deamino-arginine-vasopressin and Hemorrhagic-Disorders

Unclassified bleeding disorders account for 2.6% of all new bleeding disorder registrations in the UK. The management of the bleeding phenotype associated with these disorders is poorly described. Systematic review and meta-analysis to determine the bleeding rates associated with tranexamic acid, desmopressin, platelet transfusion, plasma transfusion and recombinant activated factor VII, for patients with unclassified bleeding disorders undergoing surgery, childbirth or with menorrhagia. We searched for randomized controlled trials in MEDLINE, Embase, The Cochrane Central Register of Controlled Trials, PubMed, ISI Web of Science and the Transfusion Evidence Library from inception to 24 February 2020. Wherever appropriate, data were pooled using the metaprop function of STATA. Two studies with 157 participants with unclassified bleeding disorders were identified. The pooled risk of minor bleeding for patients undergoing surgery treated with peri-operative tranexamic acid was 11% (95% confidence interval 3--20%; n = 52; I2 = 0%); the risk for desmopressin and tranexamic acid in combination was 3% (95% confidence interval 0--7%; n = 71; I2 = 0%). There were no instances of major bleeding. In one procedure, 1 of 71 (1.4%), treated with a combination of desmopressin and tranexamic acid, the patient had a line-related deep vein thrombosis. There were too few patients treated to prevent postpartum haemorrhage or for menorrhagia to draw conclusions. The GRADE quality of evidence was very low suggesting considerable uncertainty over the results. However, both tranexamic acid, and the combination of tranexamic and desmopressin have high rates of haemostatic efficacy and have few adverse events.. PROSPERO CRD42020169727.

Topics: Antifibrinolytic Agents; Blood Component Transfusion; Deamino Arginine Vasopressin; Disease Management; Female; Hemorrhage; Hemorrhagic Disorders; Hemostatics; Humans; Menorrhagia; Postoperative Hemorrhage; Postpartum Hemorrhage; Pregnancy; Tranexamic Acid

In Canada, care for individuals with inherited bleeding disorders, including Von Willebrand disease, is provided by 26 tertiary care multidisciplinary Inherited Bleeding Disorders clinics geographically spread across the country. The Association of Hemophilia Clinic Directors of Canada, the Canadian Association of Nurses in Hemophilia Care, the Canadian Physiotherapists in Hemophilia Care, the Canadian Social Workers in Hemophilia Care, and the Canadian Hemophilia Society all collaborate to provide optimal management for patients with inherited bleeding disorders. The standards of care for these patients were explicitly laid out in a 2007 document published by the Canadian Hemophilia Standards Group (with representation from all of the groups just mentioned) entitled Canadian Comprehensive Care Standards for Hemophilia and Other Inherited Bleeding Disorders. Separate Canadian guidelines for the management of patients with Hemophilia and Von Willebrand disease also exist, focused on diagnosis, comprehensive care, assessment, and treatment.

Topics: Canada; Comprehensive Health Care; Deamino Arginine Vasopressin; Factor VIII; Genetic Testing; Hemorrhagic Disorders; Hemostatics; Humans; Practice Guidelines as Topic; von Willebrand Diseases; von Willebrand Factor

Desmopressin is an analog of vasopressin that exerts a substantial haemostatic effect by inducing the release of von Willebrand factor from its storage sites in endothelial cells. It has proved useful in treating or preventing bleeding episodes in patients with von Willebrand disease, haemophilia A and platelet function defects. Its efficacy in achieving a satisfactory level of haemostasis has reduced the use of blood products to treat bleeding episodes. Clinicians need to become familiar with the use of this drug that has become a home medication for many patients with inherited bleeding disorders.

