{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,21]],"date-time":"2023-10-21T14:13:29Z","timestamp":1697897609943},"reference-count":31,"publisher":"Akademiai Kiado Zrt.","issue":"22","license":[{"start":{"date-parts":[[2023,6,4]],"date-time":"2023-06-04T00:00:00Z","timestamp":1685836800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["akjournals.com"],"crossmark-restriction":true},"short-container-title":["OH"],"published-print":{"date-parts":[[2023,6,4]]},"abstract":"<jats:p>A Good-szindr\u00f3ma igen ritka \u00e9s szokatlan immunhi\u00e1nyos \u00e1llapot, melyre a thymoma\n                    \u00e9s a hypogammaglobulinaemia t\u00e1rsul\u00e1sa jellegzetes. A k\u00f6zel 70 \u00e9ve le\u00edrt,\n                    alapvet\u0151en rossz progn\u00f3zis\u00fa betegs\u00e9gben szenved\u0151k k\u00fcl\u00f6n\u00f6sen fog\u00e9konyak\n                    visszat\u00e9r\u0151 invaz\u00edv bakteri\u00e1lis \u00e9s opportunista fert\u0151z\u00e9sekre, emellett autoimmun\n                    \u00e9s malignus megbeteged\u00e9sek is gyakrabban manifeszt\u00e1l\u00f3dnak. Els\u0151sorban\n                    k\u00f6z\u00e9pkor\u00faakat \u00e9rint. A legjellemz\u0151bb immunol\u00f3giai elt\u00e9r\u00e9sk\u00e9nt a\n                    hypogammaglobulinaemia \u00e9s a cs\u00f6kkent\/hi\u00e1nyz\u00f3 B-sejt-sz\u00e1m eml\u00edthet\u0151. Napjainkban\n                    a betegs\u00e9get szerzett kombin\u00e1lt (T, B) immunhi\u00e1nyos \u00e1llapotk\u00e9nt, fenok\u00f3piak\u00e9nt\n                    oszt\u00e1lyozz\u00e1k. A komplex immunkompromitt\u00e1lt k\u00f3r\u00e1llapot klinikailag rendk\u00edv\u00fcl\n                    heterog\u00e9n fenot\u00edpusos megjelen\u00e9sre vezethet, \u00edgy felismer\u00e9se val\u00f3di kih\u00edv\u00e1s. A\n                    thymoma t\u00f6bbnyire benignus, \u00e9s \u00e1ltal\u00e1ban v\u00e9letlenszer\u0171en ker\u00fcl felfed\u00e9sre. Mivel\n                    a thymus kulcsszerepet j\u00e1tszik az immunrendszer fejl\u0151d\u00e9s\u00e9ben, thymoma eset\u00e9n a\n                    megv\u00e1ltozott sz\u00f6veti strukt\u00fara \u00e9s mikrok\u00f6rnyezet egyar\u00e1nt hajlamos\u00edthat\n                    immundeficientia \u00e9s autoimmunit\u00e1s kialakul\u00e1s\u00e1ra. A betegs\u00e9g kialakul\u00e1s\u00e1nak oka\n                    m\u00e1ig tiszt\u00e1zatlan. Felt\u00e9telezik, hogy f\u0151k\u00e9nt epigenetikai faktorok \u00e9s szerzett\n                    genetikai t\u00e9nyez\u0151k egy\u00fcttesen tehet\u0151k felel\u0151ss\u00e9 a k\u00f3rk\u00e9p megjelen\u00e9s\u00e9\u00e9rt. A\n                    Good-szindr\u00f3m\u00e1nak jelenleg nincs specifikus kezel\u00e9se. A thymectomia mellett\n                    alapvet\u0151 az infekci\u00f3k gy\u00f3gyszeres kontrollja, esetleg szekunder prevenci\u00f3ja \u00e9s a\n                    rendszeres immunglobulin-p\u00f3tl\u00e1s. Orv Hetil. 2023; 164(22): 859\u2013863.<\/jats:p>","DOI":"10.1556\/650.2023.32800","type":"journal-article","created":{"date-parts":[[2023,6,5]],"date-time":"2023-06-05T05:57:30Z","timestamp":1685944650000},"page":"859-863","update-policy":"http:\/\/dx.doi.org\/10.1556\/policypage.crossmark.crossref.2","source":"Crossref","is-referenced-by-count":0,"title":["Good-szindr\u00f3ma: egy ritka, szokatlan immunhi\u00e1nyos \u00e1llapot"],"prefix":"10.1556","volume":"164","author":[{"given":"Gy\u00f6rgyi","family":"M\u0171zes","sequence":"first","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ferenc","family":"Sipos","sequence":"additional","affiliation":[{"name":"Semmelweis Egyetem, \u00c1ltal\u00e1nos Orvostudom\u00e1nyi Kar, Belgy\u00f3gy\u00e1szati \u00e9s Hematol\u00f3giai Klinika Budapest, Szentkir\u00e1lyi u. 46., 1088 Magyarorsz\u00e1g"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"425","reference":[{"key":"ref0","unstructured":"1 Good RA. Agammaglobulinemia - a provocative experiment of nature. Bull Univ Minn Hosp Min Med Found. 