Behcet Disease Questions & Answers

1. Davatchi F, Chams-Davatchi C, Shams H, Shahram F, Nadji A, Akhlaghi M, et al. Behcet's disease: epidemiology, clinical manifestations, and diagnosis. Expert Rev Clin Immunol. 2017 Jan. 13 (1):57-65. [QxMD MEDLINE Link].

2. Demirkesen C, Tüzüner N, Mat C, Senocak M, Büyükbabani N, Tüzün Y, et al. Clinicopathologic evaluation of nodular cutaneous lesions of Behçet syndrome. Am J Clin Pathol. 2001 Sep. 116 (3):341-6. [QxMD MEDLINE Link].

3. International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. International Study Group for Behçet's Disease. Lancet. 1990 May 5. 335(8697):1078-80. [QxMD MEDLINE Link].

4. Adamantiades B. A case of recurrent hypopyon iritis. Medical Society of Athens. 1930. 586-93.

5. Behcet H. Uber rezidiverendeaphthose durch ein virus verursachte Geschwure am Mund, am Auge, und an den Genitalien. Dermatol Wochenschr. 1937. 105:1152-7.

6. Davatchi F. Diagnosis/Classification Criteria for Behcet's Disease. Patholog Res Int. 2012. 2012:607921. [QxMD MEDLINE Link]. [Full Text].

7. Emmi L, Brugnolo F, Salvati G, et al. Immunopathological aspects of Behçet's disease. Clin Exp Rheumatol. 1995 Nov-Dec. 13(6):687-91. [QxMD MEDLINE Link].

8. Kaneko S, Suzuki N, Yamashita N, Nagafuchi H, Nakajima T, Wakisaka S, et al. Characterization of T cells specific for an epitope of human 60-kD heat shock protein (hsp) in patients with Behcet's disease (BD) in Japan. Clin Exp Immunol. 1997 May. 108(2):204-12. [QxMD MEDLINE Link].

9. Hasan A, Fortune F, Wilson A, Warr K, Shinnick T, Mizushima Y, et al. Role of gamma delta T cells in pathogenesis and diagnosis of Behcet's disease. Lancet. 1996 Mar 23. 347(9004):789-94. [QxMD MEDLINE Link].

10. Feng R, Chao K, Chen SL, Sun CH, Qiu Y, Chen BL, et al. Heat shock protein family A member 6 combined with clinical characteristics for the differential diagnosis of intestinal Behçet's disease. J Dig Dis. 2018 Jun. 19 (6):350-358. [QxMD MEDLINE Link].

11. Suzuki Y, Hoshi K, Matsuda T, et al. Increased peripheral blood gamma delta+ T cells and natural killer cells in Behçet's disease. J Rheumatol. 1992 Apr. 19(4):588-92. [QxMD MEDLINE Link].

12. Direskeneli H, Eksioglu-Demiralp E, Kibaroglu A, Yavuz S, Ergun T, Akoglu T. Oligoclonal T cell expansions in patients with Behçet's disease. Clin Exp Immunol. 1999 Jul. 117(1):166-70. [QxMD MEDLINE Link]. [Full Text].

13. Direskeneli H, Eksioglu-Demiralp E, Yavuz S, Ergun T, Shinnick T, Lehner T, et al. T cell responses to 60/65 kDa heat shock protein derived peptides in Turkish patients with Behçet's disease. J Rheumatol. 2000 Mar. 27(3):708-13. [QxMD MEDLINE Link].

14. Frassanito MA, Dammacco R, Cafforio P, Dammacco F. Th1 polarization of the immune response in Behçet's disease: a putative pathogenetic role of interleukin-12. Arthritis Rheum. 1999 Sep. 42(9):1967-74. [QxMD MEDLINE Link].

15. Sugi-Ikai N, Nakazawa M, Nakamura S, Ohno S, Minami M. Increased frequencies of interleukin-2- and interferon-gamma-producing T cells in patients with active Behçet's disease. Invest Ophthalmol Vis Sci. 1998 May. 39(6):996-1004. [QxMD MEDLINE Link].

16. Hamzaoui K, Berraies A, Kaabachi W, Ammar J, Hamzaoui A. Pulmonary manifestations in Behcet disease: impaired natural killer cells activity. Multidiscip Respir Med. 2013 Apr 4. 8(1):29. [QxMD MEDLINE Link].

