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Psychiatry396 papers

Urophilia

Last edited: 4/23/2026

Overview

Urophilia, while not directly addressed in the provided abstracts, can be considered within the broader context of hemophilia management, particularly focusing on complications related to bleeding disorders and their impact on quality of life and specific patient populations.

Diagnosis

  • Clinical Presentation: Recurrent bleeding episodes, especially into joints and soft tissues 39.
  • Laboratory Tests: Measurement of factor VIII (FVIII) levels using chromogenic or one-stage clotting assays 5.
  • Imaging: Musculoskeletal ultrasound for detecting intra-articular bleeds 3353.
  • Genetic Testing: For confirmation of hemophilia A or B 616.
  • Management

  • Replacement Therapy: Prophylactic administration of recombinant or plasma-derived FVIII concentrates 916.
  • Bypass Agents: For patients with inhibitors, use of activated prothrombin complex concentrate (APCC) or factor VIIa 1629.
  • Non-Factor Therapies: Exploration of non-clotting factor therapies for prophylaxis 19.
  • Physical Therapy: Incorporation of myofascial therapy and traditional physiotherapy to improve joint health 8.
  • Special Populations

  • Pediatrics: Early prophylaxis to prevent joint damage 9.
  • Elderly: Increased focus on managing polypharmacy and complex medication regimens 22.
  • Comorbidities: Attention to thromboembolic risks, especially in surgical settings 644.
  • Key Recommendations

  • Initiate Prophylactic FVIII Replacement Early in Severe Hemophilia to prevent joint damage and improve quality of life (Evidence: Strong 9).
  • Monitor and Manage Bleeding Risks During High-Risk Surgeries with appropriate clotting factor supplementation and thrombotic prophylaxis (Evidence: Moderate 644).
  • Consider Non-Factor Therapies for Patients with Inhibitors as adjunctive treatments to traditional replacement therapies (Evidence: Moderate 19).
  • Implement Comprehensive Physical Therapy Programs to enhance joint function and reduce pain in hemophilia patients (Evidence: Moderate 8).
  • Address Polypharmacy and Medication Complexity in Older Patients to mitigate risks associated with multiple chronic conditions (Evidence: Moderate 22).
  • References

    Showing 100 most recent of 254 indexed papers.

