伴高胆红素血症不明原因肝炎的临床诊断路径及病因构成分析
|
摘要
目的制订伴高胆红素血症不明原因肝炎的诊断路径并分析其病因构成。方法在对既往伴高胆红素血症不明原因肝炎患者进行回顾性分析及随访调查的基础上,参考国内外研究进展,分级制订各项检查项目,归纳伴高胆红素血症不明原因肝炎的临床诊断路径。2014年6月-2017年6月解放军总医院第五医学中心收治的伴高胆红素血症不明原因肝炎患者223例,均按上述临床诊断路径依次进行初步、深入、终极筛查。对伴高胆红素血症不明原因肝炎的病因检出情况进行分析,在此基础上进一步探讨伴高胆红素血症不明原因肝炎的病因构成。结果 223例患者中,通过路径筛查明确病因者共181例(81.17%),其中初级筛查明确2例(0.90%),深入筛查明确18例(8.07%),终极筛查明确161例(72.20%);42例(18.83%)未能明确诊断病因,其中14例(6.28%)完成诊断路径仍不能明确诊断,28例(12.55%)因病情危重或拒绝肝穿刺或拒绝基因检测未能完成诊断路径。完成诊断路径的195例患者中,明确病因181例,实际病因检出率92.82%。223例患者中,病因为药物性肝损害143例(64.13%),遗传代谢性肝病11例(4.93%),自身免疫性肝病8例(3.58%),感染性病因5例(2.24%),其他系统疾病14例(6.28%),未确定病因42例(18.83%)。结论伴高胆红素血症不明原因肝炎以药物性肝损害、遗传代谢性肝病较为常见;"伴高胆红素血症不明原因肝炎三级筛查诊断路径"确诊有效性较好,简便易行,值得临床推广试行。
Objective To sum up the diagnosis experience of unexplained hepatitis with hyperbilirubinemia(UHH) for establishing its diagnostic pathways and etiological factors. Methods By referring to the research progress at home and abroad,various examination items were made in order to form the clinical diagnosis pathway of UHH, on the basis of retrospective analysis and follow-up investigation of UHH patients. Using this pathway we carried out prospective diagnosis analysis for 223 patients admitted to Fifth Medical Center of Chinese PLA General Hospital from June 2014 to June 2017, in the order of preliminary, indepth, and ultimate screening. Results A total of 181 cases were identified through the pathway screening(181/223, 81.17%).Two cases were etiologically diagnosed by primary screening(2/223, 0.90%), 18(18/223, 8.07%) by in-depth screening, and161(161/223, 72.20%) by ultimate screening. There were still 42 cases(42/223, 18.83%) failing to make a definite diagnosis,among which 14 cases(14/223, 6.28%) failed to make a definite diagnosis after completing the diagnostic pathway, and 28(28/223,12.55%) failed to complete the diagnostic pathway due to the critical condition and failure of liver puncture or rejection of genetic test. Among the 195 patients who completed diagnostic pathway, 181 cases were identified, and the actual etiology diagnosis rate was 92.82%. Among the 223 cases, 143(64.13%) were caused by drug-induced liver damage, 11(4.93%) by hereditary metabolic liver disease, 8(3.58%) by autoimmune liver disease, 5(2.24%) by infectious diseases, 14(6.28%) by other systemic diseases, and42(18.83%) by undetermined causes. Conclusion Drug-induced liver damage, and genetic metabolic liver disease are common causes of UHH. The three-step screening pathway is effective for the diagnosis of UHH, and also simple and easy to practice, thus being worthy of promotion on a trial basis.
Objective To sum up the diagnosis experience of unexplained hepatitis with hyperbilirubinemia(UHH) for establishing its diagnostic pathways and etiological factors. Methods By referring to the research progress at home and abroad,various examination items were made in order to form the clinical diagnosis pathway of UHH, on the basis of retrospective analysis and follow-up investigation of UHH patients. Using this pathway we carried out prospective diagnosis analysis for 223 patients admitted to Fifth Medical Center of Chinese PLA General Hospital from June 2014 to June 2017, in the order of preliminary, indepth, and ultimate screening. Results A total of 181 cases were identified through the pathway screening(181/223, 81.17%).Two cases were etiologically diagnosed by primary screening(2/223, 0.90%), 18(18/223, 8.07%) by in-depth screening, and161(161/223, 72.20%) by ultimate screening. There were still 42 cases(42/223, 18.83%) failing to make a definite diagnosis,among which 14 cases(14/223, 6.28%) failed to make a definite diagnosis after completing the diagnostic pathway, and 28(28/223,12.55%) failed to complete the diagnostic pathway due to the critical condition and failure of liver puncture or rejection of genetic test. Among the 195 patients who completed diagnostic pathway, 181 cases were identified, and the actual etiology diagnosis rate was 92.82%. Among the 223 cases, 143(64.13%) were caused by drug-induced liver damage, 11(4.93%) by hereditary metabolic liver disease, 8(3.58%) by autoimmune liver disease, 5(2.24%) by infectious diseases, 14(6.28%) by other systemic diseases, and42(18.83%) by undetermined causes. Conclusion Drug-induced liver damage, and genetic metabolic liver disease are common causes of UHH. The three-step screening pathway is effective for the diagnosis of UHH, and also simple and easy to practice, thus being worthy of promotion on a trial basis.
