肝结节状再生性增生18例临床分析

我院自1983年至2008年共收治18例肝结节状再生性增生(NRH)患者,现将其临床资料总结分析如下. 一、临床资料 1.一般资料:本组患者男6例,女12例,发病年龄22～70岁(中位年龄39岁),病程1个月～6年.18例患者均无肝脏病史,其中3例因体检发现肝脏占位性病变就诊,15例因门静脉高压症就诊.后者中10例合并自身免疫性疾病,其中确诊系统性红斑狼疮5例,未分化型结缔组织疾病4例,炎症性肠病1例.     

先天性肝纤维化——附12例临床分析

目的分析先天性肝纤维化的临床特征。方法对12例先天性肝纤维化患者临床表现、实验室检查及病理组织学特点进行回顾性分析。结果12例患者均有明显临床症状,10例有典型门脉高压症状,11例就诊时肝功能异常,肝脏病理主要表现为：肝组织内呈现宽大致密且炎症不明显的胶原纤维间隔,或纤维束弥漫穿插于固有的肝小叶内,无典型的假小叶结构。结论对于不明原因肝功能异常的门脉高压症患者,应尽可能进行肝组织活检病理检查以协助诊断及指导治疗。     

门脉高压症的分型和检查方法

门脉高压多由肝硬化引起。凡肝脏弥漫性病变(炎症、变性、坏死)后,发生纤维组织异常增生和结节过度形成,以及门脉或肝静脉系某些疾病,能影响门脉血循环,在长期增加门脉阻力的情况下,就可导致门脉高压。近来认为,一些液递物质可增加门脉或肝静脉系血管的阻力或血流最,形成高动力循环状态。这一点对形成门脉高压亦很重要。根据门脉系统病变和受阻部位,门脉高压症一般可分为以下三类:     

左侧门脉高压症

<正>左侧门脉高压症(left-sided portal hypertension,LSPH)亦常称作"胰源性门脉高压症""区域性门脉高压症""脾胃区门脉高压症",其病因多样,但根本原因为脾静脉阻塞,脾静脉血液回流障碍,继发脾胃区静脉压增高,导致相应的临床症状。因其临床表现较隐匿,与肝硬化所致的门脉高压症在临床症状上有一定的相似性,病例相对少见,加之对该疾病认识不足,较易     

特发性门静脉高压症1例报告并文献复习

特发性门静脉高压症（Idiopathic portal hypertension,IPH）是一种病因未明,以窦前性门脉压增高为主要特征,主要临床表现为门脉高压,显著脾脏肿大伴脾功能亢进,而肝功能基本正常的综合征。IPH临床少见,现将本科近期肝脏活检确诊的1例IPH,结合文献复习报道如下。     

门脉高压症脾切除术后门静脉系统血栓形成的诊治(附18例报告)

对18例门脉高压症脾切除术后门静脉系统血栓形成患者的临床资料进行分析。结果术后13例出现不明原因发热,9例有腹痛症状;18例均有血小板计数、D-D二聚体水平增高;12例经彩色多普勒超声、6例经强化CT检查确诊;18例均经溶栓及抗凝治疗后痊愈。认为不明原因门脉高压症患者脾切除术后门静脉系统血栓形成的常见症状为发热及腹痛,血小板及D-D二聚体检测有助于早期诊断及预防,彩色多普勒超声及强化CT可以明确诊断,溶栓及抗凝治疗是有效的治疗措施。     

内镜套扎-部分脾栓塞联合术治疗门脉高压症的临床疗效观察

探讨内镜套扎-部分脾栓塞联合术治疗门脉高压症的临床应用价值。对2 5例合并食道静脉曲张和脾功能亢进的门脉高压患者进行内镜套扎-部分脾栓塞联合术,对其术前术后外周血象、脾脏大小、门静脉、脾静脉内径的变化进行对比研究。联合术后患者食道静脉曲张得到根治,脾功能亢进明显缓解,脾脏缩小(P <0 .0 1)、门静脉、脾静脉内径缩小(P <0 . 0 1)。内镜套扎-部分脾栓塞联合术治疗门脉高压症具有简便、安全、效果显著、创伤小、并发症少等优点,是治疗肝硬化门脉高压症的一种新的途径。     