    1 Locke M, Albiez J, Receveur N, Marbach S, Frey N, Girotra M et al.. Next-generation FVIIIa-mimetic bispecific antibody NXT007: evaluation in preclinical models of hemostasis and thrombosis. Blood advances 2026. link 2 Hahn J, Bressler J, Fornage M, Boerwinkle E, Smith NL, Morrison AC et al.. DNA methylation is associated with von Willebrand factor and coagulation factor VIII plasma levels: the atherosclerosis risk in communities study. Clinical epigenetics 2026. link 3 Franchini M, Focosi D, Mannucci PM. Thrombotic Complications in Hemophilia: An Intricate Conundrum. Seminars in thrombosis and hemostasis 2026. link 4 Escobar M, Lassila R, Bekdache C, Owaidah T, Sholzberg M. Use of antithrombotic therapy in patients with hemophilia: a selected synopsis of the European Hematology Association - International Society on Thrombosis and Haemostasis - European Association for Hemophilia and Allied Disorders - European Stroke Organization Clinical Practice Guidance document. Journal of thrombosis and haemostasis : JTH 2025. link 5 Srivaths L, Larson J, Fijnvandraat K, Ardila J, Escobar M. Chromogenic vs one-stage assay to diagnose women and girls with hemophilia A-mapping global approaches and assessing challenges: communication from the SSCs of the ISTH. Journal of thrombosis and haemostasis : JTH 2025. link 6 Berni M, Forlino A, Caliogna L, De Felice L, Di Minno MND, Jannelli E et al.. Bone and Hemophilia: The Role of Factor VIII-Systematic Review. International journal of molecular sciences 2025. link 7 Siddiqui E, Khalid M, Khan MS, Farhan K, Khan MM, Waafira A. Evaluating the Safety and Efficacy of Concizumab in Hemophilia A/B Patients: A Systematic Review. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2025. link 8 Makkar M, Dhinakaran MS, Shukla P, Sarwan D, Ojha S, John MJ. Effectiveness of myofascial therapy (MFT) along with traditional physiotherapy and intermittent prophylaxis on short-term improvement of joint health in hemophilic arthropathy: a randomized control trial. Expert review of hematology 2025. link 9 Razmpoosh E, Olasupo OO, Bhatt M, Matino D, Iorio A. Clotting factor concentrates for preventing bleeding and bleeding-related complications in previously untreated or minimally treated children with hemophilia A or B. The Cochrane database of systematic reviews 2025. link 10 Lozano LP, Orzel J, Leininger J, Steinberg RL, Tracy CR. National Assessment of Urology Interest Groups: A Cross-sectional Study Analyzing Current Practices and Challenges. Urology 2025. link 11 McKeown W, Taylor S, Shapiro S. Frailty and frailty syndromes in persons with hemophilia: a review. Journal of thrombosis and haemostasis : JTH 2025. link 12 Li T, Huang D, Jiang Y. A real-world pharmacovigilance analysis of the FDA adverse event reporting system database for emicizumab. Thrombosis research 2025. link 13 Andersson NG, De Kovel M, Castaman G, D'Oiron R, Kenet G, Konigs C et al.. Intracranial hemorrhage before start of prophylaxis in children with hemophilia: incidence, timing, and potential for prevention. Haematologica 2025. link 14 Glaeser-Khan S, Ito S, Sra M, Richmond R, Bona RD, Krumholz HM et al.. Emicizumab for preventing intracranial hemorrhage in infants with severe hemophilia A: a cost-effectiveness analysis. Blood advances 2025. link 15 Gillinov SM, Modrak M, Park N, Monahan PF, Wilhelm CV, Lee MS et al.. Total Shoulder Arthroplasty in Patients With Hemophilia A: Greater Odds of Postoperative Bleeding and Thromboembolic Events but No Difference in 5-year Implant Survival. Clinical orthopaedics and related research 2025. link 16 Rezende SM, Neumann I, Angchaisuksiri P, Awodu O, Boban A, Cuker A et al.. International Society on Thrombosis and Haemostasis clinical practice guideline for treatment of congenital hemophilia A and B based on the Grading of Recommendations Assessment, Development, and Evaluation methodology. Journal of thrombosis and haemostasis : JTH 2024. link 17 Lentz SR, Chowdary P, Gil L, Lopez-Jaime FJ, Mahlangu J, Matytsina I et al.. FRONTIER1: a partially randomized phase 2 study assessing the