{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T03:52:09Z","timestamp":1788580329902,"version":"build-2803163510"},"reference-count":19,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2024,10,12]],"date-time":"2024-10-12T00:00:00Z","timestamp":1728691200000},"content-version":"vor","delay-in-days":1291,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Introduction<\/jats:title>\n                  <jats:p>Patients with recessive dystrophic epidermolysis bullosa-severe subtype (RDEB-S) are at risk of vitamin K deficiency, potentially causing abnormal clotting, excessive bleeding, poor bone metabolism and abnormal vascular calcification. This study quantifies vitamin K deficiency prevalence in this cohort and identifies potential risk-factors to prevent deficiency.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Patients with RDEB-S who attended the EB service between 2014 and 2020 were included. Serum vitamin K and PIVKAII were measured as part of the usual nutritional blood screen. Dietetic and medical notes were reviewed to establish: antibiotic use, enteral feed intake and micronutrient supplementation.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>A total of 16\/25 64% (10\/16 female), of children aged 22\u2013180 months, had serum vitamin K and PIVKAII analysed. Six of sixteen (37.5%) patients had vitamin K deficiency requiring supplementation. Two of six (33.3%) normalized serum vitamin K after 12 weeks supplementation with oral menadiol diphosphate. Four of six (66.6%) await retesting following supplementation. Six of six (100%) patients with vitamin K deficiency were not consuming a gastrostomy\/sip feed. Nine of ten (90%) patients with sufficient vitamin K levels were consuming either; more than 200 ml prescribed sip feed or more than 400\u2013800 ml gastrostomy feed daily (containing 5.9\u201311 \u00b5g\/100 ml vitamin K). Patients who were consuming either more than 200 ml prescribed sip feed or more than 400\u2013800 ml gastrostomy feed daily (containing 5.9\u201311 \u00b5g\/100 ml vitamin K) were significantly less likely to suffer from vitamin K deficiency (0.08 odds ratio [(1\/7)\/(5\/3)] with significance level p = 0.0342 [95% CI: 0.0074\u20130.8275]). Sixteen of sixteen (100%) received antibiotics (range 0\u20134 courses\/year; median, 3; IQR, 3). Patients with the most frequent antibiotics (n = 4) had normal vitamin K and PIVKAII levels if they consumed a minimum of 200 ml prescribed sip feed or 400\u2013800 ml gastrostomy feed daily. Sixteen of sixteen (100%) patients took a multivitamin\/mineral supplement; none contained vitamin K.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Summary<\/jats:title>\n                  <jats:p>The prevalence of vitamin K deficiency is 37.5% in this cohort. Patients whom were not consuming gastrostomy\/sip feeds of at least 200 ml daily were at greatest risk of vitamin K deficiency. Patients on a micronutrient supplement remain at risk of vitamin K deficiency, as most contain no vitamin K. Prescribing a vitamin\/mineral supplement that contains vitamin K is recommended. Twelve-week supplementation of oral vitamin K (5 mg\/day for 1\u201310 years and 10 mg\/day for 12\u201317 years) adequately improved stores.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1002\/ski2.14","type":"journal-article","created":{"date-parts":[[2021,3,31]],"date-time":"2021-03-31T11:46:15Z","timestamp":1617191175000},"source":"Crossref","is-referenced-by-count":5,"title":["Prevalence and Treatment of Vitamin K Deficiency in Paediatric Patients with Recessive Dystrophic Epidermolysis Bullosa-Severe Subtype"],"prefix":"10.1093","volume":"1","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6058-0682","authenticated-orcid":false,"given":"N.","family":"Yerlett","sequence":"first","affiliation":[{"name":"Department of Dermatology, Great Ormond Street Hospital for Children , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Petrof","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Great Ormond Street Hospital for Children , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Holsgrove","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Great Ormond Street Hospital for Children , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Martinez","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Great Ormond Street Hospital for Children , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2021,3,31]]},"reference":[{"key":"2025010818015603600_ski214-bib-0001","doi-asserted-by":"publisher","first-page":"614","DOI":"10.1111\/bjd.18921","article-title":"Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility","volume":"183","author":"Has","year":"2020","journal-title":"Br J Dermatol"},{"key":"2025010818015603600_ski214-bib-0002","volume-title":"Clinical Paediatric Dietetics","author":"Yerlett","year":"2020","edition":"5th ed"},{"key":"2025010818015603600_ski214-bib-0003","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1067\/mpd.2002.123287","article-title":"Bone metabolism in children with epidermolysis bullosa","volume":"140","author":"Reyes","year":"2002","journal-title":"J Pediatr"},{"key":"2025010818015603600_ski214-bib-0004","doi-asserted-by":"crossref","first-page":"959","DOI":"10.1111\/j.1365-2133.2005.07123.x","article-title":"Bone mineralization in children with epidermolysis