应重视门静脉高压症的病因诊断及规范治疗

门静脉高压症是由门静脉系统阻力/血流增加或肝静脉系统回流受阻所导致的门静脉压力异常升高,常表现为脾大、脾功能亢进、食管胃静脉曲张及破裂出血、腹水及肝性脑病等。肝硬化所引起的窦性门静脉高压症临床最为常见,通常伴有肝脏合成功能障碍。而肝外、肝内门静脉系统异常所致的肝(窦)前门静脉高压,以及肝内、肝外肝静脉系统回流受阻所致的肝(窦)后性门静脉高压,多无明显肝脏合成功能障碍。可首先根据临床症状、体征及血液学指标判断有无门静脉高压症,再通过影像学、肝静脉压力梯度及组织病理学检查明确其类型和病因。应在积极治疗原发病的基础上,采取非选择性β受体阻断剂、内镜、介入或手术治疗,以防治门静脉高压症的并发症。     

肝硬化门脉高压发生机理

肝硬化门脉高压症常出现顽固性腹水、胃底与食管静脉曲张破裂出血和肝功能衰竭等严重并发症,因此对其发生机理的认识尤显重要。一、门脉高压的分类和机理以往将门脉高压分为窦前和窦后性,最近有人从血流动力学角度将门脉高压分为流出障碍（门脉系统流出血管阻力增大）和流入增加（流入门脉系统的动脉血流量增加）两大类,具体病因如表１,这一分类使对门脉高压的病因能一目了然。二、肝硬化门脉高压发生机理肝硬化是一种慢性进行性肝病,是门脉高压的最主要病因,其组织病理学有以下特点：（１）弥漫性病变,大体标本可见结节形成;（２）…     

肝硬化和门脉高压症无创性诊断方法评价

肝硬化发主要生于慢性肝脏疾病的终末阶段,且常并发门静脉高压症。门脉高压症是引起肝硬化患者发病或死亡的重要因素。本文从实验室指标、瞬时弹性成像和多普勒超声技术等方面综述目前应用于临床的肝硬化和门脉高压症的无创性诊断及预后评估的方法。虽然这些方法尚不能替代侵入性的肝活检和肝静脉压力梯度测量以及胃食管静脉曲张的内镜检查,但是它们可以协助肝硬化和门脉高压症患者的治疗并提供有价值的预后评价。     

Diagnostic problems in nodular regenerative hyperplasia (nodular transformation) of the liver. Review of the literature and report of two cases.

Nodular regenerative hyperplasia (NRH) is a rare lesion of the liver associated with portal hypertension in more than half of patients. We present two cases demonstrating complications and diagnostic problems of NRH and review the pathogenesis, clinical, radiologic, and pathologic features of 240 cases in the literature. Patient 1 died from variceal bleeding as a complication of NRH. Patient 2 presented with ascites. Sonographic, computed-tomographic and magnetic resonance findings did not differ from liver cirrhosis. Three needle biopsies showed nonspecific reactive hepatitis. Wedge liver biopsy provided the correct diagnosis of NRH and a shunt operation was performed. Non-Hodgkin's lymphoma (centroblastic type) was diagnosed three years after NRH. At present there is no clinical or radiologic evidence of progression of NRH in this patient. The diagnosis of NRH cannot be made without histologic examination. Correct diagnosis is difficult in percutaneous needle biopsy. Therefore, laparoscopically guided liver biopsy or wedge biopsy is often necessary for diagnosis. NRH should be included in the differential diagnosis of portal hypertension. Portal diversion can be considered.     

Nodular regenerative hyperplasia of the liver in rheumatic diseases: report of seven cases and review of the literature

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely pathogenetically. NRH may lead to portal hypertension. Thus, the diagnosis of Felty's syndrome must be considered with caution in patients with rheumatoid arthritis (RA) and NRH of the liver. We report seven additional cases of NRH in patients with rheumatic disorders and review the literature to determine the patterns of clinical presentation and natural history of this condition. We also report four patients (three systemic lupus erythematosus [SLE] and one primary antiphospholipid syndrome [PAPS]) in whom antiphospholipid antibodies may have played a role in the genesis of NRH.     

