儿童胆管缺失综合征行肝移植术治疗
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摘要
目的探讨胆管缺失综合征的临床诊治策略。方法回顾性分析四川大学华西医院收治的1例术前疑似胆道闭锁而行肝移植术患者的临床资料和诊治经过。结果该例患儿因肝功能衰竭而行肝移植手术,术后病理结果提示为肝内胆管缺失综合征伴继发性(不全分割性)胆汁淤积性肝硬变形成,术后恢复良好。已随访至术后11个月,肝功能、凝血功能及移植肝血流未见明显异常,身高、体质量与同龄儿童相近。结论胆管缺失综合征病因众多,发病机制复杂,肝移植为最终有效的治疗方法。
Objective To discuss diagnosis and treatment strategy of vanishing bile duct syndrome.Method The clinical data of 1 child patient with suspected biliary atresia who received the liver transplantation in the West China Hospital of Sichuan University was retrospectively analyzed. Results The child patient underwent the liver transplantation due to the liver failure, and the postoperative pathological results indicated the vanishing bile duct syndrome accompanied by the formation of secondary(incomplete segmentation) cholestatic liver cirrhosis, the postoperative recovery was good. After 11 months of follow-up, the liver function, coagulation function, and blood flow of the transplanted liver were not obviously abnormal. The height and weight were suitable for the same age.Conclusion At present, there are many etiologies and complicated pathogenesis of vanishing bile duct syndrome, and liver transplantation is an ultimate effective treatment.
Objective To discuss diagnosis and treatment strategy of vanishing bile duct syndrome.Method The clinical data of 1 child patient with suspected biliary atresia who received the liver transplantation in the West China Hospital of Sichuan University was retrospectively analyzed. Results The child patient underwent the liver transplantation due to the liver failure, and the postoperative pathological results indicated the vanishing bile duct syndrome accompanied by the formation of secondary(incomplete segmentation) cholestatic liver cirrhosis, the postoperative recovery was good. After 11 months of follow-up, the liver function, coagulation function, and blood flow of the transplanted liver were not obviously abnormal. The height and weight were suitable for the same age.Conclusion At present, there are many etiologies and complicated pathogenesis of vanishing bile duct syndrome, and liver transplantation is an ultimate effective treatment.
引文
1 Butler AE, Schreiber RA, Yanchar N, et al. The Canadian Biliary Atresia Registry:Improving the care of Canadian infants with biliary atresia. Paediatr Child Health, 2016, 21(3):131-134.
2 Reau NS, Jensen DM. Vanishing bile duct syndrome. Clin Liver Dis, 2008, 12(1):203-217.
3 Sherlock S. The syndrome of disappearing intrahepatic bile ducts.Lancet, 1987, 2(8557):493-496.
4 Moore KL. The digestive system in the developing human.Clinically oriented embryology. 4th edition. Philadelphia:WBSaunders,1988.
5 Clotman F, Libbrecht L, Gresh L, et al. Hepatic artery malformations associated with a primary defect in intrahepatic bile duct development. J Hepatol, 2003, 39(5):686-692.
6 Quiros-Tejeira RE, Ament ME, Heyman MB, et al. Variable morbidity in Alagille syndrome:a review of 43 cases. J Pediatr Gastroenterol Nutr, 1999, 29(4):431-437.
7 Jacquet A, Guiochon-Mantel A, Noel LH, et al. Alagille syndrome in adult patients:it is never too late. Am J Kidney Dis, 2007, 49(5):705-709.
8 Farid WR, de Jonge J, Slieker JC, et al. The importance of portal venous blood flow in ischemic-type biliary lesions after liver transplantation. Am J Transplant, 2011,11(4):857-862.
9 Abou-Rebyeh H, Veltzke-Schlieker W, Radke C, et al. Complete bile duct sequestration after liver transplantation, caused by ischemic-type biliary lesions. Endoscopy, 2003, 35(7):616-620.
