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Pocket Guide to the Diagnosis, Evaluation, and Management of von Willebrand Disease. Bethesda, MD: NHLBI Health Information Center. 2008. 15 p.
This pocket guide summarizes guidelines for the diagnosis, evaluation, and management of von Willebrand disease (VWD), an inherited bleeding disorder that is caused by a deficiency or dysfunction of von Willebrand factor (VWF). VWF is a plasma protein that mediates the initial adhesion of platelets at sites of vascular injury and also binds and stabilizes blood clotting factor VIII in the circulation. Defects in VWF can cause bleeding by impairing platelet adhesion or by reducing the concentration of blood clotting factor VIII. This brochure offers a summary of the clinical practice guidelines developed by an expert panel convened by the National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health (NIH) from the United States Expert Panel. Topics include the initial evaluation of the patient for VWD or other bleeding disorders, laboratory assessment, making the diagnosis of VWD, and management considerations, including general management of VWD patients, treatment of minor bleeding and prophylaxis for minor surgery, treatment of major bleeding and prophylaxis for major surgery, management of menorrhagia and hemorrhagic ovarian cysts in women with VWD, management of pregnancy and childbirth, and caring for patients with acquired von Willebrand syndrome (AVWS). All of the information is presented in figures or tabular format, or bulleted columns, for ease of access. A final section refers readers to the NHLBI Health Information Center at 301–592–8573 or www.nhlbi.nih.gov for more information. 2 figures. 1 table.
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ABC of Clinical Hematology. Williston, VT: Blackwell Publishing Inc. 2007. 99 p.
This book on clinical hematology is from a series of resource books written by specialists for nonspecialists. The book is designed to be easy to use and covers the symptoms, investigations, treatment, and management of conditions presenting in day-to-day practice. The book includes fifteen chapters covering iron deficiency anemia, macrocytic anemias, hereditary anemias, polycythemia, essential thrombocythemia and myelofibrosis, chronic myeloid leukemia, the acute leukemias, platelet disorders, the myelodysplastic syndromes, multiple myeloma and related conditions, bleeding disorders, thrombosis and anticoagulation, lymphoproliferative disorders including chronic lymphocytic leukemia, stem cell transplantation, hematological disorders at the extremes of life, hematological emergencies, and the impact of molecular biology and gene therapy on the field of hematology. The book includes full-color photographs and illustrations. Each chapter concludes with a list of references, and a detailed subject index appears at the end of the text.
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All About Von Willebrand Disease...For People With Von Willebrand Disease And Their Families. 2nd ed. Montreal, QC: Canadian Hemophilia Society. 2007. 87 p.
This monograph from the Canadian Hemophilia Society provides comprehensive information about von Willebrand disease (VWD), a disease characterized by problems in the von Willebrand factor (VWF). The VWF is a protein in the blood that is necessary for proper blood coagulation. When there is not enough VWF in the blood, or when it does not work the way it should, the blood takes longer to clot. This booklet reviews the different types of VWD, the role of heredity, the symptoms, diagnostic tests that can confirm the condition, treatment options, and strategies for healthy living with VWD. The lifestyle factors discussed include the safety of blood products, coping with nose bleeds, conception, pregnancy and childbirth, medications to avoid, exercise, fitness and sports, child care, schooling, employment, insurance, traveling, and medical identification. The booklet concludes with the contact information for the Canadian Hemophilia Society, a list of hemophilia or bleeding disorders treatment centers in Canada, a glossary of terms, a subject index, and a bibliography. The booklet includes black-and-white illustrations. 6 references.
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Bleeding Disorders, Thrombosis and Anticoagulation. IN: Provan, D., ed. ABC of Clinical Haematology. Williston, VT: Blackwell Publishing Inc. 2007. pp. 52-56.
Blood within the circulation must remain fluid, but if a blood vessel is damaged, localized coagulation must take place to prevent blood loss. This complex interacting system can be disturbed by inherited or acquired factors, resulting in bleeding or thrombotic disorders. This chapter about bleeding disorders, thrombosis, and anticoagulation is from a book on clinical hematology, written by specialists for nonspecialists. The book is designed to be easy to use and covers the symptoms, investigations, treatment, and management of conditions presenting in day-to-day practice. In this chapter, the author outlines the approach to a patient with a suspected bleeding disorder, which should include a medical history, examination, coagulation screening tests, and specialist coagulation tests. The chapter also covers congenital bleeding disorders, acquired bleeding disorders, venous thromboembolism, the inherited thrombophilias, and treatment strategies for venous thromboembolism. The chapter is illustrated with full-color photographs, drawings, and charts. 3 figures. 7 tables. 6 references.
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Care and Treatment of Carriers. Montreal, Quebec: Canadian Hemophilia Society. 2007. pp 79-92.
