脂肪性肝病的病理学

一、酒精性肝病的病理学 酒精性脂肪性肝炎(ASH)表明疾病的发生和进展,停止酗酒和积极治疗有可能使其病理学发生逆转.但在某些患者,即使继续饮酒,脂肪性肝病与其酒精性肝炎的病理学也不会进一步发展.     

肝星状细胞凋亡与肝纤维化

近十年来对肝纤维化形成的机理研究取得了令人鼓舞的进展。越来越多的临床及实验证据表明,肝纤维化是可以逆转的［１］,这一观点逐渐被广泛接受。肝星状细胞（ｈｏｐａｔｉｃｓｔｅｌｌａｔｅｃｅｌｌ,ＨＳＣ）的激活是肝纤维化形成的中心环节［２］,ＨＳＣ在肝纤维化逆转过程中的作用也成为研究热点之一。Ｂｕｒｔ［３］认为,肝纤维化逆转时ＨＳＣ的减少是由于激活状态的ＨＳＣ转化为静止状态。但最新的研究表明,肝纤维化恢复期,激活状态的ＨＳＣ减少主要通过凋亡机制,而不是表型的转化［４］。几位学者分别对ＨＳＣ凋亡的机制进行…     

抗肝纤维化治疗研究进展

肝纤维化是各种慢性肝病发展至肝硬化的必经阶段,是由于各种致病因子（常见病因为病毒性肝炎、慢性酒精中毒、遗传和代谢疾病、化学毒物或药物、肝脏淤血、寄生虫、脂肪肝等）引起肝脏损伤和炎症,导致细胞外基质（ECM）在肝脏过度沉积。其中肝星状细胞（HSC）的活化是纤维化的中心事件。随着肝纤维化发生机制进一步阐明,防治策略也进行了相应调整,抑制HSC活化和抑制ECM合成,促进降解,成为当今抗肝纤维化的关键措施。     

小儿病毒性肝炎的病理学

与成人相比，小儿肝损害的病因更多，其中以病毒性肝炎最为主要。小儿病毒性肝炎由于侵犯肝脏的病毒种类繁多，各年龄段各种肝病罹病的机会不同，临床表现变化较大．尤其小儿免疫功能发育来完全，肝细胞有旺盛的再生能力等都是形成小儿病毒性肝炎病理特征的因素。近年来，丙型病毒性肝炎的研究有了突破性的进展，因此，对小儿病毒性肝炎的病理也取得了更多的了解。     

代谢相关脂肪性肝病无创血清学诊断的研究进展

代谢相关脂肪性肝病是以代谢异常相关的肝脂肪变性为特征的慢性肝病, 可进展至代谢相关脂肪性肝炎、肝纤维化和肝硬化, 甚至肝细胞癌。目前, 肝活组织检查仍是诊断的金标准, 但由于肝活组织检查的有创性和并发症风险, 开发血清学诊断指标以实现无创性诊断是近年来研究的热点。现对目前研究较充分的脂肪肝无创性血清学诊断指标进行综述。     

肝原发性鳞状细胞癌临床病理学观察

肝原发性鳞状细胞癌（PSCCL）是一种罕见的肝脏异源性恶性肿瘤，迄今国外文献仅20余例。国内文献约有15例报道。我们报道1例，结合文献重点阐述其组织发生、诊断及鉴别诊断。     

中药以肝星状细胞为靶点的抗肝纤维化研究进展

肝纤维化的形成主要是由于肝炎病毒、酒精、药物与毒物、血吸虫、代谢和遗传、胆汁郁积、自身免疫性肝病等多种损伤因素长期慢性刺激肝脏，使肝窦内肝星状细胞（HSCs）活化，胶原等细胞外基质（ECM）代谢失衡，生成大于降解，促使肝脏ECM沉积与组织结构重构。而HSCs活化和功能改变是肝纤维化发生发展的关键，对HSCs的干预无疑将成为防治肝纤维化的核心措施之一。     

酒精性肝病的病理学

酒精引起的肝损害，其病理学分类目前国际上尚不一致。习惯上把它分为脂肪肝，酒精性肝炎和纤维化及肝硬变，三者组织学表现有相互重叠之处。1979年日本学者武内五郎等根据对日本人酒精性肝病的研究，主张将其组织学诊断分为酒精性脂肪肝，酒精性肝炎，酒精性肝纤维化，嗜酒者慢性肝炎，酒     

非酒精性脂肪性肝病合并2型糖尿病患者肝组织病理学变化研究

目的 研究非酒精性脂肪性肝病（NAFLD）合并2型糖尿病（T2DM）患者肝脏组织病理学变化。方法 分析240例行肝活检术的NAFLD患者临床资料,比较NAFLD合并T2DM与未合并T2DM患者肝组织病理学表现和评分的差异。结果 在240例NAFLD患者中,合并2型糖尿病者80例（33.3%）,未合并T2DM 者160例（66.7%）;在NAFLD合并T2DM患者中非酒精性脂肪性肝炎（NASH）60例、肝纤维化20例,未合并T2DM患者中分别为68例和92例;在NAFLD合并T2DM患者中检出肝纤维化评分≥2者30例（37.5%）、肝细胞气球样变评分≥2者23例（28.8%）和马洛里小体22例（27.5%）,均显著高于未合并T2DM患者的【20例（12.5%）、22例（13.8%）和30例（18.8%）,P<0.05】;T2DM为发生NASH（OR=3.27,95%CI：1.42~7.55）和肝纤维化（OR=3.35,95%CI：1.55~7.63）的独立危险因素。结论 合并T2DM的NAFLD患者肝组织病理学损伤更趋严重,应注意防治。     

