Changes in branched-chain amino acids in an infant with maple syrup urine disease during perioperative pediatric liver transplant: A case report

被引：0

作者：

Saeyup, Pipat ^{[1
]}

Tubjaroen, Chomchanat ^{[2
,3
,6
]}

Kamolvisit, Wuttichart ^{[4
,5
]}

Chongsrisawat, Voranush ^{[2
,3
]}

Thaveepunsan, Winnie ^{[1
]}

机构：

[1] Chulalongkorn Univ, King Chulalongkorn Mem Hosp, Fac Med, Dept Anesthesiol, Bangkok, Thailand

[2] Chulalongkorn Univ, King Chulalongkorn Mem Hosp, Fac Med, Dept Pediat,Div Gastroenterol & Hepatol, Bangkok, Thailand

[3] King Chulalongkorn Mem Hosp, Excellence Ctr Organ Transplantat, Bangkok, Thailand

[4] Chulalongkorn Univ, Fac Med, Ctr Excellence Med Genom Med Genom Cluster, Dept Pediat, Bangkok, Thailand

[5] King Chulalongkorn Mem Hosp, Excellence Ctr Genom & Precis Med, Thai Red Cross Soc, Bangkok, Thailand

[6] Chulalongkorn Univ, King Chulalongkorn Mem Hosp, Fac Med, 1873 Rama 4 Rd, Bangkok 10330, Thailand

来源：

PEDIATRIC ANESTHESIA | 2024年 / 34卷 / 04期

关键词：

liver transplantation; maple syrup urine disease; metabolic crisis; ANESTHETIC MANAGEMENT;

D O I：

10.1111/pan.14842

中图分类号：

R614 [麻醉学];

学科分类号：

100217 ;

摘要：

An 11-month-old female infant diagnosed with classic subtype IB maple syrup urine disease underwent living donor liver transplantation. Blood samples for plasma amino acid analysis were collected during the three phases of the operation. Despite the perioperative prophylactic administration of 12.5% hypertonic dextrose solution with insulin and a 20% intralipid emulsion, the blood levels of the branched-chain amino acids increased dramatically during surgery, consistent with an acute intraoperative metabolic decompensation. However, these blood levels normalized soon after liver transplantation with an excellent outcome. We suggest that the occurrence of an intraoperative metabolic crisis during liver transplantation is not necessarily a sign of graft failure.

引用

页码：366 / 370

页数：5

共 50 条

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