Topics: Deamino Arginine Vasopressin; Drug Administration Routes; Hemorrhagic Disorders; Hemostatics; Humans; von Willebrand Factor

Topics: Child; Deamino Arginine Vasopressin; Hemorrhagic Disorders; Hemostatics; Humans; Treatment Outcome

Bleeding disorders in women are an underestimated problem that deserves increased attention. About 9%-14% of females have menorrhagia and, amongst them, there is a significant over-representation of von Willebrand disease (VWD), with a prevalence of 13% in this group as compared with about 1% in the general population. The bleeding disorder has not been diagnosed in most of these women and they may therefore be withheld from treatment with desmopressin, which is effective in most cases of VWD and also in platelet dysfunctions and mild factor VIII deficiency. This paper is a review of the haemostatic use of desmopressin with special reference to women's bleeding disorders, the mechanisms of action, modes of administration, clinical indications, dosage recommendations, and hospital or home treatment. Desmopressin stimulates endogenous release of FVIII and von Willebrand factor (VWF), it increases platelet adhesiveness and shortens bleeding time. It can be given as intravenous or subcutaneous injection, but the intranasal spray is probably the most practical mode of administration for females with bleeding disorders as it is simple to administer and suitable for home treatment. The spray has been used successfully in connection with menorrhagia and other bleeding symptoms.

Topics: Blood Loss, Surgical; Deamino Arginine Vasopressin; Female; Hemorrhagic Disorders; Hemostatics; Humans; Postpartum Hemorrhage; Pregnancy; Self Administration

Topics: Blood Loss, Surgical; Blood Platelets; Cardiovascular Surgical Procedures; Deamino Arginine Vasopressin; Hemorrhagic Disorders; Hemostasis, Surgical; Hemostatics; Humans

Topics: Animals; Blood Loss, Surgical; Deamino Arginine Vasopressin; Factor VIII; Female; Hemophilia A; Hemorrhage; Hemorrhagic Disorders; History, 18th Century; History, 20th Century; Humans; Italy; Kidney Diseases; Liver Diseases; Male; Platelet Adhesiveness; Rabbits; von Willebrand Diseases; von Willebrand Factor

Over the past several years, the use of 1-deamino-8-D-arginine vasopressin (DDAVP), a synthetic analogue of vasopressin, has been found to be useful in the treatment of patients with abnormal bleeding tendency. This article is a review of inherited and acquired disorders with prolonged bleeding time in which DDAVP is supposed to shorten the bleeding time. DDAVP is established as effective therapy of the abnormal haemostasis in mild or moderate haemophilia A and von Willebrand's disease. Frequently, DDAVP infusions are used to control bleeding in patients with uraemia. Bleeding time is also significantly shortened in patients with liver cirrhosis, although randomized trials of DDAVP therapy of gastrointestinal bleeding in this group of patients are still needed. Shortening or normalization of the bleeding time with DDAVP has also been observed in patients with inherited platelet dysfunctions, acquired disorders of haemostasis and abnormal haemostasis in chronic myeloproliferative diseases. In addition, preoperative treatment with DDAVP seems to reduce blood loss during surgery.

Topics: Deamino Arginine Vasopressin; Hemorrhagic Disorders; Humans; Hypoglycemic Agents; Renal Agents

Topics: Aged; Blood Coagulation Disorders; Blood Coagulation Factors; Cardiopulmonary Bypass; Deamino Arginine Vasopressin; Hemorrhagic Disorders; Hemostasis; Humans; Male; Platelet Adhesiveness; Postoperative Complications; Signal Transduction; Thrombosis

A broad, open, inquisitive, and semiskeptical mind must be used when approaching the bleeding patient. As in most endeavors in medicine, the history and physical examination provide an important baseline. Key laboratory tests must be quickly ordered and interpreted. Using this data base, one can quickly determine whether the hemorrhagic disorder is congenital or acquired, severe or mild, and progressive or stable. Hemostasis may fail owing to deficiencies of platelets, the plasma coagulation protein system, or endothelial disturbances. A precise diagnosis and appreciation of the tempo of the disorder will guide specific therapy.

Topics: Blood Coagulation Factors; Deamino Arginine Vasopressin; Diagnosis, Differential; Hemophilia A; Hemorrhage; Hemorrhagic Disorders; Humans

Desmopressin (1-deamino-8-D-arginine vasopressin, DDAVP) is a synthetic analogue of the antidiuretic hormone L-arginine vasopressin. Because it can raise circulating levels of Factor VIII and of von Willebrand's factor, DDAVP is used for nontransfusional treatment of mild and moderate hemophilia and von Willebrand's disease. DDAVP also shortens the prolonged skin bleeding time in patients with uremia, liver cirrhosis, and platelet dysfunctions and is given to prevent or stop excessive bleeding in such conditions. Finally, there is evidence that DDAVP can reduce blood loss and transfusion requirements during and after surgical operations in which blood losses are unusually large. Hence DDAVP is useful as a nontransfusional hemostatic agent in many of the bleeding disorders frequently encountered in clinical practice.

