INTRODUCTION Oculocutaneous albinism (OCA) in combination with a platelet function defect caused by a disturbed release reaction from platelet δ-granules (storage pool defect - SPD) is typical for the autosomal recessive inherited Hermansky-Pudlak syndrome (HPS). CASE REPORT A girl (age: 13 years) with OCA was hospitalized with transfusion-requiring menorrhagia. The suspicion of HPS was confi...

In order to determine anticoagulation strategies in OPCAB a questionnaire survey among 750 European cardio-thoracic surgeons was performed. Questions addressed volume of OPCAB procedures performed, intra- and perioperative heparinization and antiplatelet therapy, as well as perioperative management. A total of 325 (43.7%) questionnaires were returned and validated. Perioperative protocols for a...

BACKGROUND Open heart surgeries under cardiopulmonary bypass are associated with excessive perioperative bleeding that often requires reoperation. Antifibrinolytics like epsilon aminocaproic acid and tranexamic acid are widely used to control bleeding. There are limited studies primarily showing the impact of these drugs on the incidence of reopening following open heart surgical procedures. Th...

PURPOSE To evaluate the patient satisfaction and health related quality of life (HRQoL) for levonorgestrel-releasing intrauterine system (LNG-IUS) versus conventional medical treatments ([CMTs] combined oral contraceptives, oral progestins, and antifibrinolytics, alone or in combination) in Asian women with heavy menstrual bleeding (HMB). PATIENTS AND METHODS A total of 647 patients diagnosed...

Factor V (FV; proaccelerin or labile factor) is the plasma cofactor for the prothrombinase complex that activates prothrombin to thrombin. FV deficiency can be caused by mutations in the FV gene or in genes encoding components of a putative cargo receptor that transports FV (and factor VIII) from the endoplasmic reticulum to the Golgi. Because FV is present in platelet alpha-granules as well as...

In June 2011 the Canadian National Advisory Committee on Blood and Blood Products sponsored an international consensus conference on transfusion and trauma. A panel of 10 experts and two external advisors reviewed the current medical literature and information presented at the conference by invited international speakers and attendees. The Consensus Panel addressed six specific questions on the...

Significant variability in transfusion practice persists despite guidelines. Although the lysine analogues are effective antifibrinolytics, safety concerns exist with high doses tranexamic acid. Despite recombinant activated factor VII promising results in massive bleeding after cardiac surgery, it significantly increases arterial thromboembolic risk. Aortic valve repair may evolve to standard ...

Venous air embolism (VAE) is a potential complication during cranial vault remodeling requiring early detection and prompt therapeutic intervention. The incidence of VAE has been reported to be as high as 82.6% during open craniectomy for craniosynostosis repair. On the other hand, two separate studies reported a much lower incidence of VAE (8% and 2%) during endoscopic strip craniectomy. As su...