hemorrhage; Humans; Infant; Infant, Newborn; Prenatal Diagnosis; Prevalence; Recombinant Proteins; Virus Diseases

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

Approximately 50% of female carriers of hemophilia A have factor VIII (FVIII) levels below 0.5 IU/dL and may be categorized as having mild hemophilia. Females with hemophilia may go undiagnosed for years because the most common symptoms - menorrhagia and bleeding after childbirth - also occur in females without hemophilia. Females with hemophilia can exhibit increased bleeding tendencies despite current guidelines of expected, adequate FVIII levels. The cases described illustrate the clinical variability and presentation of hemophilia in females and highlight the importance of a timely diagnosis, effective management, and monitoring. Prophylactic factor replacement therapy is recommended in females with hemophilia, particularly those with joint disease or gynecologic complications. Affected individuals should receive infusion training and education on treatment options, physical activities, the importance of treatment adherence, and recognizing bleeding symptoms warranting treatment. Further study is needed to increase awareness of hemophilia in females and reassess current guidelines for their management and monitoring.

Topics: Adolescent; Aged; Chromosome Inversion; Deamino Arginine Vasopressin; Disease Management; Drug Substitution; Factor VIII; Female; Hemarthrosis; Hemophilia A; Heterozygote; Humans; Introns; Male; Menorrhagia; Middle Aged; Pedigree; Recombinant Proteins; Tranexamic Acid; Young Adult

A 26 year old woman presented to the accident and emergency department with a painful right elbow. There had been no history of trauma. Clinical examination suggested an effusion, which was confirmed on radiological examination. Her elbow was aspirated and revealed a haemarthrosis. Subsequent investigations revealed a diagnosis of von Willebrand's disease (vWD). A spontaneously occurring effusion of the elbow may be due to a haemarthrosis. Aspiration of blood in the absence of trauma may lead to a diagnosis of an occult coagulopathy in addition to relieving pain. The diagnosis and treatment of vWD is discussed.

Topics: Acute Disease; Adult; Arthralgia; Deamino Arginine Vasopressin; Diagnosis, Differential; Elbow Joint; Female; Hemarthrosis; Hemostatics; Humans; von Willebrand Diseases

Topics: Administration, Intranasal; Adolescent; Adult; Child; Child, Preschool; Cost-Benefit Analysis; Deamino Arginine Vasopressin; Europe; Factor VIII; Female; Health Care Costs; Hemarthrosis; Hemophilia A; Humans; Infant; Infusion Pumps; Injections, Intravenous; Japan; Male; Radiography; Severity of Illness Index; Treatment Outcome; United States; von Willebrand Factor

Haemophilia is a rare and complex disorder and its successful management will depend upon the establishment of a network of 'comprehensive care' including the services of haematologists, orthopaedic surgeons, rheumatologists, dental surgeons, physiotherapists, specialised nurses and counsellors. One of the major lessons to be learned from the HIV epidemic in haemophilia is that it is critical to strive to obtain the safest and purest forms of blood products for these patients. The advent of clinically available recombinant factor VIII is expected soon; in the meantime there is a move towards treating all patients with high purity products.

Topics: Cerebral Hemorrhage; Deamino Arginine Vasopressin; Factor IX; Factor VIII; Genetic Counseling; Hemarthrosis; Hemophilia A; Hemophilia B; Humans; Male; Patient Care Team; Prevalence; Transfusion Reaction; Virus Diseases; von Willebrand Diseases

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