{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,23]],"date-time":"2026-05-23T05:07:54Z","timestamp":1779512874303,"version":"3.53.1"},"reference-count":20,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T00:00:00Z","timestamp":1778025600000},"content-version":"vor","delay-in-days":5,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Molec Gen &amp;amp; Gen Med"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Severe hypertriglyceridemia (HTG) is rare in children and is often caused by monogenic disorders. However, autoimmune mechanisms, particularly antibodies against glycosylphosphatidylinositol\u2010anchored high\u2010density lipoprotein\u2010binding protein 1 (GPIHBP1), have emerged as rare causes. Pediatric\u2010onset GPIHBP1 autoantibody\u2010related HTG remains poorly characterized.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We evaluated two 3\u2010year\u2010old children with extreme HTG (triglyceride level\u2009&gt;\u20093000\u2009mg\/dL). Whole\u2010exome sequencing was performed to investigate monogenic etiologies. Autoimmune testing included antinuclear antibodies (ANAs) and anti\u2010Sj\u00f6gren's syndrome\u2010related antigen A (SSA) antibodies, as well as an enzyme\u2010linked immunosorbent assay (ELISA) for anti\u2010GPIHBP1 antibodies. A clinical response to immunosuppressive therapy was assessed. A literature review of previously reported pediatric cases was conducted.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>No pathogenic variants were identified by whole\u2010exome sequencing. Both patients were positive for ANAs (1:320, speckled), and one had high\u2010titer anti\u2010SSA antibodies. The ELISA confirmed the presence of anti\u2010GPIHBP1 antibodies in both cases. Immunosuppressive therapy with hydroxychloroquine effectively reduced the triglyceride level in one patient, while the other required additional prednisolone. At follow\u2010ups (ages 9 and 10), neither patient developed overt systemic autoimmune disease. A literature review revealed 13 previously reported pediatric cases, most of which were diagnosed after the age of 11\u2009years and were frequently associated with systemic lupus erythematosus or other autoimmune disorders.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>GPIHBP1 autoantibody\u2010related HTG is important and potentially treatable severe pediatric HTG, representing an early manifestation of autoimmune dysregulation that requires an accurate diagnosis and longitudinal surveillance.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mgg3.70228","type":"journal-article","created":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T04:58:47Z","timestamp":1778129927000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["<scp>GPIHBP1<\/scp>\n                    Autoantibody\u2010Related Hypertriglyceridemia in Children: A Report of Two Cases and a Review of Pediatric Cases From the Literature"],"prefix":"10.1002","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4510-9953","authenticated-orcid":false,"given":"Rai\u2010Hseng","family":"Hsu","sequence":"first","affiliation":[{"name":"Department of Medical Genetics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Pediatrics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Pediatrics National Taiwan University College of Medicine  Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wuh\u2010Liang","family":"Hwu","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Pediatrics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Center for Precision Medicine, China Medical University Hospital  Taichung Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Feng\u2010Jung","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Division of Nephrology, Department of Internal Medicine National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Internal Medicine National Taiwan University College of Medicine  Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5011-7499","authenticated-orcid":false,"given":"Ni\u2010Chung","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Pediatrics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Pediatrics National Taiwan University College of Medicine  Taipei Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8802-5728","authenticated-orcid":false,"given":"Yin\u2010Hsiu","family":"Chien","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Pediatrics National Taiwan University Hospital  Taipei Taiwan"},{"name":"Department of Pediatrics National Taiwan University College of Medicine  Taipei 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