This chapter on the care and treatment of carriers is from a monograph that provides information for female carriers of hemophilia A and B, a type of genetic bleeding disorder traditionally experienced by males but passed on genetically by females. Recent understanding of this disorder shows that females can have the same problems as males with mild hemophilia, such as hemorrhaging after surgery or trauma. Women with clotting factor levels as high as 60 percent can have abnormal bleeding problems, including but not restricted to gynecological and obstetrical bleeding. This chapter describes the multidisciplinary clinics that are being created in Canada for women with bleeding disorders and the medical and surgical options available to women to treat their bleeding symptoms. The chapter briefly reviews the roles of the patient care team, including a hematologist, a gynecologist, an obstetrician, and a nurse coordinator who arranges testing and meetings with the specialists. Medical options discussed include hormone therapy, desmopressin, antifibrinolytic agents such as tranexamic acid (Cyklokapron), clotting factor concentrates, and complementary medicines. Surgical options for menorrhagia discussed include uterine ablation, hysterectomy, laparoscopic endometrial removal, oophorectomy, and dilation and curettage (D and C). 1 figure. 4 tables.
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Complementary and Alternative Medicine. Montreal, Quebec: Canadian Hemophilia Society. 2007. pp 93-102.
This chapter on complementary and alternative medicine is from a monograph that provides information for female carriers of hemophilia A and B, a type of genetic bleeding disorder traditionally experienced by males but passed on genetically by females. Recent understanding of this disorder shows that females can have the same problems as males with mild hemophilia, such as hemorrhaging after surgery or trauma. Women with clotting factor levels as high as 60 percent can have abnormal bleeding problems, including but not restricted to gynecological and obstetrical bleeding. This chapter describes alternative or holistic treatments people can use to complement their conventional medical care. The authors review many natural and pharmaceutical products that can actually cause or worsen bleeding in carriers of hemophilia A and B. Specific complementary practices discussed include stress reduction techniques, aromatherapy, massage, Ayurvedic medicine, naturopathic medicine, traditional Chinese/Asian medicine, homeopathy, and botanical medicine. One section briefly discusses remedies that are contraindicated for people with bleeding disorders, including alcohol; aspirin; danshen; dong quai, or Chinese angelica; ephedra ephedrine, or Ma Huang; fenugreek; feverfew; garlic in capsule form, safe as a food; ginger in capsule form, safe as a food; ginkgo biloba; ginseng; horse chestnut; ibuprofen; jingui huayu; kava; matricaria recutita, or chamomile; omega 3 essential fatty acids; papaya; pineapple; and vitamin E. Readers are encouraged to work in tandem with their health care providers when incorporating any alternative or complementary approaches.
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Factor XI Deficiency: An Inherited Bleeding Disorder. Montreal, QC: Canadian Hemophilia Society. 2007. 24 p.
This document provides comprehensive information about Factor XI deficiency, a rare blood coagulation disorder. The authors review the history of this disorder, which was described in 1953, and consider the transmission of Factor XI deficiency, which is an inherited disorder. A series of charts show the percentage possibility of passing on the disorder, depending on the genes carried by each parent. Additional topics describe normal blood clotting and the role of coagulation factors, the incidence of Factor XI deficiency, diagnostic strategies, symptoms, problems specific to women with Factor XI deficiency, and treatment options. A variety of treatment and prevention options are available, including fresh frozen plasma, Factor XI concentrate, hormone therapy, desmopressin (DDAVP), and tranexamic acid––Cyklokapron. One section offers practical strategies for preventing excessive bleeding during dental care and reviews first aid to be administered in common situations such as nosebleeds. People with Factor XI are advised to wear a medical identification tag, to be vaccinated regularly, and to work closely with their team of health care providers. The document concludes with the contact information for the Canadian Hemophilia Society and a bibliography. 6 figures. 12 references.
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Fibrinolytic Inhibitors in the Management of Bleeding Disorders. Montreal, Quebec: World Federation of Hemophilia. 2007. 16 p.
This monograph describes the use of fibrinolytic inhibitors in the management of bleeding disorders. The author focuses on the synthetic derivative of the amino acid lysine, called epsilon-aminocaproic acid (EACA), and the more potent tranexamic acid (TA, also known as AMCA). The naturally occurring aprotinin is only briefly discussed because it is not principally used in patients with bleeding disorders. Topics include the activation of the fibrinolytic system, inhibitors in the fibrinolytic system, the pharmaceutical fibrinolytic inhibitors EACA and TA, and clinical uses in gynecology, bleeding in the gastrointestinal tract, bleeding in the nose and mouth, dental surgery, and major surgery. The author concludes that research studies support the beneficial effects of antifibrinolytic agents, most often TA, in patients apparently without congenital or acquired bleeding diseases. TA can be administered intravenously, orally, or topically, and no severe side effects have been documented. A table of abbreviations and list of references conclude the document. 1 figure. 1 table. 110 references.
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Haemophilia A and Haemophilia B. IN: Roberts H.R., ed. Haemophilia and Haemostasis: A Case-Based Approach to Management. Williston, VT: Blackwell Publishing Inc. 2007. pp 3-92 .
This chapter about hemophilia A and hemophilia B is from a book that compiles a breadth of questions relating to perplexing or complicated management questions in the fields of hemophilia and hemostasis. The contributing authors have provided practical, hands-on answers to the questions sent in by practitioners in the field. This section discusses hemophilia and immune tolerance therapy, the hemophilic ankle and knee, combined hemophilia A and B carriers, hemophilia with HIV and hepatitis C, a premature infant with hemophilia, pregnancy in women with hemophilia, mild hemophilia in women, anticoagulation and hemophilia, hemophilia and scuba diving, and treatment of hemophilia and specific conditions, including hepatitis C, physical therapy, renal bleeds, ventricular septal defect repair, hepatitis C and recurrent bleeding, isotretinoin use, laser eye surgery, pseudotumors, and continuous infusion. A final section considers complications of treatment for hemophilia A and hemophilia B. Much of the information is presented through case studies. 11 figures. 55 references.
