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1.
We report three cases of nodular regenerative hyperplasia ofthe liver: the clinical onset of hepatic disease occurred between24 and 30 months after renal transplantation. Nodular regenerativehyperplasia was associated with peliosis hepatis in two cases,and with veno-occiusive disease in one case. Two patients developedportal hypertension, but are doing well. The third patient developedjaundice and died of septic shock. We discuss the aetiologicalrole of renal transplantation, cytomegalovirus infection, andazathioprine in the development of nodular regenerative hyperplasiaof the liver.  相似文献   

2.
Results of liver transplantation for nodular regenerative hyperplasia   总被引:1,自引:0,他引:1  
Liver transplantation has been performed in individuals with a pretransplant clinical diagnosis of cirrhosis but with nodular regenerative hyperplasia histologically. The purpose of this report is to investigate the results of liver transplantation in patients proven to have nodular regenerative hyperplasia post-transplant. A retrospective review was undertaken of four patients who underwent liver transplantation with a histologic diagnosis of nodular regenerative hyperplasia. All were felt to be cirrhotic on clinical grounds. Final histology of the explanted liver was confirmed by a single pathologist. Their ages ranged from 39 to 54 years, and three of the four were male. Three had pretransplant needle liver biopsies, two percutaneous and one transjugular. All revealed nonspecific reactive changes. Ultrasound and MRI were interpreted as consistent with cirrhosis in four of four and three of four cases, respectively. Portal vein flow was hepatopedal in three and absent in one. Pretransplant clinical characteristics and frequency were as follows: bleeding varices two, clinical ascites three, encephalopathy three, and impaired hepatic synthetic function two. All four patients underwent successful liver transplantation. There were no episodes of acute rejection. All are alive and well with normal graft function 2 to 4 years post-transplant. We conclude the following. 1) Patients with clinical end-stage liver disease due to underlying nodular regenerative hyperplasia can successfully undergo transplantation. 2) Nodular regenerative hyperplasia can present with signs and symptoms of liver failure, is difficult to diagnose by needle biopsy, and can be difficult to discriminate clinically from cirrhosis. 3) Although each case must be individually evaluated transplantation may be the optimal therapy in patients presenting with complications of liver failure.  相似文献   

3.
目的 分析总结肝结节性再生性增生(nodular regenerative hyperplasia of the liver,NRH)的临床诊治经验,以提高临床医师对本病的认识.方法 回顾性分析我院近26年连续收治的18例:NRH的临床表现、影像学及实验室检查、诊治及预后资料.结果 本组18例NRH患者中有15例表现为门静脉高压,4例表现为肝脏单发、多发占位,8例合并自身免疫疾病,3例可疑合并血液系统疾病.本组患者中术前13例被诊断为肝硬化,2例诊断为肝癌或局灶性结节性增生(focal nodular hyperplasia FNH).所有18例患者均行肝楔形活检,并且3例行脾切除,4例行断流术/Phemister术,3例行肝占位/肝叶切除术,1例行部分小肠切除术,1例行脾动脉缩窄限流手术.术后门静脉高压症状明显缓解.随访多数患者症状稳定,说明肝脏占位的:NRH患者预后良好.结论 NRH可能与肝脏血供紊乱有关,临床最常表现为门静脉高压,并可伴发免疫、血液系统性疾病.临床表现为单发、或多发性肝脏占位,应注意与肝硬化、局灶性结节性增生、特发性门静脉高压等鉴别,诊断依靠肝楔形活检.手术对于治疗门静脉高压疗效确切.
Abstract:
Objective To summarize the clinical diagnosis and treatment of nodular regenerative hyperplasia of the liver. Methods Retrospective analysis was made on the clinical manifestations,imagings, laboratory tests, diagnosis, treatment and prognosis of 18 consecutive cases finally established as NRH during the past 26 years. Results 15 of the 18 cases showed portal hypertension, 4 cases showed mono or multiple occupations of the liver, 8 cases suffered from concurrent autoimmune diseases, 3 cases were suspected of blood diseases. Preoperatively, 13 cases were diagnosed as cirrhosis, 2 cases were diagnosed as liver cancer or focal nodular hyperplasia ( FNH). All cases were diagnosed by operative wedging biopsy. 3 cases received splenectomy, 4 cases received disconnection /Phemister surgery, 3 cases received liver occupation/liver lobe resection, 1 case received partial small bowel resection, and 1 case received spleen artery restrictive surgery. Postoperatively, symptoms of portal hypertension relieved obviously. Follow-up study showed most of the patients were stable and prognosis of the NRH was good.Conclusions NRH may relate to the disturbance of liver blood supply, and most common clinical manifestation is portal hypertension, and can combine with immune diseases, hematopathy also can present single or multiple liver occupations. Differential diagnoses include liver cirrhosis, FNH, idiopathic portal hypertension. Diagnosis of NRH relies on liver wedging biopsy. Surgery can relive concurrent portal hypertension.  相似文献   

