肝结节性再生性增生1例

肝结节性再生性增生(Noducar regenerative hyperplasia of liver,NRHL)是一种临床少见的肝脏疾病,临床主要表现为非肝硬化性肝源性门静脉高压症,因对其临床病理特征缺乏认识,易引起漏诊或误诊,我院收治1例,现报道如下。     

特发性门静脉高压症的诊断与治疗

回顾性分析5例特发性门静脉高压症（IPH）患者的临床资料并进行随访。结果5例患者临床表现主要为反复消化道出血、脾大，肝功能各项指标基本正常；均伴脾功能亢进及食道（胃底）静脉曲张；病理表现为肝门静脉纤维化、肝脏结节再生性增生和不全分隔样硬化。术中发现所有患者肝脏形态、大小基本正常，术后均无再出血发生。     

特发性门静脉高压的肝脏病理学分析

耳的观察特发性门静脉高压的肝脏病理改变,明确病理诊断标准,并探讨临床病理联系。方法收集中日友好医院2005年1月-2007年3月病理确诊的特发性门静脉高压病例29例,对其肝组织行HE、网织纤维加Masson三色染色,以及α-平滑肌肌动蛋白、细胞角蛋白7、细胞角蛋白19免疫组织化学染色,并分析病变特点。结果29例中男9例,女20例。临床有门静脉高压、脾大等症状、体征。23例临床误诊为肝硬化。主要病理改变有：明显的汇管区纤维化,伴终末门静脉细小分支闭锁（缺乏）及不完全细纤维隔形成。部分门静脉支扩张并疝入小叶内。肝细胞萎缩及结节状再生相伴。结论特发性门静脉高压的肝脏病变具有一定的形态学特征,汇管区纤维化、门静脉小支闭锁,部分门静脉分支疝入肝实质,肝细胞萎缩及结节状再生相伴,较具诊断价值。     

对肝结节状再生性增生的再认识—附18例临床分析

目的通过对18例肝结节状再生性增生(nodular regenerative hyperplasia of liver,NRH)病例进行总结分析,以提高临床医师对此类疾病的认识。方法对北京协和医院1983年1月~2008年12月住院的18例NRH患者的临床表现、实验室检查、影像学检查及病理学检查等多项资料进行总结和分析。结果NRH临床上可表现为肝脏弥漫性病变和占位性病变,但以前者多见。门脉高压症是NRH常见的临床表现,以脾大、脾亢较为突出,肝功能仅轻度异常。NRH常合并自身免疫或胶原血管性疾病,此类患者常伴有多种自身抗体、ESR增快、Ig增高、γ%增高等异常。病理学检查肝脏内弥漫分布小的无纤维分隔的再生结节,其门静脉分支有不同程度的狭窄或闭塞。结论对临床上难以解释或不明原因的门脉高压症患者,尤其是合并自身免疫性疾病的患者,要警惕NRH的可能性。     

肝脏局灶性结节增生1例

肝脏局灶性结节状增生(focal nodularhyperplasia,FNH)临床少见,本文报道1例女性患者,因“体检发现肝脏占位4 d多”入院,常规体检行计算机断层扫描检查提示肝脏占位:肝细胞腺瘤?行手术探查及术后病理检查提示局灶性结节状增生,术后随访9mo,无复发及转移.本文系统探讨FNH的病因、发病机制、鉴别诊断及治疗等.     

