Acquired Diaphragmatic Hernia and Intestinal Obstruction in a Child with Methylmalonic Acidemia Following Pediatric Liver Transplantation

IF 0.8 4区医学 Q4 IMMUNOLOGY

Transplantation proceedings Pub Date : 2025-01-01 DOI:10.1016/j.transproceed.2025.01.001

Huilan Ye , Xingfei Chen , Yongmin Lin , Peidan Hu , Lihua Wen , Yiyu Yang , Nuoheng Liu , Run Dang

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引用次数: 0

Abstract

Background

An acquired diaphragmatic hernia (ADH) is an uncommon event following pediatric liver transplantation. Pediatric liver transplantation proves effective in prolonging survival and improving quality of life for children with methylmalonic acidemia. Remarkably, there have been no previous reports documenting the occurrence of post-liver transplant ADH in patients diagnosed with methylmalonic acidemia.

Methods

We present a case of a child with methylmalonic acidemia who underwent pediatric liver transplantation at the age of 19 months, followed by choledochoenterostomy due to bile leakage. Three months later, during a subsequent computed tomography (CT) scan, a focal protrusion of the right diaphragmatic muscle was observed. Subsequently, a severe intestinal obstruction emerged a year later, which was diagnosed as an ADH.

Results

Following an emergency assessment of the right hemithorax, necrotic bowel resection and repair of the diaphragmatic hernia (DH) were conducted. Consequently, the hernia repair procedure was successful, and the child was discharged on the 18th postoperative day.

Conclusions

The clinical presentation and laboratory tests of ADH resembles metabolic decompensation in methylmalonic acidemia, primarily impacting the gastrointestinal and respiratory systems. It can result in severe complications, including intestinal obstruction, and should be considered a potential late complication.

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来源期刊

Transplantation proceedings 医学-免疫学

CiteScore

1.70

自引率

0.00%

发文量

502

审稿时长

60 days

期刊介绍： Transplantation Proceedings publishes several different categories of manuscripts, all of which undergo extensive peer review by recognized authorities in the field prior to their acceptance for publication. The first type of manuscripts consists of sets of papers providing an in-depth expression of the current state of the art in various rapidly developing components of world transplantation biology and medicine. These manuscripts emanate from congresses of the affiliated transplantation societies, from Symposia sponsored by the Societies, as well as special Conferences and Workshops covering related topics. Transplantation Proceedings also publishes several special sections including publication of Clinical Transplantation Proceedings, being rapid original contributions of preclinical and clinical experiences. These manuscripts undergo review by members of the Editorial Board. Original basic or clinical science articles, clinical trials and case studies can be submitted to the journal?s open access companion title Transplantation Reports.