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Haemophilia and Haemostasis: A Case-Based Approach to Management. Williston, VT: Blackwell Publishing Inc. 2007. 229 p.
This book compiles a breadth of questions relating to perplexing or complicated management questions in the fields of hemophilia and hemostasis. The contributing authors have provided practical, hands-on answers to the questions sent in by practitioners in the field. The book is organized into seven sections: hemophilia A and hemophilia B, Von Willebrand disease, factor deficiencies, rare platelet and coagulation disorders, acquired bleeding diatheses, miscellaneous questions, and thrombotic disorders. Specific topics covered include the hemophilic ankle and knee, hemophilia with HIV, pregnancy in women with hemophilia, hemophilia and scuba diving, hemodialysis, hepatitis in people with hemophilia, VWD and pregnancy, Factor VIII deficiency, Factor X deficiency, Gardner-Diamond syndrome, cocaine and DDAVP, selective serotonin reuptake inhibitors (SSRIs) and clotting disturbances, the work-up for children with intracranial bleeding, treatment of antithrombin deficiency, recurrent thrombosis, and thromboembolic disease. Most sections include references, and a detailed subject index concludes the volume.
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Introduction to Hemophilia. Montreal, Quebec: Canadian Hemophilia Society. 2007. pp 1-20.
This introductory chapter is from a monograph that provides information for female carriers of hemophilia A and B, a type of genetic bleeding disorder traditionally experienced by males but passed on genetically by females. Recent understanding of this disorder shows that females can have the same problems as males with mild hemophilia, such as hemorrhaging after surgery or trauma. Women with clotting factor levels as high as 60 percent can have abnormal bleeding problems, including but not restricted to gynecological and obstetrical bleeding. This chapter answers common questions about who is affected by hemophilia, the number of people affected, the symptoms of hemophilia, the types and severity of hemophilia, other bleeding disorders, blood clotting, the treatments for hemophilia, common complications, the life expectancy of people with hemophilia, and the future of hemophilia care. Treatments discussed including clotting factor therapy, desmopressin acetate for factor VIII deficiency, and antifibrinolytics for both factor VIII and IX deficiency. Complications can include the development of inhibitors, joint disease, gynecological complications, and bloodborne infections. Readers are referred to a more detailed guide for families at www.hemophilia.ca/en/13.1.php.
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Miscellaneous Questions. IN: Roberts, H.R., ed. Haemophilia and Haemostasis: A Case-Based Approach to Management. Williston, VT: Blackwell Publishing Inc. 2007. pp 173-189.
This chapter of miscellaneous topics is from a book that compiles a breadth of questions relating to perplexing or complicated management questions in the fields of hemophilia and hemostasis. The contributing authors have provided practical, hands-on answers to the questions sent in by practitioners in the field. This section discusses bleeding time versus PFA-100, cocaine and DDAVP, hyponatremia and DDAVP, the use of the international normalized ratio (INR) to assess patients both on and not on coumadin, selective serotonin reuptake inhibitors (SSRIs) and clotting disturbances, and the recommended work-up for children with intracranial bleeding. Much of the information is presented through case studies. 18 references.
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Symptomatic Carriers. Montreal, Quebec: Canadian Hemophilia Society. 2007. pp 35-42.
This chapter on symptomatic carriers is from a monograph that provides information for female carriers of hemophilia A and B, a type of genetic bleeding disorder traditionally experienced by males but passed on genetically by females. Recent understanding of this disorder shows that females can have the same problems as males with mild hemophilia, such as hemorrhaging after surgery or trauma. Women with clotting factor levels as high as 60 percent can have abnormal bleeding problems, including but not restricted to gynecological and obstetrical bleeding. This chapter explains why hemophilia carriers have bleeding problems, why some carriers bleed more than others, and what types of bleeding carriers may experience. Specific topics include symptoms, menorrhagia, the process of X-inactivation, and the link between menorrhagia and bleeding disorders. The authors note that approximately 20 percent of carriers are symptomatic to some degree. However, clotting factor VIII and IX levels can range from 5 percent to 200 percent of normal. Carriers of hemophilia experience more spontaneous and unprovoked hemorrhages and the risk is highest in those with the lowest clotting factor levels. 1 table.
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Bleeding Disorders Caused by Vascular and Platelet Abnormalities. IN: Hoffbrand, A.V.; Moss, P.A.H.; Pettit, J.E. Essential Haematology. 5th ed. Williston, VT: Blackwell Publishing Inc. 2006. pp. 278-289.
This chapter on bleeding disorders caused by vascular and platelet abnormalities is from a hematology textbook that offers a comprehensive look at the biochemical, physiological, and immunological processes involved in normal blood cell formation and function and the disturbances that may occur in different diseases. The authors consider vascular bleeding disorders, thrombocytopenia, disorders of platelet function, the diagnosis of platelet disorders, and the use of platelet transfusions. Vascular and platelet disorders tend to be associated with bleeding from mucous membranes and into the skin, whereas in coagulation disorders, the bleeding is often into joints or soft tissue. The chapter features full-color illustrations and photographs. 11 figures. 3 tables. 11 references.