4.

Background

Hepatopulmomary syndrome is defined by the triad of chronic liver disease, increased alveolar-arterial gradient, and evidence of intrapulmonary vasodilation. It is commonly seen in association with cirrhosis (90%). Four percent to 8% of the hepatopulmomary syndrome cases are reported in noncirrhotic portal hypertension. The management of patients with hepatopulmomary syndrome due to noncirrhotic portal hypertension is not well described.

Methods

We report a case of a 26-year-old woman who underwent liver transplantation for hepatopulmomary syndrome due to noncirrhotic portal hypertension. The patient presented with dyspnea and platypnea, requiring home oxygen therapy. She had orthodexia, severe hypoxemia, and positive bubble echocardiography consistent with hepatopulmomary syndrome. Her Model for End-stage Liver Disease score was 10. Liver biopsy revealed diffuse nodular regenerative hyperplasia.

Results

The patient underwent liver transplantation with Model for End-stage Liver Disease exception points. Her oxygen requirements gradually improved during the postoperative period. The patient's symptoms and hypoxemia resolved at 15-month follow-up posttransplantation.

Conclusion

We suggest hepatopulmonary syndrome in this setting is an indication for liver transplantation despite the absence of cirrhosis.  相似文献   

5.
背景与目的:肝海绵状血管瘤是最常见的肝脏原发肿瘤。尽管通过影像学检查,针对该病的诊断程序已经非常成熟,但在罕见疾病组合的情形中仍有被误诊为肝细胞癌的风险。本文通过回顾1例术后被诊断为海绵状血管瘤合并结节性再生性增生的肝移植病例,分析诊疗过程中的关键疑难点,复习海绵状血管瘤和结节性再生性增生的临床病理特征以及其与肝细胞癌和肝硬化的鉴别要点,旨为今后临床工作提供经验与教训。方法:回顾性分析武汉大学中南医院收治的1例肝移植患者的临床病史资料、影像学特点及病理组织学诊断,结合国内外文献中肝海绵状血管瘤和结节性再生性增生的特点,对本病例诊疗过程进行分析和归纳。结果:患者,男性,71岁,因腹水及肝右叶占位性病变入院。影像学检查提示肝硬化、腹水及肝右叶占位性病变。全腹部CT平扫和增强诊断肝硬化合并肝细胞癌。经全科会诊与患者充分沟通后,患者进行了肝移植术。术中检查和术后大体检查发现肝脏呈弥漫结节性改变伴肝右叶占位性病变。组织学发现前者为结节性再生性增生,而占位性病变则为海绵状血管瘤。结论:通常情况下肝海绵状血管瘤不存在影像学诊断困难,但在弥漫结节性背景下,海绵状血管瘤仍可被误诊为肝细胞癌。结节性再生性...  相似文献   