Topics: Deamino Arginine Vasopressin; Hemorrhagic Disorders; Hemostasis; Humans

Arginine vasopressin preparations have been used in the treatment of diabetes insipidus for many years. Compared with older antidiuretic agents, the synthetic analog desmopressin is more potent, longer acting and easier to use. It is available for intravenous, subcutaneous and intranasal administration. Desmopressin may be useful in the treatment of hemostatic disorders such as von Willebrand's disease and hemophilia A. It has also been used for nocturnal enuresis. The vasopressor effects of arginine vasopressin preparations have been exploited for use as a temporizing measure in controlling acute gastrointestinal bleeding. Side effects such as hyponatremia and water intoxication are uncommon when these drugs are used with proper precautions.

Topics: Deamino Arginine Vasopressin; Diabetes Insipidus; Enuresis; Gastrointestinal Hemorrhage; Hemorrhagic Disorders; Humans; Vasopressins

Topics: Animals; Antigens; Autoantibodies; Autoimmune Diseases; Deamino Arginine Vasopressin; Factor IX; Factor VIII; Female; Hemophilia A; Hemorrhagic Disorders; Humans; Immunoglobulin G; Immunosuppressive Agents; Isoantibodies; Plasmapheresis; Postpartum Period; Pregnancy; Swine

Topics: Antigens; Deamino Arginine Vasopressin; Endothelium, Vascular; Factor VIII; Hemophilia A; Hemorrhagic Disorders; Hemostasis; Hemostasis, Surgical; Hemostatics; Humans; Receptors, Angiotensin; Receptors, Vasopressin; Thrombosis; von Willebrand Factor

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)

Topics: Deamino Arginine Vasopressin; Factor VIII; Hemophilia A; Hemorrhagic Disorders; Humans; Uremia; von Willebrand Diseases

There is an unmet need to characterize the diagnosis and management of patients with an unclassified bleeding disorder (UBD).. Retrospective review of registered patients with UBD at our centre. Assessment including rotational thromboelastometry (ROTEM) and thrombin generation (TG) were used.. A total of 124 patients were identified; 91% female. Mean age of presentation was 38.3 years. Mean bleeding score was 8.8 (standard deviation [SD] 3.8); 6.6 in men (SD 1.4) and 9.7 in women (SD 3.3), which was significantly different (P < .05). In women, after deduction of scores for menorrhagia and postpartum haemorrhage, the mean score was 6.4 which was not significantly different to the male score (P = .11). Twenty-three percent of patients have been transfused, 61% women had treatment for menorrhagia and 17% for epistaxis. TxA and desmopressin were effective at preventing bleeding in 69 procedures and 13 deliveries. TG revealed 26% patients with a long lag time and 19% with a decreased endogenous thrombin potential but no diagnostic pattern was seen. ROTEM (NATEM) was unable to characterize patients; 9% had a prolonged clot time or maximum lysis. ThromboGenomics was normal in 45 tested patients.. We provide data which shows the bleeding score is biased towards gynaecological bleeding but which remains elevated even when the bleeding score is deducted. Tranexamic acid and desmopressin are effective as haemostatic prophylaxis but there is an urgent need for clinical trials. In conclusion, we describe the use of the bleeding score in these patients and phenotype, diagnosis (including ThromboGenomic testing) and management with practice recommendations.

Topics: Adult; Aged; Deamino Arginine Vasopressin; Female; Hemorrhagic Disorders; Humans; Male; Middle Aged; Retrospective Studies; Tranexamic Acid

The objective of this study was to investigate the efficacy and safety of desmopressin (1-desamino-8-D-arginine vasopressin) compared with placebo in the reduction of menstrual blood loss in women with menorrhagia and prolonged bleeding time, but without common coagulation factor deficiencies. We performed a randomized, double-blind, cross-over study using 300 microg desmopressin nasal inhalation or placebo treatment in one of the two first treatment cycles. Desmopressin was given only for the 2 days during which the bleeding had been at a maximum in the previous baseline cycle. A third open cycle involved combined treatment with desmopressin and tranexamic acid during the 2 days for all patients. Menstrual blood loss during the treatment periods was compared with blood loss during placebo-treated periods using objective measurement. A significant reduction of menstrual blood loss was found in the cycles treated with combined desmopressin and tranexamic acid compared with placebo. When analyzing the blood loss during the two treatment days, there was a significant reduction in blood loss for the 2 days with desmopressin alone versus placebo. The treatment was well tolerated and no serious adverse events were recorded. In conclusion, we find that nasal desmopressin is a possible complement for the medical treatment of menorrhagia.

