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CASE REPORT
Management of haemostasis during dental extraction in a Bernard-Soulier syndrome child
  1. Supriya Sharma,
  2. Rakesh Kumar Chak and
  3. Richa Khanna
  1. Department of Paediatric and Preventive Dentistry, King George’s Medical University, Lucknow, Uttar Pradesh, India
  1. Correspondence to Dr Supriya Sharma, drsupriyasharma26{at}gmail.com

Abstract

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.

  • dentistry and oral medicine
  • congenital disorders
  • haematology (incl blood transfusion)
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Footnotes

  • Contributors RKC and RK allotted the patient, of a rare disease after diagnosis to SS. The treatment procedure was performed by SS under the guidance of RKC and RK.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Parental/guardian consent obtained.

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