Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sa...Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis.Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry.The occurrence of jaundice in sarcoidosis is rare;extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic.Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver-related death.We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded.The patient follow up was 8 years.Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis.A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.展开更多
BACKGROUND Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease ofunknown etiology that usually presents as unexplained jaundice. It ischaracterized by adult onset, lack of autoantibodies, infl...BACKGROUND Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease ofunknown etiology that usually presents as unexplained jaundice. It ischaracterized by adult onset, lack of autoantibodies, inflammatory bowel diseaseand loss of interlobular bile ducts.CASE SUMMARY This case presents a 27-year-old woman with elevation of transaminases andalkaline phosphatase without clinical symptoms. Five years ago, the patient hadabnormal transaminases but no cholestasis. Three months before admission,physical examination revealed an increase in transaminases. Oralhepatoprotective drugs did not show any significant improvement, and she wasadmitted to hospital for further diagnosis and treatment. Liver biopsy confirmedIAD. After about 2 wk of ursodeoxycholic acid treatment, serological andhistological examination showed a significant response.CONCLUSION IAD is a manifestation of cholestasis, but also may be an abnormal increase intransaminase in the early stage.展开更多
Mild idiopathic adulthood ductopenia(IAD)is a rare cholestatic disease of unknown cause and characterized by interlobular bile duct loss in less than 50%of the portal tracts.We describe the case of a middLe-aged male ...Mild idiopathic adulthood ductopenia(IAD)is a rare cholestatic disease of unknown cause and characterized by interlobular bile duct loss in less than 50%of the portal tracts.We describe the case of a middLe-aged male who presented with persistent elevation of transaminases and alkaline phosphatase.He had a normal biliary tree on endoscopic retrograde cholangiopathy and negative anti-mitochondrial antibody.His liver biopsy specimen showed chronic biliary disease,duct loss in 4 out of 15 portal tracts and prominent cholestasis.Based on the work-up,he likely had mild IAD.Liver transplantation would be necessary if his disease becomes progressive.展开更多
Vanishing bile duct syndrome(VBDS) is a group of rare disorders characterized by ductopenia,the progressive destruction and disappearance of intrahepatic bile ducts leading to cholestasis.Described in association with...Vanishing bile duct syndrome(VBDS) is a group of rare disorders characterized by ductopenia,the progressive destruction and disappearance of intrahepatic bile ducts leading to cholestasis.Described in association with medications,autoimmune disorders,cancer,transplantation,and infections,the specific mechanisms of disease are not known.To date,only 4 cases of VBDS have been reported in human immunodeficiency virus(HIV) infected patients.We report 2 additional cases of HIV-associated VBDS and review the features common to the HIV-associated cases.Presentation includes hyperbilirubinemia,normal liver imaging,and negative viral and autoimmune hepatitis studies.In HIV-infected subjects,VBDS occurred at a range of CD4+ T-cell counts,in some cases following initiation or change in antiretroviral therapy.Lymphoma was associated with two cases;nevirapine,antibiotics,and viral co-infection were suggested as etiologies in the other cases.In HIV-positive patients with progressive cholestasis,early identification of VBDS and referral for transplantation may improve outcomes.展开更多
