12例肝结节状再生性增生临床分析

目的对肝结节状再生性增生（NRHL）的临床、病理及诊断分析总结，期望提高临床医师对本病的认识。方法从300例因脾功能亢进而行脾切除和肝脏活组织检查病例中选出病理诊断符合NRHL的病例12例，分析其病史、临床症状和体征、实验室检查、诊断及处理等资料，且随访治疗效果。结果12例NRHL中6例明确诊断为系统性红斑狼疮，1例克罗恩病，1例疑诊溃疡性结肠炎。应用肾上腺皮质激素治疗6例，免疫抑制剂3例。11例有门脉高压；所有患者均无病毒性肝炎史；肝功能轻度受损；病理特征为肝实质内结节状再生性增生，门脉周围轻度纤维化和汇管区散在淋巴细胞浸润，门静脉分支狭窄和闭塞，无肝坏死表现；术前均被诊断为肝硬化伴门脉高压；行手术治疗后临床症状明显缓解，随访患者多数病情稳定。结论NRHL可能与免疫和肝脏血液循环障碍有关；以门脉高压为主要表现，应与肝硬化鉴别；诊断依靠肝脏楔形活检；处理门脉高压可使临床状况得到改善。     

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.     

狼疮性肾炎与肝结节状再生性增生一例

患者男 ,2 4岁。因水肿、尿少 5个月 ,加重 3ｄ ,于 2 0 0 1年 6月 1 5日入院。患者于 5个月前无诱因水肿、腹胀、尿少 ,Ｂ超示肝脾增大 ,诊为“肝硬化” ;服中药 2周 ,无效。转院后发现血肌酐 (Ｓｃｒ) 32 0 μｍｏｌ/Ｌ ,双肾增大 ,肾穿刺活检怀疑“新月体肾炎” ,未经治疗而转入我院。入院前 3ｄ尿量减少至 2 0 0ｍｌ/ｄ ,Ｓｃｒ由 32 0 μｍｏｌ/Ｌ升至 72 6μｍｏｌ/Ｌ。 1 2年前发现患者乙肝病毒标志物异常 ,具体不祥 ,肝功能正常。 2年前发现脾大。无关节痛、皮疹、光过敏和口腔溃疡。体检 :ＢＰ1 4 0 / 60ｍｍＨｇ(1ｍｍＨｇ=0 1 3…     

肝脏结节状再生性增生1例

1病例报告患者,女,40岁,汉族。主因脾脏肿大5年,乏力、纳差、腹胀,3月入院。患者2000年体检时B超显示脾脏肿大,因无任何不适,未行进一步诊治。2004年12月在无明显诱因下出现乏力、纳差,每日进食减至病前3/4量,并感餐后腹胀明显。外院实验室检查显示:ALT110U/L,AST81U/L,GGT62U/     

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。     

先天性肝纤维化14例临床病理特点

目的总结先天性肝纤维化的临床特点,描述先天性肝纤维化时产生门静脉高压的病理形态学特点。方法回顾性分析2000年～2009年9月,北京友谊医院门诊及住院诊断为先天性肝纤维化患者的临床特点,对活检肝组织标本进行特殊染色及免疫组织化学染色。结果先天性肝纤维化14例,男女比例1.33：1,平均年龄（14.78±8.52）岁,临床主要表现为门脉高压症型占57.1%,胆管炎型占14.29%,混合型占7.14%。肝脾肿大为最主要的体征占85.7%。常见的合并症为多囊肾和肝内胆管扩张,即Caroli’s病各占14.28%。病理形态学观察提示先天性肝纤维化导致门脉高压的机制包括：门静脉发育不良,门脉数量减少,纤维间隔挤压门静脉。结论先天性肝纤维化是导致非肝硬化性门脉高压症的常见原因之一,掌握其临床及病理特点,有助于提高诊治水平。     

原发性肝Ca和肝局灶性增生结节的超声临床对比分析

目的比较原发性肝Ca和肝局灶性增生结节的临床特点,为临床诊断提供理论依据。方法选取我院收治的25例原发性肝Ca患者以及25例肝局灶性增生结节患者,入选病例均经病理学检查确诊,经患者允许,均给予超声造影检查,做好相关记录。结果原发性肝Ca的造影方式表现为典型的快进快退型,肝局灶性增生的造影方式呈中央向周边逐渐充填,除充盈方式不同外,肝局灶性增生病灶的消退时间显著短于原发性肝Ca（P〈0.05）。结论超声造影检查可有效显示肝局灶性结节增生及原发性肝Ca的充填方式及充填过程,能清除鉴别病变类型,值得在临床上应用并且具有较高的临床诊断价值。     

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。     

肝癌误诊为肝脏局灶性结节性增生5例临床分析

目的分析5例肝癌误诊为肝脏局灶性结节性增生(FNH)的原因,探讨FNH与肝癌的鉴别诊断。方法回顾性分析2008年1月-2011年12月间术前误诊为FNH的5例肝癌患者的临床资料。结果彩超检查发现肝内低回声或不均质回声肿物,增强CT检查3例,增强MRI检查2例,部分病例可见中央瘢痕。术前诊断为FNH,所有病例均行手术切除,病理为肝细胞癌。结论合并肝炎时诊断FNH一定谨慎,正确应用和分析增强CT和MRI检查是诊断正确的关键。     