Topics: Adult; Bleeding Time; Cross-Over Studies; Deamino Arginine Vasopressin; Double-Blind Method; Female; Flushing; Headache; Hemorrhagic Disorders; Hemostatics; Humans; Menorrhagia; Nausea; Tranexamic Acid; Treatment Outcome

Topics: Bleeding Time; Blood Coagulation Disorders; Blood Platelets; Deamino Arginine Vasopressin; Hemorrhagic Disorders; Humans

Inherited disorders of platelets constitute a group of rare diseases that give rise to bleeding syndromes of variety severity, with more severe cases being first diagnosed during infancy and childhood. To appropriate diagnose a platelet function disorder during early childhood the knowledge of the physiological characteristics of platelets in the paediatric population is mandatory. Apart from thrombocytopenia which is quite common in neonates and children the present overview is aimed to focus on inherited platelet function disorders. Furthermore, knowledge on platelet maturation and reference values according to age are given, and a diagnostic strategy specifically adapted to a pediatric population is presented on the bases of plasmatic and molecular laboratory methodologies. Finally, therapeutic approaches are briefly summarized (antifibrinolytic agents, Desmopressin, HLA-matched platelets, recombinant factor VIIa).

Topics: Antifibrinolytic Agents; Blood Platelet Disorders; Child; Deamino Arginine Vasopressin; Diagnosis, Differential; DNA Mutational Analysis; Factor VIIa; Hemorrhagic Disorders; Humans; Infant; Infant, Newborn; Molecular Motor Proteins; Myosin Heavy Chains; Partial Thromboplastin Time; Platelet Count; Platelet Function Tests; Platelet Transfusion; Recombinant Proteins; Syndrome; Thrombocytopenia; von Willebrand Diseases; von Willebrand Factor

To evaluate the possible use of desmopressin acetate (DDAVP) in haemorrhagic disorders consequent to canine monocytic ehrlichiosis (CME), three dogs infected by Ehrlichia canis, with a history of thrombocytopenia and recent bleeding, were studied. The dogs were administered desmopressin (1 μg/kg b.w. s.c.) every 24 h on three occasions. Blood samples were collected immediately before, and after 2 and 48 h the first DDAVP administration, to assess haematological, clinical chemistry and clotting time parameters. Spontaneous bleeding stopped within 1 h after the first DDAVP injection. Buccal mucosa bleeding time (BMBT) was shortened from 9.6 to 2.3 min within 2 h after the treatment. A statistically significant increase in platelet PLT count and fibrinogen, and a statistically significant decrease of PT and aPTT were observed after DDAVP administration. The haemorrhagic disorders caused by CME appear to be quickly corrected by DDAVP administration, giving the clinician the time necessary to administer appropriate chemotherapy.

Topics: Animals; Blood Coagulation Tests; Deamino Arginine Vasopressin; Dog Diseases; Dogs; Ehrlichiosis; Hemorrhagic Disorders; Hemostatics; Male; Thrombocytopenia

Topics: Aged, 80 and over; Aspirin; Blood Gas Analysis; Combined Modality Therapy; Deamino Arginine Vasopressin; Erythrocyte Transfusion; Hematoma; Hemorrhage; Hemorrhagic Disorders; Humans; Kidney Failure, Chronic; Male; Platelet Aggregation Inhibitors; Platelet Transfusion; Punctures; Radial Artery; Respiratory Insufficiency

