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。     

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。     

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。     

肝结节性再生性增生

肝结节性再生性增生南京军区南京总医院病理科印洪林综述周晓军审校肝结节性再生性增生（ｎｏｄｕｌａｒｒｅｇｅｎｅｒａｔｉｖｅｈｙｐｅｒｐｌａｓｉａＮＲＨ）是一种非常罕见的肝弥漫性不伴纤维化的结节性增生性病变，由于其临表现Ｂ超影像特征与肝硬化相似，病理组织...     

肝结节性再生性增生

肝结节性再生性增生(Nodular regenerative hy-perplasia,NRH)是一种非常罕见的肝弥漫性不伴纤维化的增生性病变.由于病因不清,临床表现影像特征与肝硬化相似,病理形态与肝灶性结节性增生(Focal nodular hyperplasia,FNH)、肝细胞腺瘤很难鉴别.本院收治1例,本文就其临床病理、诊断、鉴别诊断结合文献报告如下.     

部分脾动脉栓塞治疗肝硬化脾功能亢进的作用机制

脾功能亢进(脾亢)是由肝硬化、门静脉高压引起的常见并发症,目前临床治疗脾亢的主要方式是脾切除和部分脾动脉栓塞(PSE)。脾切除治疗脾亢效果确切,可显著改善脾亢患者临床症状。相比于脾切除,部分脾动脉栓塞通过栓塞脾动脉分支使部分脾实质梗死,能够达到与部分脾切除术相似的临床疗效,并保留脾脏及脾脏自身功能。虽然PSE是治疗脾亢的有效方法,但是目前国内外关于PSE对肝纤维化、免疫及肝再生影响的研究报道并不多。本文总结了脾亢发生的常见病因、PSE治疗脾亢机制、不同栓塞方法和材料的治疗效果,以及PSE对肝纤维化、免疫及肝再生的影响,为临床脾亢治疗提供理论依据和新的思路。     

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。     

肝前性门脉高压合并脾动脉盗血综合征的诊治

1一般资料患儿男性,12岁。因间断性呕血4年而入院。钡餐X线检查示食管胃底静脉重度曲张。血常规：WBC 0.78×109/L,RBC 2.72×1012/L,PLT 40.4×109/L,Hb 68 g/L。肝功能Child分级A级。术前CT动脉造影示脾动脉粗大,肝动脉分支纤细。CT静脉造影示脾静脉粗大,汇合进入门静脉左支发出的粗大脐旁静脉下行与右髂静脉交通,     

Nodular regenerative hyperplasia of the liver: an under-recognized cause of portal hypertension in hematological disorders

Portal hypertension has been described in a wide variety of hematological disorders, especially myeloproliferative and lymphoproliferative disorders. Its clinical manifestations may include bleeding esophageal varices, ascites, or hepatic encephalopathy. In patients with hematological disorders, there are a number of potential causes of portal hypertension, including nodular regenerative hyperplasia of the liver (NRH). This lesion is characterized by diffuse replacement of normal hepatic parenchyma by multiple small nodules composed of regenerating hepatocytes with minimal or no fibrosis. This lack of fibrosis distinguishes NRH from cirrhosis. Unlike cirrhosis, NRH only rarely results in compromised hepatic synthetic function. The major manifestation is portal hypertension related to increased resistance to blood flow within hepatic sinusoids. NRH has been linked to a variety of systemic diseases including collagen vascular diseases, myeloproliferative and lymphoproliferative disorders, as well as various medications. Although NRH is commonly associated with blood dyscrasias, the diagnosis is overlooked because of the complexity and wide differential diagnosis of liver diseases in the setting of hematological malignancy. We review herein nodular regenerative hyperplasia of the liver, including aspects of epidemiology, pathogenesis, differential diagnosis, clinical course, and treatment. We highlight its association with different forms of hematological disease, aiming to increase the awareness of this entity to the internist and the treating hematologist/oncologist.     

Nodular regenerative hyperplasia of the liver diagnosed by needle biopsy of the liver: Report of a case and review of the literature

The authors report the case of a 45 year old woman in whom the diagnosis of nodular regenerative hyperplasia of the liver was made by needle biopsy of the liver, laparoscopy and hepatic venography. The patient did not have any underlying disorders and had not ingested any of the many drugs and chemicals which may produce the lesion. Clinical manifestations were hepatosplenomegaly, portal hypertension and iron deficiency anaemia, and her condition remains unchanged 5 years after diagnosis.     

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.     

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...     

Nodular regenerative hyperplasia: a deleterious consequence of chemotherapy for colorectal liver metastases?

AIMS: This report describes three patients suffering from nodular regenerative hyperplasia (NRH). METHODS: These patients have received six, 16 and 20 cycles of neoadjuvant 5-fluorouracil and oxaliplatin-based chemotherapy before planned extended hepatectomy. Two patients underwent uneventful portal vein embolization to hypertrophy the future remnant liver. RESULTS: At the end of chemotherapy, liver function tests deteriorated and portal hypertension appeared in two patients, including ascites, splenomegaly and oesophageal varices. Liver biopsy was performed through a percutaneous (two patients) or a transjugular approach (one patient) and allowed the diagnosis of NRH, which was considered to be a contraindication for major liver resection in all three patients, associated with extrahepatic disease progression in one patient. All patients died from neoplastic disease progression despite further chemotherapy at 6, 17 and 31 months following the diagnosis of NRH. One patient developed liver failure and ascites at the time of death. CONCLUSIONS: Physicians should be aware of the potential occurrence and therapeutic impact of NRH in patients suffering from CRLM and treated by neoadjuvant 5FU-oxaliplatin-based chemotherapy before major liver surgery.     

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.