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Glanzmann's thrombasthenia: A nightmare for hernia surgeons.

作者信息

Gandhi Jignesh, Gajjar Aarsh, Shinde Pravin, Takalkar Yogesh

机构信息

Department of General Surgery, Seth GSMC and KEM Hospital, Mumbai, Maharashtra, India.

Intern Doctor, Seth GSMC and KEM Hospital, Mumbai, Maharashtra, India.

出版信息

J Minim Access Surg. 2024 Jul 30. doi: 10.4103/jmas.jmas_230_23.

DOI:10.4103/jmas.jmas_230_23
PMID:39095966
Abstract

Glanzmann's thrombasthenia is a rare inherited disorder affecting one in one million. It is characterised by a lack of platelet aggregation due to a defect in the platelet membrane receptor complex (αIIb/βIIIa), which mediates the aggregation of platelets at the site of vessel injury. We report here the first case of successful perioperative haemostatic management of a male patient with Glanzmann's thrombasthenia, who underwent an elective laparoscopic hernia repair. The patient was posted for elective surgery considering the availability of expertise in minimally invasive surgery and accessibility to recombinant activated factor VII. The patient was operated using the extended-view totally extraperitoneal technique for inguinal hernia repair. The patient recovered successfully and reported no complication during follow-up. We conclude that with proper perioperative haematological consultation, and careful coordination between anaesthetists and surgeons, elective laparoscopic procedure can be done in patients with Glanzmann's thrombasthenia, with only recombinant factor VIIa support.

摘要

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