Vanishing bile duct syndrome (VBDS) refers to a group of disorders characterized by prolonged cholestasis as a result of destruction and disappearance ofintrahepatic bile ducts. Multiple etiologies have been indentifi...Vanishing bile duct syndrome (VBDS) refers to a group of disorders characterized by prolonged cholestasis as a result of destruction and disappearance ofintrahepatic bile ducts. Multiple etiologies have been indentifi ed including infections, neoplastic disorders, autoimmune conditions and drugs. The natural history of this condition is variable and may involve resolution of cholestasis or progression with irreversible damage. VBDS is extremely rare in human immunodeficiency virus (HIV)-infected patients and anti-retroviral therapy has never been implicated as a cause. We encountered a young pregnant female with HIV and VBDS secondary to anti-retroviral therapy. Here, we report her clinical course and outcome.展开更多
Amoxicillin/clavulanate is associated with liver injury, mostly of a cholestatic pattern. While outcomes are usually benign, progression to cirrhosis and death has been reported. The role of immunosuppressive therapy ...Amoxicillin/clavulanate is associated with liver injury, mostly of a cholestatic pattern. While outcomes are usually benign, progression to cirrhosis and death has been reported. The role of immunosuppressive therapy for patients with a protracted course is unclear. We report the case of an elderly patient who developed prolonged cholestasis secondary to amoxicillin/clavulanate. Vanishing bile duct syndrome was confirmed by sequential liver biopsies. The patient responded to prednisone treatment, but could not be weaned off corticosteroids, even when azathioprine was added. Complete withdrawal of both prednisone and azathioprine was possible by using mycophenolate mofetil, an inosine monophosphate dehydrogenase inhibitor. Sustained remission has been maintained for more than 3 years with low-dose mycophenolate mofetil.展开更多
Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated...Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin’s lymphoma (HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage IIB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.展开更多
BACKGROUND: Nonsteroidal anti-inflammatory drugs (NSAIDs) have been reported to induce liver injury. Patterns of the injury usually range from mild elevations of liver enzymes to sometimes severe fulminant hepatic fai...BACKGROUND: Nonsteroidal anti-inflammatory drugs (NSAIDs) have been reported to induce liver injury. Patterns of the injury usually range from mild elevations of liver enzymes to sometimes severe fulminant hepatic failure. Likewise, naproxen is a propionic acid derivative NSAID that was introduced in 1980 and has been available as an over-the- counter medication since 1994, but has rarely been reported to cause liver injury. METHODS: We treated a 30-year-old woman with jaundice and intractable pruritus that developed shortly after taking naproxen. We reviewed the medical history and liver histopathology of the patient as well as all previously published case reports of naproxen-associated liver toxicity in the English language literature. RESULTS: The liver biochemical profile of the patient revealed a mixed cholestasis and hepatitis pattern. Consecutive liver biopsies demonstrated focal lobular inflammation, hepatocyte drop-out, and a progressive loss of the small interlobular bile ducts (ductopenia). The biopsy performed two years after onset of the disease showed partial recovery of a small number of bile ducts; however, 10 years passed before the biochemical profile returned to near normal. CONCLUSIONS: Naproxen-associated liver toxicity remains a rare entity, but should be considered in any patient presenting with cholestasis shortly after its use. Liver injury is most commonly seen in a mixed pattern characterized by cholestasis and hepatitis. The resulting liver damage may take years to resolve.展开更多
病例：患者男，24岁。因“反复皮下瘀斑8年，身目黄染3个月”于2012年3月11日收治入院。患者8年前四肢受到轻微碰撞后出现大面积皮下瘀斑伴牙龈出血，外院予止血治疗（具体不详）后瘀斑消退，但该症状常反复。1年前再次因皮下瘀斑至外...病例：患者男，24岁。因“反复皮下瘀斑8年，身目黄染3个月”于2012年3月11日收治入院。患者8年前四肢受到轻微碰撞后出现大面积皮下瘀斑伴牙龈出血，外院予止血治疗（具体不详）后瘀斑消退，但该症状常反复。1年前再次因皮下瘀斑至外院就诊，检查显示凝血酶原时间（PT）120 s，活化部分凝血活酶时间（APTT ）107．8 s，凝血因子 FⅦ、FⅨ下降，ALT 372 U ／L，AST 386 U ／L，ALP 1633 U ／L，甲状旁腺素173．9 pg／mL，骨扫描未见异常，骨髓穿刺活检提示刺激性骨髓象，予“止血、护肝”等对症治疗，皮下出血症状好转后出院。11个月前，复查 ALT 298 U ／L，AST 246 U ／L，继续口服多烯磷脂酰胆碱治疗。10个月前，患者自行停药并服用中草药（具体不详）治疗半年，4个月前出现排便次数增多（2～3次／d），量少，黄色水样便，不含黏液脓血，气味恶臭，禁食后缓解。予止泻药物治疗效果不佳。3个月前，患者再次出现四肢皮下瘀斑，伴进行性加重的身目黄染，无腹胀、纳差，无恶心、呕吐，无畏寒、发热。外院复查：ALT 540 U ／L， AST 413．2 U ／L，ALP 3236 U ／L，总胆红素（TBIL）339μmol／L，直接胆红素（DBIL）268μmol／L，PT 17．2 s，总胆固醇14．2 mmol／L，予“止血、护肝、退黄”治疗效果不佳，遂转入我院。患者否认“肝炎、结核”病史，否认输血史、饮酒史、静脉注射毒品史、高危性行为史，否认曾服用“氯磺丙脲、丙氯拉嗪、甲苯磺丁脲”等药物，无已知毒物暴露史，无肝脏疾病家族史，否认食物、药物过敏史。展开更多