1954; 26: 1-19."},{"key":"ref1","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1136\/jcp.56.1.12","article-title":"What is Good's syndrome? Immunological abnormalities in patients with thymoma","volume":"56","author":"Kelleher P","year":"2003","unstructured":"2 Kelleher P, Misbah SA. What is Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol. 2003; 56: 12-16.","journal-title":"J Clin Pathol"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"343","DOI":"10.1007\/s00277-003-0635-z","article-title":"B-cell lymphopenia and hypogammaglobulinemia in thymoma patients","volume":"82","author":"3 Montella","year":"2003","unstructured":"3 Montella L, Masci AM, Merkabaoui G, et al. B-cell lymphopenia and hypogammaglobulinemia in thymoma patients. Ann Hematol. 2003; 82: 343-347.","journal-title":"Ann Hematol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"679556","DOI":"10.3389\/fimmu.2021.679556","article-title":"When the Good syndrome goes bad: a systematic literature review","volume":"12","author":"Shi Y","year":"2021","unstructured":"4 Shi Y, Wang C. When the Good syndrome goes bad: a systematic literature review. Front Immunol. 2021; 12: 679556.","journal-title":"Front Immunol"},{"key":"ref4","doi-asserted-by":"publisher","unstructured":"5 Malphettes M, G\u00e9rard L, Galicier L, et al. Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications. Clin Infect Dis. 2015; 61: e13-e19.","DOI":"10.1093\/cid\/civ269"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"347","DOI":"10.1016\/j.clim.2010.01.006","article-title":"Good's syndrome remains a mystery after 55 years: a systematic review of the scientific evidence","volume":"135","author":"Kelesidis T","year":"2010","unstructured":"6 Kelesidis T, Yang O. Good's syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol. 2010; 135: 347-363.","journal-title":"Clin Immunol"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"546","DOI":"10.1002\/ijc.11099","article-title":"Malignant thymoma in the United States: demographic patterns in incidence and associations with subsequent malignancies","volume":"105","author":"Engels EA","year":"2003","unstructured":"7 Engels EA, Pfeiffer RM. Malignant thymoma in the United States: demographic patterns in incidence and associations with subsequent malignancies. Int J Cancer 2003; 105: 546-551.","journal-title":"Int J Cancer"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1007\/s13244-012-0201-0","article-title":"A diagnostic approach to the mediastinal masses","volume":"4","author":"8 Juanpere","year":"2013","unstructured":"8 Juanpere S, Ca\u00f1ete N, Ortu\u00f1o P, et al. A diagnostic approach to the mediastinal masses. Insights Imaging 2013; 4: 29-52.","journal-title":"Insights Imaging"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"844","DOI":"10.1038\/ng.3016","article-title":"A specific missense mutation in GTF2I occurs at high frequency in thymic epithelial tumors","volume":"46","author":"9 Petrini","year":"2014","unstructured":"9 Petrini I, Meltzer PS, Kim IK, et al. A specific missense mutation in GTF2I occurs at high frequency in thymic epithelial tumors. Nat Genet. 2014; 46: 844-849.","journal-title":"Nat Genet"},{"key":"ref9","unstructured":"10 Radovich M, Pickering CR, Felau I, et al. The integrated genomic landscape of thymic epithelial tumors. Cancer Cell 2018; 33: 244-258.e10."},{"key":"ref10","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1016\/j.autrev.2015.09.005","article-title":"Thymoma associated with autoimmune diseases: 85 cases and literature review","volume":"15","author":"11 Bernard","year":"2016","unstructured":"11 Bernard C, Frih H, Pasquet F, et al. Thymoma associated with autoimmune diseases: 85 cases and literature review. Autoimmun Rev. 2016; 15: 82-92.","journal-title":"Autoimmun Rev"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1038\/cmi.2010.74","article-title":"Thymoma and autoimmunity","volume":"8","author":"12 Shelly","year":"2011","unstructured":"12 Shelly S, Agmon-Levin N, Altman A, et al. Thymoma and autoimmunity. Cell Mol Immunol. 