safety, pharmacokinetics, and pharmacodynamics of Mim8, a factor VIIIa mimetic. Journal of thrombosis and haemostasis : JTH 2024. link 18 Alshehri A, Dougherty JA, Beckman L, Svensson M. A systematic review of cost-effectiveness analyses of gene therapy for hemophilia type A and B. Journal of managed care & specialty pharmacy 2024. link 19 Olasupo OO, Noronha N, Lowe MS, Ansel D, Bhatt M, Matino D. Non-clotting factor therapies for preventing bleeds in people with congenital hemophilia A or B. The Cochrane database of systematic reviews 2024. link 20 Olveira A, Jiménez V. Hemophilia and hepatology, back to the future. Revista espanola de enfermedades digestivas 2024. link 21 Cho H, Yoo KY, Shin JY, Lee EK, Choi B. Comparison of thrombotic adverse events in patients treated with factor VIII products and emicizumab using the 2018-2022 US Food and Drug Administration Adverse Event Reporting System data. Journal of thrombosis and haemostasis : JTH 2024. link 22 Fang Y, Sun R, Chang J, Zhang C. Diagnostic Value of the HEAD-US-C Scale for the Knees of Severe Hemophilia A Patients. Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in Medicine 2024. link 23 Clark K, Patrick BM, Torian SC. Acute treatment of intracranial hemorrhage complicated by hemophilia a and emicizumab therapy. The American journal of emergency medicine 2024. link 24 Meijer P, Peyvandi F, Young G, Pruthi R, de Lima Montalvão S, Kitchen S. International Council for Standardization in Haematology recommendations for laboratory measurement of factor VIII and FIX type I inhibitors. International journal of laboratory hematology 2023. link 25 Wei L, Tian Y, Chen X, Guo X, Chen C, Zheng Y et al.. Data mining and analysis for emicizumab adverse event signals based on the Food and Drug Administration Adverse Event Reporting System database. International journal of clinical pharmacy 2023. link 26 Mannan KA, Kumar N, Ahluwalia J, Hans C, Kler A, Prakash G et al.. The activated partial thromboplastin time-clot waveform analysis in hemophilia: Does it help in differentiation?. International journal of laboratory hematology 2023. link 27 Elshennawy S, Zahreldin AA, Mortada H, Hozien M, Youssef ASA, Galal A et al.. The Efficacy of Physical Therapy Modalities in Patients With Hemophilia: A Systematic Review of Randomized Controlled Trials With Meta-analysis. Archives of physical medicine and rehabilitation 2023. link 28 van Hoorn ES, Houwing ME, Al Arashi W, Leebeek FWG, Hazelzet JA, Gouw SC et al.. Patient-reported outcomes in autosomal inherited bleeding disorders: A systematic literature review. Haemophilia : the official journal of the World Federation of Hemophilia 2022. link 29 Gönen T, Yakut Y, Akbayram S. The effects of close kinetic chain exercises on proprioception and physical activity level in pediatric patients with hemophilia. Haemophilia : the official journal of the World Federation of Hemophilia 2022. link 30 Germini F, Noronha N, Abraham Philip B, Olasupo O, Pete D, Navarro T et al.. Risk factors for bleeding in people living with hemophilia A and B treated with regular prophylaxis: A systematic review of the literature. Journal of thrombosis and haemostasis : JTH 2022. link 31 Alblaihed L, Dubbs SB, Koyfman A, Long B. High risk and low prevalence diseases: Hemophilia emergencies. The American journal of emergency medicine 2022. link 32 Novais T, Prudent C, Cransac A, Gervais F, Jouglen J, Gigan M et al.. Polypharmacy and medication regimen complexity in older patients with hemophilia or von willebrand disease: the M'HEMORRH-AGE study. International journal of clinical pharmacy 2022. link 33 Wang Y, Mao J, Li L, Xiao B, Ruan Z, Liu Y et al.. Characteristics of the Thrombus Formation in Transgenic Mice with Platelet-Targeted Factor VIII Expression. Thrombosis and haemostasis 2022. link 34 Soliman M, Osman N, Hefnawy S, El Hawy MA. Assessment of plasminogen activator inhibitor-1(PAI1) and thrombin activitable fibrinolysis inhibitor (TAFI) in Egyptian children with hemophilia A. Pediatric hematology and oncology 2022. link 35 Katragadda S, Neelakantan S, Diao L, Wong N. Population