Topics: Adenocarcinoma; Anesthesia, Intravenous; Anesthetics, Intravenous; Anticoagulants; Aprotinin; Bernard-Soulier Syndrome; Colectomy; Colonic Neoplasms; Coronary Artery Bypass; Deamino Arginine Vasopressin; Drainage; Erythrocyte Transfusion; Extracorporeal Circulation; Genes, Dominant; Hemorrhagic Disorders; Hemostatics; Heparin; Humans; Leukopenia; Male; Middle Aged; Molecular Motor Proteins; Myosin Heavy Chains; Peptic Ulcer Hemorrhage; Plasma; Platelet Count; Platelet Transfusion; Pleural Effusion; Postoperative Complications; Postoperative Hemorrhage; Propofol; Sclerotherapy; Syndrome

Hermansky-Pudlak syndrome (HPS) is a genetic disorder characterized by oculocutaneous albinism, a bleeding diathesis, and in a subset of patients, pulmonary fibrosis. Lung transplantation, the only curative therapy for pulmonary fibrosis, has not been previously reported as a successful treatment strategy for patients with HPS because the bleeding diathesis was thought to contraindicate major thoracic surgery. We successfully performed bilateral sequential lung transplantation in a patient with pulmonary fibrosis and HPS after transfusion of 6 units of platelets. Lung transplantation is a viable therapeutic option in patients with pulmonary fibrosis and only a mild bleeding diathesis associated with HPS.

Topics: Adult; Blood Platelet Disorders; Deamino Arginine Vasopressin; Hemorrhagic Disorders; Hemostatics; Hermanski-Pudlak Syndrome; Humans; Lung Transplantation; Male; Membrane Proteins; Platelet Transfusion; Pulmonary Fibrosis; Treatment Outcome

This study evaluated the effectiveness of a protocol to prevent bleeding after dental extraction in patients with hemophilia, von Willebrand's disease (VWD), or platelet disorders.. Replacement therapy was used in cases involving general anesthesia, and nerve trunk infiltration was used in patients with severe bleeding disorders (severe-to-moderate hemophilia or type 2-3 VWD). Desmopressin was used in good responders with mild hemophilia A, type 1 VWD, and platelet disorders. Local hemostatic measures and antifibrinolytic treatment were used systematically.. Ninety-three patients underwent 103 dental extractions; 2 of these patients had secondary bleeding requiring surgical hemostasis.. The indication for replacement therapy depended on the type of anesthesia that was used. Coagulation factor concentrates or desmopressin were necessary to avoid upper airway hematoma with general anesthesia or nerve trunk infiltration. With local anesthesia, substitutive treatment was indicated in patients with severe-to-moderate hemophilia and type 2-3 VWD. Inexpensive desmopressin was effective in those who responded well. Local hemostatic measures and antifibrinolytic treatment were performed systematically.

Topics: Adolescent; Adult; Aged; Antifibrinolytic Agents; Blood Platelet Disorders; Blood Transfusion; Child; Clinical Protocols; Deamino Arginine Vasopressin; Dental Care for Chronically Ill; Female; Hemophilia A; Hemorrhagic Disorders; Hemostatics; Humans; Male; Middle Aged; Oral Hemorrhage; Retrospective Studies; Tooth Extraction; von Willebrand Diseases

Topics: Carcinoma, Papillary; Carcinoma, Renal Cell; Deamino Arginine Vasopressin; Female; Hemorrhagic Disorders; Humans; Hyponatremia; Kidney Neoplasms; Laparoscopy; Middle Aged; Neoplasms, Multiple Primary; Nephrectomy; Postoperative Complications; Seizures; Thyroid Neoplasms; Thyroidectomy; von Willebrand Diseases

The May-Hegglin anomaly is an extremely rare, autosomal dominant inherited disorder characterized by alterations in white cells and in blood platelets. The granulocytes show basophilic inclusion bodies of no clinical importance. Usually moderate thrombocytopenia with variable platelet size, including giant platelets, is also found. Clinically a mild hemorrhagic diathesis may occur. We report on a so far asymptomatic patient from the second family described by Hegglin et al. in 1964 [1] who had to be treated for repeated life-threatening bleedings. A moderate prolongation of bleeding time was found, corresponding to the reduced platelet count; platelet aggregation induced by ADP, collagen, ristocetin or arachidonic acid was not impaired. Therefore, there is at present no evidence of a congenital platelet function defect in the May-Hegglin anomaly. The bleeding time improved temporarily in our patient on administration of DDAVP (Minirin); platelet substitution is indicated in special situations only.