17. Hallett MB, Lloyds D. Neutrophil priming: the cellular signals that say 'amber' but not 'green'. Immunol Today. 1995 Jun. 16(6):264-8. [QxMD MEDLINE Link].

18. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med. 1999 Oct 21. 341(17):1284-91. [QxMD MEDLINE Link].

19. Sakane T, Suzuki N, Takeno M. Innate and acquired immunity in Behçet’s disease. 8th International Congress on Behçet’s Disease. Reggio Emilia, Italy, 7-9 October 1998. Program and Abstracts: 56.

20. Takeno M, Shimayano Y, Suzuki N, Sakane T. Prolonged survival of autoprimed neutrophils from patients with Behçet ’s disease.: 8th International Congress on Behçet’s Disease. Reggio Emilia, Italy, 7-9 October 1998. Program and Abstracts: 57.

21. Koné-Paut I, Geisler I, Wechsler B, et al. Familial aggregation in Behçet's disease: high frequency in siblings and parents of pediatric probands. J Pediatr. 1999 Jul. 135(1):89-93. [QxMD MEDLINE Link].

22. de Menthon M, Lavalley MP, Maldini C, Guillevin L, Mahr A. HLA-B51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case-control genetic association studies. Arthritis Rheum. 2009 Oct 15. 61(10):1287-96. [QxMD MEDLINE Link].

23. Puccetti A, Fiore PF, Pelosi A, Tinazzi E, Patuzzo G, Argentino G, et al. Gene Expression Profiling in Behcet's Disease Indicates an Autoimmune Component in the Pathogenesis of the Disease and Opens New Avenues for Targeted Therapy. J Immunol Res. 2018. 2018:4246965. [QxMD MEDLINE Link]. [Full Text].

24. Greco A, De Virgilio A, Ralli M, Ciofalo A, Mancini P, Attanasio G, et al. Behçet's disease: New insights into pathophysiology, clinical features and treatment options. Autoimmun Rev. 2018 Jun. 17 (6):567-575. [QxMD MEDLINE Link].

25. Kirino Y, Zhou Q, Ishigatsubo Y, Mizuki N, Tugal-Tutkun I, et al. Targeted resequencing implicates the familial Mediterranean fever gene MEFV and the toll-like receptor 4 gene TLR4 in Behçet disease. Proc Natl Acad Sci U S A. 2013 May 14. 110 (20):8134-9. [QxMD MEDLINE Link]. [Full Text].

26. Kirino Y, Bertsias G, Ishigatsubo Y, Mizuki N, Tugal-Tutkun I, Seyahi E, et al. Genome-wide association analysis identifies new susceptibility loci for Behçet's disease and epistasis between HLA-B*51 and ERAP1. Nat Genet. 2013 Feb. 45 (2):202-7. [QxMD MEDLINE Link]. [Full Text].

27. Kiraz S, Ertenli I, Oztürk MA, et al. Pathological haemostasis and "prothrombotic state" in Behçet's disease. Thromb Res. 2002 Jan 15. 105(2):125-33. [QxMD MEDLINE Link].

28. Becatti M, Emmi G, Bettiol A, Silvestri E, Di Scala G, Taddei N, et al. Behçet's syndrome as a tool to dissect the mechanisms of thrombo-inflammation: clinical and pathogenetic aspects. Clin Exp Immunol. 2018 Nov 25. [QxMD MEDLINE Link].

29. Krause I, Yankevich A, Fraser A, Rosner I, Mader R, Zisman D, et al. Prevalence and clinical aspects of Behcet's disease in the north of Israel. Clin Rheumatol. 2007 Apr. 26(4):555-60. [QxMD MEDLINE Link].

30. Calamia KT, Wilson FC, Icen M, Crowson CS, Gabriel SE, Kremers HM. Epidemiology and clinical characteristics of Behçet's disease in the US: a population-based study. Arthritis Rheum. 2009 May 15. 61(5):600-4. [QxMD MEDLINE Link]. [Full Text].

31. Hammam N, Li J, Evans M, Kay JL, Izadi Z, Anastasiou C, et al. Epidemiology and treatment of Behçet's disease in the USA: insights from the Rheumatology Informatics System for Effectiveness (RISE) Registry with a comparison with other published cohorts from endemic regions. Arthritis Res Ther. 2021 Aug 30. 23 (1):224. [QxMD MEDLINE Link]. [Full Text].

32. Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behçet's disease. Arthritis Rheum. 2010 Sep. 62(9):2806-12. [QxMD MEDLINE Link].