10 Xu Y, Yu J, Zhang R, et al. The perinatal infection of cytomegalovirus is an important etiology for biliary atresia in China. Clin Pediatr(Phila), 2012, 51(2):109-113.
11 Ballonoff A, Kavanagh B, Nash R, et al. Hodgkin lymphomarelated vanishing bile duct syndrome and idiopathic cholestasis:statistical analysis of all published cases and literature review. Acta Oncol, 2008, 47(5):962-970.
12 Hubscher SG, Lumley MA, Elias E. Vanishing bile duct syndrome:a possible mechanism for intrahepatic cholestasis in Hodgkin's lymphoma. Hepatology, 1993,17(1):70-77.
13 Wong KM, Chang CS, Wu CC, et al. Hodgkin's lymphomarelated vanishing bile duct syndrome:a case report and literature review. Kaohsiung J Med Sci,2013, 29(11):636-641.
14 Das A, Mitra S, Ghosh D, et al. Vanishing bile duct syndrome following cytomegalovirus infection in a child with Hodgkin lymphoma. J Pediatr Hematol Oncol, 2018, 40(1):83-84.
15陈芳,涂相林.胆管消失综合征的再认识.世界华人消化杂志,2016,24(23):3445-3453.
16 Desmet VJ. Vanishing bile duct syndrome in drug-induced liver disease. J Hepatol, 1997, 26(Suppl 1):31-35.
17 United States National Library of Medicine; National Insitute of Diabetes and Digestive and Kidney Diseases. LiverTox.http://livertox.nlm.nih.gov. Accessed March 2017.
18 Ramos AM, Gayotto LC, Clemente CM, et al. Reversible vanishing bile duct syndrome induced by carbamazepine. Eur J Gastroenterol Hepatol, 2002, 14(9):1019-1022.
19 Vuppalanchi R, Chalasani N, Saxena R. Restoration of bile ducts in drug-induced vanishing bile duct syndrome due to zonisamide.Am J Surg Pathol, 2006, 30(12):1619-1623.
20 Bonkovsky HL, Kleiner DE, Gu J, et al. Clinical presentations and outcomes of bile duct loss caused by drugs and herbal and dietary supplements. Hepatology, 2017, 65(4):1267-1277.
21 Sgro C, Clinard F, Ouazir K, et al. Incidence of drug-induced hepatic injuries:a French population-based study. Hepatology,2002, 36(2):451-455.
22 Ludwig J, Wiesner RH, LaRusso NF. Idiopathic adulthood ductopenia. A cause of chronic cholestatic liver disease and biliary cirrhosis. J Hepatol, 1988, 7(2):193-199.
23张文胜,王宝恩,贾继东.特发性成人肝内胆管缺失症.肝脏,2004,9(4):264-266.
24孙玥,赵新颜,贾继东.胆管消失综合征病因学诊断及预后进展.肝脏,2014, 19(2):137-139.
25 Lee WS, Chai PF. Clinical features differentiating biliary atresia from other causes of neonatal cholestasis, Ann Acad Med Singapore, 2010, 39(8):648-654.
26 Jancelewicz T, Barmherzig R, Chung CT, et al. A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice. J Pediatr Surg, 2015, 50(3):363-370.
27 Ludwig J, Wiesner RH, LaRusso NF. Idiopathic adulthood ductopenia. A cause of chronic cholestatic liver disease and biliary cirrhosis. J Hepatol, 1988, 7(2):193-199.
28 Ludwig J, Wiesner RH, Batts KP, et al. The acute vanishing bile duct syndrome(acute irreversible rejection)after orthotopic liver transplantation. Hepatology, 1987, 7(3):476-483.
29 Herman HK, Abramowsky CR, Caltharp S, et al. Identification of bile duct paucity in Alagille syndrome:using CK7 and EMA immunohistochemistry as a reliable panel for accurate diagnosis.Pediatr Dev Pathol, 2016,19(1):47-50.