Topics: Adult; Bleeding Time; Child; Chromosome Aberrations; Chromosome Disorders; Deamino Arginine Vasopressin; Female; Genes, Dominant; Hemorrhagic Disorders; Humans; Male; Middle Aged; Phenotype; Platelet Aggregation; Platelet Function Tests; Thrombocytopenia

Platelets of anticoagulated whole blood forced at 40 mmHg through a fine filter are activated, aggregated and retained, so block the filter (platelet filter test, O'Brien JR, Salmon GP. Blood 1987; 1354-1361). Our clinical experiences with this simple and quick haemostasis test are summarized. Patients were investigated with different types of vWD (type-1 = 35, type-2A = 7, type-2B = 7, type-3 = 1), Glanzmann's thrombasthenia, congenital deficiency of cyclo-oxygenase, acquired Bernard-Soulier syndrome, FXII-, FXIII-deficiency and a control group. The cumulative drop count and the platelet retention were carefully measured during two phases of the filter test. Platelet count, bleeding time, vWF:Ag and vWF:Rcof activity were measured along with the platelet filter test. The filter was not blocked and the platelet retention was abnormally low in all patients with thrombasthenia, type-2a, type-2B, type-3 vWD. Treatment with 1-desamino-8-D-arginine-vasopressin (DDAVP) caused enhanced platelet retention in 16 patients with type-1 vWD. The test is simple, quick and cheap, has good reproducibility, and may be useful in clinical haemostasis laboratories for examination of high shear induced platelet functions.

Topics: Adolescent; Adult; Aged; Deamino Arginine Vasopressin; Factor XII Deficiency; Factor XIII Deficiency; Female; Hemorrhagic Disorders; Humans; Male; Middle Aged; Platelet Aggregation; Platelet Function Tests; Thrombasthenia; von Willebrand Diseases

Grey-platelet syndrome is a rare familial platelet impairment characterised by lack of alpha granules and giant vacuolated platelets. A Mexican family with grey-platelet syndrome associated to Marfan disease is presented. The family was comprised of 22 members, of whom 3 (the propositus and two of his nephews) could be studied. Two of them, with haemorrhagic symptoms since childhood, had moderate prolongation of the Ivy bleeding time which improved after DDAVP administration, plus moderate thrombocytopenia, giant platelets and abnormal platelet aggregation induced by adrenalin, ADP and collagen. Platelet factor 4 was normal. Electron microscope examination of platelets showed lack of alpha granules and increased dense bodies. The rarity of the casual association of two low-frequency genetic diseases, namely Marfan disease and the grey-platelet syndrome, is commented, along with the response attained with DDAVP in the two affected individuals.

Topics: Adult; Bleeding Time; Blood Platelets; Cytoplasmic Granules; Deamino Arginine Vasopressin; Female; Hemorrhagic Disorders; Humans; Infant; Male; Marfan Syndrome; Mexico; Pedigree; Platelet Aggregation

Haemorrhagic diathesis is a serious complication of uraemia. Desmopressin is known to shorten prolonged bleeding time in uraemia but mechanism of the haemostatic action of this drug remains still unknown. The aim of the work was to study the effect of desmopressin on some haemostatic parameters in relation to plasma and platelet serotonin. Desmopressin was administered i.v. to 33 haemodialysed patients (age range 27-66 years) in a dose of 0.4 microgram/kg b.w., 90 minutes after desmopressin infusion bleeding time became significantly shorter (p < 0.001) and correlated with the shortening of the euglobulin clot lysis time (r = -0.43, p < 0.05). Tissue plasminogen activator activity increased (p < 0.01) and its inhibitor (PAI) activity decreased (p < 0.001) after desmopressin infusion. A correlation between the fall in platelet serotonin content and changes in tissue plasminogen activator and PAI activities was found (r = 0.55, p < 0.01 respectively). A rise in plasma serotonin concentration was observed. In vitro desmopressin inhibited 14C serotonin uptake in a dose-dependent manner. After 2 hours of platelet incubation with desmopressin in a concentration of 4 ng/ml 16% of 14C serotonin was released. A possibility of serotoninergic mechanism in the haemostatic action of desmopressin is suggested.