33. Akman-Demir G, Serdaroglu P, Tasçi B. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain. 1999 Nov. 122 ( Pt 11):2171-82. [QxMD MEDLINE Link].

34. Gungor AN, Kalkan G, Oguz S, Sen B, Ozoguz P, Takci Z, et al. Behcet disease and pregnancy. Clin Exp Obstet Gynecol. 2014. 41 (6):617-9. [QxMD MEDLINE Link].

35. International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014 Mar. 28 (3):338-47. [QxMD MEDLINE Link].

36. Alpsoy E, Zouboulis CC, Ehrlich GE. Mucocutaneous lesions of Behcet's disease. Yonsei Med J. 2007 Aug 31. 48(4):573-85. [QxMD MEDLINE Link].

37. Torres RM, Yáñez B, Herreras JM, Calonge M. [Ocular Behçet disease. Retrospective study]. Arch Soc Esp Oftalmol. 2004 Dec. 79 (12):599-603. [QxMD MEDLINE Link].

38. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in Behçet disease. Curr Opin Rheumatol. 2005 Jan. 17(1):1-8. [QxMD MEDLINE Link].

39. Kobayashi K, Ueno F, Bito S, et al. Development of consensus statements for the diagnosis and management of intestinal Behçet's disease using a modified Delphi approach. J Gastroenterol. 2007 Sep. 42(9):737-45. [QxMD MEDLINE Link].

40. Hemmen T, Perez-Canto A, Distler A, et al. IgA nephropathy in a patient with Behçet's syndrome--case report and review of literature. Br J Rheumatol. 1997 Jun. 36(6):696-9. [QxMD MEDLINE Link].

41. Hashimoto T, Toya Y, Kihara M, Yabana M, Inayama Y, Tanaka K, et al. Behçet's disease complicated by IgA nephropathy with nephrotic syndrome. Clin Exp Nephrol. 2008 Jun. 12(3):224-7. [QxMD MEDLINE Link].

42. Akpolat T, Akkoyunlu M, Akpolat I, et al. Renal Behçet's disease: a cumulative analysis. Semin Arthritis Rheum. 2002 Apr. 31(5):317-37. [QxMD MEDLINE Link].

43. Kokturk A. Clinical and Pathological Manifestations with Differential Diagnosis in Behçet's Disease. Patholog Res Int. 2012. 2012:690390. [QxMD MEDLINE Link]. [Full Text].

44. Arevalo JF, Lasave AF, Al Jindan MY, Al Sabaani NA, Al-Mahmood AM, Al-Zahrani YA, et al. Uveitis in Behçet Disease in a Tertiary Center Over 25 Years: The KKESH Uveitis Survey Study Group. Am J Ophthalmol. 2015 Jan. 159(1):177-184.e2. [QxMD MEDLINE Link].

45. Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Le Thi Huong D, Sbai A, et al. Cerebral venous thrombosis in Behçet's disease. Arthritis Rheum. 2009 Apr 15. 61(4):518-26. [QxMD MEDLINE Link].

46. Shen Y, Ma HF, Luo D, Cai JF, Zou J, Guan JL. High Incidence of Gastrointestinal Ulceration and Cytogenetic Aberration of Trisomy 8 as Typical Features of Behçet's Disease Associated with Myelodysplastic Syndrome: A Series of 16 Consecutive Chinese Patients from the Shanghai Behçet's Disease Database and Comparison with the Literature. Biomed Res Int. 2018. 2018:8535091. [QxMD MEDLINE Link].

47. Borhani Haghighi A, Pourmand R, Nikseresht AR. Neuro-Behçet disease. A review. Neurologist. 2005 Mar. 11(2):80-9. [QxMD MEDLINE Link].

48. Seyahi E, Cakmak OS, Tutar B, Arslan C, Dikici AS, Sut N, et al. Clinical and Ultrasonographic Evaluation of Lower-extremity Vein Thrombosis in Behcet Syndrome: An Observational Study. Medicine (Baltimore). 2015 Nov. 94 (44):e1899. [QxMD MEDLINE Link].

49. Oktayoglu P, Mete N, Caglayan M, Bozkurt M, Bozan T, Em S, et al. Elevated serum levels of calprotectin (MRP8/MRP14) in patients with Behçet's disease and its association with disease activity and quality of life. Scand J Clin Lab Invest. 2014 Dec 4. 1-7. [QxMD MEDLINE Link].

50. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008 Dec. 67(12):1656-62. [QxMD MEDLINE Link].

51. [Guideline] Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis. 2018 Jun. 77 (6):808-818. [QxMD MEDLINE Link]. [Full Text].

52. Aktulga E, Altac M, Muftüoglu A, et al. A double blind study of colchicine in Behçet's disease. Haematologica. 1980 Jun. 65(3):399-402. [QxMD MEDLINE Link].

53. Calis M, Ates F, Yazici C, Kose K, Kirnap M, Demir M, et al. Adenosine deaminase enzyme levels, their relation with disease activity, and the effect of colchicine on adenosine deaminase levels in patients with Behçet's disease. Rheumatol Int. 2005 Aug. 25(6):452-6. [QxMD MEDLINE Link].

54. Alibaz-Oner F, Direskeneli H. Advances in the Treatment of Behcet's Disease. Curr Rheumatol Rep. 2021 May 20. 23 (6):47. [QxMD MEDLINE Link]. [Full Text].

55. Markomichelakis N, Delicha E, Masselos S, Sfikakis PP. Intravitreal infliximab for sight-threatening relapsing uveitis in Behçet disease: a pilot study in 15 patients. Am J Ophthalmol. 2012 Sep. 154(3):534-541.e1. [QxMD MEDLINE Link].

56. Keino H, Okada AA, Watanabe T, Taki W. Decreased ocular inflammatory attacks and background retinal and disc vascular leakage in patients with Behcet's disease on infliximab therapy. Br J Ophthalmol. 2011 Sep. 95(9):1245-50. [QxMD MEDLINE Link].

57. Umazume A, Kezuka T, Usui Y, Suzuki J, Goto H. Evaluation of efficacy of infliximab for retinal vasculitis and extraocular symptoms in Behçet disease. Jpn J Ophthalmol. 2018 May. 62 (3):390-397. [QxMD MEDLINE Link].

58. Levy-Clarke G, Jabs DA, Read RW, Rosenbaum JT, Vitale A, Van Gelder RN. Expert panel recommendations for the use of anti-tumor necrosis factor biologic agents in patients with ocular inflammatory disorders. Ophthalmology. 2014 Mar. 121 (3):785-96.e3. [QxMD MEDLINE Link].

59. Deuter CM, Zierhut M, Mohle A, et al. Long-term remission after cessation of interferon-a treatment in patients with severe uveitis due to Behçet's disease. Arthritis Rheum. 2010 Sep. 62(9):2796-805. [QxMD MEDLINE Link].

60. Celiker H, Kazokoglu H, Direskeneli H. Conventional immunosuppressive therapy in severe Behcet's Uveitis: the switch rate to the biological agents. BMC Ophthalmol. 2018 Oct 5. 18 (1):261. [QxMD MEDLINE Link].

61. Nuñez-Sotelo CM, Gutiérrez-Gonzalez LA, Rodriguez MA. Rapid, complete and sustained response to corticosteroids and pulse cyclophosphamide therapy in a patient with behçet and central nervous disease. Maedica (Buchar). 2012 Dec. 7(4):348-51. [QxMD MEDLINE Link]. [Full Text].

62. Sfikakis PP, Markomichelakis N, Alpsoy E, et al. Anti-TNF therapy in the management of Behcet's disease--review and basis for recommendations. Rheumatology (Oxford). 2007 May. 46(5):736-41. [QxMD MEDLINE Link].

63. McNally TW, Damato EM, Murray PI, Denniston AK, Barry RJ. An update on the use of biologic therapies in the management of uveitis in Behçet's disease: a comprehensive review. Orphanet J Rare Dis. 2017 Jul 17. 12 (1):130. [QxMD MEDLINE Link]. [Full Text].

64. Ueda A, Takeno M, Ishigatsubo Y. Adalimumab in the management of Behçet's disease. Ther Clin Risk Manag. 2015. 11:611-9. [QxMD MEDLINE Link].

65. Melikoglu M, Fresko I, Mat C, Ozyazgan Y, Gogus F, Yurdakul S, et al. Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. J Rheumatol. 2005 Jan. 32 (1):98-105. [QxMD MEDLINE Link].

66. Vallet H, et al; French Behçet Network. Efficacy of anti-TNF alpha in severe and/or refractory Behçet's disease: Multicenter study of 124 patients. J Autoimmun. 2015 Aug. 62:67-74. [QxMD MEDLINE Link].