bullosa","volume":"154","author":"Fewtrell","year":"2006","journal-title":"Br J Dermatol"},{"key":"2025010818015603600_ski214-bib-0005","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.1016\/j.jaad.2010.08.028","article-title":"Correlates of low bone mass in children with generalized forms of epidermolysis bullosa","volume":"65","author":"Bruckner","year":"2011","journal-title":"J Am Acad Dermatol"},{"key":"2025010818015603600_ski214-bib-0006","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.1111\/j.1365-2133.2008.08507.x","article-title":"Gastrointestinal complications of epidermolysis bullosa in children","volume":"158","author":"Freeman","year":"2008","journal-title":"Br J Dermatol"},{"key":"2025010818015603600_ski214-bib-0007","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1160\/TH08-03-0147","article-title":"Metabolism and cell biology of vitamin K","volume":"100","author":"Shearer","year":"2008","journal-title":"Thromb Haemostasis"},{"key":"2025010818015603600_ski214-bib-0008","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1136\/jclinpath-2019-205997","volume":"73","author":"Card","year":"2020","journal-title":"J Clin Pathol"},{"key":"2025010818015603600_ski214-bib-0009","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1097\/00004630-199801000-00017","article-title":"A prospective analysis of serum vitamin K in severely burned paediatric patients","volume":"19","author":"Jenkins","year":"1998","journal-title":"J Burn Care Rehabil"},{"issue":"1","key":"2025010818015603600_ski214-bib-0010","first-page":"3","article-title":"Vitamin K dependent proteins involved in bone and cardiovascular health","volume":"18","author":"Wen","year":"2018","journal-title":"Mol Med Rep"},{"issue":"4","key":"2025010818015603600_ski214-bib-0011","first-page":"307","article-title":"The production of menaquinones (vitamin K2) by intestinal bacteria and their role in maintaining coagulation homeostasis","volume":"16","author":"Conly","year":"1992","journal-title":"Prog Food Nutr Sci"},{"key":"2025010818015603600_ski214-bib-0012","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1038\/ki.2013.26","article-title":"Vitamin K: key vitamin in controlling vascular calcification in chronic kidney disease","volume":"83","author":"Schurgers","year":"2013","journal-title":"Kidney Int"},{"key":"2025010818015603600_ski214-bib-0013","doi-asserted-by":"crossref","first-page":"1181S","DOI":"10.1093\/jn\/126.suppl_4.1181S","article-title":"Chemistry, nutritional sources, tissue distribution and metabolism of vitamin K with special reference to bone health","volume":"126","author":"Shearer","year":"1996","journal-title":"J Nutr"},{"key":"2025010818015603600_ski214-bib-0014","doi-asserted-by":"crossref","first-page":"463","DOI":"10.3945\/an.113.003855","article-title":"Menaquinones, bacteria, and the food supply: the relevance of dairy and fermented food products to vitamin K requirements","volume":"4","author":"Walther","year":"2013","journal-title":"Adv Nutr"},{"key":"2025010818015603600_ski214-bib-0015","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-017-00612-y","article-title":"Potential role of fecal microbiota from patients with slow transit constipation in the regulation of gastrointestinal motility","volume":"7","author":"Ge","year":"2017","journal-title":"Sci Rep"},{"issue":"2","key":"2025010818015603600_ski214-bib-0016","first-page":"180","article-title":"The causes of intestinal dysbiosis: a review","volume":"9","author":"Hawrelak","year":"2004","journal-title":"Alternative Med Rev"},{"issue":"10","key":"2025010818015603600_ski214-bib-0017","doi-asserted-by":"publisher","first-page":"1122","DOI":"10.1111\/exd.13771","article-title":"Prevalence and pathogenesis of osteopenia and osteoporosis in epidermolysis bullosa: an evidence-based review [published correction appears in Exp Dermatol. 2020 Jun;29(6):575]","volume":"28","author":"Chen","year":"2019","journal-title":"Exp Dermatol"},{"key":"2025010818015603600_ski214-bib-0018","volume-title":"Joint Formulary Committee British National Formulary for Children","year":"2020"},{"key":"2025010818015603600_ski214-bib-0019","volume-title":"Dietary Reference Values for Food, Energy and Nutrients for the UK. Report on health and social subjects. No 41, Department of Health","year":"1991"}],"container-title":["Skin Health and Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ski2.14","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ski2.14","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/academic.oup.com\/skinhd\/article-pdf\/1\/2\/ski2.14\/59728592\/skinhd_1_2_e14.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/skinhd\/article-pdf\/1\/2\/ski2.14\/59728592\/skinhd_1_2_e14.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,1,8]],"date-time":"2025-01-08T18:02:18Z","timestamp":1736359338000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/skinhd\/article\/doi\/10.1002\/ski2.14\/7733932"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,31]]},"references-count":19,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2021,6,1]]}},"URL":"https:\/\/doi.org\/10.1002\/ski2.14","relation":{},"ISSN":["2690-442X"],"issn-type":[{"value":"2690-442X","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2021,6]]},"published":{"date-parts":[[2021,3,31]]},"article-number":"ski2.14"}}