6.
Intrahepatic noncirrhotic portal hypertension can be idiopathic or associated with known toxic, developmental, vascular, or biliary tract diseases. Most patients are successfully managed medically or with shunting procedures. The goal of this study was to explore the reasons some patients require orthotopic liver transplantation (OLT). The clinical features, gross and microscopic liver explant pathology, and posttransplantation course in 16 patients who underwent OLT for intrahepatic noncirrhotic portal hypertension were studied. There were 11 men and 5 women with a mean age of 47 years. Clinical manifestations included gastrointestinal varices (n = 12), ascites (n = 8), encephalopathy (n = 3), and hepatopulmonary syndrome (n = 3). Cirrhosis was misdiagnosed clinically, radiographically and/or histologically in 13 patients (81%). Grossly, liver explants weighed a mean of 1,100 g, and 12 had a nodular appearance. Histologically, all 16 livers had portal tract vascular abnormalities, 15 had nodular regenerative hyperplasia (NRH), and 9 had incomplete septal cirrhosis. After OLT, mild NRH features were noted in 2 patients, and 1 of these patients developed evidence of portal hypertension. This study demonstrates that a subset of patients with intrahepatic noncirrhotic portal hypertension have severe symptoms requiring OLT. Accurate pre-OLT diagnosis is frequently difficult at advanced stages of the disease; 81% of our patients carried a diagnosis of cirrhosis. Morphologically, the explanted livers showed evidence of vascular abnormalities, NRH, and increased fibrosis, but not cirrhosis. Importantly, however, a diagnosis of cirrhosis is not required in this group of patients to qualify them for OLT, and these patients have good long-term graft function after OLT.  相似文献   

7.
目的:研究辅助式肝移植行功能性分流治疗门静脉高压症肝脏血流动力学的变化规律及病理生理学机制。方法:回顾性分析2014年7月至2018年12月在首都医科大学附属北京友谊医院以活体供肝方式的小体积移植物行功能性分流治疗门静脉高压症患者临床资料,共纳入6例患者为研究对象,其中4例男性,2例女性,中位年龄35.5(29.0~5...  相似文献   

8.
Chronic biliary tract disease is the third most common indication for orthotopic liver transplantation (OLT) in the United States. Most patients undergoing OLT for chronic biliary tract disease have end-stage liver disease associated with cirrhosis, but a minority are transplanted in the precirrhotic stage for indications that can include poor quality of life (eg, intractable pruritis or fatigue), recurrent ascending cholangitis, or cholangiocarcinoma. A smaller subset of these patients suffer from severe noncirrhotic portal hypertension that can be associated with histologic features of nodular regenerative hyperplasia (NRH) and/or obliterative portal venopathy. We reviewed 306 liver explants performed for chronic biliary tract disease at 2 institutions during 1995 to 2003 to identify patients who were transplanted in the precirrhotic stage. The following clinical data were recorded: age, sex, type of biliary tract disease, radiology, clinical symptoms, signs of portal hypertension, pretransplant shunting procedures, time between diagnosis and OLT, and primary indication for OLT. Histopathologic data included: explant weight, gross appearance, fibrosis stage (1 to 4), cholangitis, bile duct dysplasia, malignancy, portal vein thrombi, presence of NRH, and presence of obliterative portal venopathy. Twenty-six of 306 (8.5%) patients underwent OLT in the precirrhotic stage (12 females: 14 males, mean age of 46 y, age range 12 to 68 y). At explant, fibrosis stage ranged from 1 to 2 (portal and periportal fibrosis) to 3 (multiple bridging fibrosis). Underlying biliary tract disease included primary sclerosing cholangitis (18 cases), primary biliary cirrhosis (5 cases), autoimmune cholangitis (2 cases), and secondary sclerosing cholangitis (1 case). Primary indications for OLT were recurrent cholangitis and/or decreased quality of life (11 cases), complications of portal hypertension (6 cases), portal hypertension plus cholangitis/decreased quality of life (5 cases), and malignancy (4 cases). Of the 11 patients with portal hypertension as a major indication for transplant, 2 had undergone transjugular intrahepatic portal-systemic shunting and 3 others had portal vein thrombi. Histopathologically, NRH was prominent in 8 of these 11 patients (73%) and obliterative portal venopathy in 6 (55%). NRH was also present in 4 of the 15 (27%) patients who were transplanted for other indications. These results indicate that precirrhotic portal hypertension is a predominant or major contributing factor to OLT in a significant minority (11 of 306, 3.3%) of patients with chronic biliary tract disease. The occurrence of NRH in some patients transplanted for other indications suggests it is a histologic pattern that can precede the development of clinically significant portal hypertension.  相似文献   