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Bleeding Disorders in Premenopausal Women: (Another) Public Health Crisis for Hematology?. Washington, DC: American Society of Hematology. 2006. pp. 474-485.
This article reviews bleeding disorders in premenopausal women, a potentially major public health problem. The authors note that up to 20 percent of women with menorrhagia may have an underlying bleeding disorder, which is a prevalence of 1.5 to 4 million American women. Von Willebrand disease (VWD) is the most common bleeding disorder among women with menorrhagia, affecting up to 20 percent of such patients. Besides menorrhagia, important complications of bleeding disorders in premenopausal women include iron deficiency anemia, miscarriage, postpartum bleeding, uterine bleeding and hysterectomy. These patients face many obstacles in achieving optimum care. Recognition is difficult as women may consider their symptoms a normal part of their monthly menstrual cycle and thus come to medical attention only after serious bleeding events. Symptoms of VWD may also overlap with benign conditions; primary providers may not suspect the diagnosis, and convenient hematologic input may be unavailable. Diagnosis is difficult as there is no single definitive test for VWD, and test results are variable, often being affected by factors such as stress, contraceptives, hormones, and pregnancy. Hemostatic treatment is limited by DDAVP tachyphylaxis, the lack of recombinant VWD concentrates, and the ineffectiveness of hormonal therapy, leading to unnecessary procedures and early hysterectomy. The authors conclude by discussing the significant controversy that exists regarding classification of type 1 VWD as a disease: given the overlap in symptoms and laboratory assays within the normal population, evaluation for those with VWD might be seen as identification of potential bleeding risk rather than detection of a disease. The authors offer the combined perspectives of the obstetrician-gynecologist and the hematologist to promote a better public health approach to this problem. 8 tables. 60 references.
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Bleeding Disorders. IN: Nilsson, K.R.; Piccini, J.P., eds. Osler Medical Handbook. Philadelphia, PA: Saunders. 2006. pp. 516-525.
This chapter on bleeding disorders is from a handbook that provides the essentials of diagnosis and treatment, as well as the latest in evidence-based medicine, for residents working bedside, in-patient care. The chapter begins with a presentation of essential Fast Facts and concludes with Pearls and Pitfalls useful to the practicing internist. The body of the chapter is divided into sections: Epidemiology, Clinical Presentation, Diagnosis, and Management. Specific topics covered in this chapter include inherited bleeding disorders, such as von Willebrand disease (vWD) and hemophilia; acquired bleeding disorders, which can be caused by liver disease, vitamin K deficiency, and antiplatelet or anticoagulation therapy, among other causes; the symptoms of clinically significant vWD, including mucosal bleeding and easy bruising; the role of family history of bleeding diathesis or a need for chronic transfusions or iron replacement in identifying bleeding disorders; and hemorrhagic death due to cerebral bleeding in patients with hemophilia. The chapter concludes with a list of references, each labeled with a 'strength of evidence' grade to help readers determine the type of research available in that reference source. 5 figures. 1 table. 9 references.
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Coagulation Disorders. IN: Hoffbrand, A.V.; Moss, P.A.H.; Pettit, J.E. Essential Haematology. 5th ed. Williston, VT: Blackwell Publishing Inc. 2006. pp. 290-302.
This chapter on coagulation disorders is from a hematology textbook that offers a comprehensive look at the biochemical, physiological, and immunological processes involved in normal blood cell formation and function and the disturbances that may occur in different diseases. The authors discuss hereditary coagulation disorders, notably hemophilia, and acquired coagulation disorders. Specific topics include hemophilia A, factor IX deficiency, von Willebrand disease, vitamin K deficiency, disseminated intravascular coagulation, coagulation deficiency, and massive transfusion syndrome. A final section describes thromboelastrography, a technique for the global assessment of hemostatic function of a single blood sample in which the reaction of platelets with the protein coagulation cascade is observed. The chapter features full-color illustrations. 10 figures. 6 tables. 22 references.
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Essential Haematology. 5th ed. Williston, VT: Blackwell Publishing Inc. 2006. 380 p.
This hematology textbook offers a comprehensive look at the biochemical, physiological, and immunological processes involved in normal blood cell formation and function and the disturbances that may occur in different diseases. The book is designed to help medical students grasp the essential features of modern clinical and laboratory hematology. The book includes 28 chapters on hemopoiesis, erythropoiesis and general aspects of anemia, hypochromic anemias and iron overload, megaloblastic anemias and other macrocytic anemias, hemolytic anemias, genetic disorders of hemoglobin, granulocytes and monocytes, lymphocytes and their benign disorders, the spleen, the etiology and genetics of hematological malignancies, the management of hematological malignancy, acute leukemias, chronic myeloid leukemia, myelodysplasia, the chronic lymphoid leukemias, Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, multiple myeloma and related disorders, myeloproliferative disorders, aplastic anemia and bone marrow failure, stem cell transplantation, platelets and blood coagulation, bleeding disorders caused by vascular and platelet abnormalities, coagulation disorders, thrombosis and antithrombotic therapy, hematological changes in systemic disease, blood transfusion, and pregnancy and neonatal hematology. Each chapter includes full-color photographs and illustrations and concludes with a list of references. The book concludes with three appendices and a detailed subject index.