30 Pusl T, Beuers U. Ursodeoxycholic acid treatment of vanishing bileduct syndromes. World J Gastroenterol, 2006, 12(22):3487-3495.
31 Rota Scalabrini D, Caravelli D, Carnevale Schianca F, et al.Complete remission of paraneoplastic vanishing bile duct syndrome after the successful treatment of Hodgkin's lymphoma:a case report and review of the literature. BMC Res Notes, 2014, 7:529.
32 Hallen K, Sangfelt P, Nilsson T, et al. Vanishing bile duct-like syndrome in a patient with Hodgkin lymphoma-pathological development and restitution. Acta Oncol, 2014, 53(9):1271-1275.
33 Crosbie OM, Crown JP, Nolan NP, et al. Resolution of paraneoplastic bile duct paucity following successful treatment of Hodgkin's disease. Hepatology, 1997, 26(1):5-8.
34 Leeuwenburgh I, Lugtenburg EP, van Buuren HR, et al. Severe jaundice, due to vanishing bile duct syndrome, as presenting symptom of Hodgkin's lymphoma, fully reversible after chemotherapy. Eur J Gastroenterol Hepatol, 2008, 20(2):145-147.
2 Reau NS, Jensen DM. Vanishing bile duct syndrome. Clin Liver Dis, 2008, 12(1):203-217.
3 Sherlock S. The syndrome of disappearing intrahepatic bile ducts.Lancet, 1987, 2(8557):493-496.
4 Moore KL. The digestive system in the developing human.Clinically oriented embryology. 4th edition. Philadelphia:WBSaunders,1988.
5 Clotman F, Libbrecht L, Gresh L, et al. Hepatic artery malformations associated with a primary defect in intrahepatic bile duct development. J Hepatol, 2003, 39(5):686-692.
6 Quiros-Tejeira RE, Ament ME, Heyman MB, et al. Variable morbidity in Alagille syndrome:a review of 43 cases. J Pediatr Gastroenterol Nutr, 1999, 29(4):431-437.
7 Jacquet A, Guiochon-Mantel A, Noel LH, et al. Alagille syndrome in adult patients:it is never too late. Am J Kidney Dis, 2007, 49(5):705-709.
8 Farid WR, de Jonge J, Slieker JC, et al. The importance of portal venous blood flow in ischemic-type biliary lesions after liver transplantation. Am J Transplant, 2011,11(4):857-862.
9 Abou-Rebyeh H, Veltzke-Schlieker W, Radke C, et al. Complete bile duct sequestration after liver transplantation, caused by ischemic-type biliary lesions. Endoscopy, 2003, 35(7):616-620.
10 Xu Y, Yu J, Zhang R, et al. The perinatal infection of cytomegalovirus is an important etiology for biliary atresia in China. Clin Pediatr(Phila), 2012, 51(2):109-113.
11 Ballonoff A, Kavanagh B, Nash R, et al. Hodgkin lymphomarelated vanishing bile duct syndrome and idiopathic cholestasis:statistical analysis of all published cases and literature review. Acta Oncol, 2008, 47(5):962-970.
12 Hubscher SG, Lumley MA, Elias E. Vanishing bile duct syndrome:a possible mechanism for intrahepatic cholestasis in Hodgkin's lymphoma. Hepatology, 1993,17(1):70-77.
13 Wong KM, Chang CS, Wu CC, et al. Hodgkin's lymphomarelated vanishing bile duct syndrome:a case report and literature review. Kaohsiung J Med Sci,2013, 29(11):636-641.
14 Das A, Mitra S, Ghosh D, et al. Vanishing bile duct syndrome following cytomegalovirus infection in a child with Hodgkin lymphoma. J Pediatr Hematol Oncol, 2018, 40(1):83-84.
15陈芳,涂相林.胆管消失综合征的再认识.世界华人消化杂志,2016,24(23):3445-3453.