BACKGROUND Muscle growth promoters are being developed for the treatment of diseaseinduced loss of muscle mass.Ligandrol and ostarine are selective androgen receptor modulators(SARMs)with a non-steroidal structure and...BACKGROUND Muscle growth promoters are being developed for the treatment of diseaseinduced loss of muscle mass.Ligandrol and ostarine are selective androgen receptor modulators(SARMs)with a non-steroidal structure and a presumably more favorable side effect profile.In recent years,these substances with or without“post-cycle therapy”(PCT)are often misused by amateur athletes aiming to promote muscle growth.At the same time,reports on their toxic effects on organ systems are emerging.CASE SUMMARY We report two cases of liver injury in young men who used ligandrol and/or ostarine for a few weeks followed by the use of substances for PCT.Acute liver injury occurred in both cases after stopping SARMs while on PCT.The clinical picture was dominated by jaundice and fatigue.The biochemical pattern showed a mixed type of injury with normal alkaline phosphatase and high concentrations of bilirubin and serum bile acids. Histological evidence showed predominantlycholestatic injury with canalicular bile plugs, ductopenia, and mild hepatocellulardamage without significant fibrosis. The patients recovered from the conditionafter 3 mo. The off target effects of SARMs were likely idiosyncratic, but ourreport highlights the yet unrecognized effects of other toxic substances used forPCT, supra-therapeutic doses, and the complete absence of monitoring foradverse effects.CONCLUSIONAmong muscle-building amateur athletes, SARMs (ligandrol or ostarine) and/orsubstances in PCT may cause cholestatic liver injury with prolonged recovery.展开更多
肝内胆管缺失是指肝内胆管数量的减少或消失,是胆管树基本病理改变之一。免疫紊乱、肿瘤、感染、药物、缺血、遗传等因素都有可能引起胆管缺失。临床上,通过肝活组织检查,在10个及以上门管区的标本里,发现50%以上的小叶间胆管缺失,即可...肝内胆管缺失是指肝内胆管数量的减少或消失,是胆管树基本病理改变之一。免疫紊乱、肿瘤、感染、药物、缺血、遗传等因素都有可能引起胆管缺失。临床上,通过肝活组织检查,在10个及以上门管区的标本里,发现50%以上的小叶间胆管缺失,即可确诊[1]。胆管缺失的预后取决于病因和损伤程度。晚期出现不可逆的广泛胆管缺失甚至胆管消失时,该病理综合征称为胆管消失综合征(vanishing bile duct syndrome,VBDS),仅发生在0.5%小胆管病[2]。随后,可进展至广泛的胆管纤维化或肝硬化。有趣的是,也有部分患者表现为胆管上皮细胞再生,在数月或数年后得到恢复。本文总结了胆管缺失发病的分子机制,并重点强调近年来免疫介导的胆管病和胆管缺失的研究进展。展开更多
文摘Sarcoidosis is a systemic granulomatous disease which may involve many organs.In approximately 95% of patients there is liver involvement,with noncaseating hepatic granulomas occurring in 21 to 99% of patients with sarcoidosis.Liver involvement is usually asymptomatic and limited to mild to moderate abnormalities in liver biochemistry.The occurrence of jaundice in sarcoidosis is rare;extensive imaging procedures and the examination of liver biopsies permit a precise diagnostic.Ductopenia associated with sarcoidosis has been reported in less than 20 cases and can lead to biliary cirrhosis and liver-related death.We report here on a case of ductopenia-related sarcoidosis in which primary biliary cirrhosis and extrahepatic cholestasis have been carefully excluded.The patient follow up was 8 years.Although ursodesoxycholic acid appears to improve liver biochemistry it does not preclude the rapid occurrence of extensive fibrosis.A review of the literature of reported cases of ductopenia related to sarcoidosis is provided.
文摘BACKGROUND Idiopathic adulthood ductopenia (IAD) is a chronic cholestatic liver disease ofunknown etiology that usually presents as unexplained jaundice. It ischaracterized by adult onset, lack of autoantibodies, inflammatory bowel diseaseand loss of interlobular bile ducts.CASE SUMMARY This case presents a 27-year-old woman with elevation of transaminases andalkaline phosphatase without clinical symptoms. Five years ago, the patient hadabnormal transaminases but no cholestasis. Three months before admission,physical examination revealed an increase in transaminases. Oralhepatoprotective drugs did not show any significant improvement, and she wasadmitted to hospital for further diagnosis and treatment. Liver biopsy confirmedIAD. After about 2 wk of ursodeoxycholic acid treatment, serological andhistological examination showed a significant response.CONCLUSION IAD is a manifestation of cholestasis, but also may be an abnormal increase intransaminase in the early stage.
