Jesús Quintero Bernabeu, Javier Juamperez Goñi, Maria Mercadal Hally, Cristina Padrós Fornieles, Juan Ortega López, Mauricio Larrarte King, José A Molino Gahete, María T Salcedo Allende, Ernest Hidalgo Llompart, Itxarone Bilbao Aguirre, Ramon Charco Torra
{"title":"西罗莫司治疗小儿肝移植中的慢性和类固醇耐药的异体移植排斥相关纤维化。","authors":"Jesús Quintero Bernabeu, Javier Juamperez Goñi, Maria Mercadal Hally, Cristina Padrós Fornieles, Juan Ortega López, Mauricio Larrarte King, José A Molino Gahete, María T Salcedo Allende, Ernest Hidalgo Llompart, Itxarone Bilbao Aguirre, Ramon Charco Torra","doi":"10.1002/jpn3.12286","DOIUrl":null,"url":null,"abstract":"<p><p>This study aimed to report our experience with the use of Sirolimus (SRL) in pediatric liver transplant patients with chronic rejection or steroid-resistant rejection with hepatic fibrosis, focusing on their histological evolution. All pediatric liver transplant recipients who received off-label treatment with SRL for chronic ductopenic rejection or cortico-resistant rejection between July 2003 and July 2022 were included in the study. All nine patients included in the study showed improvement in liver enzymes and cholestasis parameters as soon as 1-month after post-SRL introduction. A decrease in fibrosis stage was observed in 7/9 (77.7%) patients at 36 months. All but one patient experienced an improvement in the Rejection Activity Index and ductopenia at 12 months. A single patient had to discontinue SRL treatment owing to nephrotic proteinuria. In conclusion, SRL may be a safe and effective treatment for chronic and steroid-resistant rejection and may improve allograft rejection-related fibrosis and ductal damage.</p>","PeriodicalId":16694,"journal":{"name":"Journal of Pediatric Gastroenterology and Nutrition","volume":" ","pages":"962-968"},"PeriodicalIF":2.4000,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Sirolimus to treat chronic and steroid-resistant allograft rejection-related fibrosis in pediatric liver transplantation.\",\"authors\":\"Jesús Quintero Bernabeu, Javier Juamperez Goñi, Maria Mercadal Hally, Cristina Padrós Fornieles, Juan Ortega López, Mauricio Larrarte King, José A Molino Gahete, María T Salcedo Allende, Ernest Hidalgo Llompart, Itxarone Bilbao Aguirre, Ramon Charco Torra\",\"doi\":\"10.1002/jpn3.12286\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>This study aimed to report our experience with the use of Sirolimus (SRL) in pediatric liver transplant patients with chronic rejection or steroid-resistant rejection with hepatic fibrosis, focusing on their histological evolution. All pediatric liver transplant recipients who received off-label treatment with SRL for chronic ductopenic rejection or cortico-resistant rejection between July 2003 and July 2022 were included in the study. All nine patients included in the study showed improvement in liver enzymes and cholestasis parameters as soon as 1-month after post-SRL introduction. A decrease in fibrosis stage was observed in 7/9 (77.7%) patients at 36 months. All but one patient experienced an improvement in the Rejection Activity Index and ductopenia at 12 months. A single patient had to discontinue SRL treatment owing to nephrotic proteinuria. In conclusion, SRL may be a safe and effective treatment for chronic and steroid-resistant rejection and may improve allograft rejection-related fibrosis and ductal damage.</p>\",\"PeriodicalId\":16694,\"journal\":{\"name\":\"Journal of Pediatric Gastroenterology and Nutrition\",\"volume\":\" \",\"pages\":\"962-968\"},\"PeriodicalIF\":2.4000,\"publicationDate\":\"2024-11-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Pediatric Gastroenterology and Nutrition\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1002/jpn3.12286\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/7/8 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q3\",\"JCRName\":\"GASTROENTEROLOGY & HEPATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Pediatric Gastroenterology and Nutrition","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1002/jpn3.12286","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/7/8 0:00:00","PubModel":"Epub","JCR":"Q3","JCRName":"GASTROENTEROLOGY & HEPATOLOGY","Score":null,"Total":0}
Sirolimus to treat chronic and steroid-resistant allograft rejection-related fibrosis in pediatric liver transplantation.
This study aimed to report our experience with the use of Sirolimus (SRL) in pediatric liver transplant patients with chronic rejection or steroid-resistant rejection with hepatic fibrosis, focusing on their histological evolution. All pediatric liver transplant recipients who received off-label treatment with SRL for chronic ductopenic rejection or cortico-resistant rejection between July 2003 and July 2022 were included in the study. All nine patients included in the study showed improvement in liver enzymes and cholestasis parameters as soon as 1-month after post-SRL introduction. A decrease in fibrosis stage was observed in 7/9 (77.7%) patients at 36 months. All but one patient experienced an improvement in the Rejection Activity Index and ductopenia at 12 months. A single patient had to discontinue SRL treatment owing to nephrotic proteinuria. In conclusion, SRL may be a safe and effective treatment for chronic and steroid-resistant rejection and may improve allograft rejection-related fibrosis and ductal damage.
期刊介绍:
The Journal of Pediatric Gastroenterology and Nutrition (JPGN) provides a forum for original papers and reviews dealing with pediatric gastroenterology and nutrition, including normal and abnormal functions of the alimentary tract and its associated organs, including the salivary glands, pancreas, gallbladder, and liver. Particular emphasis is on development and its relation to infant and childhood nutrition.