deamino-arginine-vasopressin and Gastrointestinal-Hemorrhage

Gastrointestinal (GI) bleeding is distinctive of severe von Willebrand disease (VWD), generally arising in older patients; in most cases, blood transfusion and hospitalization are required. The presence of arteriovenous malformations is often described when endoscopic examinations are performed. Patients with congenital type 3, 2A, and 2B are those most frequently affected by this symptom, possibly due to the loss of high-molecular-weight multimers of von Willebrand factor (VWF). GI bleeding can also occur in patients affected by acquired von Willebrand syndrome. Endoscopic examination of the GI tract is necessary to exclude ulcers and polyps or cancer as possible causes of GI bleeding. In congenital VWD, prophylaxis with VWF/factor VIII concentrates is generally started after GI-bleeding events, but this therapy is not always successful. Iron supplementation must be prescribed to avoid chronic iron deficiency. Possible rescue therapies (high-dose statins, octreotide, thalidomide, lenalidomide, and tamoxifen) were described in a few case reports and series; however, surgery may be necessary in emergency situations or if medical treatment fails to stop bleeding. In this article, we present several clinical cases that highlight the clinical challenges of these patients and possible strategies for their long-term management.

Topics: Adult; Aged; Aged, 80 and over; Blood Transfusion; Deamino Arginine Vasopressin; Drug Combinations; Factor VIII; Female; Gastrointestinal Hemorrhage; Humans; Male; Middle Aged; Prognosis; von Willebrand Diseases; von Willebrand Factor

The management of von Willebrand disease (VWD) is based upon the dual correction of the primary hemostasis defect, due to the inherited deficiency of von Willebrand factor (VWF), and of the secondary defect of factor VIII coagulant activity (FVIII:C), due to the loss of binding and stabilization by VWF of this intrinsic coagulation factor in flowing blood. The traditional therapeutic weapons (the synthetic derivative of the antidiuretic hormone desmopressin and plasma-derived VWF/FVIII concentrates) are able to transiently correct both the defects. With the goal of tackling the primary deficiency in the disease, that is, VWF, but at the same time exploiting the normal capacity of patients to produce FVIII, the novel approach of replacing only VWF was implemented in the last 10 years. Following the manufacturing of a concentrate fractionated from human plasma and of one obtained by recombinant DNA technology, clinical studies have shown that VWF-only products correct not only the primary VWF deficiency but also the secondary FVIII:C deficiency. The demonstrated efficacy of these products in various clinical situations and, ultimately, in such a hemostasis-challenging context as surgery testifies to the effectiveness and safety of this approach. It remains to be seen whether VWF-only products are efficacious and safe in still-unexplored situations, such as use in children; the long-term use for prophylaxis; and in recurrent gastrointestinal (GI) bleeding due to angiodysplasia, a major therapeutic problem in VWD.

Topics: Aged; Deamino Arginine Vasopressin; Factor VIII; Female; Gastrointestinal Hemorrhage; Humans; von Willebrand Diseases; von Willebrand Factor

Topics: Deamino Arginine Vasopressin; Gastrointestinal Hemorrhage; Hemostatics; Humans; Reference Standards; 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

Approximately one-fifth of the patients, referred to the acute gastrointestinal bleeding unit took antithrombotic drugs (anticoagulants and antiplatelet agents) in a dose or with an effect, which made the causal relationship with acute bleeding episodes unequivocal. This paper analyzes the data of the first 100 such patients of the ward. The majority used acethyl-salicylic acid derivatives, however, a substantial number were on coumarol, and some bleeding patients with prosthetic heart valves were also observed. Haemostasis was forced by urgent, accurate, intervention based endoscopy along with simultaneous rapid efforts to correct the underlying clotting abnormalities. Prosthetic valve patients needed special care and attendance, and the reinstitution of anticoagulant treatment as soon as possible to maintain valve patency. The experiences and schedules of the authors, as well as the proposals of the literature are reviewed and summarized.

