Isolated intestinal transplantation for megacystis microcolon intestinal hypoperistalsis syndrome: Case report

被引：7

作者：

Huang, Chen-Ming ^{[1
]}

Tseng, Sheng-Hong ^{[2
,3
]}

Weng, Chia-Chee ^{[1
]}

Chen, Yun ^{[1
,4
,5
]}

机构：

[1] Far Eastern Mem Hosp, Div Pediat Surg, Dept Surg, New Taipei 220, Taiwan

[2] Natl Taiwan Univ Hosp, Dept Surg, Taipei 100, Taiwan

[3] Natl Taiwan Univ, Coll Med, Taipei 10764, Taiwan

[4] Yuan Ze Univ, Dept Chem Engn & Mat Sci, Tao Yuan, Taiwan

[5] Natl Yang Ming Univ, Sch Med, Dept Surg, Taipei 112, Taiwan

来源：

PEDIATRIC TRANSPLANTATION | 2013年 / 17卷 / 01期

关键词：

isolated intestinal transplantation; megacystis microcolon intestinal hypoperistalsis syndrome; multivisceral transplantation; MULTIVISCERAL TRANSPLANTATION; BOWEL TRANSPLANTATION; CHILDREN; LIVER; NUTRITION;

D O I：

10.1111/petr.12019

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

MMIHS is a rare congenital disease. It is characterized by distended urinary bladder, small colon and intestinal hypoperistalsis, or aperistalsis with normal morphology. There is no specific treatment for MMIHS, and most patients have to be maintained by TPN, which frequently causes TPN-related liver failure, loss of venous access, or catheter-related sepsis. The prognosis of patients with MMIHS is poor, and most patients die early. Multivisceral transplantation including stomach, duodenum, intestine, and liver has been used for the treatment of patients with MMIHS because these patients often have liver failure. We report an eight-yr-old patient with MMIHS who was treated with isolated intestinal transplantation. She had completely oral intake during the fouryr of follow-up. The experience in this case suggests isolated intestinal transplantation may be indicated in selected cases with MMIHS.

引用

页码：E4 / E8

页数：5

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