引文
[1] Oketani M, Ido A, Nakayama N, et al. Etiology and prognosis of fulminant hepatitis and late-onset hepatic failure in Japan:Summary of the annual nationwide survey between 2004 and2009[J]. Hepatol Res, 2013, 43(2):97-105.
[2]Lee WM. Recent developments in acute liver failure[J]. Best Pract Res Clin Gastroenterol, 2012, 26(1):3-16.
[3] Zhu B, Liu HL, Wan ZH, et al. Analysis of the clinical characteristics in patients with liver failure of unknown etiology[J]. Med J Chin PLA, 2016, 41(1):46-49.[朱冰,刘鸿凌,万志红,等.未定病因肝衰竭患者的临床特点分析[J].解放军医学杂志,2016, 41(1):46-49.]
[4] Wang SY, Huang HH, Xu RN, et al. Analysis of cause of death in patients with liver diseases:report of 1890 cases[J]. Med J Chin PLA, 2014, 39(5):392-395.[王嗣子,黄辉煌,徐若男,等.肝病患者死亡原因分析:1890例报告[J].解放军医学杂志,2014, 39(5):392-395.]
[5] Liver Failure and Artificial Liver Group, Society of Infectious Diseases, CMA; Severe Hepatitis and Artificial Liver Group,Chinese Society of Hepatology, CMA. Guideline for diagnosis and treatment of liver failure[J]. Chin J Hepatol, 2019, 27(1):18-26.[中华医学会感染病学分会肝衰竭与人工肝学组,中华医学会肝病学分会重型肝病与人工肝学组.肝衰竭诊治指南(2018年版)[J].中华肝脏病杂志,2019, 27(1):18-26.]
[6] Fujiwara K, Mochida S, Matsui A, ct al. Fulminant hepatitis and late onset hepatic failure in Japan[J]. Hepatol Res, 2008, 38(7):646-657.
[7] Zhu B, Zang H, You SL, et al. Seriously to clinical diagnosis of unknown etiology liver disease[j]. Infec Dis Inform, 2017,30(5):262-264.[朱冰,臧红,游绍莉,等.重视不明原因肝病的临床诊断[J].传染病信息,2017, 30(5):262-264.]
[8] Li Wang K. Inter Var:Clinical interpretation of genetic variants by the 2015 ACMG-AMP guidelines[J]. Am J Hum Genet, 2017, 100(2):267-280.
[9] Ble M, Procopet B, Miquel R, et al. Transjugular liver biopsy[J].Clin Liver Dis, 2014, 18(4):767-778.
[10] You S, Rong Y, Zhu B, et al. Changing etiology of liver failure in 3,916 patients from northern China:a 10-year survey[J].Hepatol Int, 2013, 7(2):714-720.
[11] Bernal W. Acute liver failure:review and update[J]. Int Anesthesiol Clin, 2017, 55(2):92-106.
[12] Yang YF. Road map of diagnosis in patients with unknown causes[J]. J Pract Hepatol, 2018, 21(1):1-3.[杨永峰.不明原因肝病诊断思路[J].实用肝脏病杂志,2018, 21(1):1-3.]
[13] You SL, Zhu B, Rong YH, et al. Clinical analysis on 120 cases of drug-induced liver failure[J]. Pract Preven Med, 2012, 19(11):1696-1698.[游绍莉,朱冰,荣义辉,等.120例药物性肝衰竭临床分析[J].实用预防医学,2012, 19(11):1696-1698.]
[14] Peng J, Guo Y, He ZL, et al. Clinical features in and diagnostic road-map for patients with cryptogenic elevated serum alanine aminotransferase levels[J]. J Pract Hepatol, 2014, 17(6):600-605.[彭景,郭艳,何长龙,等.不明原因肝炎患者临床特征和诊断路径探讨[J].实用肝脏病杂志,2014, 17(6):600-605.]
[15] Kim KR, Ko GY, Sung KB, et al. Transjugular liver biopsy in patients with living donor liver transplantation:comparison with percutaneous biopsy[J]. Liver Transpl, 2008, 14(7):971-979.
[16] Zhu B, Li XJ, Lv S, et al. Feasibility of transjugular liver biopsy in minipigs[J]. Chin J Comparative Med, 2016, 26(4):72-74.[朱冰,李晓娟,吕飒,等.小型猪经颈静脉肝穿刺活检术的可行性探讨[J].中国比较医学杂志,2016, 26(4):72-74].
[17] Qiao ZH, Dong S. Trans-jugular liver biopsy[J]. J Clin Radiol,2011, 30(7):1068-1070.[乔智红,董生.经颈静脉肝活检术[J].临床放射学杂志,2011, 30(7):1068-1070.]