2011; 8: 199-202.","journal-title":"Cell Mol Immunol"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"659","DOI":"10.1038\/s41590-018-0128-9","article-title":"Thymic tolerance as a key brake on autoimmunity","volume":"19","author":"Cheng M","year":"2018","unstructured":"13 Cheng M, Anderson MS. Thymic tolerance as a key brake on autoimmunity. Nat Immunol. 2018; 19: 659-664.","journal-title":"Nat Immunol"},{"key":"ref13","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1016\/j.imlet.2020.12.010","article-title":"Immunodeficiency and thymoma in Good syndrome: two sides of the same coin","volume":"231","author":"14 Guevara-Hoyer","year":"2021","unstructured":"14 Guevara-Hoyer K, Fuentes-Antr\u00e1s J, Calatayud Gastardi J, et al. Immunodeficiency and thymoma in Good syndrome: two sides of the same coin. Immunol Lett. 2021; 231: 11-17.","journal-title":"Immunol Lett"},{"key":"ref14","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1016\/j.clim.2016.07.025","article-title":"Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective","volume":"171","author":"15 Jansen","year":"2016","unstructured":"15 Jansen A, van Deuren M, Miller J, et al. Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. Clin Immunol. 2016; 171: 12-17.","journal-title":"Clin Immunol"},{"key":"ref15","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1177\/039463201002300129","article-title":"Thymoma-associated immunodeficiency: a syndrome characterized by severe alterations in NK, T and B-cells and progressive increase in na\u00efve CD8+ T cells","volume":"23","author":"16 Vitiello","year":"2010","unstructured":"16 Vitiello L, Masci AM, Montella L, et al. Thymoma-associated immunodeficiency: a syndrome characterized by severe alterations in NK, T and B-cells and progressive increase in na\u00efve CD8+ T cells. Int J Immunopathol Pharmacol. 2010; 23: 307-316.","journal-title":"Int J Immunopathol Pharmacol"},{"key":"ref16","doi-asserted-by":"publisher","first-page":"3106","DOI":"10.1182\/blood-2002-08-2638","article-title":"Clonal expansion of CD8+ BV8 T lymphocytes in bone marrow characterizes thymoma-associated B lymphopenia","volume":"101","author":"17 Masci","year":"2003","unstructured":"17 Masci AM, Palmieri G, Vitiello L, et al. Clonal expansion of CD8+ BV8 T lymphocytes in bone marrow characterizes thymoma-associated B lymphopenia. Blood 2003; 101: 3106-3108.","journal-title":"Blood"},{"key":"ref17","doi-asserted-by":"publisher","first-page":"659","DOI":"10.1038\/76233","article-title":"Limitin: an interferon-like cytokine that preferentially influences B-lymphocyte precursors","volume":"6","author":"18 Oritani","year":"2000","unstructured":"18 Oritani K, Medina KL, Tomiyama Y, et al. Limitin: an interferon-like cytokine that preferentially influences B-lymphocyte precursors. Nat Med. 2000; 6: 659-666.","journal-title":"Nat Med"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"3045","DOI":"10.4049\/jimmunol.1402805","article-title":"A novel thymoma-associated immunodeficiency with increased naive T cells and reduced CD247 expression","volume":"194","author":"19 Christopoulos","year":"2015","unstructured":"19 Christopoulos P, Dopfer EP, Malkovsky M, et al. A novel thymoma-associated immunodeficiency with increased naive T cells and reduced CD247 expression. J Immunol. 