Mobile computed tomography at the Munich Oktoberfest : From idea to implementation.

Background: The Munich Oktoberfest, the largest folk festival in the world, attracts around 6.2 million visitors over 16-18 days. Some of the visitors exhibit certain risky behaviors, leading to a significantly increased number of medical emergencies during the festival.

Invasive procedures and surgery following etranacogene dezaparvovec gene therapy in people with hemophilia B.

Background: Little information regarding the management of invasive procedures in people with hemophilia B (HB) after undergoing gene therapy is available. Here, we report the management of invasive procedures in people with severe or moderately severe HB who had previously been treated with etranacogene dezaparvovec in the phase 2b and phase 3 Health Outcomes with Padua Gene; Evaluation in Hemophilia B clinical trials (NCT03489291 and NCT03569891).

Objectives: The objective of this study was to describe the use of exogenous FIX, endogenous FIX activity prior to invasive procedures, and peri- and postoperative bleeds in participants who underwent invasive procedures after receiving etranacogene dezaparvovec gene therapy.

Fitusiran prophylaxis in people with hemophilia A or B who switched from prior BPA/CFC prophylaxis: the ATLAS-PPX trial.

Fitusiran, a subcutaneous investigational small interfering RNA therapeutic, targets antithrombin to rebalance hemostasis in people with hemophilia A or B (PwHA/B), irrespective of inhibitor status. This phase 3, open-label study evaluated the efficacy and safety of fitusiran prophylaxis in males aged ≥12 years with hemophilia A or B, with or without inhibitors, who received prior bypassing agent (BPA)/clotting factor concentrate (CFC) prophylaxis. Participants continued their prior BPA/CFC prophylaxis for 6 months before switching to once-monthly 80 mg fitusiran prophylaxis for 7 months (onset and efficacy periods).

Infrastructural considerations of implementing gene therapy for hemophilia in the Nordic context.

Background: Despite improvements in hemophilia care, challenges remain, including treatment burden and impaired quality of life. Gene therapy may overcome these. However, its introduction presents a challenge.

Inspection and maintenance of industrial infrastructure with autonomous underwater robots.

Underwater infrastructure, such as pipelines, requires regular inspection and maintenance including cleaning, welding of defects and valve-turning or hot-stabbing. At the moment, these tasks are mostly performed by divers and Remotely Operated Vehicles (ROVs) but the use of intervention Autonomous Underwater Vehicles (intervention-AUVs) can greatly reduce operation time, risk, and cost. However, autonomous underwater manipulation has not yet reached a high technological readiness and is an intensively researched topic.

Prostatic artery embolization in men with severe hemophilia a: a case report of two patients.

Background: This is the first case report describing the peri- and postoperative hemostasis plans in two men with severe hemophilia A (HA) who underwent prostatic artery embolization (PAE) for symptomatic benign prostatic hyperplasia (BPH).

Case Presentation: Two patients with severe HA and lower urinary tract symptoms (LUTS) not responding to medical therapy underwent PAE at our institution. In both patients, intermittent administration of decreasing doses of extended half-life recombinant factor VIII (EHL rFVIII) concentrate from 30 min before to 7 days after the PAE resulted in good hemostatic control.

A national Danish proof of concept on feasibility and safety of home -based intensive chemotherapy in patients with acute myeloid leukemia.

Technological advances have made it possible to offer home-based chemotherapy to patients without health care professionals being present. Prior studies on effects of home-based treatment lack inclusion of patients with hematologic malignancies. We present data from a multicenter single-arm feasibility and safety study of home-based intensive chemotherapy in patients with newly diagnosed acute myeloid leukemia and their quality of life and psychological wellbeing.

[SARS-CoV-2 vaccine-induced immune thrombosis and thrombocytopenia].

Vaccine-induced immune thrombosis and thrombocytopenia (VITT) is a new syndrome, which has emerged after introduction of the adenovirus vector-based COVID-19 vaccines ChAdOx1 nCoV-19 og Ad26.COV2-SVITT is characterised by venous thrombosis at unusual, and often multiple localisations, especially including cerebral venous sinus thrombosis. Besides, bleeding manifestations often occur.

A case of thrombocytopenia and multiple thromboses after vaccination with ChAdOx1 nCoV-19 against SARS-CoV-2.

Recently, reports of severe thromboses, thrombocytopenia, and hemorrhage in persons vaccinated with the chimpanzee adenovirus-vectored vaccine (ChAdOx1 nCoV-19, AZD1222, Vaxzevria; Oxford/AstraZeneca) against severe acute respiratory syndrome coronavirus 2 have emerged. We describe an otherwise healthy 30-year-old woman who developed thrombocytopenia, ecchymosis, portal vein thrombosis, and cerebral venous sinus thrombosis the second week after she received the ChAdOx1 nCoV-19 vaccine. Extensive diagnostic workup for thrombosis predispositions showed heterozygosity for the prothrombin mutation, but no evidence of myeloproliferative neoplasia or infectious or autoimmune diseases.

Increments in DNA-thioguanine level during thiopurine-enhanced maintenance therapy of acute lymphoblastic leukemia.

Maintenance therapy containing methotrexate and 6-mercapto - purine is essential to cure acute lymphoblastic leukemia (ALL). Cytotoxicity is elicited by incorporation of thioguanine nucleotides into DNA (DNA-TG), and higher leukocyte DNA-TG is associated with increased relapse-free survival. As 6-thioguanine provides 6- fold higher cytosolic levels of thioguanine nucleotides than does 6- mercapto purine, we added low-dose 6-thioguanine to methotrexate/6- mercapto purine maintenance therapy to explore if this combination results in significantly higher DNA-TG.