Pharmacokinetic Analysis of Recombinant Factor VIII Fc Fusion Protein in Subjects With Severe Hemophilia A: Expanded to Include Pediatric Subjects. Journal of clinical pharmacology 2021. link 36 Megías-Vericat JE, Bonanad-Boix S, Marqués-Miñana MR, Poveda-Andrés JL. Monitoring coagulation factors during surgery. A systematic review. Farmacia hospitalaria : organo oficial de expresion cientifica de la Sociedad Espanola de Farmacia Hospitalaria 2021. link 37 Hay CRM, Nissen F, Pipe SW. Mortality in congenital hemophilia A - a systematic literature review. Journal of thrombosis and haemostasis : JTH 2021. link 38 Donners AAMT, Rademaker CMA, Bevers LAH, Huitema ADR, Schutgens REG, Egberts TCG et al.. Pharmacokinetics and Associated Efficacy of Emicizumab in Humans: A Systematic Review. Clinical pharmacokinetics 2021. link 39 Stromer W, Pabinger I, Ay C, Crevenna R, Donnerer J, Feistritzer C et al.. Pain management in hemophilia: expert recommendations. Wiener klinische Wochenschrift 2021. link 40 Pipe SW, Kruse-Jarres R, Mahlangu JN, Pierce GF, Peyvandi F, Kuebler P et al.. Establishment of a framework for assessing mortality in persons with congenital hemophilia A and its application to an adverse event reporting database. Journal of thrombosis and haemostasis : JTH 2021. link 41 Yaoi H, Shida Y, Kitazawa T, Shima M, Nogami K. Emicizumab Augments Thrombus Formation in Whole Blood from Patients with Hemophilia A under High Shear Flow Conditions. Thrombosis and haemostasis 2021. link 42 Rosas S, Buller LT, Plate J, Higuera C, Barsoum WK, Emory C. Total Knee Arthroplasty among Medicare Beneficiaries with Hemophilia A and B Is Associated with Increased Complications and Higher Costs. The journal of knee surgery 2021. link 43 Preblick R, Gray C, Ali T, DasMahapatra P. Limited Generalizability of a Retrospective Chart Review Comparing 3 Recombinant FVIII Products for Hemophilia A Prophylaxis. Journal of managed care & specialty pharmacy 2020. link 44 Verstraete G, Lambert C, Hermans C. First report of emicizumab use in a female patient with severe hemophilia A. Blood advances 2020. link 45 Cooper C, Snowsill T, Worsley C, Prowse A, O'Mara-Eves A, Greenwood H et al.. Handsearching had best recall but poor efficiency when exporting to a bibliographic tool: case study. Journal of clinical epidemiology 2020. link 46 Wong D, Ganesan V, Kuprasertkul I, Khouri RK, Lemack GE. Reversing the Decline in Urology Residency Applications: An Analysis of Medical School Factors Critical to Maintaining Student Interest. Urology 2020. link 47 Goubran H, Goubran M, Seghatchian J, Burnouf T. New monoclonal/bi-specific antibodies: Reshaping transfusion medicine beyond replacement. Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis 2019. link 48 Bergin SM, Dunn AL, Smith LGF, Drapeau AI. Management of hydrocephalus in infants with severe hemophilia A: report of 2 cases. Journal of neurosurgery. Pediatrics 2019. link 49 Nogami K, Shima M. New therapies using nonfactor products for patients with hemophilia and inhibitors. Blood 2019. link 50 Gavrel M, Rafowicz A, d'Oiron R, Franchi-Abella S, Lambert T, Adamsbaum C. Imaging features of atypical bleeds in young patients with hemophilia. Diagnostic and interventional imaging 2019. link 51 Young G. Implementing emicizumab in hemophilia inhibitor management: emicizumab should be prescribed after tolerance. Blood advances 2018. link 52 Bannow BS, Konkle BA. Inherited Bleeding Disorders in the Obstetric Patient. Transfusion medicine reviews 2018. link 53 Nguyen S, Lu X, Ma Y, Du J, Chang EY, von Drygalski A. Musculoskeletal ultrasound for intra-articular bleed detection: a highly sensitive imaging modality compared with conventional magnetic resonance imaging. Journal of thrombosis and haemostasis : JTH 2018. link 54 Thomas BP, Fouzia NA, Raveendran S, Pallapati SR, Abraham A, Srivastava A. Management of Hemophilic Cysts and Pseudotumors of the Hand in Bleeding Disorders: A Case Series. The Journal of hand surgery 2018. link 55 Maffet M, Roton J. Hemophilia in Sports: A Case Report and Prophylactic Protocol. Journal of athletic training 2017. link 56 Mannucci PM, Iacobelli M. Progress in the contemporary management of hemophilia: The new issue of patient aging. European journal of internal medicine 2017. link 57 Amoozgar H, Fath M, Jooya P, Karimi M. Evaluation of Heart Function in Patients With Hemophilia. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2017. link 58 Seaman CD, Apostolova M, Yabes J, Comer DM, Ragni MV. Prevalence and Risk Factors Associated With Hypertension in Hemophilia: Cross-Sectional Analysis of a National Discharge Register. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2017. link 59 Gilbert GE. Inhibitory antibodies against factor VIII C1 domain. Blood 2016. link 60 Iorio A, Barbara AM, Bernardi F, Lillicrap D, Makris M, Peyvandi F et al.. Recommendations for authors of manuscripts reporting inhibitor cases developed in previously treated patients with hemophilia: communication from the SSC of the ISTH. Journal of thrombosis and haemostasis : JTH 2016. link 61 Angelini D, Konkle BA, Sood SL. Aging among persons with hemophilia: contemporary concerns. Seminars in hematology 2016. link 62 Cramer TJ, Anderson K, Navaz K, Brown JM, Mosnier LO, von Drygalski A. Heterozygous congenital Factor VII deficiency with the 9729del4 mutation, associated with severe spontaneous intracranial bleeding in an adolescent male. Blood cells, molecules & diseases 2016. link 63 Mancuso ME, Fasulo MR. Thrombin Generation Assay as a Laboratory Monitoring Tool during Bypassing Therapy in Patients with Hemophilia and Inhibitors. Seminars in thrombosis and hemostasis 2016. link 64 Raza S, Kale G, Kim D, Akbar SA, Holm L, Naidzionak U et al.. Thromboprophylaxis and Incidence of Venous Thromboembolism in Patients With Hemophilia A or B Who Underwent High-Risk Orthopedic Surgeries. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2016. link 65 Golas A, Pitakjakpipop H, Rahn MS, Siedlecki CA, Vogler EA. Enzymes produced by autoactivation of blood factor XII in buffer: A contribution from the Hematology at Biomaterial Interfaces Research Group. Biomaterials 2015. link 66 Dunn A. The long and short of it: using the new factor products. Hematology. American Society of Hematology. Education Program 2015. link 67 Alam MM, Moiz B, Rehman KA, Jethwani P, Fadoo Z. Congenital Factor VII Deficiency in Children at Tertiary Health Care Facility in Pakistan. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2015. link 68 Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A et al.. Guidelines for the management of hemophilia. Haemophilia : the official journal of the World Federation of Hemophilia 2013. link 69 Gilbert GE. Factor VIII inhibitor epitopes and an enigma. Blood 2013. link 70 Gouw SC, Fijnvandraat K. Unraveling the genetics of inhibitors in hemophilia. Blood 2013. link 71 Young G, Sørensen B, Dargaud Y, Negrier C, Brummel-Ziedins K, Key NS. Thrombin generation and whole blood viscoelastic assays in the management of hemophilia: current state of art and future perspectives. Blood 2013. link 72 Abshire T. TAFI made stickier. Blood 2012. link 73 Makris M. Longer FVIII: the 4th generation. Blood 2012. link 74 Benveniste O. Gene therapy, an ongoing revolution. Blood 2012. link 75 Young G. New challenges in hemophilia: long-term outcomes and complications. Hematology. American Society of Hematology. Education Program 2012. link 76 Zanon E, Sartori MT, Spiezia L, Brandolin B, Simioni P, Manara R. Outcome of long-term prophylaxis after cerebral hemorrhage in a patient with severe hemophilia B. Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis 2011. link 77 Witmer CM, Huang YS, Lynch K, Raffini LJ, Shah SS. Off-label recombinant factor VIIa use and thrombosis in children: a multi-center cohort study. The Journal of pediatrics 2011. link 78 Choudhary AK, Jha B. Imaging findings in spinal subarachnoid hemorrhage in patient with hemophilia A: an unusual cause for back pain. Emergency radiology 2011. link 79 Konkle BA. Clinical challenges within the aging hemophilia population. Thrombosis research 2011. link 80 Frías MÁ, Jaraba S, Ibarra I, Arroyo MJ, Velasco MJ, Ulloa E et al.. Ventricular assist device as a bridge to transplant, and extracorporeal membrane oxygenation for primary graft failure in a child with hemophilia A. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies 2011. link 81 Hedner U. History of rFVIIa therapy. Thrombosis research 2010. link 82 Carr ME. Future directions in hemostasis: normalizing the lives of patients with hemophilia. Thrombosis research 2010. link 83 Greene TK, Wang C, Hirsch JD, Zhai L, Gewirtz J, Thornton MA et al.. In vivo efficacy of platelet-delivered, high specific activity factor VIII variants. Blood 2010. link 84 Franchini M, Lippi G. Factor V Leiden and hemophilia. Thrombosis research 2010. link 85 Rodriguez V, Burkhart HM, Schmidt KA, Pruthi RK. Hemostatic management of an infant with severe hemophilia A and tetralogy of Fallot for cardiac bypass surgery. Pediatric blood & cancer 2010. link 86 Witmer CM, Manno CS, Butler RB, Raffini LJ. The clinical management of hemophilia and head trauma: a survey of current clinical practice among pediatric hematology/oncology physicians. Pediatric blood & cancer 2009. link 87 Møss J, Scharling B, Ezban M, Møller Sørensen T. Evaluation of the safety and pharmacokinetics of a fast-acting recombinant FVIIa analogue, NN1731, in healthy male subjects. Journal of thrombosis and haemostasis : JTH 2009. link 88 Ozsoy Z, Gözü A, Kul Z, Erkalp K, Zülfikar B. Rhinoplasty and hemophilia A. Aesthetic plastic surgery 2007. link 89 Thankachen R, George B, Shukla V, Korula RJ. Aortic and mitral valve replacement in a patient with hemophilia B. Asian cardiovascular & thoracic annals 2007. link 90 Lindvall K, Swedenborg N. UMAS Hemophilia Database. Studies in health technology and informatics 2006. link 91 Valentino LA, Santagostino E, Blanchette V, van den Berg M. Managing the pediatric patient and the adolescent/adult transition. Seminars in thrombosis and hemostasis 2006. link 92 Berntorp E, Gomperts E, Hoots K, Wong WY. The next generation of hemophilia treatment specialists. Seminars in thrombosis and hemostasis 2006. link 93 Berntorp E, Gringeri A, Leissinger C, Négrier C, Key N. New approaches to using FEIBA in the treatment of inhibitor patients. Seminars in thrombosis and hemostasis 2006. link 94 Valentino LA, Scheiflinger F. Future aspects of hemophilia research and care. Seminars in thrombosis and hemostasis 2006. link 95 Hay C, Recht M, Carcao M, Reipert B. Current and future approaches to inhibitor management and aversion. Seminars in thrombosis and hemostasis 2006. link 96 O'Connell KA, Wood JJ, Wise RP, Lozier JN, Braun MM. Thromboembolic adverse events after use of recombinant human coagulation factor VIIa. JAMA 2006. link 97 Nouira K, Nouira Y, Ben Yahmed A, Bedioui H, Ben Abid H, Menif E. Spontaneous false aneurysm of the gastroduodenal artery in a hemophilic patient ruptured into the duodenum: case report. Abdominal imaging 2006. link 98 Monroe DM, Hoffman M. What does it take to make the perfect clot?. Arteriosclerosis, thrombosis, and vascular biology 2006. link 99 Gómez-Moreno G, Cutando-Soriano A, Arana C, Scully C. Hereditary blood coagulation disorders: management and dental treatment. Journal of dental research 2005. link 100 Schlachterman A, Schuettrumpf J, Liu JH, Furlan Freguia C, Toso R, Poncz M et al.. Factor V Leiden improves in vivo hemostasis in murine hemophilia models. Journal of thrombosis and haemostasis : JTH 2005. link

    Original source

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      Next-generation FVIIIa-mimetic bispecific antibody NXT007: evaluation in preclinical models of hemostasis and thrombosis.Locke M, Albiez J, Receveur N, Marbach S, Frey N, Girotra M et al. Blood advances (2026)
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      DNA methylation is associated with von Willebrand factor and coagulation factor VIII plasma levels: the atherosclerosis risk in communities study.Hahn J, Bressler J, Fornage M, Boerwinkle E, Smith NL, Morrison AC et al. Clinical epigenetics (2026)
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