文摘Mild idiopathic adulthood ductopenia(IAD)is a rare cholestatic disease of unknown cause and characterized by interlobular bile duct loss in less than 50%of the portal tracts.We describe the case of a middLe-aged male who presented with persistent elevation of transaminases and alkaline phosphatase.He had a normal biliary tree on endoscopic retrograde cholangiopathy and negative anti-mitochondrial antibody.His liver biopsy specimen showed chronic biliary disease,duct loss in 4 out of 15 portal tracts and prominent cholestasis.Based on the work-up,he likely had mild IAD.Liver transplantation would be necessary if his disease becomes progressive.
基金Supported by The Intramural Research Programs of the National Institutes of Health Clinical Center and the National Institute of Allergy and Infectious Diseases
文摘Vanishing bile duct syndrome(VBDS) is a group of rare disorders characterized by ductopenia,the progressive destruction and disappearance of intrahepatic bile ducts leading to cholestasis.Described in association with medications,autoimmune disorders,cancer,transplantation,and infections,the specific mechanisms of disease are not known.To date,only 4 cases of VBDS have been reported in human immunodeficiency virus(HIV) infected patients.We report 2 additional cases of HIV-associated VBDS and review the features common to the HIV-associated cases.Presentation includes hyperbilirubinemia,normal liver imaging,and negative viral and autoimmune hepatitis studies.In HIV-infected subjects,VBDS occurred at a range of CD4+ T-cell counts,in some cases following initiation or change in antiretroviral therapy.Lymphoma was associated with two cases;nevirapine,antibiotics,and viral co-infection were suggested as etiologies in the other cases.In HIV-positive patients with progressive cholestasis,early identification of VBDS and referral for transplantation may improve outcomes.
文摘Vanishing bile duct syndrome (VBDS) refers to a group of disorders characterized by prolonged cholestasis as a result of destruction and disappearance ofintrahepatic bile ducts. Multiple etiologies have been indentifi ed including infections, neoplastic disorders, autoimmune conditions and drugs. The natural history of this condition is variable and may involve resolution of cholestasis or progression with irreversible damage. VBDS is extremely rare in human immunodeficiency virus (HIV)-infected patients and anti-retroviral therapy has never been implicated as a cause. We encountered a young pregnant female with HIV and VBDS secondary to anti-retroviral therapy. Here, we report her clinical course and outcome.
文摘Amoxicillin/clavulanate is associated with liver injury, mostly of a cholestatic pattern. While outcomes are usually benign, progression to cirrhosis and death has been reported. The role of immunosuppressive therapy for patients with a protracted course is unclear. We report the case of an elderly patient who developed prolonged cholestasis secondary to amoxicillin/clavulanate. Vanishing bile duct syndrome was confirmed by sequential liver biopsies. The patient responded to prednisone treatment, but could not be weaned off corticosteroids, even when azathioprine was added. Complete withdrawal of both prednisone and azathioprine was possible by using mycophenolate mofetil, an inosine monophosphate dehydrogenase inhibitor. Sustained remission has been maintained for more than 3 years with low-dose mycophenolate mofetil.
基金Supported by National Institute of Health,NIH 5 T32 DK 7356-37(BN)
文摘Vanishing bile duct syndrome (VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin’s lymphoma (HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage IIB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.
文摘BACKGROUND: Nonsteroidal anti-inflammatory drugs (NSAIDs) have been reported to induce liver injury. Patterns of the injury usually range from mild elevations of liver enzymes to sometimes severe fulminant hepatic failure. Likewise, naproxen is a propionic acid derivative NSAID that was introduced in 1980 and has been available as an over-the- counter medication since 1994, but has rarely been reported to cause liver injury. METHODS: We treated a 30-year-old woman with jaundice and intractable pruritus that developed shortly after taking naproxen. We reviewed the medical history and liver histopathology of the patient as well as all previously published case reports of naproxen-associated liver toxicity in the English language literature. RESULTS: The liver biochemical profile of the patient revealed a mixed cholestasis and hepatitis pattern. Consecutive liver biopsies demonstrated focal lobular inflammation, hepatocyte drop-out, and a progressive loss of the small interlobular bile ducts (ductopenia). The biopsy performed two years after onset of the disease showed partial recovery of a small number of bile ducts; however, 10 years passed before the biochemical profile returned to near normal. CONCLUSIONS: Naproxen-associated liver toxicity remains a rare entity, but should be considered in any patient presenting with cholestasis shortly after its use. Liver injury is most commonly seen in a mixed pattern characterized by cholestasis and hepatitis. The resulting liver damage may take years to resolve.