[Work-up of thrombophilia].

The number of thrombophilia investigations has been steadily increasing over the past decade. However, it is debatable to what extent treatment of thromboembolic events is further qualified by thrombophilia investigations. Based of the most recent literature as well as international and national expert opinions, this review provides a status on the frequency and severity of inherited and acquired thrombophilia, discusses indication for thrombophilia assessment, states the analyses to be included in the test panel, and the clinical implications that presence of thrombophilia may have.

Histiocytic Sarcoma: Challenging Course, Dismal Outcome.

Histiocytic sarcoma (HS) is a rare hematopoietic neoplasm derived from non-Langerhans histiocytic cells of the monocyte/macrophage system. With an incidence of 0.17/million individuals and a slight male preference, HS presents with a wide age distribution.

Introduction of a Comprehensive Diagnostic and Interdisciplinary Management Approach in Haematological Patients with Mucormycosis: A Pre and Post-Intervention Analysis.

Mucormycosis is a life threatening infection in patients with haematological disease. We introduced a Mucorales-PCR and an aggressive, multidisciplinary management approach for mucormycosis during 2016-2017 and evaluated patient outcomes in 13 patients diagnosed and treated in 2012-2019. Management principle: repeated surgical debridement until biopsies from the resection margins were clean as defined by negative Blankophor microscopy, Mucorales-PCR (both reported within 24 h), and cultures.

bacteraemia in a patient with multiple haematological malignancies.

We present a case of sepsis in a woman suffering from multiple myeloma and myelodysplastic syndrome. , a Gram-positive coccus and a gut commensal, has been described in nine cases of infection in the literature, with most infections having occurred in patients with either gastrointestinal symptoms or prosthesis infections. In this case, was identified by mass spectrometry, and showed susceptibility to penicillin, meropenem, tetracycline, metronidazole and clindamycin.

[Venous thromboembolism in patients with COVID-19].

The importance of venous thromboembolism (VTE) as a major complication in patients with severe corona virus disease 2019 (COVID-19) is becoming increasingly evident. In this review, we describe the proposed pathophysiology of the prothrombotic coagulation changes observed in patients with COVID-19. Further, based on a review of the currently available evidence on VTE prevalence in patients with COVID-19, we present and discuss the recommendations from the Danish Society of Thrombosis and Haemostasis on the use of thromboprophylaxis in patients with COVID-19.

Systematic patient involvement for homebased outpatient administration of complex chemotherapy in acute leukemia and lymphoma.

Based on experience with comprehensive patient involvement, we present data from implementation of portable, programmable infusion pumps (PPP) for home-based chemotherapy administration in patients with acute leukaemia and in lymphoma patients receiving (carmustine, etoposide, cytarabine, melphalan) BEAM regimen. Data from 84 patients, receiving 177 cycles of PPP administered chemotherapy, showed convincing safety with minor equipment errors encountered and with high patient satisfaction. In-hospital days could be reduced with 52% out of a total of 1197 treatment days.

Transfusion transmission of hepatitis A virus with fecal shedding in a previously hepatitis A vaccinated recipient.

We describe a rare case of hepatitis A virus (HAV) replication in feces despite presence of hepatitis A antibodies in an acute myeloid leukemia (AML) patient after transfusion with HAV contaminated platelets. The patient has been vaccinated against HAV years before the AML diagnosis. Transient infection and reshedding should thus be considered in antibody-positive hematological patients.

Gene therapy with adeno-associated virus vector 5-human factor IX in adults with hemophilia B.

Gene therapy for hemophilia B aims to ameliorate bleeding risk and provide endogenous factor IX (FIX) activity/synthesis through a single treatment, eliminating the requirement for FIX concentrate. AMT-060 combines an adeno-associated virus-5 (AAV5) vector with a liver-specific promoter driving expression of a codon-optimized wild-type human FIX gene. This multinational, open-label study included 10 adults with hemophilia B (FIX ≤2% of normal) and severe-bleeding phenotype.

Prediction of bleeding and thrombosis by standard biochemical coagulation variables in haematological intensive care patients.

Purpose: We assessed the value of standard biochemical coagulation parameters in predicting bleeding, thrombosis and mortality in adult Intensive Care Unit (ICU) patients with haematological malignancies.

Methods: We screened all patients with acute leukaemia and myelodysplastic syndrome admitted to a university hospital ICU during 2008-2012. Data were obtained from the clinical chemistry laboratory database and patient files.

Application of whole-exome sequencing to direct the specific functional testing and diagnosis of rare inherited bleeding disorders in patients from the Öresund Region, Scandinavia.

Rare inherited bleeding disorders (IBD) are a common cause of bleeding tendency. To ensure a correct diagnosis, specialized laboratory analyses are necessary. This study reports the results of an upfront diagnostic strategy using targeted whole exome sequencing.

Integration of fiber-optic sensor arrays into a multi-modal tactile sensor processing system for robotic end-effectors.

With the increasing complexity of robotic missions and the development towards long-term autonomous systems, the need for multi-modal sensing of the environment increases. Until now, the use of tactile sensor systems has been mostly based on sensing one modality of forces in the robotic end-effector. The use of a multi-modal tactile sensory system is motivated, which combines static and dynamic force sensor arrays together with an absolute force measurement system.