Topics: Adult; Aged; Blood Platelets; Deamino Arginine Vasopressin; Dose-Response Relationship, Drug; Hemorrhagic Disorders; Hemostasis; Humans; Middle Aged; Serotonin; Tissue Plasminogen Activator; Uremia

Topics: Benzamidines; Deamino Arginine Vasopressin; Estrogens; Factor VIII; Fibrinogen; Guanidines; Hemorrhagic Disorders; Heparin; Humans; Kidney Failure, Chronic; Renal Dialysis; Thrombocytopenia

Two important haematological problems were found in an otherwise healthy 78 year old man: chronic myelomonocytic leukaemia; and a complex, acquired, hyperfibrinolytic bleeding disorder characterized by prolonged coagulation times, deficiency of coagulation factors V, X, and XI, anti-thrombin III and proteins C and S, with high concentrations of circulating tissue plasminogen activator, and low concentrations of plasminogen activator inhibitor. There may be a causal relation between the two conditions, with the peripheral blood monocytes mediating the hyperfibrinolytic process by the abnormal production of tissue plasminogen activator, though no previous description of a similar association has been reported.

Topics: Aged; Blood Coagulation Tests; Blood Transfusion; Deamino Arginine Vasopressin; Hemorrhagic Disorders; Humans; Leukemia, Myelomonocytic, Chronic; Male

A 73-year old patient suffering from IgM-paraproteinemia, plasma hyperviscosity, and acquired v. Willebrand's disease developed two consecutive bleeding episodes. He was treated by double-bag plasmapheresis and double-bag plasmapheresis combined with DDAVP. This combined therapy was superior to plasmapheresis alone, since bleeding ultimately stopped and did not reoccur. Thus, labour and expense of large volume plasma exchange and the costs of F VIII therapy could be prevented.

Topics: Aged; Blood Viscosity; Combined Modality Therapy; Deamino Arginine Vasopressin; Hemorrhagic Disorders; Humans; Male; Plasmapheresis; von Willebrand Diseases; Waldenstrom Macroglobulinemia

Topics: Administration, Intranasal; Administration, Topical; Antifibrinolytic Agents; Deamino Arginine Vasopressin; Hemophilia A; Hemorrhage; Hemorrhagic Disorders; Hemostatics; Humans; Injections, Subcutaneous; Liver Cirrhosis; Surgical Procedures, Operative; Uremia; von Willebrand Diseases

Between 1981 and 1986, we evaluated 59 patients who presented with isolated prolongation of bleeding time with normal platelet counts, platelet aggregation and coagulation parameters (including von Willebrand's factor), and without evidence of liver or kidney disease, or exposure to anti-platelet agents. These patients, termed as vascular fragility syndrome (VFS), were analyzed to further characterize their bleeding patterns. The pattern of bleeding manifestations was similar to that of patients with platelet dysfunction, such as mucocutaneous bleeding or excessive post-operative bleeding. In 16 patients, desmopressin (1-desamino-8-d-arginine vasopressin, DDAVP) was infused to control active bleeding or as a part of pre-surgical evaluation. Bleeding time improved (pre-DDAVP bleeding time 15.3 +/- 3.4 min, mean +/- S.D.; post-DDAVP bleeding time 10.7 +/- 3.9 min; p less than 0.01) within 30 minutes following the DDAVP infusion with either satisfactory arrest of acute bleeding or good control of subsequent hemostasis with surgery. Side effects with DDAVP were transient and minor, i.e. facial flushing, or conjunctival erythema. These findings indicate that VFS with isolated prolongation of bleeding time is a frequently encountered bleeding disorder and that DDAVP is effective in achieving hemostasis for the management of acute bleeding and may be useful prior to surgical procedures.

Topics: Bleeding Time; Deamino Arginine Vasopressin; Female; Hemorrhagic Disorders; Hemostasis; Humans; Infusions, Intravenous; Male; Pedigree

Desmopressin acetate (DDAVP) is efficacious in patients with von Willebrand's disease. It additionally appears to have value in patients with uremic or aspirin-induced platelet dysfunction. We report here three patients with primary platelet defects who had previously experienced grossly inadequate hemostasis to whom we administered DDAVP. Each successfully underwent surgical procedures with DDAVP as the only hemostatic agent. Although the mechanism of these salutary effects is unclear, DDAVP may exert an influence directly on the endothelium independent of correcting abnormalities of the factor VIII:von Willebrand complex associated with von Willebrand's disease.