9.
Introduction  Liver disease and the development of hepatic fibrosis are complications associated with total parenteral nutrition (TPN). Patients developing cirrhosis and portal hypertension in the setting of intestinal failure have a high mortality and may require combined liver and intestinal transplantation which carries much higher morbidity and mortality than isolated intestinal transplantation. Discussion  Recently, regression of hepatic fibrosis in patients with TPN liver disease has been described following intestinal transplantation. To date, there has been no demonstration of the reversal of established cirrhosis due to long-term TPN injury. Herein, we describe a patient with intestinal failure who developed cirrhosis from long-standing TPN injury and underwent isolated intestinal transplantation. He had no overt clinical stigmata of portal hypertension and had preserved liver function. Serial liver biopsies were reviewed and assessed with standard histology and quantitation of fibrosis using image analysis. Dramatic regression of fibrosis and reversal of cirrhosis were observed 17 months posttransplantation. Image analysis demonstrated a 14% total decrease in the percentage area of fibrosis. Conclusions  Cirrhosis related to TPN may be rapidly reversible after isolated intestinal transplantation. Such patients may be able to undergo isolated intestinal transplantation if they do not have hepatic synthetic compromise or clinical stigmata of portal hypertension.  相似文献   

10.
BACKGROUND: Intrahepatic Osler's disease with multiple arteriovenous malformations and high intrahepatic shunting may lead to secondary pulmonary hypertension followed by right-heart stress and insufficiency. Until now, therapy with arterial embolization, banding, or ligation of the hepatic arteries is still limited and provides unsatisfactory long-term results. Liver transplantation offers another therapeutic option. METHODS: We report on four patients with intrahepatic involvement of Osler's disease who were liver transplanted between 1995 and 1999. All patients suffered from restricted liver function and right-heart insufficiency with multiple cardiac decompensations. One patient received one course of embolization, and another received six courses of embolization and then banding of the main hepatic artery before transplantation. In both patients, the clinical symptoms improved for only a few months. RESULTS: All patients had high degrees of intrahepatic arteriovenous shunting, and cardiac output measurements were between 8.0 to 13.3 L/min preoperatively. Preoperative mean pulmonary artery pressure was between 24 to 35 mmHg. After liver transplantation, cardiac output and right-heart diameter decreased or normalized and pulmonary pressure reached the normal range after 2 months. All patients received tacrolimus and steroids for primary immunosuppression. In one case, temporary hemodialysis was necessary for 2 weeks after transplantation, but renal function recovered completely. After follow-up time of 12 to 65 months, all patients had normal graft function and good cardiopulmonary condition. CONCLUSIONS: Indication for liver transplantation should be considered in patients with intrahepatic Osler's disease, high arteriovenous shunting with right-heart stress, and restricted liver function before irreversible fixed pulmonary hypertension leads to severe right-heart insufficiency or failure. Our therapeutic regimen of early liver transplantation in the case of intrahepatic Osler's disease in four patients has promising results.  相似文献   

11.
A 33-year-old man presented with hepatosplenomegaly and ascites26 months after receiving a successful renal transplant. Immunosuppressionconsisted of low-dose prednisolone and azathioprine. Biopsyshowed characteristic features of nodular regenerative hyperplasiaof liver (NRHL), a condition which may be related to azathioprinetherapy but has rarely been reported in association with renaltransplantation.  相似文献   

12.
Diffuse thrombosis of the entire portal system (PVT) and cavernomatous transformation of the portal vein (CTPV) represents a demanding challenge in liver transplantation. We present the case of a patient with nodular regenerative hyperplasia and recurrent episodes of type B hepatic encephalopathy concomitant with PVT as well as CTPV, successfully treated with orthotopic liver transplantation. The portal inflow to the graft was carried out through the confluence of 2 thin paracholedochal varicose veins, obtaining good early graft function and recovery of the encephalopatic episodes. This alternative should be kept in mind as an option to assure hepatopetal splanchnic flow in those cases of diffuse thrombosis and cavernomatous transformation of portal vein.  相似文献   