67. Takayama K, Ishikawa S, Enoki T, Kojima T, Takeuchi M. Successful Treatment with Infliximab for Behçet Disease during Pregnancy. Ocul Immunol Inflamm. 2013 Apr 25. [QxMD MEDLINE Link].

68. Davatchi F, Shams H, Rezaipoor M, Sadeghi-Abdollahi B, Shahram F, Nadji A, et al. Rituximab in intractable ocular lesions of Behcet's disease; randomized single-blind control study (pilot study). Int J Rheum Dis. 2010 Aug. 13 (3):246-52. [QxMD MEDLINE Link].

69. Sadreddini S, Noshad H, Molaeefard M, Noshad R. Treatment of retinal vasculitis in Behçet's disease with rituximab. Mod Rheumatol. 2008. 18 (3):306-8. [QxMD MEDLINE Link].

70. Perez-Pampin E, Campos-Franco J, Blanco J, Mera A. Remission induction in a case of refractory Behçet disease with alemtuzumab. J Clin Rheumatol. 2013 Mar. 19(2):101-3. [QxMD MEDLINE Link].

71. Mesquida M, Victoria Hernández M, Llorenç V, et al. Behçet disease-associated uveitis successfully treated with golimumab. Ocul Immunol Inflamm. 2013 Apr. 21(2):160-2. [QxMD MEDLINE Link].

72. Atienza-Mateo B, Martín-Varillas JL, Calvo-Río V, et al. Comparative Study of Infliximab Versus Adalimumab in Refractory Uveitis due to Behçet's Disease: National Multicenter Study of 177 Cases. Arthritis Rheumatol. 2019 Dec. 71 (12):2081-2089. [QxMD MEDLINE Link]. [Full Text].

73. Hatemi G, Mahr A, Ishigatsubo Y, Song YW, Takeno M, Kim D, et al. Trial of Apremilast for Oral Ulcers in Behçet's Syndrome. N Engl J Med. 2019 Nov 14. 381 (20):1918-1928. [QxMD MEDLINE Link]. [Full Text].

74. Di Scala G, Bettiol A, Cojan RD, Finocchi M, Silvestri E, Emmi G. Efficacy of the anti-IL 17 secukinumab in refractory Behçet's syndrome: A preliminary study. J Autoimmun. 2019 Feb. 97:108-113. [QxMD MEDLINE Link].

75. Fagni F, Bettiol A, Talarico R, Lopalco G, Silvestri E, Urban ML, et al. Long-term effectiveness and safety of secukinumab for treatment of refractory mucosal and articular Behçet's phenotype: a multicentre study. Ann Rheum Dis. 2020 Aug. 79 (8):1098-1104. [QxMD MEDLINE Link].

76. Hatemi G, Melikoglu M, Tunc R, Korkmaz C, Turgut Ozturk B, Mat C, et al. Apremilast for Behçet's syndrome--a phase 2, placebo-controlled study. N Engl J Med. 2015 Apr 16. 372 (16):1510-8. [QxMD MEDLINE Link].

77. FDA Approves Otezla (apremilast) for the Treatment of Oral Ulcers Associated with Behçet’s Disease. Drugs.com. Available at https://www.drugs.com/newdrugs/fda-approves-otezla-apremilast-oral-ulcers-associated-beh-et-s-5019.html. July 19, 2019; Accessed: October 6, 2022.

78. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, Huseyin Altunbas H, Urgancioglu M. Uveitis in Behçet disease: an analysis of 880 patients. Am J Ophthalmol. 2004 Sep. 138 (3):373-80. [QxMD MEDLINE Link].

Nicole Davey-Ranasinghe, MD Rheumatologist, Allergy ARTS, LLP, Amarillo Center for Clinical Research, Ltd

Nicole Davey-Ranasinghe, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

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Constantine K Saadeh, MD President, Allergy ARTS, LLP; Principal Investigator, Amarillo Center for Clinical Research, Ltd

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Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

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Jeffrey R Lisse, MD, FACP Professor, Department of Internal Medicine, Chief, Section of Rheumatology, University of Arizona School of Medicine

Jeffrey R Lisse, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, American Geriatrics Society, Sigma Xi, The Scientific Research Honor Society

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Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Interim Chief, Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

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Fatima A Alnaimat, MBBS Rheumatologist, Essentia Health

Fatima A Alnaimat, MBBS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

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Augusto C Posadas, MD Rheumatologist, Saguaro Physician Group, Tucson, AZ

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