{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,20]],"date-time":"2025-10-20T10:29:33Z","timestamp":1760956173511,"version":"3.44.0"},"reference-count":14,"publisher":"BMJ","issue":"7","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2019,5,30]]},"published-print":{"date-parts":[[2019,7]]},"abstract":"<jats:p>The Bernard-Soulier syndrome also known as hemorrhagioparous thrombocytic dystrophy, is a rare autosomal recessive bleeding disorder. Macrothrombocytopenia and increased bleeding time are the two main features of this syndrome. There is a reduced adherence of giant platelets to the subendothelium due to a deficiency of glycoprotein GPIb-V-IX complex. Routine dental extraction in a patient with a bleeding disorder can lead to life-threatening complications and there are very few specific protocols for the same. Here, we present a case report describing haemostasis management during dental extraction in a 7-year-old female child, suffering from this syndrome. It was observed that the preoperative and postoperative human leucocyte antigen-matched platelet transfusion, systemic intraoperative tranexamic acid and minimally traumatic procedure improved bleeding control and management.<\/jats:p>","DOI":"10.1136\/bcr-2018-229082","type":"journal-article","created":{"date-parts":[[2023,3,14]],"date-time":"2023-03-14T00:10:54Z","timestamp":1678752654000},"page":"e229082","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":8,"title":["Management of haemostasis during dental extraction in a Bernard-Soulier syndrome child"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1972-4282","authenticated-orcid":false,"given":"Supriya","family":"Sharma","sequence":"first","affiliation":[{"name":"Department of Paediatric and Preventive Dentistry, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rakesh Kumar","family":"Chak","sequence":"additional","affiliation":[{"name":"Department of Paediatric and Preventive Dentistry, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richa","family":"Khanna","sequence":"additional","affiliation":[{"name":"Department of Paediatric and Preventive Dentistry, King George\u2019s Medical University, Lucknow, Uttar Pradesh, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2019,7,8]]},"reference":[{"key":"2025082802201709000_12.7.e229082.1","first-page":"3217","article-title":"Sur une nouvelle vari\u00e9t\u00e9 de dystrophie thrombocytaire-h\u00e9morragipare cong\u00e9nitale","volume":"24","author":"Bernard","year":"1948","journal-title":"Semin Hop Paris"},{"key":"2025082802201709000_12.7.e229082.2","first-page":"860","article-title":"Inherited thrombocytopenias: from genes to therapy","volume":"87","author":"Balduini","year":"2002","journal-title":"Haematologica"},{"key":"2025082802201709000_12.7.e229082.3","doi-asserted-by":"crossref","first-page":"4397","DOI":"10.1182\/blood.V91.12.4397","article-title":"Bernard-Soulier syndrome","volume":"91","author":"L\u00f3pez","year":"1998","journal-title":"Blood"},{"key":"2025082802201709000_12.7.e229082.4","doi-asserted-by":"crossref","DOI":"10.1186\/1750-1172-1-46","article-title":"hemorrhagiparous thrombocytic dystrophy)","volume":"1","author":"Lanza","year":"2006","journal-title":"Orphanet journal of rare diseases"},{"key":"2025082802201709000_12.7.e229082.5","unstructured":"Guyton AC , Hall JE . 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