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Exercises for People With Hemophilia. Montréal, QC: World Federation of Hemophilia. 2006. 46 p.
This document provides a guide to exercise for people with hemophilia. The guide is intended for physical therapists with little experience working with people with bleeding disorders; other health care workers, including community rehabilitation workers; and people with hemophilia and their families. The author stresses that there is really no such thing as specific exercises for hemophilia. Exercise, like medicine, works best when there has been a complete assessment done by a trained professional, and specific exercises have been chosen that suit the individual's goals and abilities. The document includes four sections: how joints and muscles are affected by hemophilia, guidelines for exercise and hemophilia, suggested joint exercises, and suggested muscles exercises. Terms highlighted in the text are defined in a glossary at the end of the booklet. The author reminds readers of the importance of exercise for building healthy bones and strengthening the muscles that support joints, which is important in all people but vitally so in those with hemophilia. Most of the exercises are illustrated with simple line drawings demonstrating how to perform each movement. The document concludes with the contact information for the World Federation of Hemophilia, based in Montreal, Canada, and a bibliography. 37 figures. 1 table. 5 references.
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Guidelines for Dental Treatment of Patients With Inherited Bleeding Disorders. Montreal, Quebec: World Federation of Hemophilia. 2006. 9 p.
This monograph presents guidelines for the dental treatment of patients with inherited bleeding disorders, including hemophilia A and hemophilia B. The author notes that successful patient care protocols are the result of cooperation between hematologists and dentists. Most guidelines recommend the use of clotting factor replacement therapy before invasive oral surgery and the use of the inferior alveolar nerve block for restorative dental treatment. The use of local hemostatic techniques, such as suturing, and the use of oxidized cellulose has resulted in certain minor oral surgery procedures being done with minimal or no coagulation factor replacement. Topics in this document include prevention, dental treatment, periodontal treatment, removable prosthodontics, orthodontic treatment, restorative procedures, endodontics, anesthesia and pain management, surgery, postextraction hemorrhage, management of oral infections, and dental emergencies. 1 table. 35 references.
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Bleeding Disorder Basics. Pediatric Nursing. 30(5): 402-405, 428-429. September-October 2005.
This article helps pediatric nurses understand the basics of bleeding disorders in children. The most common genetic bleeding disorder is von Willebrand disease, a genetic disorder characterized by easy bruising and frequent and prolonged bleeding from mucous membranes, such as nosebleeds, menorrhagia (excessive menstrual bleeding), or bleeding after oral surgery. Other disorders discussed include the hemophilias, and platelet or fibrinogen; the latter are extremely rare. The author discusses the products and treatments used for bleeding episodes, the physiology of the clotting process, and the management of children with bleeding disorders. Five patient management strategies are advocated: prompt and adequate treatment, testing for bloodborne viruses, immunizations, physical activity, and regular visits to a hemophilia treatment center. The author concludes with a section describing current research initiatives and resources for readers wishing to obtain additional information. 5 figures. 2 tables. 25 references.
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Diagnosis and Management of Mild Bleeding Disorders. IN: Hematology 2005. Washington, DC: American Society of Hematology. 2005. pp. 423-428.
This article reviews the diagnosis and management of mild bleeding disorders, which are a common reason for a referral to a hematologist. The author notes that some bleeding symptoms are quite common in the general population, and there is great variety in the symptoms among individuals with defined bleeding problems. Risks for many bleeding disorders are unknown. The author reviews symptoms and problems that can be considered suspicious of a mild form of bleeding disorder and the diagnostic investigations that may be used to further investigate these disorders. The author presents a stepwise approach for the diagnostic evaluation of common and rare coagulation and fibrinolytic defects and adequate assessments of potential von Willebrand factor and platelet problems. The author includes a section on diagnostic difficulties, including the common failure to establish a diagnosis with standard laboratory testing. Patient education approaches are also reviewed. 40 references.
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Management of Bleeding Disorders in Children. IN: Hematology 2005. Washington, DC: American Society of Hematology. 2005. pp. 416-422.
This article, from a special issue on the clinical management of bleeding disorders, considers the management of bleeding disorders in children. The author emphasizes that the diagnosis and management of congenital and acquired bleeding disorders in children requires an understanding of the unique characteristics of pediatric hemostasis as well as the natural course of bleeding disorders in children, which may differ from the course observed in adults. The author focuses on three bleeding disorders: neonatal alloimmune thrombocytopenia (NAIT), hemophilia, and immune-mediated thrombocytopenia purpura (ITP). The unique physiology of transplacental transfer of maternally derived anti-platelet antibodies can result in NAIT, which is a significant cause of morbidity and mortality from bleeding in affected infants. For patients with hemophilia, treatment strategies have changed from on-demand therapy to primary or secondary prevention, which results in the delay of onset or even a complete avoidance of hemophilic arthropathy. Regarding the management of ITP of childhood, although several effective management strategies have been developed, the side effects associated with these treatments can require the clinician to also consider the option of carefully observing, but not treating, children with ITP. 1 table. 43 references.