肝脏局灶性结节增生的影像学表现、临床诊疗及预后分析

目的总结肝脏局灶性结节增生的临床特点、影像学表现(超声、CT、MRI)、临床诊治方法及预后。方法对我院病理证实的31例肝脏局灶性结节增生患者的临床病理资料进行回顾性分析。收集患者的性别、年龄、临床表现、影像学检查、病变部位、病灶最大径线、病理诊断等资料,统计超声、CT、MRI等检查方法对肝脏局灶性结节增生的正确诊断情况,并进行随访,统计并发症,观察疗效。结果 31例患者中,体检发现占位者21例,有症状表现者10例;AST轻度异常者2例,HBsAg阳性者6例,CA19-9水平升高者1例。影像学检查:(1)31例患者均行腹部彩超检查,诊断肝脏局灶性结节增生10例,诊断率为32.26%;9例患者加行超声造影检查,诊断肝脏局灶性结节增生7例,诊断率为77.78%;(2)31例患者均行肝脏平扫增强CT扫描,诊断肝脏局灶性结节增生12例,诊断率38.71%;(3)11例患者行上腹部MRI平扫及增强扫描,诊断肝脏局灶性结节增生8例,诊断率72.72%。MRI的诊断率明显高于彩超及CT(均P0.05)。病理学检查:所有病例均经病理学诊断为肝脏局灶性结节增生,病灶直径为2~11 cm,平均(4.6±2.1)cm。治疗方法及预后:31例患者均接受手术治疗,其中腹腔镜肝切除18例,开腹肝切除13例。与开腹肝切除比较,腹腔镜肝切除术中出血量更少(P0.05)、肠道排气时间和住院时间更短(P0.05);31例患者随访29例(93.55%),随访时间3~24个月,均存活,生活质量良好,无复发转移。结论肝脏局灶性结节增生是一种预后较好的肝脏良性增生性病变,多种影像学及病理学联合检查可提高诊断率,对于不能排除肝细胞癌等恶性肿瘤或其他肝脏占位性病变者应行手术切除治疗。     

非肝硬化性门静脉高压症的研究现状

肝硬化是门静脉高压的最常见原因,但仍有约20%的门静脉高压继发于非肝硬化因素,称为非肝硬化性门静脉高压症(NCPH),在发展中国家发病率较高。NCPH是一组异源性的肝脏血管疾病,临床上多见的是特发性门静脉高压(IPH)、肝外门静脉血管阻塞(EHPVO),以及布加综合征、先天性肝纤维化和结节再生性增生等少见病。此类患者常常具有门静脉高压的证据,如反复发生的静脉曲张出血和脾脏肿大,但肝功能保存尚好。目前尚无诊断NCPH的统一标准,对其诊断仍是一个挑战。临床上往往采用排除性诊断,必要时可行肝穿刺活组织检查来确诊。介绍了IPH和EHPVO的发病机制、病理表现、诊断方法及治疗策略的选择,若能有效控制上消化道出血,NCPH被认为是预后相对良好的一类疾病。     

特发性门脉高压肝移植术后随访第3年再发1例报告及文献复习

目的 复习1例特发性门脉高压(IPH)患者接受肝移植(LT)后第3年出现病情“再发”,并进行了相关文献复习,以提高对该病的认识。方法 报道1例我们诊治的IPH患者的病例资料,并检索MEDLINE、EMBASE、万方等数据库经LT治疗的IPH患者的研究报道,分析其治疗和转归。结果 本文报道的病例为57岁女性,因消化道出血、腹水行LT术,组织病理学检查诊断为IPH;术后随访第3年病情复发,行经皮肝穿刺活检术,病理学检查提示结节性再生性增生(NRH)、轻度汇管区炎症及纤维化,提示IPH再发;文献检索到81例LT治疗的IPH患者,其中42例在LT前诊断为肝硬化;LT后最长随访时间为248个月,8例死亡,其中5例分别在首次LT后3.5月～14年进行肝活检,组织病理学检查提示NRH,3例分别于LT后第7月、第3年和第14年出现具有门脉高压表现的NRH。结论 具有严重的门脉高压或肝功能衰竭的IPH患者需要LT治疗。少数患者在LT后可能出现IPH“再发”。     