13.
Caroli's disease (including Caroli's syndrome) is a rare autosomal recessive disorder of the liver characterized by diffuse cystic dilatation of the intrahepatic bile ducts. The disease may present at any age and is characterized by recurrent episodes of biliary obstruction, cholangitis, hepaticolithiasis, and liver abscesses. Caroli's syndrome is further associated with congenital hepatic fibrosis and portal hypertension. Patients with recurrent complications or cirrhosis may die because of recurrent infection, portal hypertension, liver failure, or cholangiocarcinoma. Liver transplantation is the treatment of choice for these complicated patients. Here we describe the youngest reported patient with Caroli's syndrome treated successfully using liver transplantation and review the recent literature.  相似文献   

14.
目的 探讨肝移植治疗肝硬化门静脉高压症的临床疗效.方法 回顾性分析2000年1月至2012年1月北京大学人民医院收治的181例肝硬化门静脉高压症患者的临床资料.肝移植手术适应证为反复发作上消化道大出血,经内、外科和介入治疗无效,或合并肝功能失代偿的门静脉高压症患者.根据患者情况选择行经典原位肝移植或背驼式肝移植.术中于移植肝植入前后分别经胃网膜右血管置入套管针,连接测压管测压.观察手术前后门静脉压力变化情况,术后并发症的发生情况.术后通过肝移植随访中心定期随访,并根据具体指标调整用药,随访时间截至2012年12月,监测患者食管静脉曲张再出血及生存情况.Kaplan-Meier法计算生存率,计量资料采用(x)±s表示,均值比较采用t检验.结果 181例患者中,65例行经典原位肝移植,116例行背驮式肝移植.手术时间为(485±97) min,术中出血量为(4 380±1 993) mL,无肝期时间为(56±24) min.157例患者留置T管,24例患者未留置T管.102例患者术中经胃网膜右静脉测量了肝移植前后的门静脉压力,术前门静脉压力为(32±11)cmH2O(1 cmH2O =0.098 kPa),术后门静脉压力为(21±6)cmH20,手术前后门静脉压力比较,差异有统计学意义(t=2.412,P<0.05).肝移植术后严重感染23例、急性肾衰竭20例、严重腹腔内出血6例、血管相关并发症5例和移植物原发无功能2例.181例患者均获得随访,随访时间为6 ~131个月.138例患者术后1年复查内镜或行上消化道造影检查,112例曲张静脉完全消失,其余26例较术前明显减轻,总改善率为85.71%(138/161).术后1年内4例患者出现了上消化道再出血,再出血率为3.70%(4/108),其中3例经止血药物或内镜治疗后得到缓解,1例死于再次出血导致的肝衰竭.随访患者术后1个月、1年及5年生存率分别为86.8%、84.9%、77.4%.23例死亡患者中,15例死于MODS,5例死?  相似文献   

15.
A 50-year-old White man with noncirrhotic portal hypertension presented with bleeding from gastric varices. Bleeding was initially managed with band ligation and subsequent transjugular intrahepatic portosystemic shunt (TIPS). Over the next few months, the patient had recurrent episodes of anemia, jaundice, fever and polymicrobial bacteremia. Computed tomography (CT) of the abdomen and chest, upper and lower endoscopy, endoscopic retrograde cholangiopancreatography (ERCP), and echocardiography failed to explain the bacteremia and anemia. Follow-up CT scan and Doppler sonography 9 months after placement showed TIPS was occluded. Repeat ERCP showed a bile leak with free run-off of contrast from the left hepatic duct into a vascular structure. The patient's status was upgraded for liver transplantation with Regional Review Board agreement and subsequently received a liver transplant. Gross examination of the native liver demonstrated a fistula between the left bile duct and the middle hepatic vein. Pathologic evaluation confirmed focal necrosis of the left hepatic duct communicating with an occluded TIPS and nodular regenerative hyperplasia consistent with noncirrhotic portal hypertension. Infection is rarely reported in a totally occluded TIPS. Biliary fistulas in patent TIPS have been treated by endoluminal stent graft and endoscopic sphincterotomy with biliary stent placement. Liver transplantation may be the preferred treatment if TIPS becomes infected following its complete occlusion.  相似文献   