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New Perspectives in Hemophilia Treatment. IN: Hematology 2005. Washington, DC: American Society of Hematology. 2005. pp. 429-435.
This article explores new perspectives in hemophilia treatment, focusing on the variety of factor concentrates that are available for replacement therapy. The author notes that the choice of replacement product reflects both safety issues of pathogen transmission or inhibitor development and personal preferences of the patient and physician. In general, available products are viral pathogen-free, although there is debate about the risk of transmission of parvovirus B19 and prion pathogens. Because of this very small risk, recombinant factor is the treatment of choice in previously untreated patients. The management of hemophilia patients with inhibitors is an ongoing challenge. Immune tolerance induction using a desensitization technique is successful in up to 90 percent of patients with alloantibodies against factor VIII, with greatest success seen in patients with low titer inhibitors who are treated soon after detection of an alloantibody and in whom treatment includes administration of immunosuppression along with repeated infusions of high titer concentrates. Such therapy is less successful in patients with factor IX alloantibodies. A final section discusses the management of nonhemophiliac patients with acquired inhibitors. These patients have a mortality rate that approaches 25 percent because of the association of acquired inhibitors with severe bleeding complications, occurrence in a largely elderly population, and the frequent presence of an underlying, often serious, primary medical condition. Treatment consists of immunosuppression with steroids, chemotherapy, or intravenous immunoglobulin. 2 figures. 2 tables. 14 references.
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Basic Science, Diagnosis And Clinical Management of Von Willebrand Disease. Montreal, Quebec: World Federation of Hemophilia. 2004. 12 p.
This monograph offers an overview of the basic science, diagnosis, and clinical management of von Willebrand disease (VWD), an inherited bleeding disorder that is distinct from hemophilia. Topics include an historical perspective of VWD, the VWD factor gene and factor protein, biological functions of von Willebrand factor, clinical features, epidemiology of VWD, diagnostic approaches, family history, the classification of VWD, VWD during pregnancy, and the prevention and treatment of bleeding patients with VWD, including the use of desmopressin and factor concentrates. The diagnosis of VWD requires three components: personal history of excessive mucocutaneous bleeding, a family history of excessive bleeding, and a laboratory evaluation that is consistent with a quantitative or qualitative defect in von Willebrand factor. Type 1 VWD is the most common form of the disease. Adjunctive therapies such as antifibrinolytic agents and the application of topical hemostatic preparations are used with significant benefit in VWD; in women with menorrhagia, the administration of estrogens often results in significant clinical benefit. 6 figures. 23 references.
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Acute Bleeding from Diverticulosis and Ischemic Colitis. In: Chung, P. and Kim, K.E. Acute Gastrointestinal Bleeding: Diagnosis and Treatment. Totowa, NJ: Humana Press. 2003. p. 163-174.
Lower gastrointestinal (GI) bleeding is a common medical problem in the United States. Two common causes of such bleeding are diverticulosis and ischemic colitis. This chapter reviews the pathogenesis, diagnosis, and management of bleeding associated with these two conditions. The chapter is from a textbook in which leading experts in the fields of gastroenterology, surgery, and radiology comprehensively review the pathophysiology, diagnosis, management, and treatment of acute bleeding disorders of the GI tract. The authors of this chapter note that acute diverticular bleeding is the most common cause of lower GI bleeding. Most patients are otherwise asymptomatic at presentation, and bleeding ceases spontaneously 70 to 80 percent of the time. Ischemic colitis is the most common form of intestinal ischemic injury, most often occurring in the left side of the colon. Colonoscopy is the diagnostic procedure of choice since it allows direct visualization of the mucosa and tissue sampling. Management is usually merely supportive, consisting of bowel rest and intravenous fluid and antibiotics, with most patients recovering in 24 to 48 hours. 2 figures. 39 references.
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Acute Gastrointestinal Bleeding: Diagnosis and Treatment. Totowa, NJ: Humana Press. 2003. 288 p.
Acute gastrointestinal (GI) bleeding accounts for over 300,000 hospitalizations per year and has high morbidity and mortality in those who go undiagnosed. In this textbook, leading experts in the fields of gastroenterology, surgery, and radiology comprehensively review the pathophysiology, diagnosis, management, and treatment of acute bleeding disorders of the GI tract. The authors categorize acute bleeding into upper and lower GI tract sources and provide a differential diagnosis for each disease, evidence-based algorithms for clinical practice, treatment modalities for its management, and standards of care. General approaches to the acute GI bleeding patient are discussed in terms of supportive care, early detection, and determination of upper versus lower GI bleed, when to transfuse, as well as early predictors of morbidity and mortality. The authors outline the many dilemmas faced by physicians in their approach to their patients, such as localization of the bleeding source (upper versus lower), the need and timing for emergency endoscopy, and the timing for radiologic intervention or surgery. Each of the chapters concludes with a list of references; a subject index concludes the volume.
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Congenital Bleeding Disorders. IN: Hematology 2003. Washington, DC: American Society of Hematology. 2003. pp. 559-574.
