deamino-arginine-vasopressin and Epistaxis

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

Delayed parkinsonism and dystonia are recognized phenomena in osmotic demyelinating syndrome (ODS). Dopamine receptor agonists and levodopa have been reported to benefit select patients.. We report a patient with ODS with severe pseudobulbar deficits, parkinsonism and dystonia, poorly responsive to levodopa, who experienced a remarkable improvement with pramipexole.. A marked response to pramipexole with lack of response to levodopa suggests a pre-synaptic source for his deficits coupled with injuries to non-nigral compensatory structures.. This case highlights a dramatic response of osmotic demyelination-induced parkinsonism/dystonia to pramipexole. A lack of response to levodopa suggests deficits in the pre-synaptic nigral as well as non-nigral compensatory structures.

Topics: Adult; Antiparkinson Agents; Deamino Arginine Vasopressin; Demyelinating Diseases; Dystonia; Epistaxis; Hemostatics; Humans; Hyponatremia; Levodopa; Locked-In Syndrome; Male; Myelinolysis, Central Pontine; Osmotic Pressure; Parkinsonian Disorders; Postoperative Hemorrhage; Pramipexole; Pseudobulbar Palsy; Rhinoplasty; Tetrahydronaphthalenes; Thiophenes; Treatment Failure; Treatment Outcome; von Willebrand Disease, Type 1

Cauterization, nasal packing, and topical and/or injection of intranasal vasoconstrictors have been the mainstay of treatment for epistaxis following outpatient nasal surgery. In this study, the authors report the clinical outcomes in a cohort of patients with postoperative epistaxis managed with a single dose of intravenous desmopressin.. A retrospective chart review of 268 consecutive nasal operations (rhinoplasty, septoplasty, and/or turbinectomy for cosmetic and/or functional purposes) was conducted. Information on demographics, perioperative blood pressure, postoperative management, and effectiveness of the measures used was assessed. The primary outcome variable was cessation of bleeding.. Nine patients were identified who experienced excessive postoperative bleeding following discharge from the surgical facility. Each patient received 0.3 μg/kg of intravenous desmopressin over 30 minutes under the supervision of the local emergency room physician with verbal instructions from the treating plastic surgeon. After administration of desmopressin, bleeding either stopped completely (eight patients) or slowed down significantly to allow discharge (one patient). No significant adverse side effects of desmopressin were observed. No patient was known to be taking medication negatively affecting coagulation perioperatively. Preoperatively, two patients were documented to have von Willebrand disease and thus received desmopressin preoperatively. Average blood pressure was 116/71 mmHg intraoperatively (range, 109 to 126/66 to 83 mmHg) and 118/74 mmHg postoperatively (range, 105 to 129/65 to 85 mmHg).. Unremitting postoperative epistaxis following outpatient nasal surgery can be successfully controlled by a protocol using intravenous desmopressin without the need for alternative maneuvers.

Topics: Adolescent; Adult; Cohort Studies; Deamino Arginine Vasopressin; Epistaxis; Female; Follow-Up Studies; Hemostatics; Humans; Infusions, Intravenous; Intraoperative Complications; Male; Middle Aged; Nasal Septum; Postoperative Complications; Postoperative Hemorrhage; Rhinoplasty; Treatment Outcome; Turbinates; von Willebrand Diseases; Young Adult

Recent studies indicate that bleeding disorders, particularly von Willebrand disease (VWD) is more prevalent than previously thought in adolescents with menorrhagia. Menorrhagia management in undiagnosed disorders of hemostasis may be associated with unwanted risks and complications. The prevalence of symptomatic VWD in the pediatric primary care setting appears to be 0.11% (95% CI, 0.04-0.25%). Studies evaluating the prevalence of VWD in adolescents with menorrhagia have included over 500 patients with a prevalence range from 3 to 36% depending on the clinical setting studied, with the highest prevalence seen in adolescents referred to an outpatient Hemophilia Center, while the lowest prevalence is seen in the acute hospital setting. Recently, the diagnosis of VWD has been facilitated by the use of pediatric bleeding questionnaires that have proved useful in quantifying the severity of bleeding symptoms. Treatment of VWD is often complex because a combination of therapies is often required. Potential treatment options include estrogen-progesterone preparations, desmopressin, antifibrinolytic agents and von Willebrand factor concentrates. More research is needed to evaluate the effectiveness of the various treatment modalities in the adolescent population.