Nodular regenerative hyperplasia of the liver and the Budd-Chiari syndrome. Case report, review of the literature and reappraisal of pathogenesis

We report a case of nodular regenerative hyperplasia (NRH) of the liver associated with the Budd-Chiari syndrome in a patient whose clinical and radiological presentation suggested a diagnosis of multiple liver tumours. Based on both our study and a review of the literature, it appears that, in a number of cases of NRH associated with various clinical conditions, blood stasis at the sinusoidal level is the common denominator. We postulate that, in this situation, the prolonged exposure of hepatocytes to blood-borne hepatotrophic substances, such as hepatopoietins, glucagon and insulin, in combination with functional loss due to pressure injury within the congested areas, may be one of the mechanisms leading to the development of NRH.     

Nodular regenerative hyperplasia of the liver. Study of 15 cases and review of the literature

The authors report 15 cases of nodular regenerative hyperplasia (NRH) of the liver observed in 10 women and 5 men during a 9 year period. Gastrointestinal bleeding due to ruptured esophageal varices revealed the liver disease in 11 cases. Hepatomegaly and splenomegaly were noted in 9 cases and ascites in 7. Anicteric cholestasis was demonstrated in 10 cases. Another disease, e. g. myelofibrosis and monoclonal gammapathy, was present in 11 patients. In 10 patients, portal diversion was performed; outcome being favorable with a follow-up of one to six years. The analysis of these cases and of the 113 previously published reports calls for the following comments: 1) In most cases, NRH is characterized by small-sized hepatocytic nodules scattered throughout the entire liver with no surrounding fibrosis; however this histological pattern may vary somewhat, with adjacent normal zones being found adjacent to typical cirrhotic fibrosis; although a precise morphometric study was not performed in our patients, obstruction of the tiny branches of intrahepatic portal veins was not observed. 2) Histological diagnosis of NRH is difficult and in most cases requires surgical biopsy specimens and specific coloration of the reticulin network. 3) NRH must be considered as a new cause of intrahepatic (sinusoidal or presinusoidal) portal hypertension and/or of chronic anicteric cholestasis. 4) A number of various conditions may be associated with NRH, the most frequent being Felty's syndrome and myeloproliferative disorders. 5) The pathogenesis of NRH remains unknown. 6) Portal diversion generally has a favorable outcome in this disease.     

6-thioguanine associated nodular regenerative hyperplasia in patients with inflammatory bowel disease may induce portal hypertension

BACKGROUND: Recent studies suggest an association between 6-thioguanine (6-TG) therapy and hepatic nodular regenerative hyperplasia (NRH) in patients with inflammatory bowel disease (IBD). An influence of 6-TG on portal pressure remains to be determined. The aim of the study was to examine the functional relevance of long-term 6-TG treatment on hepatic hemodynamics in IBD patients and its association with NRH. METHODS: Patients treated with 6-TG for IBD underwent measurement of the hepatic venous pressure gradient (HVPG) and liver biopsy. 6-TG therapy was stopped when NRH was diagnosed. If elevated, HVPG measurement was repeated after 1 yr. RESULTS: Twenty-six patients (15 women, 11 men; median age 41 yr, range 23-76) treated with 6-TG for 38 months (median; range 12-45) were included. Among 24 patients with sufficient liver biopsy, 6 patients (25%) were diagnosed with NRH. In these 6 patients, the HVPG was higher (median HVPG 7 mmHg, range 3-14) than in the 18 patients without NRH (median 3 mmHg, range 2-5; P < 0.001). In the patients with NRH, two had clinically significant portal hypertension (CSPH) (13 and 14 mmHg, respectively); in one patient the HVPG was slightly elevated (7 mmHg). No overt clinical signs of portal hypertension were observed. One year after stopping 6-TG therapy, HVPG decreased in all 3 patients with initially elevated HVPG levels. CONCLUSIONS: We demonstrate that IBD patients under long-term 6-TG therapy are at a substantial risk for developing NRH. NRH results in elevation of HVPG and may cause CSPH. Discontinuation of 6-TG therapy extenuates portal hypertension and may thus reduce the risk of complications.     

