一名因 ABCA3 基因突变导致肺间质纤维化的五岁儿童成功接受了双侧肺移植手术

IF 1.6 4区医学 Q4 IMMUNOLOGY

Transplant immunology Pub Date : 2024-05-24 DOI:10.1016/j.trim.2024.102056

Feifan Ge , Jialong Liang , Jintao Zhou , Yuan Chen, Jingyu Chen, Shugao Ye

{"title":"一名因 ABCA3 基因突变导致肺间质纤维化的五岁儿童成功接受了双侧肺移植手术","authors":"Feifan Ge , Jialong Liang , Jintao Zhou , Yuan Chen, Jingyu Chen, Shugao Ye","doi":"10.1016/j.trim.2024.102056","DOIUrl":null,"url":null,"abstract":"<div><p>The ATP-binding cassette subfamily A member 3 (ABCA3) protein plays a fundamental role in surfactant homeostasis. Most children with ABCA3 gene mutations develop pulmonary interstitial fibrosis leading to the development of interstitial lung disease. Since traditional medicine does not offer effective therapy, the best option is lung transplantations, especially bilateral lung transplantations. We are reporting the case of a successful bilateral lung transplantation in a five-year-old child with pulmonary interstitial fibrosis caused by ABCA3 gene mutations. This successful transplantation enabled the patient to get rid of chronic cough and tachypnea.</p></div>","PeriodicalId":23304,"journal":{"name":"Transplant immunology","volume":"85 ","pages":"Article 102056"},"PeriodicalIF":1.6000,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Successful bilateral lung transplantation in a five-year-old child with pulmonary interstitial fibrosis caused by an ABCA3 gene mutation\",\"authors\":\"Feifan Ge , Jialong Liang , Jintao Zhou , Yuan Chen, Jingyu Chen, Shugao Ye\",\"doi\":\"10.1016/j.trim.2024.102056\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p>The ATP-binding cassette subfamily A member 3 (ABCA3) protein plays a fundamental role in surfactant homeostasis. Most children with ABCA3 gene mutations develop pulmonary interstitial fibrosis leading to the development of interstitial lung disease. Since traditional medicine does not offer effective therapy, the best option is lung transplantations, especially bilateral lung transplantations. We are reporting the case of a successful bilateral lung transplantation in a five-year-old child with pulmonary interstitial fibrosis caused by ABCA3 gene mutations. This successful transplantation enabled the patient to get rid of chronic cough and tachypnea.</p></div>\",\"PeriodicalId\":23304,\"journal\":{\"name\":\"Transplant immunology\",\"volume\":\"85 \",\"pages\":\"Article 102056\"},\"PeriodicalIF\":1.6000,\"publicationDate\":\"2024-05-24\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Transplant immunology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S0966327424000728\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"IMMUNOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Transplant immunology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0966327424000728","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"IMMUNOLOGY","Score":null,"Total":0}

引用次数: 0

摘要

ATP结合盒A亚家族成员3（ABCA3）蛋白在表面活性物质平衡中发挥着重要作用。大多数 ABCA3 基因突变的儿童都会出现肺间质纤维化，导致间质性肺病。由于传统医学无法提供有效的治疗，最好的选择是肺移植，尤其是双侧肺移植。我们报告了一例成功进行双肺移植的病例，患者是一名五岁儿童，因ABCA3基因突变导致肺间质纤维化。这次成功的移植使患者摆脱了慢性咳嗽和呼吸急促。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

Successful bilateral lung transplantation in a five-year-old child with pulmonary interstitial fibrosis caused by an ABCA3 gene mutation

The ATP-binding cassette subfamily A member 3 (ABCA3) protein plays a fundamental role in surfactant homeostasis. Most children with ABCA3 gene mutations develop pulmonary interstitial fibrosis leading to the development of interstitial lung disease. Since traditional medicine does not offer effective therapy, the best option is lung transplantations, especially bilateral lung transplantations. We are reporting the case of a successful bilateral lung transplantation in a five-year-old child with pulmonary interstitial fibrosis caused by ABCA3 gene mutations. This successful transplantation enabled the patient to get rid of chronic cough and tachypnea.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

Transplant immunology 医学-免疫学

CiteScore

2.10

自引率

13.30%

发文量

198

审稿时长

48 days

期刊介绍： Transplant Immunology will publish up-to-date information on all aspects of the broad field it encompasses. The journal will be directed at (basic) scientists, tissue typers, transplant physicians and surgeons, and research and data on all immunological aspects of organ-, tissue- and (haematopoietic) stem cell transplantation are of potential interest to the readers of Transplant Immunology. Original papers, Review articles and Hypotheses will be considered for publication and submitted manuscripts will be rapidly peer-reviewed and published. They will be judged on the basis of scientific merit, originality, timeliness and quality.