ArticleJournal of orthopaedic case reports
Total Hip Arthroplasty in A Young Patient with Bernard-Soulier Syndrome.
Article in Journal of orthopaedic case reports. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
2 citing papers in PubMed, 3 citations in OpenAlex.
- Article
- Utilization of paravertebral nerve blocks as part of a multimodal analgesic regimen in a patient with Bernard-Soulier syndrome undergoing a Nuss procedure.Romanian journal of anaesthesia and intensive care · 2016Article
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Authors and funding
2 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionThe management of patients with coagulopathic disorders undergoing orthopaedic surgery requires a dedicated, multi-disciplinary team with detailed perioperative planning. Bernard-Soulier Syndrome (BSS) is an extremely rare disorder, affecting 1 in 1 million individuals worldwide. It is caused by a deficiency in glycoprotein 1b-V-IX which is required for normal platelet-mediated clot formation. The deficiency results in prolonged bleeding time with high risk of spontaneous bleeds. Few reports exist in the clinical literature of BSS patients undergoing major surgery. CASE REPORT: A 40 year old, female with known BSS and developmental dysplasia of her left hip (DDH) was referred to us for consideration of left total hip arthroplasty (THA). Consultation with her Haematologist for pre-operative optimization of platelets and related clotting times together with detailed discussions of her intended anaesthesia protocol and surgery resulted in a successful operation with less than anticipated blood loss. She entered our rehabilitation program just one week after surgery.
conclusionBSS is an extremely rare bleeding disorder that puts patients at very high risk of blood loss following surgery. This is the first report that we are aware of describing a BSS patient undergoing a THA. A cohesive, highly specialized, multi-disciplinary team is crucial to the success of these patients.
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