Document Type

Report

Department

Anaesthesia

Abstract

Homocystinuria is a rare autosomal recessive genetic disease. It is caused by a deficiency in cystathionine-b-synthase leading to a defect in methionine metabolism. High levels of plasma homocysteine are associated with vascular injury via mechanisms of oxidative damage, vascular smooth muscle proliferation, promotion of platelet activation and aggregation,and disruption of normal procoagulant-anticoagulant balance favouring thrombosis. This is a case of 8 years old boy, a known case of homocystinuria, who was scheduled for bilateral lensectomy and intraocular lens placement. The major anaesthetic considerations include the development of thromboembolism, need to avoid nitrous oxide in balanced anaesthesia regimen and hypoglycaemia.

Publication

Journal of the College of Physicians and Surgeons Pakistan

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