Bernard–Soulier syndrome and anesthetic management

Bernard–Soulier syndrome and anesthetic management

Bernard–Soulier syndrome (BSS) is a rare autosomal recessive disease with bleeding diathesis due to platelet dysfunction secondary to the absence of the von Willebrand factor receptor on the platelet surface called glycoprotein Ib–V–IX. The deficiency results in prolonged bleeding time and spontaneo...

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Veröffentlicht in:MRIMS journal of health sciences 2020-10, Vol.8 (4), p.96-98
Hauptverfasser: Ramsali, Manjula, Hadigal, Raj, Koshy, P, Senthil1, Sirisha, Surender, P, Devi, V
Format: Artikel
Sprache:eng
Schlagworte:
anesthesia
bernard–soulier syndrome
bleeding disorder
Blood coagulation factors
Enflurane
platelet transfusion
surgery
Tranexamic acid
Von Willebrand factor
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Zusammenfassung:Bernard–Soulier syndrome (BSS) is a rare autosomal recessive disease with bleeding diathesis due to platelet dysfunction secondary to the absence of the von Willebrand factor receptor on the platelet surface called glycoprotein Ib–V–IX. The deficiency results in prolonged bleeding time and spontaneous bleeds and may be with high risk for anesthesia and surgery. Here, we report a case of BSS coming for trabeculectomy.
ISSN:2321-7006
2321-7294
DOI:10.4103/mjhs.mjhs_16_20