16.
Herein we report a case of liver dysfunction caused by consumption of vitamin A supplements leading to liver transplantation. The patient was a 48-year-old male with a medical history of congenital ichthyosiform erythroderma in treatment with vitamin A until 12 years of age, at which point he discontinued the supplements because he had developed ascites. Liver cirrhosis was diagnosed as secondary to hypervitaminosis A on the basis of histologic examination of liver biopsy and the absence of other potential causes of chronic liver disease. Despite interruption of administration of vitamin A, the patient continued to deteriorate over the years, with development of portal hypertension signs. His medical conditions were aggravated with the development of hepatic insufficiency manifested by refractory ascites, renal insufficiency, and severe encephalopathy and he underwent orthotopic liver transplantation, followed by disappearance of all signs of portal hypertension. This case highlights the need to take a careful history of consumption of vitamin A when evaluating a patient with liver failure.  相似文献   

17.
门静脉动脉化是一种为防止肝脏缺血导致的肝损害而将动脉血灌注入门静脉的方法.本文就其在肝移植、肝门部肿瘤和门脉高压症外科治疗、急性肝功能衰竭治疗中的临床应用情况及存在的问题做一综述.  相似文献   

18.
目的探讨肝硬变门静脉高压及其合并症对肝移植的影响及其治疗原则。方法对近年来有关肝硬变门静脉高压及其合并症对肝移植的影响的文献进行系统分析。结果肝硬变门静脉高压时脾肿大、脾功能亢进、门静脉血栓、门体分流、侧支循环等对肝移植手术产生不同程度的影响,增加了手术的难度和复杂性,术前和术中正确处理这些合并症,可有效提高肝移植的成功率。结论正确处理终末期肝硬变门静脉高压及其合并症,可拓宽肝移植的适应证、提高移植的成功率和远期疗效。  相似文献   

19.
In the present study, we investigated the effect of decompression of excessive portal hypertension by inferior mesenteric venous (IMV) left renal vein shunting in 7 cirrhotic patients with esophago-gastric varices and 2 patients who underwent adult living related donor liver transplantation (ALRDLT). The portal pressure remarkably decreased after shunting in all patients with esophago-gastric varices (388 ± 42 mm H2O vs. 247 ± 57 mm H2O; P < .05). It also decreased after a shunt operation in patients who had undergone liver transplantation. We report that the excessive shear stress by portal hypertension after small-for-size LRDLT induces a liver injury and the decompression of portal hypertension by splenic arterial ligation or splenectomy prevents postoperative liver injury following massive hepatectomy and small-for-size LRDLT. Our present studies suggested that IMV left renal vein shunting might prevent postoperative liver injury by partial decompression of excessive portal hypertension following small-for-size LRDLT.  相似文献   

20.
杨翔  郎韧  贺强  陈大志  李宁 《腹部外科》2004,17(6):324-326
目的 探讨肝肾序贯移植和同期联合移植的手术难点及围手术期处理要点。方法 对2例肾移植术后发生药物性肝损害的病例实施肝移植 ,并对 1例巨大多囊肝、多囊肾的病例实施肝肾联合移植。结果  2例肾移植术后实施肝移植的病例 ,其中 1例因术后肾功能衰竭导致多器官功能衰竭死亡 ;另 1例术后肝、肾功能良好 ,现已存活 1年。肝肾联合移植病例术中采用肝后腔静脉直接阻断法 ,使重达 10kg的巨大病肝得以顺利切除 ,并采用腔静脉成型术完成改良背驮式肝移植。术后免疫方案采用人源化单克隆抗体达利珠单抗免疫诱导下的以FK5 0 6、霉酚酸酯 (MMF)和激素的三联用药 ,肝、肾功能恢复良好 ,现为术后 6个月。结论 序贯性肝肾移植在术前应该准确评估移植肾功能 ,如果移植肾功能不良 ,应果断选择实施肝肾联合移植。肝后下腔静脉直接阻断法在实施巨大病肝切除时具有较大优势。肝肾联合移植术中及术后建议采用达利珠单抗免疫诱导下的免疫三联用药。  相似文献   

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