先天性肝纤维化24例临床分析并文献复习

目的分析先天性肝纤维化的临床特征。方法对24例先天性肝纤维化患者临床症状、体征、实验室化验检查及病理学特点进行回顾性分析。结果 24例患者均有门脉高压表现,8例曾有消化道出血。化验肝功能正常或轻度异常。影像学检查提示7例存在肝肾囊肿。22例病理结果均提示先天性肝纤维化,其中9例合并先天性肝内胆管扩张（Caroli’s）病。结论对于病因不明门脉高压,尤其门脉高压与肝功损害程度不一致的患者应尽量行肝组织活检病理检查以协助诊断。先天性肝纤维化与肝肾囊肿、Caroli’s病常常伴发。     

慢性乙型肝炎256例肝穿刺活检分析

经临床血清学诊断为慢性乙型肝炎（慢乙肝），其中慢乙肝轻度126例，中度72例，重度11例，肝炎肝硬化47例。采用超声引导下肝自动活检术取得患者肝组织行病理检查，结果256例中，病理诊断慢乙肝轻度111例，中度30例，重度6例，肝硬化20例；与病理诊断比较，临床诊断慢乙肝轻度、中度、重度、肝硬化的符合率分别为88．1％、41．67％、54，55％、42．55％。认为慢乙肝患者只要无肝穿禁忌证，都应进行肝穿刺活检行病理检查确定诊断。     

Nodular regenerative hyperplasia of the liver. A review of 14 cases.

BACKGROUND/AIMS: Nodular regenerative hyperplasia of the liver, is a noncirrhotic liver disease, characterized by nodules in the hepatic parenchyma, which clinically presents primarily with manifestations of portal hypertension. The aims of this study are i) to review the clinical, histological and diagnostic aspects of 14 documented cases of NRHL, and ii) to assess the evolution and management of this condition in the cases reviewed. METHODOLOGY: The diagnosis of nodular regenerative hyperplasia of the liver was based on liver biopsy in all cases. Imaging studies (ultrasonography, computed tomography scan and magnetic resonance imaging scan) were performed as part of the diagnostic evaluation. Clinical manifestations and biochemical tests were recorded at the time of diagnosis. Management and prognosis were also reviewed. RESULTS: The most common clinical manifestations were those of portal hypertension, namely splenomegaly, esophageal varices and variceal bleeding. The histological findings were nodules in the hepatic parenchyma, the typical histologic feature of nodular regenerative hyperplasia of the liver, with mild periportal fibrosis and intraportal lymphocytic infiltration. Biochemical tests showed normal synthetic liver function, as evidenced by normal serum albumin, bilirubin and prothrombin time. Elevation of gamma-glutamyl transpeptidase and alkaline phosphatase due to cholestasis was noted. Management was directed to portal hypertension and variceal bleeding, with beta-blockers, sclerotherapy, mesenteric-caval shunt and transjugular intrahepatic portosystemic shunt with satisfactory results. CONCLUSIONS: Nodular regenerative hyperplasia of the liver is an uncommon condition but it should be considered in patients with unexplained portal hypertension and distinguished from liver cirrhosis, in view of the differences in the natural history and prognosis. Liver biopsy confirms the diagnosis. Management is directed primarily to portal hypertension and variceal bleeding, which is the main source of mortality. Liver failure is uncommon due to satisfactory preservation of liver function.     

Nodular regenerative hyperplasia of the liver in a patient with systemic sclerosis

Summary We report on a 33-year-old female patient with systemic sclerosis and nodular regenerative hyperplasia of the liver (NRHL). A needle biopsy of the patient's liver did not reveal the histology of NRHL or liver cirrhosis at her first visit to our hospital, when portal hypertension was demonstrated by percutaneous transhepatic portography. After 11 years, the patient died of hepatic and renal failure. At the time of autopsy, multiple nodules were found in the liver, and a microscopic examination showed a histology compatible with NRHL. It is suggested that the immunological disturbance was related to the patient's portal hypertension and NRHL.     