Topics: Acute Disease; Anticoagulants; Aspirin; Coumarins; Deamino Arginine Vasopressin; Gastrointestinal Hemorrhage; Heart Valve Prosthesis; Hemostatics; Heparin; Humans; Thrombolytic Therapy; Ticlopidine

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

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

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

Intraoperative blood loss more than 400 mL during gastrointestinal surgery is an independent predictor of mortality. Desmopressin acetate (DDAVP) could reduce perioperative blood loss. Few studies have prompted concerning the effects of DDAVP on gastrointestinal surgery. This study was to investigate whether DDAVP can decrease blood loss in patients with massive hemorrhage undergoing gastrointestinal surgery.. A multiple-centers, double-blind clinical trial was conducted, patients who underwent gastrointestinal surgery were recruited from 3 hospitals, randomly assigned to two different groups. Patients in the treatment group received desmopressin 0.3 ug/kg,30 min once a day after surgery, patients in the control group received 50 ml saline for 30 min. The primary outcome was the changes of hemoglobin at 24 hours after the surgery. And the secondary outcomes included coagulation function, urine volume, serum creatinine, and safety.. There were 59 patients enrolled between 1 June 2015 and 1 June 2017. At 24hr.after surgery, a decrease in hemoglobin in the DDAVP group was significantly lower than that in the NS group (-5.0±6.9 g/L vs. -10.2±9.3g/L, p=0.03). Sonoclot® showed that the platelet function in the DDAVP group was higher than that in NS group at 24 hr. (2.56 ±0.59 vs. 1.91 ±0.72, p<0.05). There was no difference in urine volume and serum creatinine at 24 hr. between two group.. DDAVP could reduce post-operation blood loss in patients with massive hemorrhage undergoing surgery by improving the platelet function. We observed no difference in urine volume and serum creatinine in two groups.

Topics: Aged; Blood Platelets; Creatinine; Deamino Arginine Vasopressin; Digestive System Surgical Procedures; Double-Blind Method; Female; Gastrointestinal Hemorrhage; Hemoglobins; Hemostasis, Surgical; Humans; Male; Middle Aged; Postoperative Care; Postoperative Hemorrhage; Treatment Outcome; Urine

1-Deamino-8-D-arginine vasopressin (DDAVP, desmopressin), a synthetic analog of the antidiuretic hormone L-arginine vasopressin, improves hemostasis parameters in cirrhotic patients. Hence its use in combination with a vasoactive drug such as terlipressin might improve the performance of this drug in controlling variceal bleeding. The aim of this trial was to compare the efficacy of desmopressin plus terlipressin with that of terlipressin alone in controlling acute variceal hemorrhage. Cirrhotic patients with active variceal hemorrhage diagnosed endoscopically were randomized within 2 hr of admission to receive desmopressin plus terlipressin or placebo plus terlipressin. Terlipressin (2 mg, intravenous bolus) was given at time 0 and every 4 hr thereafter for 24 hr. Desmopressin (0.3 microgram/kg, intravenously) or placebo was given in saline solution over 30 min at time 0 and at 26 hr. Patients were monitored for 24 hr after cessation of treatment. Treatment failure was defined as recurrence of active bleeding during treatment or within the 24 hr after treatment. After enrolling 51 of the planned 84 patients, we carried out an interim analysis. Treatment failure occurred in 13 of 24 patients randomized to receive desmopressin plus terlipressin (54.2%) and in 6 of 22 patients randomized to receive terlipressin (27.3%) (p = 0.06, Fisher's exact test). The trial was interrupted at this stage because patients treated with the "new" therapy fared worse than those treated with the standard therapy, and the possibility of reversing this trend by completing the trial was deemed remote. The addition of desmopressin does not improve and may worsen the efficacy of terlipressin in controlling acute variceal bleeding in cirrhotic patients.

Topics: Acute Disease; Deamino Arginine Vasopressin; Double-Blind Method; Drug Therapy, Combination; Esophageal and Gastric Varices; Female; Gastrointestinal Hemorrhage; Humans; Liver Cirrhosis; Lypressin; Male; Middle Aged; Terlipressin; Treatment Failure

Topics: Angiodysplasia; Aortic Valve; Aortic Valve Stenosis; Deamino Arginine Vasopressin; Female; Gastrointestinal Hemorrhage; Heart Valve Prosthesis Implantation; Hemostatics; Humans; Middle Aged; Postoperative Care; Postoperative Hemorrhage

