{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T13:13:18Z","timestamp":1768569198773,"version":"3.49.0"},"reference-count":11,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2022,1,7]],"date-time":"2022-01-07T00:00:00Z","timestamp":1641513600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,1,7]],"date-time":"2022-01-07T00:00:00Z","timestamp":1641513600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Allergy Asthma Clin Immunol"],"published-print":{"date-parts":[[2022,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Nodular regenerating hyperplasia (NRH) is the most common liver involvement in common variable immunodeficiency (CVID<jats:bold>).<\/jats:bold> Most patients are asymptomatic with gradually increasing alkaline phosphatase (ALP) and mildly elevated transaminase enzymes over the years. We report the first case of fatal liver mass rupture in a CVID patient with probable NRH<jats:bold>.<\/jats:bold><\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 24-year-old man was diagnosed with CVID at the age of 1.25\u00a0years. Genetic testing revealed a transmembrane activator and calcium-modulator and cyclophilin-ligand interactor (TACI) mutation. He had been receiving intravenous immunoglobulin (IVIg) replacement therapy ever since then. The trough level of serum IgG ranged between 750\u20131200\u00a0mg\/dL. However, he still had occasional episodes of lower respiratory tract infection until bronchiectasis developed. At 22\u00a0years old, computed tomography (CT) chest and abdomen as an investigation for lung infection revealed incidental findings of numerous nodular arterial-enhancing lesions in the liver and mild splenomegaly suggestive of NRH with portal hypertension. Seven months later, he developed sudden hypotension and tense bloody ascites. Emergency CT angiography of the abdomen showed NRH with intrahepatic hemorrhage and hemoperitoneum. Despite successful gel foam embolization, the patient died from prolonged shock and multiple organ failure.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Although CVID patients with NRH are generally asymptomatic, late complications including portal hypertension, hepatic failure, and hepatic rupture could occur. Therefore, an evaluation of liver function should be included in the regular follow-up of CVID patients.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13223-021-00643-1","type":"journal-article","created":{"date-parts":[[2022,1,7]],"date-time":"2022-01-07T18:03:04Z","timestamp":1641578584000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Fatal liver mass rupture in a common-variable-immunodeficiency patient with probable nodular regenerating hyperplasia"],"prefix":"10.1186","volume":"18","author":[{"given":"Mongkhon","family":"Sompornrattanaphan","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ranista","family":"Tongdee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chamard","family":"Wongsa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anupop","family":"Jitmuang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3182-4614","authenticated-orcid":false,"given":"Torpong","family":"Thongngarm","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,1,7]]},"reference":[{"issue":"1","key":"643_CR1","doi-asserted-by":"publisher","first-page":"116","DOI":"10.1016\/j.jaci.2013.12.1077","volume":"134","author":"B Gathmann","year":"2014","unstructured":"Gathmann B, Mahlaoui N, Gerard L, Oksenhendler E, Warnatz K, et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014;134(1):116\u201326.","journal-title":"J Allergy Clin Immunol"},{"key":"643_CR2","doi-asserted-by":"publisher","first-page":"1740","DOI":"10.3389\/fimmu.2017.01740","volume":"8","author":"JR Farmer","year":"2017","unstructured":"Farmer JR, Ong MS, Barmettler S, Yonker LM, Fuleihan R, Sullivan KE, et al. Common variable immunodeficiency non-infectious disease endotypes redefined using unbiased network clustering in large electronic datasets. Front Immunol. 2017;8:1740.","journal-title":"Front Immunol"},{"key":"643_CR3","doi-asserted-by":"publisher","first-page":"149","DOI":"10.3389\/fimmu.2020.00149","volume":"11","author":"HE Ho","year":"2020","unstructured":"Ho HE, Cunningham-Rundles C. Non-infectious complications of common variable immunodeficiency: updated clinical spectrum, sequelae, and insights to pathogenesis. 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The patient\u2019s relative was informed that de-identified data would be used in scientific research and publications.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"2"}}