16 Desmet VJ. Vanishing bile duct syndrome in drug-induced liver disease. J Hepatol, 1997, 26(Suppl 1):31-35.
17 United States National Library of Medicine; National Insitute of Diabetes and Digestive and Kidney Diseases. LiverTox.http://livertox.nlm.nih.gov. Accessed March 2017.
18 Ramos AM, Gayotto LC, Clemente CM, et al. Reversible vanishing bile duct syndrome induced by carbamazepine. Eur J Gastroenterol Hepatol, 2002, 14(9):1019-1022.
19 Vuppalanchi R, Chalasani N, Saxena R. Restoration of bile ducts in drug-induced vanishing bile duct syndrome due to zonisamide.Am J Surg Pathol, 2006, 30(12):1619-1623.
20 Bonkovsky HL, Kleiner DE, Gu J, et al. Clinical presentations and outcomes of bile duct loss caused by drugs and herbal and dietary supplements. Hepatology, 2017, 65(4):1267-1277.
21 Sgro C, Clinard F, Ouazir K, et al. Incidence of drug-induced hepatic injuries:a French population-based study. Hepatology,2002, 36(2):451-455.
22 Ludwig J, Wiesner RH, LaRusso NF. Idiopathic adulthood ductopenia. A cause of chronic cholestatic liver disease and biliary cirrhosis. J Hepatol, 1988, 7(2):193-199.
23张文胜,王宝恩,贾继东.特发性成人肝内胆管缺失症.肝脏,2004,9(4):264-266.
24孙玥,赵新颜,贾继东.胆管消失综合征病因学诊断及预后进展.肝脏,2014, 19(2):137-139.
25 Lee WS, Chai PF. Clinical features differentiating biliary atresia from other causes of neonatal cholestasis, Ann Acad Med Singapore, 2010, 39(8):648-654.
26 Jancelewicz T, Barmherzig R, Chung CT, et al. A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice. J Pediatr Surg, 2015, 50(3):363-370.
27 Ludwig J, Wiesner RH, LaRusso NF. Idiopathic adulthood ductopenia. A cause of chronic cholestatic liver disease and biliary cirrhosis. J Hepatol, 1988, 7(2):193-199.
28 Ludwig J, Wiesner RH, Batts KP, et al. The acute vanishing bile duct syndrome(acute irreversible rejection)after orthotopic liver transplantation. Hepatology, 1987, 7(3):476-483.
29 Herman HK, Abramowsky CR, Caltharp S, et al. Identification of bile duct paucity in Alagille syndrome:using CK7 and EMA immunohistochemistry as a reliable panel for accurate diagnosis.Pediatr Dev Pathol, 2016,19(1):47-50.
30 Pusl T, Beuers U. Ursodeoxycholic acid treatment of vanishing bileduct syndromes. World J Gastroenterol, 2006, 12(22):3487-3495.
31 Rota Scalabrini D, Caravelli D, Carnevale Schianca F, et al.Complete remission of paraneoplastic vanishing bile duct syndrome after the successful treatment of Hodgkin's lymphoma:a case report and review of the literature. BMC Res Notes, 2014, 7:529.
32 Hallen K, Sangfelt P, Nilsson T, et al. Vanishing bile duct-like syndrome in a patient with Hodgkin lymphoma-pathological development and restitution. Acta Oncol, 2014, 53(9):1271-1275.
33 Crosbie OM, Crown JP, Nolan NP, et al. Resolution of paraneoplastic bile duct paucity following successful treatment of Hodgkin's disease. Hepatology, 1997, 26(1):5-8.
34 Leeuwenburgh I, Lugtenburg EP, van Buuren HR, et al. Severe jaundice, due to vanishing bile duct syndrome, as presenting symptom of Hodgkin's lymphoma, fully reversible after chemotherapy. Eur J Gastroenterol Hepatol, 2008, 20(2):145-147.
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