The pathogenesis of nodular regenerative hyperplasia of the liver associated with rheumatoid vasculitis.

Nodular regenerative hyperplasia (NRH) is an uncommon liver disease that typically develops in the setting of a chronic illness such as Felty's syndrome. Although the pathogenesis of NRH has not been defined, vasculitis has been postulated to play an important pathogenetic role in some cases of NRH, even though the association of NRH and vasculitis has been reported rarely. We describe two unusual cases of NRH in patients with Felty's syndrome complicated by rheumatoid vasculitis. Morphometric analysis demonstrated evidence of injury to the hepatic vasculature in the form of loss of small intrahepatic arteries and portal spaces, findings that support the hypothesis that hepatic arteritis plays a role in the development of NRH in cases associated with systemic vasculitis.     

Coexistence of nodular regenerative hyperplasia of the liver and pulmonary arterial hypertension in patients with connective tissue diseases: report of three cases and review of the literature

Nodular regenerative hyperplasia of the liver (NRH) is known to be a rare condition in patients with connective tissue diseases (CTD). In this report, we document three patients with CTD who had both NRH and pulmonary hypertension (PH). All three patients developed PH during their course and thereafter developed NRH. The clinical course of these patients suggests that circulatory disturbance caused by PH might be a trigger for NRH.     

Ascites as the sole clinical manifestation in a patient with nodular regenerative hyperplasia

Nodular regenerative hyperplasia (NRH) of the liver is an infrequent entity that is usually diagnosed after the appearance of clinical signs of portal hypertension such as hepatomegaly, splenomegaly or upper gastrointestinal bleeding due to esophageal varices, which are the most frequently found clinical manifestations in NRH. Ascites is a less frequent finding and has always been described in association with other manifestations of portal hypertension. We describe a new case of NRH with atypical presentation in which ascites was the sole clinical manifestation.     

MRI联合血清GPC3水平鉴别肝脏结节性增生病灶临床应用价值研究*

目的 探究磁共振成像(MRI)联合血清磷脂酰肌醇蛋白聚糖3(GPC3)水平在鉴别诊断肝脏结节性增生(NRH)病灶方面的临床价值。方法 2016年3月～2019年8月我院诊治的NRH患者79例,经手术组织病理学检查结果为局灶性结节性增生(FNH)25例,肝细胞腺瘤(LCA)18例,异型增生结节(DN)20例和肝细胞癌(HCC)16例。采用ELISA法检测血清GPC3水平,使用西门子Avanto 3.0 T超导MRI检查设备和ADW 4.6后处理工作站行MRI检查。应用受试者工作特征曲线(ROC)分析MRI联合血清GPC3对四种病变的鉴别诊断价值。结果 HCC组血清GPC3水平为(2.2±0.4)ng/ml,显著高于FNH组或LCA组【分别为(1.0±0.2)ng/ml和(1.2±0.2)ng/ml,P<0.05】,而与DN组血清GPC3水平【(1.8±0.3)ng/ml,P>0.05】比,无显著性差异;MRI鉴别HCC、DN、FNH与LCA的准确率分别为88.0%(22/25)、88.9%(16/18)、70.0%(14/20)和75.0%(12/16),无显著性差异(P>0.05);MRI联合血清GPC3水平鉴别诊断HCC的AUC为0.760,显著大于诊断其他三种病变(分别为0.610、0.611和0.614,P<0.05)。结论 MRI联合血清GPC3水平检测诊断HCC具有较大的临床应用价值,值得进一步验证。     

Nodular regenerative hyperplasia of the liver and coeliac disease: potential role of IgA anticardiolipin antibody

Nodular regenerative hyperplasia (NRH) of the liver is a rare disorder that is often associated with connective tissue disorders, haematological malignancy, or drugs, and is a cause of non-cirrhotic portal hypertension. We describe two cases of NRH in individuals with adult coeliac disease and IgA anticardiolipin antibodies. We discuss the potential impact of this observation on the understanding of the pathogenesis of NRH.