Hepatic complications of obesity

Obesity increases the risk and severity of liver disease. The most common form of liver disease that occurs in obesity is NAFLD. A better understanding of the basic disease mechanisms and natural histories ofNAFLD is needed to guide management and treatment of obese patients and others with this disorder. Large, prospective, randomized, controlled treatment trials also are needed. Ideally, such studies will focus on well-defined patient subsets and monitor the impact of therapy on clinically relevant endpoints, such as liver-related morbidity and mortality.     

Hepatic morphology in obesity

Changes in hepatic morphology were evaluated in 242 consecutive liver biopsy specimens obtained from nonalcoholic obese subjects. Steatosis occurred in 59.7% of the subjects but was moderate to severe in 28.7%. An alcoholic hepatitis-like picture was present in 8.7% and cirrhosis in 2.7%. Fibrosis of mild and moderate degrees was encountered in 26.1% and 5.8%, respectively. This study indicates that the histopathological changes that occur in the livers of obese subjects are similar to alcoholic liver injury.     

Hepatic lipid metabolism in severe human obesity

The rate of incorporation of glycerolcarbon into triglyceride (TG) in liver slices obtained from 17 severely obese normolipoproteinemic patients during jejunoileal bypass was significantly elevated compared to normal controls. The obese patients had an almost sevenfold increase in hepatic TG content as well. None of the patients had manifest diabetes, though increased basal levels of insulin and impaired glucose tolerance during oral glucose tolerance tests (OGTT) were common. There were statistically significant positive Spearman rank correlations between plasma insulin levels and TG synthesis (r = 0.57; p < 0.01), TG content (r = 0.54; p < 0.01), and serum fatty acids (r = 0.52; p < 0.05). The basal insulin levels were also positively correlated to the incorporation rate of fructose-carbon into fatty acids (r = 0.47; p < 0.05). The sum of insulin values during OGTT showed a trend toward positive correlation with the hepatic cholesterol content (r = 0.34; p < 0.10). The hepatic protein content as well as the rate of leucinecarbon incorporation into proteins did not differ from controls. However, there was a trend toward a negative correlation between protein synthesis and basal plasma insulin levels (r = ?0.33; p < 0.10). It is suggested that liver steatosis in severely obese patients is due to significantly increased hepatic lipid synthesis in the face of elevated levels of serum fatty acids. The possible importance of plasma insulin is also emphasized.     

Clinical and pathologic features of familial interstitial pneumonia

RATIONALE: Several lines of evidence suggest that genetic factors and environmental exposures play a role in the development of pulmonary fibrosis. OBJECTIVES: We evaluated families with 2 or more cases of idiopathic interstitial pneumonia among first-degree family members (familial interstitial pneumonia, or FIP), and identified 111 families with FIP having 309 affected and 360 unaffected individuals. METHODS: The presence of probable or definite FIP was based on medical record review in 28 cases (9.1%); clinical history, diffusing capacity of carbon monoxide (DL(CO)), and chest X-ray in 16 cases (5.2%); clinical history, DL(CO), and high-resolution computed tomography chest scan in 191 cases (61.8%); clinical history and surgical lung biopsy in 56 cases (18.1%); and clinical history and autopsy in 18 cases (5.8%). RESULTS: Older age (68.3 vs. 53.1; p < 0.0001), male sex (55.7 vs. 37.2%; p < 0.0001), and having ever smoked cigarettes (67.3 vs. 34.1%; p < 0.0001) were associated with the development of FIP. After controlling for age and sex, having ever smoked cigarettes remained strongly associated with the development of FIP (odds ratio(adj), 3.6; 95% confidence interval, 1.3-9.8). Evidence of aggregation of disease was highly significant (p < 0.001) among sibling pairs, and 20 pedigrees demonstrated vertical transmission, consistent with autosomal dominant inheritance. Forty-five percent of pedigrees demonstrated phenotypic heterogeneity, with some pedigrees demonstrating several subtypes of idiopathic interstitial pneumonia occurring within the same families. CONCLUSIONS: These findings suggest that FIP may be caused by an interaction between a specific environmental exposure and a gene (or genes) that predisposes to the development of several subtypes of idiopathic interstitial pneumonia.     

腓骨肌萎缩症的临床与病理

目的：探讨腓骨肌萎缩症(CMT)的临床与病理特点。方法：综合分析20例腓骨肌萎缩症患者的临床资料,神经电生理改变及神经肌肉活检的病理特点。结果：腓骨肌萎缩症的1、2型在临床表现上基本相同,但神经电生理改变及神经肌肉活检的病理特点明显不同。16例CMT1型（肥大型或脱骨鞘型）表现为神经传导速度减慢和脱髓鞘,4例CMT2型（轴索型）表现为神经传导速度正常和轴索变性。结论：神经电生理检查和神经肌肉活检对腓骨肌萎缩症诊断和分型有重要的意义。