文摘病例：患者男，24岁。因“反复皮下瘀斑8年，身目黄染3个月”于2012年3月11日收治入院。患者8年前四肢受到轻微碰撞后出现大面积皮下瘀斑伴牙龈出血，外院予止血治疗（具体不详）后瘀斑消退，但该症状常反复。1年前再次因皮下瘀斑至外院就诊，检查显示凝血酶原时间（PT）120 s，活化部分凝血活酶时间（APTT ）107．8 s，凝血因子 FⅦ、FⅨ下降，ALT 372 U ／L，AST 386 U ／L，ALP 1633 U ／L，甲状旁腺素173．9 pg／mL，骨扫描未见异常，骨髓穿刺活检提示刺激性骨髓象，予“止血、护肝”等对症治疗，皮下出血症状好转后出院。11个月前，复查 ALT 298 U ／L，AST 246 U ／L，继续口服多烯磷脂酰胆碱治疗。10个月前，患者自行停药并服用中草药（具体不详）治疗半年，4个月前出现排便次数增多（2～3次／d），量少，黄色水样便，不含黏液脓血，气味恶臭，禁食后缓解。予止泻药物治疗效果不佳。3个月前，患者再次出现四肢皮下瘀斑，伴进行性加重的身目黄染，无腹胀、纳差，无恶心、呕吐，无畏寒、发热。外院复查：ALT 540 U ／L， AST 413．2 U ／L，ALP 3236 U ／L，总胆红素（TBIL）339μmol／L，直接胆红素（DBIL）268μmol／L，PT 17．2 s，总胆固醇14．2 mmol／L，予“止血、护肝、退黄”治疗效果不佳，遂转入我院。患者否认“肝炎、结核”病史，否认输血史、饮酒史、静脉注射毒品史、高危性行为史，否认曾服用“氯磺丙脲、丙氯拉嗪、甲苯磺丁脲”等药物，无已知毒物暴露史，无肝脏疾病家族史，否认食物、药物过敏史。
文摘BACKGROUND Muscle growth promoters are being developed for the treatment of diseaseinduced loss of muscle mass.Ligandrol and ostarine are selective androgen receptor modulators(SARMs)with a non-steroidal structure and a presumably more favorable side effect profile.In recent years,these substances with or without“post-cycle therapy”(PCT)are often misused by amateur athletes aiming to promote muscle growth.At the same time,reports on their toxic effects on organ systems are emerging.CASE SUMMARY We report two cases of liver injury in young men who used ligandrol and/or ostarine for a few weeks followed by the use of substances for PCT.Acute liver injury occurred in both cases after stopping SARMs while on PCT.The clinical picture was dominated by jaundice and fatigue.The biochemical pattern showed a mixed type of injury with normal alkaline phosphatase and high concentrations of bilirubin and serum bile acids. Histological evidence showed predominantlycholestatic injury with canalicular bile plugs, ductopenia, and mild hepatocellulardamage without significant fibrosis. The patients recovered from the conditionafter 3 mo. The off target effects of SARMs were likely idiosyncratic, but ourreport highlights the yet unrecognized effects of other toxic substances used forPCT, supra-therapeutic doses, and the complete absence of monitoring foradverse effects.CONCLUSIONAmong muscle-building amateur athletes, SARMs (ligandrol or ostarine) and/orsubstances in PCT may cause cholestatic liver injury with prolonged recovery.
文摘肝内胆管缺失是指肝内胆管数量的减少或消失,是胆管树基本病理改变之一。免疫紊乱、肿瘤、感染、药物、缺血、遗传等因素都有可能引起胆管缺失。临床上,通过肝活组织检查,在10个及以上门管区的标本里,发现50%以上的小叶间胆管缺失,即可确诊[1]。胆管缺失的预后取决于病因和损伤程度。晚期出现不可逆的广泛胆管缺失甚至胆管消失时,该病理综合征称为胆管消失综合征(vanishing bile duct syndrome,VBDS),仅发生在0.5%小胆管病[2]。随后,可进展至广泛的胆管纤维化或肝硬化。有趣的是,也有部分患者表现为胆管上皮细胞再生,在数月或数年后得到恢复。本文总结了胆管缺失发病的分子机制,并重点强调近年来免疫介导的胆管病和胆管缺失的研究进展。