先天性肝纤维化1例

<正>患者,女,21岁,安徽省临泉县人。因"体检发现贫血、脾大十天"入院。患者于2014年7月14日因上呼吸道感染到河北医科大学第二医院就诊,查血常规:WBC 3.64×109/L、N0.745、RBC 3.56×1012/L、HGB 96 g/L、PLT 47×109/L。进一步查肝功能:TP 60.5 g/L,A/G 36.5/24 g/L,TBil/DBil 5.7/2.9μmol/L,ALT 13 U/L、AST 16 U/L、ALP 33 U/L、GGT 11 U/L,GLU 4.86 mmol/L,血脂:CHOL 3.14 mmol/L,TG 0.72 mmol/L;     

Nodular regenerative hyperplasia: evolving concepts on underdiagnosed cause of portal hypertension

Nodular regenerative hyperplasia(NRH)is a rare liver condition characterized by a widespread benign transformation of the hepatic parenchyma into small regenerative nodules.NRH may lead to the development of non-cirrhotic portal hypertension.There are no published systematic population studies on NRH and our current knowledge is limited to case reports and case series.NRH may develop via autoimmune,hematological,infectious,neoplastic,or drug-related causes.The disease is usually asymptomatic,slowly or nonpr...     

Human immunodeficiency virus and nodular regenerative hyperplasia of liver: A systematic review

AIM: To investigate the diagnosis, pathogenesis, natural history, and management of nodular regenerative hyperplasia(NRH) in patients with human immunodeficiency virus(HIV). METHODS: We performed a systematic review of the medical literature regarding NRH in patients with HIV. Inclusion criteria include reports with biopsy proven NRH. We studied the clinical features of NRH, in particular, related to its presenting manifestation and laboratory values. Combinations of the following keywords were implemented: "nodular regenerative hyperplasia", "human immunodeficiency virus", "noncirrhotic portal hypertension", "idiopathic portal hypertension", "cryptogenic liver disease", "highly active antiretroviral therapy" and "didanosine". The bibliographies of these studies were subsequently searched for any additional relevant publications.RESULTS: The clinical presentation of patients with NRH varies from patients being completely asymptomatic to the development of portal hypertension – namely esophageal variceal bleeding and ascites. Liver associated enzymes are generally normal and synthetic function well preserved. There is a strong association between the occurrence of NRH and the use of antiviral therapies such as didanosine. The management of NRH revolves around treating the manifestations of portal hypertension. The prognosis of NRH is generally good since liver function is preserved. A high index of suspicion is required to make a identify NRH. CONCLUSION: The appropriate management of HIVinfected persons with suspected NRH is yet to be outlined. However, NRH is a clinically subtle condition that is difficult to diagnose, and it is important to be able to manage it according to the best available evidence.     

Human immunodeficiency virus and nodular regenerative hyperplasia of liver:A systematic review简

AIM: To investigate the diagnosis, pathogenesis, natural history, and management of nodular regenerative hyperplasia (NRH) in patients with human immunodeficiency virus (HIV).METHODS: We performed a systematic review of the medical literature regarding NRH in patients with HIV. Inclusion criteria include reports with biopsy proven NRH. We studied the clinical features of NRH, in particular, related to its presenting manifestation and laboratory values. Combinations of the following keywords were implemented: “nodular regenerative hyperplasia”, “human immunodeficiency virus”, “noncirrhotic portal hypertension”, “idiopathic portal hypertension”, “cryptogenic liver disease”, “highly active antiretroviral therapy” and “didanosine”. The bibliographies of these studies were subsequently searched for any additional relevant publications.RESULTS: The clinical presentation of patients with NRH varies from patients being completely asymptomatic to the development of portal hypertension – namely esophageal variceal bleeding and ascites. Liver associated enzymes are generally normal and synthetic function well preserved. There is a strong association between the occurrence of NRH and the use of antiviral therapies such as didanosine. The management of NRH revolves around treating the manifestations of portal hypertension. The prognosis of NRH is generally good since liver function is preserved. A high index of suspicion is required to make a identify NRH.CONCLUSION: The appropriate management of HIV-infected persons with suspected NRH is yet to be outlined. However, NRH is a clinically subtle condition that is difficult to diagnose, and it is important to be able to manage it according to the best available evidence.     

Nodular regenerative hyperplasia related portal hypertension in a patient with hypogammaglobulinaemia

Nodular regenerative hyperplasia (NRH) of liver is a relatively rare liver disorder, but a frequent cause of noncirrhotic portal hypertension. We present a lady with common variable immune deficiency who presented with upper gastrointestinal bleeding and deranged liver function tests but preserved synthetic function. Upper gastrointestinal endoscope showed bleeding gastric varices and non-bleeding oesophageal varices. Although her oesophageal varices were eradicated by repeated endoscopic band ligation, the gastric varices failed to resolve after repeated endoscopic histocryl injection and she eventually needed transjugular intrahepatic portosystemic shunt placement. Liver biopsy showed NRH. We review the association of hypogammaglobinaemia and NRH and discuss the appropriate management of portal hypertension in NRH.     

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

Abstract

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.     

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.