表现为严重贫血的自身免疫性肝炎。

JPGN reports Pub Date : 2024-04-29 eCollection Date: 2024-08-01 DOI:10.1002/jpr3.12076
Brandon J Calley, Alexandra Polovneff, Kathryn Henry, Paula North, David C Moe, Cara L Mack
{"title":"表现为严重贫血的自身免疫性肝炎。","authors":"Brandon J Calley, Alexandra Polovneff, Kathryn Henry, Paula North, David C Moe, Cara L Mack","doi":"10.1002/jpr3.12076","DOIUrl":null,"url":null,"abstract":"<p><p>Autoimmune hepatitis (AIH) is relatively rare in children. Herein, our case demonstrates a unique presentation of AIH in a previously healthy 18-year-old female presenting with a mild cough, fatigue, and severe anemia (hemoglobin 2.9 g/dL). Initial evaluation revealed jaundice and scleral icterus, prompting transfer of care and further testing, which demonstrated severe microcytic anemia, pancytopenia, elevated liver enzymes, direct hyperbilirubinemia, and marked splenomegaly. Concern for autoimmune hemolytic anemia resulted in a delayed diagnosis. The combination of triple antibody positivity (anti-nuclear antibodies, anti-actin, and anti-liver-kidney microsomal-1) and liver histology findings confirmed the diagnosis of AIH. Intravenous methylprednisolone was initiated to induce remission. Due to pancytopenia and persistently elevated international normalized ratio, tacrolimus was chosen as the maintenance immunosuppression instead of azathioprine. This case highlights several significant considerations for clinicians, including the importance of a timely clinicopathologic diagnosis, the severe anemia presentation secondary to hypersplenism, and the rare finding of triple autoantibody-positive AIH.</p>","PeriodicalId":501015,"journal":{"name":"JPGN reports","volume":"5 3","pages":"402-406"},"PeriodicalIF":0.0000,"publicationDate":"2024-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11322014/pdf/","citationCount":"0","resultStr":"{\"title\":\"Autoimmune hepatitis presenting as severe anemia.\",\"authors\":\"Brandon J Calley, Alexandra Polovneff, Kathryn Henry, Paula North, David C Moe, Cara L Mack\",\"doi\":\"10.1002/jpr3.12076\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Autoimmune hepatitis (AIH) is relatively rare in children. Herein, our case demonstrates a unique presentation of AIH in a previously healthy 18-year-old female presenting with a mild cough, fatigue, and severe anemia (hemoglobin 2.9 g/dL). Initial evaluation revealed jaundice and scleral icterus, prompting transfer of care and further testing, which demonstrated severe microcytic anemia, pancytopenia, elevated liver enzymes, direct hyperbilirubinemia, and marked splenomegaly. Concern for autoimmune hemolytic anemia resulted in a delayed diagnosis. The combination of triple antibody positivity (anti-nuclear antibodies, anti-actin, and anti-liver-kidney microsomal-1) and liver histology findings confirmed the diagnosis of AIH. Intravenous methylprednisolone was initiated to induce remission. Due to pancytopenia and persistently elevated international normalized ratio, tacrolimus was chosen as the maintenance immunosuppression instead of azathioprine. This case highlights several significant considerations for clinicians, including the importance of a timely clinicopathologic diagnosis, the severe anemia presentation secondary to hypersplenism, and the rare finding of triple autoantibody-positive AIH.</p>\",\"PeriodicalId\":501015,\"journal\":{\"name\":\"JPGN reports\",\"volume\":\"5 3\",\"pages\":\"402-406\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-04-29\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11322014/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"JPGN reports\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1002/jpr3.12076\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/8/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"JPGN reports","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1002/jpr3.12076","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/8/1 0:00:00","PubModel":"eCollection","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0

摘要

自身免疫性肝炎(AIH)在儿童中相对罕见。在本病例中,一名 18 岁的健康女性因轻微咳嗽、乏力和严重贫血(血红蛋白 2.9 g/dL)而患上了自身免疫性肝炎。初步评估发现该患者出现黄疸和巩膜黄疸,于是转院并接受进一步检查,结果显示该患者存在严重的小细胞性贫血、泛血细胞减少、肝酶升高、直接高胆红素血症和明显的脾脏肿大。由于担心是自身免疫性溶血性贫血,诊断被推迟。三重抗体阳性(抗核抗体、抗肌动蛋白、抗肝肾微粒体-1)和肝脏组织学检查结果共同证实了 AIH 的诊断。患者开始静脉注射甲基强的松龙以诱导病情缓解。由于全血细胞减少和国际正常化比率持续升高,患者选择他克莫司代替硫唑嘌呤作为维持性免疫抑制剂。本病例强调了临床医生需要考虑的几个重要因素,包括及时进行临床病理诊断的重要性、继发于脾功能亢进的严重贫血表现以及罕见的三联自身抗体阳性 AIH。
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
分享 分享
微信好友 朋友圈 QQ好友 复制链接
本刊更多论文
Autoimmune hepatitis presenting as severe anemia.

Autoimmune hepatitis (AIH) is relatively rare in children. Herein, our case demonstrates a unique presentation of AIH in a previously healthy 18-year-old female presenting with a mild cough, fatigue, and severe anemia (hemoglobin 2.9 g/dL). Initial evaluation revealed jaundice and scleral icterus, prompting transfer of care and further testing, which demonstrated severe microcytic anemia, pancytopenia, elevated liver enzymes, direct hyperbilirubinemia, and marked splenomegaly. Concern for autoimmune hemolytic anemia resulted in a delayed diagnosis. The combination of triple antibody positivity (anti-nuclear antibodies, anti-actin, and anti-liver-kidney microsomal-1) and liver histology findings confirmed the diagnosis of AIH. Intravenous methylprednisolone was initiated to induce remission. Due to pancytopenia and persistently elevated international normalized ratio, tacrolimus was chosen as the maintenance immunosuppression instead of azathioprine. This case highlights several significant considerations for clinicians, including the importance of a timely clinicopathologic diagnosis, the severe anemia presentation secondary to hypersplenism, and the rare finding of triple autoantibody-positive AIH.

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
自引率
0.00%
发文量
0
期刊最新文献
Long‐term implications of a multidisciplinary tube‐weaning program: Parental perspectives The first paediatric living donor auxiliary orthotopic liver transplant for dengue acute liver failure Anterior abdominal wall defects and their management outcomes in Tikur Anbessa Specialized Hospital, neonatal intensive care unit Eosinophilic myenteric ganglionitis: A case in a 14‐year‐old‐male Thirty‐two‐month‐old with multiple duodenal webs diagnosed after failed gastrojejunostomy exchange successfully treated with combination endoscopic therapy
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
现在去查看 取消
×
提示
确定
0
微信
客服QQ
Book学术公众号 扫码关注我们
反馈
×
意见反馈
请填写您的意见或建议
请填写您的手机或邮箱
已复制链接
已复制链接
快去分享给好友吧!
我知道了
×
扫码分享
扫码分享
Book学术官方微信
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术
文献互助 智能选刊 最新文献 互助须知 联系我们:info@booksci.cn
Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。
Copyright © 2023 Book学术 All rights reserved.
ghs 京公网安备 11010802042870号 京ICP备2023020795号-1