[18] Lv S, You SL, Yu et al. Feasibility and significance of transjugular liver biopsy in diagnosis and treatment of complicated and severe liver diseases:a single-center analysis of5 cases[J]. Chin J Hepatol, 2017, 25(10):772-774.[吕飒,游绍莉,余强,等.经颈静脉肝穿刺活组织检查在疑难重症肝病诊治中的可行性及意义:单中心5例患者分析[J].中华肝脏病杂志,2017, 25(10):772-774.]
[2]Lee WM. Recent developments in acute liver failure[J]. Best Pract Res Clin Gastroenterol, 2012, 26(1):3-16.
[3] Zhu B, Liu HL, Wan ZH, et al. Analysis of the clinical characteristics in patients with liver failure of unknown etiology[J]. Med J Chin PLA, 2016, 41(1):46-49.[朱冰,刘鸿凌,万志红,等.未定病因肝衰竭患者的临床特点分析[J].解放军医学杂志,2016, 41(1):46-49.]
[4] Wang SY, Huang HH, Xu RN, et al. Analysis of cause of death in patients with liver diseases:report of 1890 cases[J]. Med J Chin PLA, 2014, 39(5):392-395.[王嗣子,黄辉煌,徐若男,等.肝病患者死亡原因分析:1890例报告[J].解放军医学杂志,2014, 39(5):392-395.]
[5] Liver Failure and Artificial Liver Group, Society of Infectious Diseases, CMA; Severe Hepatitis and Artificial Liver Group,Chinese Society of Hepatology, CMA. Guideline for diagnosis and treatment of liver failure[J]. Chin J Hepatol, 2019, 27(1):18-26.[中华医学会感染病学分会肝衰竭与人工肝学组,中华医学会肝病学分会重型肝病与人工肝学组.肝衰竭诊治指南(2018年版)[J].中华肝脏病杂志,2019, 27(1):18-26.]
[6] Fujiwara K, Mochida S, Matsui A, ct al. Fulminant hepatitis and late onset hepatic failure in Japan[J]. Hepatol Res, 2008, 38(7):646-657.
[7] Zhu B, Zang H, You SL, et al. Seriously to clinical diagnosis of unknown etiology liver disease[j]. Infec Dis Inform, 2017,30(5):262-264.[朱冰,臧红,游绍莉,等.重视不明原因肝病的临床诊断[J].传染病信息,2017, 30(5):262-264.]
[8] Li Wang K. Inter Var:Clinical interpretation of genetic variants by the 2015 ACMG-AMP guidelines[J]. Am J Hum Genet, 2017, 100(2):267-280.
[9] Ble M, Procopet B, Miquel R, et al. Transjugular liver biopsy[J].Clin Liver Dis, 2014, 18(4):767-778.
[10] You S, Rong Y, Zhu B, et al. Changing etiology of liver failure in 3,916 patients from northern China:a 10-year survey[J].Hepatol Int, 2013, 7(2):714-720.
[11] Bernal W. Acute liver failure:review and update[J]. Int Anesthesiol Clin, 2017, 55(2):92-106.
[12] Yang YF. Road map of diagnosis in patients with unknown causes[J]. J Pract Hepatol, 2018, 21(1):1-3.[杨永峰.不明原因肝病诊断思路[J].实用肝脏病杂志,2018, 21(1):1-3.]
[13] You SL, Zhu B, Rong YH, et al. Clinical analysis on 120 cases of drug-induced liver failure[J]. Pract Preven Med, 2012, 19(11):1696-1698.[游绍莉,朱冰,荣义辉,等.120例药物性肝衰竭临床分析[J].实用预防医学,2012, 19(11):1696-1698.]
[14] Peng J, Guo Y, He ZL, et al. Clinical features in and diagnostic road-map for patients with cryptogenic elevated serum alanine aminotransferase levels[J]. J Pract Hepatol, 2014, 17(6):600-605.[彭景,郭艳,何长龙,等.不明原因肝炎患者临床特征和诊断路径探讨[J].实用肝脏病杂志,2014, 17(6):600-605.]
[15] Kim KR, Ko GY, Sung KB, et al. Transjugular liver biopsy in patients with living donor liver transplantation:comparison with percutaneous biopsy[J]. Liver Transpl, 2008, 14(7):971-979.
[16] Zhu B, Li XJ, Lv S, et al. Feasibility of transjugular liver biopsy in minipigs[J]. Chin J Comparative Med, 2016, 26(4):72-74.[朱冰,李晓娟,吕飒,等.小型猪经颈静脉肝穿刺活检术的可行性探讨[J].中国比较医学杂志,2016, 26(4):72-74].
[17] Qiao ZH, Dong S. Trans-jugular liver biopsy[J]. J Clin Radiol,2011, 30(7):1068-1070.[乔智红,董生.经颈静脉肝活检术[J].临床放射学杂志,2011, 30(7):1068-1070.]
[18] Lv S, You SL, Yu et al. Feasibility and significance of transjugular liver biopsy in diagnosis and treatment of complicated and severe liver diseases:a single-center analysis of5 cases[J]. Chin J Hepatol, 2017, 25(10):772-774.[吕飒,游绍莉,余强,等.经颈静脉肝穿刺活组织检查在疑难重症肝病诊治中的可行性及意义:单中心5例患者分析[J].中华肝脏病杂志,2017, 25(10):772-774.]