吡咯生物碱相关肝窦阻塞综合征 9 例报告并文献复习
Journal: Basic Medical Theory Research DOI: 10.32629/bmtr.v8i3.20481
Abstract
目的：本文旨在分析由吡咯生物碱（pyrrolizidine alkaloids,PAs）诱发的肝窦阻塞综合征（Hepatic sinusoidal obstruction syndrome,HSOS），围绕其临床特征、干预措施及预后相关因子展开研究，进而深化对该疾病临床诊治的认识。方法：对9例肝窦阻塞综合征患者的临床资料进行回顾性研究，系统阐述其致病相关因素、临床表征、实验室指标、影像学征象、治疗策略及预后转归。结果：9例患者均存在含吡咯生物碱类中草药用药史，主诉症状涵盖腹部胀痛、乏力、水肿等临床表现；多数患者实验室检查为肝功能异常、凝血时间延长、血小板数量下降等，肝脏彩超、CT或MRI提示肝脏增大、腹水，CT、MRI增强见肝脏强化不均匀，肝实质呈花斑状、地图样强化，肝静脉及门静脉显示不清；9例患者均给予护肝、利尿、补充白蛋白治疗，2例未使用抗生素，1例未行抗凝治疗，1例未行抑酸治疗，8例行腹腔穿刺术，治疗后4例患者好转出院，3例回当地医院继续治疗，2例转上级医院治疗。结论：吡咯生物碱相关肝窦综合征引起特殊类型的药物性肝损伤罕见，诱因多为服用如土三七类吡咯生物碱，其特征性临床表现与影像学检查结果，在诊断与鉴别诊断中发挥着重要作用。临床医生要加深对该病的认识，避免漏诊、误诊，治疗上早期及时干预能有效改善预后。
Keywords
肝窦阻塞综合征；吡咯生物碱；影像学检查
Full Text
PDF - Viewed/Downloaded: 0 TimesReferences
[1] Zhu C, Ren X, Liu D, et al. Oxaliplatin-induced hepatic sinusoidal obstruction syndrome[J]. Toxicology, 2021, 460:152882.
[2] 刘少松,周晓东.吡咯烷生物碱诱导肝窦阻塞综合征的研究进展[J].临床内科杂志,2020,37(04):256-259.
[3] 邓泽平,杨茜,覃凤燕,等.一点红致肝窦阻塞综合征1例并文献复习[J].肝脏,2021,26(08):947-949.
[4] 郭严,张世荣,文良志,等.我国吡咯烷生物碱致肝窦阻塞综合征的临床特征分析[J].临床肝胆病杂志,2018,34(06):1277-1281.
[5] 吡咯生物碱相关肝窦阻塞综合征诊断和治疗专家共识意见(2017年,南京)[J].中华消化杂志,2017,37(08):513-522.
[6] Mohty M, Malard F, Abecassis M, et al. Revised diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a new classification from the European Society for Blood and Marrow Transplantation[J]. Bone Marrow Transplant, 2016, 51(7): 906- 12.
[7] 陈依然,张玮,张明,等.PA-HSOS 严重度分级预测经颈静脉肝内门体分流术治疗PA-HSOS 患者预后的研究[J].中华肝脏病杂志,2021,29(01):46-53.
[8] Wang J Y, Gao H. Tusanqi and hepatic sinusoidal obstruction syndrome[J].J Dig Dis, 2014, 15(3): 105-7.
[9] Wang X, Zhou Y, Sun Q, et al. Thymosin β4 Exerts a Cytoprotective Function and Attenuates Liver Injury in Murine Hepatic Sinusoidal Obstruction Syndrome after Hematopoietic Stem Cell Transplantation[J].Transplant Cell Ther, 2023, 29(8):492.e1-492.e10.
[10] Liu F, Rong X, Guo H, et al. Clinical characteristics, CT signs, and pathological findings of Pyrrolizidine alkaloids-induced sinusoidal obstructive syndrome: a retrospective study[J]. BMC Gastroenterol, 2020, 20(1): 30.
[11] Wang W, Yang X, Chen Y, et al. Seneciphylline, a main pyrrolizidine alkaloid in Gynura japonica, induces hepatotoxicity in mice and primary hepatocytes via activating mitochondria-mediated apoptosis[J].J Appl Toxicol,2020,40(11):1534-1544.
[12] Bao Y, Phan M, Zhu J, et al. Alterations of Cytochrome P450-Mediated Drug Metabolism during Liver Repair and Regeneration after Acetaminophen-Induced Liver Injury in Mice[J]. Drug Metab Dispos, 2022, 50(5): 694-703.
[13] 贾翠宇,赵大伟,冯骥良,等.肝窦阻塞综合征的临床和影像学表现[J].临床放射学杂志,2022,41(02):284-288.
[14] Zhuge Y, Liu Y, Xie W, et al. Expert consensus on the clinical management of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome[J]. J Gastroenterol Hepatol, 2019, 34(4): 634-642.
[15] Masuda T, Nakaura T, Funama Y, et al. Enhancement rate of venous phase to portal venousphase computed tomography and its correlation with ultrasound elastography determination of liver fibrosis[J]. Radiography (Lond), 2022, 28(2): 412-419.
