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. 2009 Apr;53(2):219-22.

Anaesthetic management of a newborn with galactosaemia for congenital heart surgery

Arindam Choudhury  1 , Sambhunath Das, Usha Kiran
Affiliations

Anaesthetic management of a newborn with galactosaemia for congenital heart surgery

Arindam Choudhury et al. Indian J Anaesth. 2009 Apr.

Abstract

Galactosaemia is an autosomal recessive metabolic disorder that occurs due to galactose-1-phosphate uridyl transferase enzyme deficiency that leads to hepatic, ophthalmic, neural and renal derangements. Association of cyanotic congenital heart disease with galactosaemia is a rare occurence and a matter of great concern for the anaesthesiologist during open heart surgery. In this case report, the perioperative management of a newborn with galactosaemia operated for correction of transposition of great arteries (TGA) is discussed.

Keywords: Anaesthetic management; Congenital heart surgery; Galactosaemia; Newborn; Transposition of Great arteries.

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