【目的】报道一例误诊为原发性肾病综合征的成人脂蛋白肾病（lipoprotein glomerulopathy ， LPG），以提高诊断与鉴别诊断水平。【方法】对肾穿组织进行光镜、电镜及免疫荧光观察。探讨LPG的临床表现与病理学特征（免疫组织化学、组织...【目的】报道一例误诊为原发性肾病综合征的成人脂蛋白肾病（lipoprotein glomerulopathy ， LPG），以提高诊断与鉴别诊断水平。【方法】对肾穿组织进行光镜、电镜及免疫荧光观察。探讨LPG的临床表现与病理学特征（免疫组织化学、组织学特征）。【结果】患者临床表现为严重水肿及低蛋白血症，大量蛋白尿及镜下血尿，血清胆固醇正常，三酰甘油及载脂蛋白B （A po B ）、A po E 水平显著上升，肾活检显示可见22个肾小球，体积均明显增大，其中1个肾小球球性硬化。肾小球毛细血管腔重度扩张，其内可见充满大小不一的淡染网状物质充填，多形性，呈网眼样、层状、羽毛状，其间可见巨大“栓子”，经免疫荧光染色证实“栓子”富含A po B、A po E。【结论】L PG是一种罕见的原发性肾小球疾病，发病率低，临床上表现酷似肾病综合征，易误诊，应引起重视。展开更多
Acute fatty liver of pregnancy (AFLP) is a serious maternal illness occurring in the third trimester of pregnancy with significant perinatal and maternal mortality. Till recently, it has been considered a mysterious i...Acute fatty liver of pregnancy (AFLP) is a serious maternal illness occurring in the third trimester of pregnancy with significant perinatal and maternal mortality. Till recently, it has been considered a mysterious illness. In this editorial, we review the recent advances in understanding the pathogenesis of AFLP and discuss the studies documenting a fetal-maternal interaction with a causative association between carrying a fetus with a defect in mitochondrial fatty acid oxidation and development of AFLP. Further, we discuss the impact of these recent advances on the offspring born to women who develop AFLP, such that screening for a genetic defect can be life saving to the newborn and would allow genetic counseling in subsequent pregnancies. The molecular basis and underlying mechanism for this unique fetal-maternal interaction causing maternal liver disease is discussed.展开更多
AIM： To compare the clinical outcome and pathologic features of non-alcoholic steatohepatitis （NASH） patients with hepatocellular carcinoma （HCC） and hepatitic C virus （HCV） patients with HCC （another group in...AIM： To compare the clinical outcome and pathologic features of non-alcoholic steatohepatitis （NASH） patients with hepatocellular carcinoma （HCC） and hepatitic C virus （HCV） patients with HCC （another group in which HCC is commonly seen） undergoing liver transplantation. METHODS： Patients transplanted for HCV and NASH at our institution from January 2000 to April 2011 were analyzed. All explanted liver histology and pre-trans- plant liver biopsies were examined by two specialist liver histopathologists. Patient demographics, disease free survival, explant liver characteristics and HCC features （tumour number, cumulative tumour size, vascular invasion and differentiation） were compared between HCV and NASH liver transplant recipients. RESULTS： A total of 102 patients with NA^SH and 283 patients with HCV were transplanted. The incidence of HCC in NASH transplant recipients was 16.7% （17/102）. The incidence of HCC in HCV transplant recipients was 22.6% （64/283）. Patients with NASH-HCC were statisti- cally older than HCV-HCC patients （P 〈 0.001）. A signif- icantly higher proportion of HCV-HCC patients had vas- cular invasion （23.4% vs 6.4%, P = 0.002） and poorly differentiated HCC （4.7% vs 0%, P 〈 0.001） compared to the NASH-HCC group. A trend of poorer recurrence free survival at 5 years was seen in HCV-HCC patients compared to NASH-HCC who underwent a Liver trans- plantation （P = 0.11）. CONCLUSION： Patients transplanted for NASH-HCC appear to have less aggressive turnout features com- pared to those with HCV-HCC, which likely in part ac- counts for their improved recurrence free survival.展开更多
文摘【目的】报道一例误诊为原发性肾病综合征的成人脂蛋白肾病（lipoprotein glomerulopathy ， LPG），以提高诊断与鉴别诊断水平。【方法】对肾穿组织进行光镜、电镜及免疫荧光观察。探讨LPG的临床表现与病理学特征（免疫组织化学、组织学特征）。【结果】患者临床表现为严重水肿及低蛋白血症，大量蛋白尿及镜下血尿，血清胆固醇正常，三酰甘油及载脂蛋白B （A po B ）、A po E 水平显著上升，肾活检显示可见22个肾小球，体积均明显增大，其中1个肾小球球性硬化。肾小球毛细血管腔重度扩张，其内可见充满大小不一的淡染网状物质充填，多形性，呈网眼样、层状、羽毛状，其间可见巨大“栓子”，经免疫荧光染色证实“栓子”富含A po B、A po E。【结论】L PG是一种罕见的原发性肾小球疾病，发病率低，临床上表现酷似肾病综合征，易误诊，应引起重视。
文摘Acute fatty liver of pregnancy (AFLP) is a serious maternal illness occurring in the third trimester of pregnancy with significant perinatal and maternal mortality. Till recently, it has been considered a mysterious illness. In this editorial, we review the recent advances in understanding the pathogenesis of AFLP and discuss the studies documenting a fetal-maternal interaction with a causative association between carrying a fetus with a defect in mitochondrial fatty acid oxidation and development of AFLP. Further, we discuss the impact of these recent advances on the offspring born to women who develop AFLP, such that screening for a genetic defect can be life saving to the newborn and would allow genetic counseling in subsequent pregnancies. The molecular basis and underlying mechanism for this unique fetal-maternal interaction causing maternal liver disease is discussed.
文摘AIM： To compare the clinical outcome and pathologic features of non-alcoholic steatohepatitis （NASH） patients with hepatocellular carcinoma （HCC） and hepatitic C virus （HCV） patients with HCC （another group in which HCC is commonly seen） undergoing liver transplantation. METHODS： Patients transplanted for HCV and NASH at our institution from January 2000 to April 2011 were analyzed. All explanted liver histology and pre-trans- plant liver biopsies were examined by two specialist liver histopathologists. Patient demographics, disease free survival, explant liver characteristics and HCC features （tumour number, cumulative tumour size, vascular invasion and differentiation） were compared between HCV and NASH liver transplant recipients. RESULTS： A total of 102 patients with NA^SH and 283 patients with HCV were transplanted. The incidence of HCC in NASH transplant recipients was 16.7% （17/102）. The incidence of HCC in HCV transplant recipients was 22.6% （64/283）. Patients with NASH-HCC were statisti- cally older than HCV-HCC patients （P 〈 0.001）. A signif- icantly higher proportion of HCV-HCC patients had vas- cular invasion （23.4% vs 6.4%, P = 0.002） and poorly differentiated HCC （4.7% vs 0%, P 〈 0.001） compared to the NASH-HCC group. A trend of poorer recurrence free survival at 5 years was seen in HCV-HCC patients compared to NASH-HCC who underwent a Liver trans- plantation （P = 0.11）. CONCLUSION： Patients transplanted for NASH-HCC appear to have less aggressive turnout features com- pared to those with HCV-HCC, which likely in part ac- counts for their improved recurrence free survival.