This article reviews some of the clinical and basic problems related to the congenital bleeding disorders that continue to confront hematologists. In the first section, Dr. Christopher Walsh discusses general principles of effective gene transfer for the hemophilias, specific information about viral vectors and nonviral gene transfer, and alternative target tissues for factor VIII and factor IX production. He highlights information about the immune response to gene transfer, reviews data from the hemophilia gene transfer trials to date, and presents outlines of some future prospects for newer methods of therapy such as RNA repair and the use of gene-modified circulating endothelial progenitors. In the second section, Dr. Nigel Key focuses on inhibitor development in patients with hemophilia A, reviewing the progress in understanding the risk factors and the immunobiology of inhibitor development. Topics include the natural history of these inhibitors and the screening, laboratory diagnosis, and treatment, including the use of different modalities for the treatment of acute bleeding episodes. In the final section, Dr. Margaret Rick discusses the diagnosis, classification, and management of von Willebrand disease. Attention is given to the difficulty of diagnosis in patients with mild bleeding histories and borderline laboratory test results for von Willebrand factor. Practical issues of diagnosis and treatment, including clinical cases, are presented. 1 figure. 2 tables. 48 references.
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Epidemiology of Acute Gastrointestinal Bleeding. In: Chung, P. and Kim, K.E. Acute Gastrointestinal Bleeding: Diagnosis and Treatment. Totowa, NJ: Humana Press. 2003. p. 3-7.
Acute gastrointestinal (GI) bleeding accounts for over 300,000 hospitalizations per year and has high morbidity and mortality in those who go undiagnosed. This chapter on the epidemiology of acute GI bleeding is from a textbook in which leading experts in the fields of gastroenterology, surgery, and radiology comprehensively review the pathophysiology, diagnosis, management, and treatment of acute bleeding disorders of the GI tract. The authors of this chapter stress that acute GI bleeding is an extremely common clinical condition affecting a large patient population. The diverse clinical presentations, etiologic factors, and treatment modalities are important to understand, and early identification of the source of bleeding is the essential component in reducing morbidity and mortality. 32 references.
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Helicobacter Pylori and Peptic Ulcer Disease. In: Chung, P. and Kim, K.E. Acute Gastrointestinal Bleeding: Diagnosis and Treatment. Totowa, NJ: Humana Press. 2003. p. 35-56.
Peptic ulcer disease (PUD) is a common gastrointestinal (GI) problem that has a substantial impact on patient well-being and health care costs. Complications include hemorrhage, perforation, and gastric outlet obstruction. This chapter on Helicobacter pylori and PUD is from a textbook in which leading experts in the fields of gastroenterology, surgery, and radiology comprehensively review the pathophysiology, diagnosis, management, and treatment of acute bleeding disorders of the GI tract. Topics covered in this chapter include epidemiology, clinical presentation, diagnosis, etiology, treatment, and bleeding associated with PUD. Peptic ulcer can present silently with complications such as hemorrhage, particularly in patients on nonsteroidal antiinflammatory drugs (NSAIDs). Proton pump inhibitors (PPI) are the mainstays of therapy. Effective eradication of H. pylori involves regimens utilizing multiple antibiotics. Upper endoscopy effectively diagnoses peptic ulcers, reduces rebleeding, and allows for appropriate triage of patients with upper GI bleeding complications. One patient care algorithm is provided. 1 figure. 3 tables. 99 references.
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Infectious and Inflammatory Causes of Acute Gastrointestinal Bleeding. In: Chung, P. and Kim, K.E. Acute Gastrointestinal Bleeding: Diagnosis and Treatment. Totowa, NJ: Humana Press. 2003. p. 151-161.
Most patients who present to their physician with a sudden onset of diarrhea have a benign, self-limited illness. Bloody diarrhea, on the other hand, requires a thoughtful and thorough evaluation. This chapter on infectious and inflammatory causes of acute gastrointestinal (GI) bleeding is from a textbook in which leading experts in the fields of gastroenterology, surgery, and radiology comprehensively review the pathophysiology, diagnosis, management, and treatment of acute bleeding disorders of the GI tract. The authors of this chapter discuss pathophysiology, clinical presentation, diagnosis, and treatment options. The authors note that acute bleeding from either infectious colitis or inflammatory bowel disease (IBD) can present in a similar manner. The presence of an infectious agent should not preclude the workup for IBD if the patient's history supports this as an additional diagnosis. A timely diagnosis is important for proper treatment strategies. Supportive care and early surgical consultation are important in severe cases. A patient care algorithm is included. 1 figure. 3 tables. 16 references.
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Medical Therapy for Stress Ulcer Prophylaxis. In: Chung, P. and Kim, K.E. Acute Gastrointestinal Bleeding: Diagnosis and Treatment. Totowa, NJ: Humana Press. 2003. p. 57-74.
Patients admitted to intensive care units (ICUs) develop a spectrum of gastroduodenal lesions that may result in gastrointestinal (GI) bleeding. It has been assumed that mucosal damage in these critically ill patients is related to physiologic stress, hence the terms stress ulcers and stress-related GI hemorrhage. This chapter on medical therapy for stress ulcer prophylaxis is from a textbook in which leading experts in the fields of gastroenterology, surgery, and radiology comprehensively review the pathophysiology, diagnosis, management, and treatment of acute bleeding disorders of the GI tract. The author of this chapter discusses routine prophylaxis approaches, identifying patients at high risk for developing stress-related bleeding, the use of medical prophylaxis for stress-related GI hemorrhage, the safety of said medical prophylaxis, cost considerations, and patient selection issues for medical therapy for stress ulcer prophylaxis. 1 figure. 2 tables. 54 references.
