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联合肝胰脏移植术治疗囊性纤维化患者:病例报告。

Combined Liver-Pancreas Transplantation as Novel Treatment for Patient With Cystic Fibrosis: A Case Report.

机构信息

Department of General and Transplant Surgery, Medical University of Warsaw, Warsaw, Poland; Faculty of Medicine, Medical University of Warsaw, Warsaw, Poland.

Department of General and Transplant Surgery, Medical University of Warsaw, Warsaw, Poland.

出版信息

Transplant Proc. 2024 May;56(4):1013-1017. doi: 10.1016/j.transproceed.2024.03.034. Epub 2024 May 14.

DOI:10.1016/j.transproceed.2024.03.034
PMID:38749862
Abstract

BACKGROUND

A 21-year-old woman diagnosed with cystic fibrosis developed cirrhosis, exocrine pancreatic insufficiency, and insulin-dependent diabetes mellitus. The patient qualified for double organ liver-pancreas transplantation beyond typical indications. The respiratory symptoms of cystic fibrosis were moderate and well-treated. The patient was endangered mainly by liver insufficiency and recurrent hypoglycemia, which was due to the treatment of diabetes with high doses of insulin. Computed tomography showed mild bronchiectasis, cirrhotic liver, splenomegaly, and atrophy of the pancreas. Pseudomonas aeruginosa colonized the upper respiratory tract. Gastrointestinal complications were sufficient for the patient to be qualified for combined liver-pancreas transplantation.

METHODS

First, a standard hepatectomy was performed. The liver was transplanted orthotopically. Subsequently, the team performed pancreas transplantation through a separate incision. The donor's duodenum was anastomosed to the recipient's jejunum, close to the ligament of Treitz.

RESULTS

No serious complications were noted during the postoperative period. Transplanted organs started functioning without delay. The patient was discharged after 6 weeks in general good condition. Twenty months later, the patient felt well, and the grafts kept functioning properly.

CONCLUSION

Combined liver-pancreas transplantation in patients with CF restores exocrine and endocrine pancreatic function and minimizes the risk of life-threatening complications associated with liver insufficiency. Improvement of life quality coincides with the possibility of discontinuing insulin and pancreatic enzyme supplementation. The combination of liver and pancreas transplantation may prevent advanced pulmonary complications, extend the prognosis of survival, and improve the long-term life quality.

摘要

背景

一名 21 岁女性患有囊性纤维化,发展为肝硬化、外分泌胰腺功能不全和胰岛素依赖型糖尿病。该患者符合超出典型适应证的双器官肝胰脏联合移植条件。囊性纤维化的呼吸症状为中度,且治疗效果良好。该患者主要受到肝功能不全和反复低血糖的威胁,这是由于糖尿病的胰岛素高剂量治疗所致。计算机断层扫描显示轻度支气管扩张、肝硬化、脾肿大和胰腺萎缩。上呼吸道定植铜绿假单胞菌。胃肠道并发症足以使患者符合联合肝胰脏移植的条件。

方法

首先进行标准的肝切除术。将肝脏原位移植。随后,团队通过单独的切口进行胰腺移植。供体的十二指肠与受体的空肠吻合,靠近Treitz 韧带。

结果

术后期间未出现严重并发症。移植器官立即开始发挥功能。患者在一般情况良好的情况下出院,共住院 6 周。20 个月后,患者感觉良好,移植物功能正常。

结论

CF 患者的肝胰脏联合移植可恢复外分泌和内分泌胰腺功能,并最大程度降低与肝功能不全相关的危及生命的并发症风险。生活质量的改善与停止胰岛素和胰腺酶补充的可能性相吻合。肝和胰腺联合移植可能预防晚期肺部并发症,延长生存预后,并改善长期生活质量。

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