患有半乳糖血症的新生儿先天性心脏病手术的麻醉管理。
Anaesthetic management of a newborn with galactosaemia for congenital heart surgery.
作者信息
Choudhury Arindam, Das Sambhunath, Kiran Usha
机构信息
Senior resident, Department of Cardiac Anaesthesiology, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi 110029.
出版信息
Indian J Anaesth. 2009 Apr;53(2):219-22.
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.
半乳糖血症是一种常染色体隐性代谢紊乱疾病，因1-磷酸半乳糖尿苷转移酶缺乏所致，会导致肝脏、眼部、神经和肾脏功能紊乱。患有半乳糖血症的新生儿合并青紫型先天性心脏病是一种罕见情况，在心脏直视手术期间，这也是麻醉医生极为关注的问题。在本病例报告中，我们讨论了一名患有半乳糖血症的新生儿在接受大动脉转位（TGA）矫正手术围手术期的管理情况。
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