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Nonsteroidal Antiinflammatory Drug (NSAID)-Induced Gastropathy. In: Chung, P. and Kim, K.E. Acute Gastrointestinal Bleeding: Diagnosis and Treatment. Totowa, NJ: Humana Press. 2003. p. 75-96.
The term NSAID gastropathy refers to the spectrum of side effects in the upper gastrointestinal (GI) tract suffered by patients using nonsteroidal antiinflammatory drugs (NSAIDs). This chapter on NSAID- induced gastropathy is from a textbook in which leading experts in the fields of gastroenterology, surgery, and radiology comprehensively review the pathophysiology, diagnosis, management, and treatment of acute bleeding disorders of the GI tract. The author of this chapter notes that the toxicity of NSAIDs ranges from commonly experienced nuisance symptoms such as dyspepsia to much more serious events such as symptomatic and complicated ulcers. The author discusses the pathogenesis of NSAID toxicity, clinical presentation, the risks of GI complications with NSAIDs, prevention and treatment of dyspepsia associated with NSAID use, treatment of ulcers in NSAID users, and the prevention of NSAID-associated gastrointestinal ulcers and complications. 3 figures. 3 tables. 52 references.
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Nonvariceal Esophageal Bleeding. In: Chung, P. and Kim, K.E. Acute Gastrointestinal Bleeding: Diagnosis and Treatment. Totowa, NJ: Humana Press. 2003. p. 11-34.
Acute gastrointestinal (GI) bleeding accounts for over 300,000 hospitalizations per year and has high morbidity and mortality in those who go undiagnosed. This chapter on nonvariceal esophageal bleeding is from a textbook in which leading experts in the fields of gastroenterology, surgery, and radiology comprehensively review the pathophysiology, diagnosis, management, and treatment of acute bleeding disorders of the GI tract. The authors of this chapter note that the esophagus is an important site of acute upper GI bleeding. They discuss Mallory-Weiss lesions, reflux esophagitis, esophageal infections, malignant neoplasm (esophageal cancer), and miscellaneous conditions. The differential diagnosis of nonvariceal esophageal bleeding is large, and the condition often requires endoscopy for accurate diagnosis. In general, the more common causes of acute esophageal hemorrhage are self-limited or respond to conservative management. Massive, acute bleeding, however, does occur. Prompt diagnosis is important, as the treatments of the various disorders are quite diverse and include medical, endoscopic, and surgical management. 5 figures. 121 references.
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Oral Manifestations of Gastrointestinal Disease. In: Textbook of Gastroenterology. 4th ed. [2-volume set]. Hagerstown, MD: Lippincott Williams and Wilkins. 2003. p. 1009-1016.
Many gastrointestinal disease states are associated with oral findings. This chapter briefly addresses the disorders in which oropharyngeal pathology is most common. These include gastroesophageal reflux disease (GERD), polyposis syndromes, bleeding disorders, immunodeficiency states, collagen vascular disease, nutritional deficiencies and malabsorption, inflammatory bowel states, and miscellaneous disorders. The chapter is from a lengthy, two-volume textbook that integrates the various demands of science, technology, expanding information, good judgment, and common sense into the diagnosis and management of gastrointestinal patients. 7 figures 5 tables. 55 references.
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Hepatitis C in Adults and Adolescents with Hemophilia: A Randomized, Controlled Trial of Interferon Alfa-2b and Ribavirin. Hepatology. 36(4 Part 1): 967-972. October 2002.
Adolescents and adults with inherited disorders of coagulation have one of the highest prevalence rates of hepatitis C among known risk groups. Few data are available on the use of combination therapy with interferon and ribavirin in this population. This article reports on a study in which patients 13 years of age and older (n = 113) who were positive for hepatitis C virus (HCV) RNA and negative for HIV were randomized to receive interferon alfa-2b plus ribavirin or interferon alfa-2b alone for 48 weeks, with 24 weeks of posttreatment follow up. Patients started on interferon alone who remained positive for HCV RNA at week 12 crossed over to treatment with interferon plus ribavirin. Of the 113 patients, 37 were younger than 18 years. At the end of treatment, 18 of 56 patients (32 percent) treated with interferon plus ribavirin and 6 of 57 patients (11 percent) treated with interferon alone were negative for HCV RNA. Sustained virologic response in the combination arm was 29 percent (16 of 56) compared with 7 percent (4 of 57) for those started on interferon alone. Among adolescents younger than 18 years who were treated with combination therapy, 10 of 17 (59 percent) had sustained response compared with 6 of 39 (15 percent) of adult patients on the same regimen. The authors conclude that in this trial of therapy for HCV in patients with inherited bleeding disorders, sustained virologic response rate was significantly improved for patients treated with interferon and ribavirin compared with those started on interferon alone. Adolescents treated with combination therapy had a significantly higher sustained response than adults did on the same regimen. 1 figure. 5 tables. 25 references.
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