Topics: Adolescent; Deamino Arginine Vasopressin; Factor VIII; Female; Hemophilia A; Hemorrhagic Disorders; Hemostasis, Surgical; Humans; Male; Middle Aged; Platelet Aggregation; Preoperative Care; von Willebrand Diseases; von Willebrand Factor

Five patients with glycogen storage disease type I (GSD-I) were evaluated for a bleeding diathesis and subsequently were given an infusion of 1-deamino-8-D-arginine vasopressin (DDAVP). Although platelet counts were normal or slightly elevated, the baseline template bleeding times were prolonged in four of the patients. Prothrombin times and activated partial thromboplastin times were normal, while ADP- and epinephrine-induced platelet aggregations were absent in the three patients tested. Ristocetin- and collagen-induced platelet aggregations were abnormal. Laurell and immunoradiometric determinations of the factor VIII-related antigen (vWf antigen) were decreased. Glyoxyl agarose gel electrophoresis of the patients' plasma revealed abnormal multimer patterns in four of the five patients. After the DDAVP infusion the platelet aggregation abnormalities persisted; however, the bleeding time and the von Willebrand antigen and activity corrected. We conclude that GSD-Ia patients may have a metabolically acquired form of von Willebrand's syndrome as well as an acquired intrinsic platelet defect, and that DDAVP may be useful in the management of bleeding in these patients.

Topics: Adult; Bleeding Time; Child, Preschool; Deamino Arginine Vasopressin; Electrophoresis, Agar Gel; Factor VIII; Female; Glycogen Storage Disease Type I; Hemorrhagic Disorders; Humans; Infusions, Parenteral; Male; Platelet Aggregation

The treatment of the haemorrhagic tendency of acute renal failure remains unsatisfactory. To investigate a potential new therapy, 8 patients with acute renal failure and prolonged bleeding times received an infusion of 1-deamino-8-d-arginine vasopressin (0.4 microgram/kg). This resulted in significant shortening of the bleeding time at 1 and 2 hours post-infusion. The beneficial effect on bleeding time had largely disappeared by 8 hours. Pre-infusion Factor VIII activities were either within the normal range or mildly increased and a tendency to further elevation was noted post-DDAVP. In contrast, no alteration in platelet count or haematocrit was observed. No adverse effects were reported, and no significant change in heart rate, blood pressure or plasma osmolarity was noted.

Topics: Acute Kidney Injury; Arginine Vasopressin; Bleeding Time; Deamino Arginine Vasopressin; Factor VII; Female; Hemorrhagic Disorders; Humans; Male; Platelet Function Tests; Time Factors

The treatment of the bleeding diathesis of renal failure remains unsatisfactory. 1-Deamino-8-D-arginine vasopressin (DDAVP) has recently been shown to shorten the prolonged bleeding time of renal failure and may as a result diminish the risk of clinical hemorrhage. We describe 2 cases where DDAVP was used successfully in the management of hemorrhage in the setting of renal insufficiency.

Topics: Acute Kidney Injury; Aged; Arginine Vasopressin; Bleeding Time; Deamino Arginine Vasopressin; Female; Hemorrhagic Disorders; Humans; Kidney Failure, Chronic; Male

The prolonged bleeding times of 12 patients with chronic renal failure were significantly shortened at 1 and 2 h after an infusion of 0.4 microgram/kg 1-deamino-8-D-arginine vasopressin (DDAVP). In 5 of these patients, the bleeding times 24 h after infusion had returned to approximate baseline values, suggesting that the effect of DDAVP is a temporary one. The levels of factor VIII coagulant activity, factor VIII related antigen activity, and factor VIII ristocetin cofactor activity were all normal or elevated prior to infusion, and a tendency to further elevation, although significant only for the factor VIII ristocetin cofactor activity, was observed after infusion of DDAVP. The latter observation suggests that the effect of DDAVP infusion on the bleeding time is unrelated to alteration of factor VIII activities.

Topics: Adult; Arginine Vasopressin; Bleeding Time; Deamino Arginine Vasopressin; Factor VIII; Hemorrhagic Disorders; Humans; Kidney Failure, Chronic; Middle Aged; Platelet Function Tests