Due to lack of patient/health care provider awareness causing delayed diagnosis, the bleeding phenotype and provider interventions in adolescents with heavy menstrual bleeding (HMB) and bleeding disorders (BD) may be different when compared to adults.. The aim of this study was to compare/characterize bleeding phenotype and provider interventions in postmenarchal adolescents < 18 years and premenopausal adults ≥ 18 years with HMB and BD.. Patient demographics, BD, and provider interventions/therapy details for HMB were compared between both age groups enrolled in the Centers for Disease Control and Prevention (CDC) Female Universal Data Collection (UDC) surveillance project in United States hemophilia treatment centres. Cross-sectional descriptive analyses including frequency distributions, summary statistics, bivariate and logistic regression analyses were performed.. Of 269 females (79 adolescents; median age 16 years, interquartile range (IQR) = 2; 190 adults; median age 27 years, IQR = 13) evaluated, BD distribution was similar in both groups. Compared to adolescents, adults more often had family history of bleeding (Adjusted odds ratios [AOR] = 2.6, 1.3-5.6), delay in diagnosis (AOR = 2.5, 1.2-4.9), bleeding with dental procedures (AOR = 2.0, 1.0-4.0), gastrointestinal bleeding (AOR = 4.6, 1.0-21.9), anaemia (AOR = 2.7, 1.4-5.2), utilized desmopressin less often (AOR = 0.4, 0.2-0.8) and underwent gynaecologic procedure/surgery more frequently (AOR = 5.9, 1.3-27.3).. Bleeding phenotypes of adolescents and adults with HMB and BD were different with more frequent bleeding complications, anaemia, gynaecologic procedures/surgeries, less desmopressin use and more delay in diagnosing BD in adults. Longitudinal studies are needed to determine whether improved patient/provider awareness and education will translate to early diagnosis and timely management of BD/HMB in adolescents that may prevent/reduce future haematologic/gynaecologic complications.

Topics: Adolescent; Adult; Anemia; Antifibrinolytic Agents; Blood Coagulation Disorders; Cross-Sectional Studies; Deamino Arginine Vasopressin; Delayed Diagnosis; Female; Gastrointestinal Hemorrhage; Hemostatics; Humans; Logistic Models; Menopause; Menorrhagia; Odds Ratio; Phenotype; Young Adult

Bleeding originating in the gastrointestinal (GI) tract is one of the most common adverse events after left ventricular assist device (LVAD) implantation. In these patients, GI bleeding appears to be the consequence of altered hemostasis on the one hand and alterations of the GI microvasculature on the other.. We report the case of a patient who suffered repeated, severe GI bleeding early after implantation of a HeartMate II continuous-flow LVAD.. After failure of conventional treatment strategies, GI bleeding was controlled using repeated transfusions of a purified von Willebrand factor (VWF) concentrate, almost devoid of Factor VIII (Wilfactin, LFB). No episodes of pump thrombosis were noted. Subsequent to VWF transfusions, we observed a progressive normalization of circulating vascular endothelial growth factor levels.. Our data raise the possibility that, in addition to its hemostatic properties, transfusions of VWF might have acted as an antiangiogenic factor.

Topics: Aged; Combined Modality Therapy; Deamino Arginine Vasopressin; Embolization, Therapeutic; Erythrocyte Transfusion; Fibrinogen; Gastrointestinal Hemorrhage; Heart Ventricles; Heart-Assist Devices; Hemostatics; Humans; Laser Coagulation; Male; Neovascularization, Physiologic; Postoperative Hemorrhage; Recurrence; Tranexamic Acid; von Willebrand Factor

Topics: Adult; Blood Coagulation; Colonic Polyps; Deamino Arginine Vasopressin; Gastrointestinal Hemorrhage; Humans; Sigmoid Neoplasms; Sigmoidoscopy; von Willebrand Diseases

A patient with von Willebrand's disease had recurrent gastrointestinal bleeding from angiodysplasia, with inadequate response to von Willebrand factor substitution, medical and endoscopic treatment, and resection of affected bowel. Frequent blood transfusions were required. She started home treatment with recombinant activated factor VII (rFVIIa) at the onset of bleeding, in addition to her standard therapy. From then on, bleeds could be controlled rapidly and no more blood transfusions were needed. We conclude that rFVIIa is effective in this case of angiodysplasia and might be a therapeutic option in similar patients.

