{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T13:47:41Z","timestamp":1775051261969,"version":"3.50.1"},"reference-count":17,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T00:00:00Z","timestamp":1606780800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["BMC Pediatr"],"published-print":{"date-parts":[[2020,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>X-linked agammaglobulinemia (XLA, OMIM#300,300), caused by mutations in the Bruton tyrosine kinase (<jats:italic>BTK<\/jats:italic>) gene, is a rare monogenic inheritable immunodeficiency disorder. Ecthyma gangrenosum is a cutaneous lesion caused by <jats:italic>Pseudomonas aeruginosa<\/jats:italic> that typically occurs in patients with XLA and other immunodeficiencies.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>We report the case of a 20-month-old boy who presented with fever, vomiting, diarrhea, and ecthyma gangrenosum. Blood, stool, and skin lesion culture samples were positive for <jats:italic>P. aeruginosa<\/jats:italic>. A diagnosis of XLA was established, and the c.262G\u2009&gt;\u2009T mutation in exon 4 of <jats:italic>BTK<\/jats:italic> was identified with Sanger sequencing. Symptoms improved following treatment with antibiotics and immunoglobulin infusion.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Primary immunodeficiency (i.e., XLA) should be suspected in male infants with <jats:italic>P. aeruginosa<\/jats:italic> sepsis, highlighting the importance of genetic and immune testing in these patients.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s12887-020-02436-8","type":"journal-article","created":{"date-parts":[[2020,12,1]],"date-time":"2020-12-01T01:51:34Z","timestamp":1606787494000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Ecthyma gangrenosum due to Pseudomonas aeruginosa sepsis as initial manifestation of X-linked agammaglobulinemia: a case report"],"prefix":"10.1186","volume":"20","author":[{"given":"Haixia","family":"Huang","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ke","family":"Bai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yueqiang","family":"Fu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jin","family":"Yan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jing","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2020,12,1]]},"reference":[{"key":"2436_CR1","doi-asserted-by":"crossref","unstructured":"Soresina A, Moratto D, Chiarini M, Paolillo C, Baresi G, Foca\u0300 E, Bezzi M, Baronio B, Giacomelli M, Badolato R. Two X-linked agammaglobulinemia patients develop pneumonia as COVID-19 manifestation but recover. Pediatr Allergy Immunol. 2020;31(5):565\u20139.","DOI":"10.1111\/pai.13263"},{"issue":"3","key":"2436_CR2","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1007\/s12519-013-0400-x","volume":"9","author":"X Qin","year":"2013","unstructured":"Qin X, Jiang LP, Tang XM, Wang M, Liu EM, Zhao XD. Clinical features and mutation analysis of X-linked agammaglobulinemia in 20 Chinese patients. World J Pediatrics. 2013;9(3):273\u20137.","journal-title":"World J Pediatrics"},{"issue":"5","key":"2436_CR3","doi-asserted-by":"publisher","first-page":"612","DOI":"10.3346\/jkms.2011.26.5.612","volume":"26","author":"MA Yang","year":"2011","unstructured":"Yang MA, Lee J, Choi EH, Lee HJ. Pseudomonas aeruginosa bacteremia in children over ten consecutive years: analysis of clinical characteristics, risk factors of multi-drug resistance and clinical outcomes. 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