{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T09:13:06Z","timestamp":1784193186944,"version":"3.55.0"},"reference-count":27,"publisher":"SAGE Publications","issue":"1","license":[{"start":{"date-parts":[[2021,3,1]],"date-time":"2021-03-01T00:00:00Z","timestamp":1614556800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy, Immunology, and Pulmonology"],"published-print":{"date-parts":[[2021,3]]},"abstract":"<jats:sec>\n                    <jats:title>Introduction:<\/jats:title>\n                    <jats:p>\n                      Hyperimmunoglobulin E syndromes (HIESs) are characterized by a high serum immunoglobulin E (IgE) level, eczematoid rashes, recurrent staphylococcal skin abscesses, and recurrent pneumonia and pneumatocele formation. Autosomal dominant HIES is the most common form of HIES and mainly occurs due to loss-of-function mutations in the\n                      <jats:italic toggle=\"yes\">Signal Transducer and Activator of Transcription 3<\/jats:italic>\n                      (\n                      <jats:italic toggle=\"yes\">STAT3<\/jats:italic>\n                      ) gene (\n                      <jats:italic toggle=\"yes\">STAT3<\/jats:italic>\n                      LOF).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation:<\/jats:title>\n                    <jats:p>\n                      We report the case of an 11-year-old Peruvian girl diagnosed with\n                      <jats:italic toggle=\"yes\">STAT3<\/jats:italic>\n                      LOF caused by p.R382W mutation. She presented with recurrent staphylococcal pneumonia and empyema caused by the rarely reported\n                      <jats:italic toggle=\"yes\">Achromobacter xylosoxidans<\/jats:italic>\n                      , which led to severe destruction of the lung parenchyma, multiple lung surgeries, and the development of bronchopleural fistulas. A laparotomy was also performed, which showed evidence of sigmoid colon perforation. The patient received immunoglobulin replacement therapy (IRT) and antibiotic prophylaxis, and the frequency of her infections has decreased over the past 3 years.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>\n                      This is the first case of\n                      <jats:italic toggle=\"yes\">STAT3<\/jats:italic>\n                      LOF diagnosed by genomic sequencing in Peru. Patients with this mutation have recurrent pulmonary infections, and require multiple surgical procedures with frequent complications.\n                      <jats:italic toggle=\"yes\">A. xylosoxidans<\/jats:italic>\n                      infection could be related to the prolonged stay in intensive care leading to high mortality; therefore, additional care must be taken when treating patients with this infection. In addition, colonic perforation is a rare complication in\n                      <jats:italic toggle=\"yes\">STAT3<\/jats:italic>\n                      LOF patients. IRT and antibiotic prophylaxis appear to decrease the frequency of infections and hospitalizations.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1089\/ped.2020.1225","type":"journal-article","created":{"date-parts":[[2021,3,18]],"date-time":"2021-03-18T12:24:06Z","timestamp":1616070246000},"page":"33-37","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":2,"title":["Pulmonary Infections and Surgical Complications in a Young Girl with Signal Transducer and Activator of Transcription 3 Loss-of-Function Mutation Hyperimmunoglobulin E Syndrome: A Case Report"],"prefix":"10.1177","volume":"34","author":[{"given":"Crhistian","family":"Toribio-Dionicio","sequence":"first","affiliation":[{"name":"Faculty of Medicine, Universidad Nacional Mayor de San Marcos (UNMSM), Lima, Peru."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dania","family":"Cubas-Guzm\u00e1n","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Universidad Nacional Mayor de San Marcos (UNMSM), Lima, Peru."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pedro","family":"Guerra-Canchari","sequence":"additional","affiliation":[{"name":"Sociedad Cientifica de San Fernando, Faculty of Medicine, Universidad Nacional Mayor de San Marcos (UNMSM), Lima, Peru."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanuza","family":"Garc\u00eda-S\u00e1nchez","sequence":"additional","affiliation":[{"name":"Hospital Amazonico de Yarinacocha, Pucallpa, Peru."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wilmer","family":"C\u00f3rdova-Calder\u00f3n","sequence":"additional","affiliation":[{"name":"Unidad Funcional de Alergia, Asma e Inmunolog\u00eda (UFAAI), Instituto Nacional de Salud del Ni\u00f1o (INSN), Lima-Bre\u00f1a, Peru."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2021,3,17]]},"reference":[{"key":"e_1_3_5_2_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199903043400904"},{"key":"e_1_3_5_3_2","doi-asserted-by":"publisher","DOI":"10.1111\/imcb.12209"},{"key":"e_1_3_5_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccm.2016.04.016"},{"key":"e_1_3_5_5_2","doi-asserted-by":"publisher","DOI":"10.1086\/302547"},{"key":"e_1_3_5_6_2","doi-asserted-by":"publisher","DOI":"10.4161\/jkst.23435"},{"key":"e_1_3_5_7_2","unstructured":"Latin American Society for Immunodeficiencies: Online PID registry. Latin American Society for Immunodeficiencies; 2019\u20132020. The statistics general of LASID; October 2020 (about 23 screens). https:\/\/lasidregistry.org\/view\/statistics\/general\/2020-10 (accessed November27 2020)."},{"key":"e_1_3_5_8_2","first-page":"538","article-title":"Registration of patients with primary immunodeficiencies in the three main reference centers in Peru","volume":"35","author":"Garc\u00eda-Gomero DS","year":"2018","unstructured":"Garc\u00eda-Gomero DS, , C\u00f3rdova-Calder\u00f3n W, , Aldave-Becerra J. Registration of patients with primary immunodeficiencies in the three main reference centers in Peru. 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