Topics: Angiodysplasia; Blood Transfusion; Combined Modality Therapy; Deamino Arginine Vasopressin; Estrogens; Factor VIIa; Factor VIII; Female; Gastrointestinal Hemorrhage; Gastroscopy; Humans; Iron; Jejunal Diseases; Melena; Middle Aged; Omeprazole; Recombinant Proteins; Recurrence; Tranexamic Acid; Treatment Outcome; von Willebrand Diseases; von Willebrand Factor

A case of idiopathic immune-mediated von Willebrand's disease (AvWD) associated angiodysplasia and recurrent lower gastrointestinal bleeding is reported. Coagulation parameters at presentation were activated partial thromboplastin time of 41 sec, bleeding time >15 min, factor VIII procoagulant activity, 5%; von Willebrand factor antigen (WF:Ag) 5%, and vWF:ristocetirn cofactor activity 11% sodium dodecyl sulfate-agarose gel electrophoresis pattern of plasma vWF showed a pattern similar to type II vWD. An in vitro inhibitor against vWF in the immunoglobulin (Ig)G fraction of the patient's plasma was demonstrated vWF parameters showed a short-lived increase after 1-deamino-8-D-arginine vasopressin (DDAVP) administration. The patient's bleeding episodes were initially managed adequately with cryoprecipitate replacement therapy and DDAVP, to which she became refractory. No significant improvement was achieved following the institution of immunosuppressive therapy in the form of high-dose steroids and cyclophosphamide. She was then treated with intravenous immunoglobulin (IvIg) to which she showed an adequate response in terms of her clinical situation and her hemostatic parameters. The patient is on maintenance treatment with repeated courses of IvIg based on vWF parameter monitoring. To our knowledge, this is the third reported association between idiopathic immune-mediated AvWD and angiodysplasia.

Topics: Aged; Angiodysplasia; Autoantibodies; Bleeding Time; Deamino Arginine Vasopressin; Electrophoresis, Polyacrylamide Gel; Factor VIII; Female; Gastrointestinal Hemorrhage; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Metabolic Clearance Rate; Partial Thromboplastin Time; von Willebrand Diseases; von Willebrand Factor

Topics: Bias; Deamino Arginine Vasopressin; Gastric Acidity Determination; Gastrointestinal Hemorrhage; Humans; Omeprazole; Stress, Physiological

A patient with hereditary hemorrhagic telangiectasia and reduced levels of von Willebrand factor had severe recurrent gastrointestinal bleeding. Treatment with either desmopressin acetate or cryoprecipitate normalized both bleeding time and kaolin-activated partial thromboplastin time. Hematochezia (five episodes) continued, however, for a week despite daily treatment with cryoprecipitate, whereas treatment with desmopressin at the onset of three later episodes of hematochezia resulted in the prompt cessation of bleeding. After an episode of life-threatening gastrointestinal bleeding, desmopressin administration allowed a total colectomy without excessive bleeding. During the 3 years after colectomy, no further gastrointestinal bleeding occurred. In the past year, however, three episodes of severe epistaxis responded promptly to intravenous infusion of desmopressin. We conclude that desmopressin may be useful in the management of bleeding in patients with hereditary hemorrhagic telangiectasia with or without von Willebrand factor deficiency.

Topics: Adult; Deamino Arginine Vasopressin; Factor VIII; Fibrinogen; Gastrointestinal Hemorrhage; Humans; Male; Recurrence; Telangiectasia, Hereditary Hemorrhagic

Topics: Aged; Amyloidosis; Deamino Arginine Vasopressin; Gastrointestinal Hemorrhage; Humans; Male

Topics: Aged; Bundle-Branch Block; Deamino Arginine Vasopressin; Gastrointestinal Hemorrhage; Humans; Male; Myocardial Infarction

Bleeding in uraemia is frequently manifest as diffuse gastric mucosal haemorrhage. In a patient with acute renal failure, prolonged bleeding time and life-threatening bleeding of this type, effective haemostasis was established with platelet-rich blood products and 1-deamino-8-D-arginine vasopressin (DDAVP).

Topics: Adult; Deamino Arginine Vasopressin; Female; Gastrointestinal Hemorrhage; Humans; Uremia

Clinical bleeding in uremia is a frequent problem and seems to correlate with a prolonged bleeding time. The vasopressin analog deamino-8-D-arginine vasopressin has been shown to shorten the bleeding time and to decrease clinical bleeding when administered intravenously to uremic patients. In the present study we administered the readily available intranasal deamino-8-D-arginine vasopressin to 2 uremic patients and demonstrated a decreased bleeding time and improvement in clinical bleeding.

Topics: Administration, Intranasal; Aged; Arginine Vasopressin; Bleeding Time; Deamino Arginine Vasopressin; Female; Gastrointestinal Hemorrhage; Humans; Middle Aged; Time Factors; Uremia