48例疑难肝病临床与病理分析

目的：评价肝活组织穿刺检查对疑难肝病的诊断价值。方法对48例临床不能确诊的疑难肝病患者行肝活组织穿刺检查,分析其临床与病理组织学特点。结果48例疑难肝病患者中,肝脏占位性病变24例（其中肝细胞癌7例,转移性肝癌7例,胆管细胞癌2例,肝脓肿3例,慢性肝炎重度1例,肝结核1例,肝海绵状血管瘤1例,未能确诊2例;慢性肝炎与肝硬化鉴别困难者12例（慢性肝炎轻度3例,慢性肝炎中度4例,慢性肝炎重度1例,早期肝纤维化1例,肝硬化3例）;隐源性肝炎5例（急性淤胆型肝炎1例,慢性肝炎中度4例）;隐源性肝硬化3例（结节性肝硬化1例,特发性门静脉高压2例）;慢性肝炎中度伴结节性肝硬化1例;急性淤胆型肝炎1例;自身免疫性肝病1例（原发性胆汁性肝硬化）;肝脏寄生虫病1例。病理组织学确诊46例。结论疑难肝病中以肝脏占位性病变多见,其中以原发性肝癌与转移性肝癌最常见。对疑难肝病行肝活组织穿刺检查可明显提高诊断率。     

Nodular regenerative hyperplasia of the liver: an important cause of portal hypertension in non-cirrhotic patients

In our hospital over the last 10 years a diagnosis of nodular regenerative hyperplasia was made for 13 patients. Sixty-nine percent of these patients had portal hypertension, representing 27% of all our patients with portal hypertension and a non-cirrhotic liver. Nodular regenerative hyperplasia was the second most frequent cause of portal hypertension in patients without cirrhosis. To make the diagnosis, a reticulin staining of a surgical biopsy is most helpful. However, the characteristic derangement of the liver architecture on histology may still be overlooked. In this study a suggestive relation was found between malignant disease (multiple myeloma, chronic myelogenous leukaemia, Leydig cell tumour and Hodgkin's disease), the use of cytotoxic or immunosuppressive drugs and nodular regenerative hyperplasia. Furthermore, a high rate of symptomatic nodular regenerative hyperplasia was observed in patients following kidney transplantation. Liver function abnormalities developed in these patients after a period ranging from 8 months to 3 years of immunosuppressive- or chemotherapy. These liver function abnormalities were, however, usually mild. Since hepatic encephalopathy is not likely to develop in these patients with nodular regenerative hyperplasia a decompressive shunt operation is a good alternative approach, if not the treatment of choice, for the prevention of recurrent variceal haemorrhage.     

Orthotopic liver transplantation for idiopathic portal hypertension: indications and outcome

BACKGROUND: Idiopathic portal hypertension is a rare clinical syndrome which may be associated with a spectrum of histological lesions, including nodular regenerative hyperplasia and incomplete septal cirrhosis. Here, we report eight adult patients with idiopathic portal hypertension who experienced an unusually severe clinical evolution characterized by the development of progressive hepatic failure requiring orthotopic liver transplantation. Our aims are: (a) to stress the distinctive clinical presentation of these patients, (b) to describe their biological and histopathological features, and (c) to evaluate the results of orthotopic liver transplantation in this rare indication. METHODS: Complete clinical charts and histological data were available in all patients. All patients were male. Their age at diagnosis ranged from 17 to 59 years. Complications of portal hypertension revealed the disease in all cases. Medical treatment was performed in all patients and portosystemic shunt in three. RESULTS: The development of progressive hepatic failure led to the indication of liver transplantation after a delay ranging from 3 to 10 years. Explanted livers showed pure nodular regenerative hyperplasia in three patients and incomplete septal cirrhosis in five. Recovery was uneventful. All patients are alive, without recurrence of the disease. CONCLUSIONS: This report points to the existence of severe cases of idiopathic portal hypertension occurring without underlying or associated systemic disease and characterized by a poor clinical course and requiring liver transplantation.     

