{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,4,10]],"date-time":"2025-04-10T04:20:51Z","timestamp":1744258851480,"version":"3.40.4"},"reference-count":11,"publisher":"Colegio Mexicano de Inmunologia Clinica y Alergia, A. C.","issue":"4","license":[{"start":{"date-parts":[[2023,4,19]],"date-time":"2023-04-19T00:00:00Z","timestamp":1681862400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Rev Alerg Mex"],"abstract":"<jats:p>Introducci\u00f3n: El s\u00edndrome de hiper-IgM es un error innato de la inmunidad, caracterizado por un defecto en el cambio de isotipo de inmunoglobulina, con valores disminuidos de IgG, IgA e IgE, y concentraciones normales o elevadas de IgM. Predispone a procesos infecciosos en el sistema respiratorio y aparato gastrointestinal, adem\u00e1s de enfermedades autoinmunes y neoplasias.\nReporte de caso: Paciente pedi\u00e1trico de g\u00e9nero masculino, de 5 a\u00f1os y 7 meses de edad, con antecedente de dos cuadros de neumon\u00eda (uno de estos grave) y diarrea cr\u00f3nica desde los 2 a\u00f1os. Neutropenia moderada persistente, disminuci\u00f3n de la concentraci\u00f3n de IgG y elevaci\u00f3n de IgM. La citometr\u00eda de flujo confirm\u00f3 la ausencia de CD40L. Durante la evoluci\u00f3n cl\u00ednica tuvo afectaci\u00f3n hep\u00e1tica temprana.\nConclusi\u00f3n: El s\u00edndrome de hiper-IgM predispone a da\u00f1o hep\u00e1tico, por lo que se requiere la evaluaci\u00f3n completa y el diagn\u00f3stico oportuno. El tratamiento antiinfeccioso activo y el control de la respuesta inflamatoria son factores decisivos para establecer el tratamiento del da\u00f1o hep\u00e1tico.\n\u00a0<\/jats:p>","DOI":"10.29262\/ram.v69i4.1091","type":"journal-article","created":{"date-parts":[[2023,4,21]],"date-time":"2023-04-21T07:02:21Z","timestamp":1682060541000},"page":"214-219","source":"Crossref","is-referenced-by-count":0,"title":["S\u00edndrome de hiper-IgM con afectaci\u00f3n hep\u00e1tica temprana","Hyper-IgM Syndrome with Early Liver Involvement"],"prefix":"10.29262","volume":"69","author":[{"given":"Kareli Guadalupe","family":"Coronado Hern\u00e1ndez","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H\u00e9ctor Hugo","family":"Campos T\u00e9llez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana Paola","family":"Mac\u00edas Robles","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rosa Mar\u00eda","family":"Cort\u00e9s Grimaldo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carlos David","family":"Estrada Garc\u00eda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Britza","family":"Barrios D\u00edaz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adriana","family":"Ram\u00edrez Nepomuceno","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marl\u00e9n","family":"Barreto Alcal\u00e1","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Esparza Amaya","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hilda Lilian","family":"Carvajal Alonso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura","family":"Berr\u00f3n Ruiz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"11808","published-online":{"date-parts":[[2023,4,19]]},"reference":[{"key":"53617","doi-asserted-by":"crossref","unstructured":"Yazdani R, Fekrvand S, Shahkarami S, et al. The hyper IgM syndromes: Epidemiology, pathogenesis, clinical manifestations, diagnosis and management. Clin Immunol 2019; 198: 19-30. doi: 10.1016\/j.clim.2018.11.007","DOI":"10.1016\/j.clim.2018.11.007"},{"key":"53618","doi-asserted-by":"crossref","unstructured":"Freyer DR, Gowans LK, Warzynski M, et al. Flow cytometric diagnosis of X-linked hyper-IgM syndrome: application of an accurate and convenient procedure. J Pediatr Hematol Oncol 2004; 26 (6): 363-70. doi: 10.1097\/00043426-200406000-00006","DOI":"10.1097\/00043426-200406000-00006"},{"key":"53619","doi-asserted-by":"crossref","unstructured":"Fran\u00e7a TT, Barreiros LA, Al-Ramadi BK, et al. CD40 ligand deficiency: treatment strategies and novel therapeutic perspectives. Expert Rev Clin Immunol 2019; 15 (5): 529-540. doi: 10.1080\/1744666X.2019.1573674","DOI":"10.1080\/1744666X.2019.1573674"},{"key":"53620","doi-asserted-by":"crossref","unstructured":"de la Morena MT. Clinical Phenotypes of Hyper-IgM Syndromes. J Allergy Clin Immunol Pract 2016; 4 (6): 1023-1036. doi: 10.1016\/j.jaip.2016.09.013","DOI":"10.1016\/j.jaip.2016.09.013"},{"key":"53621","doi-asserted-by":"crossref","unstructured":"Fuleihan RL. The hyper IgM syndrome. Curr Allergy Asthma Rep 2001; 1 (5): 445-50. doi: 10.1007\/s11882-001-0030-6","DOI":"10.1007\/s11882-001-0030-6"},{"key":"53622","doi-asserted-by":"crossref","unstructured":"Barbouche MR, Chen Q, Carbone M, et al. Comprehensive review of autoantibodies in patients with hyper-IgM syndrome. Cell Mol Immunol 2018; 15 (6): 610-617. doi: 10.1038\/cmi.2017.140","DOI":"10.1038\/cmi.2017.140"},{"key":"53623","doi-asserted-by":"crossref","unstructured":"Bhushan A, Covey LR. CD40:CD40L interactions in X-linked and nonX-linked hyper-IgM syndromes. Immunol Res 2001; 24: 311-324. doi: 10.1385\/IR:24:3:311","DOI":"10.1385\/IR:24:3:311"},{"key":"53624","doi-asserted-by":"crossref","unstructured":"Wang TJ, Wu LF, Chen J, et al. X-linked hyper-IgM syndrome complicated with interstitial pneumonia and liver injury: a new mutation locus in the CD40LG gene. Immunol Res 2019; 67 (4-5): 454-459. doi: 10.1007\/s12026-019-09098-4","DOI":"10.1007\/s12026-019-09098-4"},{"key":"53625","unstructured":"Abinun M, Albert M, Cohen S, Buckland M, Bustamante J, Cant A, et al. ESID Registry \u2013 Working Definitions for Clinical Diagnosis of PID. 2019. https:\/\/esid.org\/Working-Parties\/Registry-Working-Party\/Diagnosis-criteria."},{"key":"53626","doi-asserted-by":"crossref","unstructured":"Rodr\u00edguez C, Carri\u00f3n F, Marinovic MA, et al. S\u00edndrome de hiper-IgM asociado a colangitis esclerosante y neoplasia vesicular: caso cl\u00ednico [X-linked hyper-IGM syndrome associated to sclerosing cholangitis and gallbladder neoplasm: clinical case]. 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