2015; 194: 3045-3053.","journal-title":"J Immunol"},{"key":"ref19","doi-asserted-by":"publisher","first-page":"4848","DOI":"10.1182\/blood-2010-05-286161","article-title":"Anti-cytokine autoantibodies are associated with opportunistic infection in patients with thymic neoplasia","volume":"116","author":"20 Burbelo","year":"2010","unstructured":"20 Burbelo PD, Browne SK, Sampaio EP, et al. Anti-cytokine autoantibodies are associated with opportunistic infection in patients with thymic neoplasia. Blood 2010; 116: 4848-4858.","journal-title":"Blood"},{"key":"ref20","doi-asserted-by":"publisher","first-page":"10","DOI":"10.1016\/j.clim.2012.10.006","article-title":"Lys154Ter identified in Good syndrome","volume":"146","author":"21 Margraf","year":"2013","unstructured":"21 Margraf RL, Coonrod EM, Durtschi JD, et al. TACI mutation p.Lys154Ter identified in Good syndrome. Clin Immunol. 2013; 146: 10-12.","journal-title":"Clin Immunol"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1016\/j.clim.2014.04.002","article-title":"BAFF-R mutations in Good's syndrome","volume":"153","author":"22 Lougaris","year":"2014","unstructured":"22 Lougaris V, Vitali M, Baronio M, et al. BAFF-R mutations in Good's syndrome. Clin Immunol. 2014; 153: 91-93.","journal-title":"Clin Immunol"},{"key":"ref22","doi-asserted-by":"publisher","unstructured":"23 Primary immunodeficiency diseases. Report of an IUIS Scientific Committee. International Union of Immunological Societies. Clin Exp Immunol. 1999; 118 (Suppl 1): 1-28.","DOI":"10.1046\/j.1365-2249.1999.00109.x"},{"key":"ref23","doi-asserted-by":"publisher","unstructured":"24 Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005; 94(5 Suppl 1): S1-S63. Erratum: Ann Allergy Asthma Immunol. 2006; 96: 504.","DOI":"10.1016\/S1081-1206(10)61142-8"},{"key":"ref24","doi-asserted-by":"publisher","first-page":"815710","DOI":"10.3389\/fimmu.2021.815710","article-title":"Good's syndrome: time to move on from reviewing the past","volume":"12","author":"25 Kabir","year":"2022","unstructured":"25 Kabir A, Alizadehfar R, Tsoukas CM. Good's syndrome: time to move on from reviewing the past. Front Immunol. 2022; 12: 815710.","journal-title":"Front Immunol"},{"key":"ref25","doi-asserted-by":"publisher","first-page":"1508","DOI":"10.1007\/s10875-022-01352-z","article-title":"The 2022 update of IUIS phenotypical classification for human inborn errors of immunity","volume":"42","author":"26 Bousfiha","year":"2022","unstructured":"26 Bousfiha A, Moundir A, Tangye SG, et al. The 2022 update of IUIS phenotypical classification for human inborn errors of immunity. J Clin Immunol. 2022; 42: 1508-1520.","journal-title":"J Clin Immunol"},{"key":"ref26","doi-asserted-by":"publisher","first-page":"132","DOI":"10.1111\/cei.13216","article-title":"Clinical and laboratory features of seventy-eight UK patients with Good's syndrome (thymoma and hypogammaglobulinaemia)","volume":"195","author":"27 Zaman","year":"2019","unstructured":"27 Zaman M, Huissoon A, Buckland M, et al. Clinical and laboratory features of seventy-eight UK patients with Good's syndrome (thymoma and hypogammaglobulinaemia). Clin Exp Immunol. 2019; 195: 132-138.","journal-title":"Clin Exp Immunol"},{"key":"ref27","unstructured":"28 Hermaszewski RA, Webster AD. Primary hypogammaglobulinaemia: a survey of clinical manifestations and complications. Q J Med. 1993; 86: 31-42."},{"key":"ref28","doi-asserted-by":"publisher","first-page":"123","DOI":"10.1097\/00005792-200103000-00005","article-title":"Report of 5 cases and review of the literature","volume":"80","author":"29 Tarr","year":"2001","unstructured":"29 Tarr PE, Sneller MC, Mechanic LJ, et al. Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. Medicine (Baltimore) 2001; 80: 123-133.","journal-title":"Medicine (Baltimore)"},{"key":"ref29","doi-asserted-by":"publisher","first-page":"307","DOI":"10.3389\/fonc.2014.00307","article-title":"Good syndrome, bad problem","volume":"4","author":"Martinez B","year":"2014","unstructured":"30 Martinez B, Browne SK. Good syndrome, bad problem. Front Oncol. 2014; 4: 307.","journal-title":"Front Oncol"},{"key":"ref30","unstructured":"31 M\u0171zes G, Sipos F. An unexpected diagnosis: Good's syndrome. 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