[16] 郑君仪,李梦亚,张月浪,等.土三七致肝小静脉闭塞症的临床特征与影像学表现分析[J].疑难病杂志,2017,16(07):724-727+757.
[17] Kernan N A, Grupp S, Smith A R, et al. Final results from a defibrotide treatment-IND study for patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome[J].Br J Haematol, 2018, 181(6): 816-827.
[18] Peng C, Zhang X, Zhang F, et al. Clinical efficacy and safety of anticoagulation therapy for Pyrrolizidine alkaloids-induced hepatic sinusoidal obstruction syndrome: a retrospective multicenter cohort study[J]. Eur J Gastroenterol Hepatol, 2020, 32(9): 1168- 1178.
[19] Karagiannakis D S. Transjugular intrahepatic portosystemic shunt for recompensating decompensated cirrhosis?[J]. World J Gastroenterol, 2024, 30(20): 2621-2623.
[2] 刘少松,周晓东.吡咯烷生物碱诱导肝窦阻塞综合征的研究进展[J].临床内科杂志,2020,37(04):256-259.
[3] 邓泽平,杨茜,覃凤燕,等.一点红致肝窦阻塞综合征1例并文献复习[J].肝脏,2021,26(08):947-949.
[4] 郭严,张世荣,文良志,等.我国吡咯烷生物碱致肝窦阻塞综合征的临床特征分析[J].临床肝胆病杂志,2018,34(06):1277-1281.
[5] 吡咯生物碱相关肝窦阻塞综合征诊断和治疗专家共识意见(2017年,南京)[J].中华消化杂志,2017,37(08):513-522.
[6] Mohty M, Malard F, Abecassis M, et al. Revised diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a new classification from the European Society for Blood and Marrow Transplantation[J]. Bone Marrow Transplant, 2016, 51(7): 906- 12.
[7] 陈依然,张玮,张明,等.PA-HSOS 严重度分级预测经颈静脉肝内门体分流术治疗PA-HSOS 患者预后的研究[J].中华肝脏病杂志,2021,29(01):46-53.
[8] Wang J Y, Gao H. Tusanqi and hepatic sinusoidal obstruction syndrome[J].J Dig Dis, 2014, 15(3): 105-7.
[9] Wang X, Zhou Y, Sun Q, et al. Thymosin β4 Exerts a Cytoprotective Function and Attenuates Liver Injury in Murine Hepatic Sinusoidal Obstruction Syndrome after Hematopoietic Stem Cell Transplantation[J].Transplant Cell Ther, 2023, 29(8):492.e1-492.e10.
[10] Liu F, Rong X, Guo H, et al. Clinical characteristics, CT signs, and pathological findings of Pyrrolizidine alkaloids-induced sinusoidal obstructive syndrome: a retrospective study[J]. BMC Gastroenterol, 2020, 20(1): 30.
[11] Wang W, Yang X, Chen Y, et al. Seneciphylline, a main pyrrolizidine alkaloid in Gynura japonica, induces hepatotoxicity in mice and primary hepatocytes via activating mitochondria-mediated apoptosis[J].J Appl Toxicol,2020,40(11):1534-1544.
[12] Bao Y, Phan M, Zhu J, et al. Alterations of Cytochrome P450-Mediated Drug Metabolism during Liver Repair and Regeneration after Acetaminophen-Induced Liver Injury in Mice[J]. Drug Metab Dispos, 2022, 50(5): 694-703.
[13] 贾翠宇,赵大伟,冯骥良,等.肝窦阻塞综合征的临床和影像学表现[J].临床放射学杂志,2022,41(02):284-288.
[14] Zhuge Y, Liu Y, Xie W, et al. Expert consensus on the clinical management of pyrrolizidine alkaloid-induced hepatic sinusoidal obstruction syndrome[J]. J Gastroenterol Hepatol, 2019, 34(4): 634-642.
[15] Masuda T, Nakaura T, Funama Y, et al. Enhancement rate of venous phase to portal venousphase computed tomography and its correlation with ultrasound elastography determination of liver fibrosis[J]. Radiography (Lond), 2022, 28(2): 412-419.
[16] 郑君仪,李梦亚,张月浪,等.土三七致肝小静脉闭塞症的临床特征与影像学表现分析[J].疑难病杂志,2017,16(07):724-727+757.
[17] Kernan N A, Grupp S, Smith A R, et al. Final results from a defibrotide treatment-IND study for patients with hepatic veno-occlusive disease/sinusoidal obstruction syndrome[J].Br J Haematol, 2018, 181(6): 816-827.
[18] Peng C, Zhang X, Zhang F, et al. Clinical efficacy and safety of anticoagulation therapy for Pyrrolizidine alkaloids-induced hepatic sinusoidal obstruction syndrome: a retrospective multicenter cohort study[J]. Eur J Gastroenterol Hepatol, 2020, 32(9): 1168- 1178.
[19] Karagiannakis D S. Transjugular intrahepatic portosystemic shunt for recompensating decompensated cirrhosis?[J]. World J Gastroenterol, 2024, 30(20): 2621-2623.
Copyright © 2026 匡旭, 张卓
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License