{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,4]],"date-time":"2023-10-04T05:41:56Z","timestamp":1696398116517},"reference-count":11,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2013,9,1]],"date-time":"2013-09-01T00:00:00Z","timestamp":1377993600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Hepatology Research"],"published-print":{"date-parts":[[2014,10]]},"abstract":"Primary biliary cirrhosis (PBC<\/jats:styled-content>) is a cholestatic liver disease with an elevated serum immunoglobulin (Ig<\/jats:styled-content>)M<\/jats:styled-content> level. Patients with PBC<\/jats:styled-content> may develop extrahepatic manifestations, including hypogammaglobulinemia. However, hypogammaglobulinemia seldom occurs, and the associated changes of lymphocytes remain unknown. Furthermore, the impact of I<\/jats:styled-content>g on the progression of PBC<\/jats:styled-content> is still unclear. Here, we describe a case of hypogammaglobulinemia developed in a female patient with PBC<\/jats:styled-content>. The patient was diagnosed with PBC<\/jats:styled-content> at the age of 46 years and treated with ursodeoxycholic acid and bezafibrate. At the age of 50 years, the patient developed bronchitis, and laboratory test results indicated a marked decrease in serum levels of IgA<\/jats:styled-content>, IgM<\/jats:styled-content> and IgG<\/jats:styled-content>. Then, the patient was diagnosed as having idiopathic hypogammaglobulinemia and treated with I<\/jats:styled-content>g replacement therapy; however, respiratory infections recurred frequently, leading to the patient's death at the age of 53 years. An autopsy revealed hyperplastic bone marrow with CD<\/jats:styled-content>3, CD<\/jats:styled-content>20 and IgG<\/jats:styled-content> positive lymphocytes. However, no CD<\/jats:styled-content>79a, CD<\/jats:styled-content>138, IgA<\/jats:styled-content> and IgM<\/jats:styled-content> positive lymphocytes were observed. Moreover, the severity of PBC<\/jats:styled-content> progressed even after the onset of hypogammaglobulinemia. In addition, CD<\/jats:styled-content>3 positive cells were seen around chronic non\u2010suppurative destructive cholangitis in the autopsy specimen of the liver. Thus, the present case demonstrated changes of lymphocytes in hypogammaglobulinemia developed in patients with PBC<\/jats:styled-content>. Furthermore, the clinical course of the present case of PBC<\/jats:styled-content> may indicate that the I<\/jats:styled-content>g\u2010mediated mechanisms may be non\u2010essential for the progression of PBC<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/hepr.12211","type":"journal-article","created":{"date-parts":[[2013,7,29]],"date-time":"2013-07-29T11:51:41Z","timestamp":1375098701000},"source":"Crossref","is-referenced-by-count":1,"title":["Decreased serum levels of immunoglobulin A<\/scp>, immunoglobulin M<\/scp> and immunoglobulin G<\/scp> in a patient with primary biliary cirrhosis: A case report"],"prefix":"10.1111","volume":"44","author":[{"given":"Takumi","family":"Kawaguchi","sequence":"first","affiliation":[{"name":"Division of Gastroenteorlogy Department of Medicine Kurume University School of Medicine Kurume Japan"},{"name":"Department of Digestive Disease Information and Research Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Toshimitsu","family":"Tanaka","sequence":"additional","affiliation":[{"name":"Division of Gastroenteorlogy Department of Medicine Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michitoshi","family":"Hashiguchi","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology Department of Medicine Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroaki","family":"Miyoshi","sequence":"additional","affiliation":[{"name":"Department of Pathology Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun","family":"Akiba","sequence":"additional","affiliation":[{"name":"Department of Pathology Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masayoshi","family":"Kage","sequence":"additional","affiliation":[{"name":"Department of Diagnostic Pathology Kurume University Hospital Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hirohisa","family":"Yano","sequence":"additional","affiliation":[{"name":"Department of Pathology Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Koichi","family":"Ohshima","sequence":"additional","affiliation":[{"name":"Department of Pathology Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takashi","family":"Okamura","sequence":"additional","affiliation":[{"name":"Division of Hematology and Oncology Department of Medicine Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michio","family":"Sata","sequence":"additional","affiliation":[{"name":"Division of Gastroenteorlogy Department of Medicine Kurume University School of Medicine Kurume Japan"},{"name":"Department of Digestive Disease Information and Research Kurume University School of Medicine Kurume Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,9]]},"reference":[{"key":"e_1_2_4_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840080128"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2001.25086"},{"key":"e_1_2_4_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840080137"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840110504"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1386-6346(01)00138-3"},{"key":"e_1_2_4_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(86)90922-4"},{"key":"e_1_2_4_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2796.1995.tb00936.x"},{"key":"e_1_2_4_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(08)61199-X"},{"key":"e_1_2_4_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1872-034X.2007.00281.x"},{"key":"e_1_2_4_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840200120"},{"key":"e_1_2_4_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.24748"}],"container-title":["Hepatology 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