Topics: Adolescent; Aminocaproic Acid; Antifibrinolytic Agents; Child; Contusions; Deamino Arginine Vasopressin; Epistaxis; Female; Hemostatics; Humans; Medical History Taking; Menorrhagia; Platelet Transfusion; Prevalence; Surveys and Questionnaires; Tranexamic Acid; von Willebrand Diseases; von Willebrand Factor

Topics: Child; Deamino Arginine Vasopressin; Epistaxis; Factor V Deficiency; Female; Hemophilia A; Humans; von Willebrand Diseases

Type 2A von Willebrand disease (VWD) is characterized by decreased platelet-dependent function of von Willebrand factor (VWF); this in turn is associated with an absence of high-molecular-weight multimers. Sequence analysis of the VWF gene from two unrelated type 2A VWD patients showed an identical, novel, heterozygous T-->G transversion at nucleotide 4508, resulting in the substitution of L1503R in the VWF A2 domain. This substitution, which was not found in 60 unrelated normal individuals, was introduced into a full-length VWF cDNA and subsequently expressed in 293T cells. Only trace amount of the mutant VWF protein was secreted but most of the same was retained in 293T cells. Co-transfection experiment of both wild-type and mutant plasmids indicated the dominant-negative mechanism of disease development; as more of mutant DNA was transfected, VWF secretion was impaired in the media, whereas more of VWF was stored in the cell lysates. Molecular dynamic simulations of structural changes induced by L1503R indicated that the mean value of all-atom root-mean-squared-deviation was shifted from those with wild type or another mutation L1503Q that has been reported to be a group II mutation, which is susceptible to ADAMTS13 proteolysis. Protein instability of L1503R may be responsible for its intracellular retention and perhaps the larger VWF multimers, containing more mutant VWF subunits, are likely to be mal-processed and retained within the cell.

Topics: Adolescent; Amino Acid Substitution; Deamino Arginine Vasopressin; DNA Mutational Analysis; Epistaxis; Exons; Female; Gene Expression; Hemostasis; Humans; Male; Middle Aged; Models, Molecular; Molecular Biology; Mutation; Platelet Adhesiveness; Polymerase Chain Reaction; Recombinant Proteins; Structure-Activity Relationship; Transfection; von Willebrand Diseases; von Willebrand Factor

Topics: Adolescent; Antifibrinolytic Agents; Bernard-Soulier Syndrome; Comorbidity; Deamino Arginine Vasopressin; Epistaxis; Female; Hemostatics; Humans; Menorrhagia; Recurrence; Thrombasthenia; Tranexamic Acid

A 58-year-old woman was brought to our emergency department with massive nasal bleeding and hemodynamic instability. The patient had been on clopidogrel treatment (75 mg/day) for 2 years, which was started after an episode of transitory ischemic attack. Blood pressure normalized following the administration of intravenous fluids, and the bleeding stopped after nasal tamponade and desmopressin therapy.

Topics: Clopidogrel; Deamino Arginine Vasopressin; Epistaxis; Female; Hemodynamics; Hemostatics; Humans; Ischemic Attack, Transient; Middle Aged; Platelet Aggregation Inhibitors; Ticlopidine

Topics: Ambulatory Care; Child; Deamino Arginine Vasopressin; Epistaxis; Follow-Up Studies; Humans; Laryngoscopes; Low-Level Light Therapy; Male; Premedication; Recurrence; Retreatment; Telangiectasia, Hereditary Hemorrhagic; Treatment Outcome

Topics: Administration, Intranasal; Blood Coagulation Disorders; Cost of Illness; Cost Savings; Deamino Arginine Vasopressin; Epistaxis; Humans; Quality of Life; Self Administration; Surveys and Questionnaires; Sweden

Of 150 consecutive patients who underwent dacryocystorhinostomy, postoperative hemorrhage requiring treatment occurred in 2 patients, both of whom had endogenous platelet dysfunction without thrombocytopenia. The first patient had macroglobulinemia, and the second patient had congenital platelet hypofunction. Prophylactic 1-deamino-8-D-arginine vasopressin (desmopressin; DDAVP) was used successfully to decrease intraoperative bleeding in the second patient. Of the 15 patients with exogenous platelet dysfunction secondary to the use of aspirin or nonsteroidal anti-inflammatory agents within 1 week of operation, none had hemorrhaging. Dacryocystorhinostomy should be undertaken cautiously and with hematologic consultation in patients with blood dyscrasias.

Topics: Adult; Aged; Aged, 80 and over; Blood Loss, Surgical; Blood Platelet Disorders; Dacryocystitis; Dacryocystorhinostomy; Deamino Arginine Vasopressin; Epistaxis; Female; Hemorrhage; Humans; Lacrimal Apparatus Diseases; Male; Nasolacrimal Duct; Postoperative Complications; Waldenstrom Macroglobulinemia

Topics: Adolescent; Child; Deamino Arginine Vasopressin; Epistaxis; Female; Humans; Male; Menorrhagia; Thrombocytopenia

Hemophilia A, von Willebrand's disease and uremia prolong the bleeding time in affected children. Management of hemorrhage is difficult in these patients, and often requires multiple therapeutic modalities. Desmopressin, a synthetic analog of arginine vasopressin (DDAVP), decreases the bleeding time in patients with these disorders. We describe a case of a child with uremia and spontaneous epistaxis originating from the adenoid. Bleeding was controlled with administration of DDAVP and other measures. DDAVP is effective for the rapid, temporary correction of prolonged bleeding time associated with hemophilia A, von Willebrand's disease or uremia. The use of DDAVP avoids the risks associated with blood component transfusion. DDAVP may be used as a single hemostatic agent in minor surgical procedures, or in combination with other therapeutic modalities in major surgical procedures.

Topics: Adenoids; Blood Coagulation; Child; Deamino Arginine Vasopressin; Drug Evaluation; Epistaxis; Female; Humans; Uremia