{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,16]],"date-time":"2026-05-16T03:41:31Z","timestamp":1778902891542,"version":"3.51.4"},"reference-count":15,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,11,14]],"date-time":"2017-11-14T00:00:00Z","timestamp":1510617600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2017,11,14]],"date-time":"2017-11-14T00:00:00Z","timestamp":1510617600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Stofwisselkracht","award":["1269"],"award-info":[{"award-number":["1269"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>Zellweger spectrum disorders (ZSDs) are caused by an impairment of peroxisome biogenesis, resulting in multiple metabolic abnormalities. This leads to a range of symptoms, including hepatic dysfunction and coagulopathy. This study evaluated the incidence and severity of coagulopathy and the effect of vitamin K supplementation orally and IV in ZSD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Data were retrospectively retrieved from the medical records of 30 ZSD patients to study coagulopathy and the effect of vitamin K orally on proteins induced by vitamin K absence (PIVKA\u2010II) levels. Five patients from the cohort with a prolonged prothrombin time, low factor VII, and elevated PIVKA\u2010II levels received 10 mg of vitamin K IV. Laboratory results, including thrombin generation, at baseline and 72 h after vitamin K administration were examined.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In the retrospective cohort, four patients (13.3%) experienced intracranial bleedings and 14 (46.7%) reported minor bleeding. No thrombotic events occurred. PIVKA\u2010II levels decreased 38% after start of vitamin K therapy orally. In the five patients with a coagulopathy, despite treatment with oral administration of vitamin K, vitamin K IV caused an additional decrease (23%) of PIVKA\u2010II levels and increased thrombin generation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Bleeding complications frequently occur in ZSD patients due to liver disease and vitamin K deficiency. Vitamin K deficiency is partly corrected by vitamin K supplementation orally, and vitamin K administered IV additionally improves vitamin K status, as shown by further decrease of PIVKA\u2010II and improved thrombin generation.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s10545-017-0113-8","type":"journal-article","created":{"date-parts":[[2017,11,14]],"date-time":"2017-11-14T07:23:29Z","timestamp":1510644209000},"page":"249-255","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Coagulopathy in Zellweger spectrum disorders: a role for vitamin K"],"prefix":"10.1002","volume":"41","author":[{"given":"Sara","family":"Zeynelabidin","sequence":"first","affiliation":[{"name":"Department of Pediatric Neurology, Emma Children's Hospital, Academic Medical Center University of Amsterdam Meibergdreef 9 Amsterdam 1105 AZ The Netherlands"},{"name":"Department of Pediatric Hematology, Emma Children's Hospital, Academic Medical Center Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Femke C. C.","family":"Klouwer","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, Emma Children's Hospital, Academic Medical Center University of Amsterdam Meibergdreef 9 Amsterdam 1105 AZ The Netherlands"},{"name":"Laboratory Genetic Metabolic Diseases, Academic Medical Center University of Amsterdam Meibergdreef 9 Amsterdam 1105 AZ The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joost C. M.","family":"Meijers","sequence":"additional","affiliation":[{"name":"Department of Experimental Vascular Medicine, Academic Medical Center University of Amsterdam Meibergdreef 9 Amsterdam 1105 AZ The Netherlands"},{"name":"Department of Plasma Proteins Sanquin Research Amsterdam the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Monique H.","family":"Suijker","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology, Emma Children's Hospital, Academic Medical Center Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marc","family":"Engelen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, Emma Children's Hospital, Academic Medical Center University of Amsterdam Meibergdreef 9 Amsterdam 1105 AZ The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8118-5419","authenticated-orcid":false,"given":"Bwee Tien","family":"Poll\u2010The","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, Emma Children's Hospital, Academic Medical Center University of Amsterdam Meibergdreef 9 Amsterdam 1105 AZ The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C. 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