CASE REPORT: Incomplete septal cirrhosis with liver cell dysplasia

A 60-year-old woman was transplanted for end-stage alcoholic cirrhosis. The diagnosis of cirrhosis was made 13 years earlier on the basis of features of portal hypertension and a wedge liver biopsy. Liver function tests were subnormal except for a low prothrombin time. Unproven possible alcohol abuse was the only aetiological factor. Her condition remained unchanged until transplantation, despite complete abstinence. Histological examination of the explant showed incomplete septal cirrhosis associated with distal obstructive portal venopathy, cirrhotic nodules predominantly in the subcapsular areas and nodular regenerative hyperplasia with septal fibrosis elsewhere. In addition, there were areas of large and small liver cell dysplasia. This observation shows the difficulty in making a diagnosis of incomplete septal cirrhosis and the hypothetical link between liver cell dysplasia (which has never been reported in incomplete septal cirrhosis but is well known to be associated with hepatocellular carcinoma in cirrhosis) and rare cases of liver adenomas and carcinomas reported in patients presenting with liver vascular disorders.     

An Adult Case of Congenital Extrahepatic Portosystemic Shunt Successfully Treated with Balloon-occluded Retrograde Transvenous Obliteration

A 42-year-old woman visited our hospital due to syncope. Contrast-enhanced CT revealed portosystemic shunt, portal vein hypoplasia, and multiple liver nodules. The histological examination of a liver biopsy specimen exhibited portal vein hypoplasia and revealed that the liver tumor was positive for glutamine synthetase. The patient was therefore diagnosed with congenital extrahepatic portosystemic shunt type II, and with focal nodular hyperplasia (FNH)-like nodules. She had the complication of severe portopulmonary hypertension and underwent complete shunt closure by balloon-occluded retrograde transvenous obliteration (B-RTO). The intrahepatic portal vein was well developed at 1 year after B-RTO, and multiple liver nodules completely regressed. Her pulmonary hypertension also improved.     

Portal hypertension in primary biliary cirrhosis

Evidence of portal hypertension was found in 50 out of 109 patients (47%) with primary biliary cirrhosis, and of these 32 bled from oesophageal varices. In four patients portal hypertension was the initial manifestation of the disease and this complication was recognized in a further 17 within two years of the first symptom of primary biliary cirrhosis. The development of portal hypertension was associated with a poor prognosis and death could frequently be attributed to variceal bleeding; the mean duration of survival from the time that portal hypertension was recognized was 14.9 months. Portal decompression operations may have improved the immediate prognosis in some patients but did not otherwise influence the progression of the disease. In 47 patients the histological findings in wedge biopsy or necropsy material were correlated with the presence or absence of varices. An association between nodular regeneration of the liver and varices was confirmed, but, in the absence of nodules, no other histological cause for portal venous obstruction could be found.     

特发性门脉高压症的临床病理学特点

目的 探讨特发性门脉高压症(idiopathic portal hypertension,IPH)的临床病理学特点.方法 回顾性分析了9例IPH的临床及病理学资料,并对其肝脏标本进行常规病理学及免疫组化研究.结果 9例IPH中,5例首发症状为上消化道出血和黑便,3例体检发现脾大脾亢而无临床症状,1例以血管瘤入院.人院检查脾肿大7例,胃底食道静脉曲张6例,腹水征4例,贫血者6例,肝功能正常或接近正常9例.病理组织学显示9例肝小叶结构基本正常.均未见假小叶形成及肝细胞坏死;9例均有不同程度汇管区纤维化,3例汇管区纤细的不全纤维间隔形成并向肝实质延伸,6例有门脉末支管壁纤维化;9例中有6例小叶内肝细胞有不同程度的水肿变性,5例肝窦有不同程度的扩张,2例肝窦扩张较明显,肝细胞萎缩,呈血管瘤样结构,2例有轻-中度肝腺胞3区大泡脂变.脾脏组织学符合淤血性脾肿大病理表现.结论 IPH的临床表现与其他原因所致的肝硬化门脉高压相似,肝穿组织病理学可除外肝硬化,并有一定的特征.诊断时应与各种原因所致肝硬化门脉高压,肝窦阻塞综合征等相鉴别.