目的 分析肝硬化患者门脉性肺动脉高压(PoPH)的发生率、临床特征及危险因素.方法 收集2021年3月至2022年3月于中国医科大学附属第一医院住院的115例肝硬化门静脉高压患者的一般临床资料、实验室化验及检查结果,根据超声心动图结果是否合并肺动脉高压为标准分为PoPH组和非PoPH组.分析两组患者的一般临床特征及多因素logistic回归分析肝硬化发生PoPH的独立危险因素.结果 (1)肝硬化患者PoPH的发生率为8.7％(10/115).(2)PoPH组10例,平均年龄(54.5±14.3)岁,平均肺动脉压力(44.0±4.3)mmHg.非PoPH组105例,平均年龄(56.9±10.7)岁.PoPH组患者血红蛋白(Hb)、血清白蛋白(ALB)显著低于非PoPH组(P＜0.05);PoPH组平均红细胞容积(MCV)、平均红细胞血红蛋白含量(MCH)、Child-Pugh评分及终末期肝病模型(MELD)评分显著高于非PoPH组(P＜0.05).(3)多因素logistic回归分析结果显示:低Hb(OR 0.95,95％CI0.90～1.00,P=0.034)是肝硬化门脉高压患者发生肺动脉高压的独立危险因素.结论 本研究肝硬化门脉高压患者中PoPH的发生率为8.7％,Hb和ALB降低以及MCV和MCH升高与肝硬化PoPH的发展密切相关,低Hb是肝硬化门脉高压患者发生肺动脉高压的独立危险因素.

The aim of this study was to observe the demographic and clinical characteristics of immunoglobulin (Ig) G4–related disease (IgG4-RD). We aimed to compare different treatment methods and to identify the risk factors for non-response and relapse after treatment. We performed a retrospective study of 201 IgG4-RD patients initially diagnosed and treated at the First Affiliated Hospital of China Medical University from January 2016 to December 2020. Patients’ sex, age, clinical manifestations, baseline biochemical values, the number of organs involved, and the type of organ involvement were recorded. All patients received glucocorticoid (GC) monotherapy or GC + immunosuppressant combination therapy. The serum IgG4 concentration as well as the details of clinical response, relapse, and side effects were recorded at 1, 3, 6, and 12 months after treatment. The incidence of IgG4-RD was primarily centered in the age group of 50–70 years old, and the proportion of affected male patients increased with age. The most common clinical symptom was swollen glands or eyes (42.79

Abstract Objective: The aim of this study was to observe the demographic and clinical characteristics of immunoglobulin (Ig) G4–related disease (IgG4-RD). We aimed to compare different treatment methods and to identify the risk factors for non-response and relapse after treatment.Methods: We performed a retrospective study of 201 IgG4-RD patients initially diagnosed and treated at the First Affiliated Hospital of China Medical University from January 2016 to December 2020. Patients’ sex, age, clinical manifestations, baseline biochemical values, the number of organs involved, and the type of organ involvement were recorded. All patients received glucocorticoid (GC) monotherapy or GC + immunosuppressant combination therapy. The serum IgG4 concentration as well as the details of clinical response, relapse, and side effects were recorded at 1, 3, 6, and 12 months after treatment.Results: The incidence of IgG4-RD was primarily centered in the age group of 50–70 years old, and the proportion of affected male patients increased with age. The most common clinical symptom was swollen glands or eyes (42.79%). The rates of single- and double-organ involvement were 34.83% and 46.27%, respectively. The pancreas (45.77%) was the most frequently involved organ in cases of single-organ involvement, and the pancreas and biliary tract (45.12%) was the most common organ combination in cases of double-organ involvement. Correlation analysis showed that the number of organs involved was positively related to the serum IgG4 concentration (r = 0.161). The effective rate of GC monotherapy was 91.82%, the recurrence rate was 31.46%, and the incidence of adverse reactions was 36.77%. Meanwhile, the effective rate of GC + immunosuppressant combination therapy was 88.52%, the recurrence rate was 19.61%, and the adverse reaction rate was 41.00%. There were no statistically significant differences in response, recurrence, and adverse reactions. The overall response rate within 12 months was 90.64%. Age (<50 years old) and aorta involvement were significantly associated with non-response. The overall recurrence rate within 12 months was 26.90%. Age (<50 years old), low serum C4 concentration, a high number of involved organs, and lymph node involvement were significantly associated with recurrence.Conclusion: The clinical features vary among different age groups and according to gender. The number of organs involved in IgG4-RD is related to the serum IgG4 concentration. Age (<50 years old), low serum C4 concentration, a high number of involved organs, and lymph node involvement are risk factors for recurrence.

Pancreatic neuroendocrine neoplasms (PNEN) are tumors that originate from neuroendocrine cells. Only about 1% patients are related to mutation of tuberous sclerosis complex gene. Here, we reported a rare case with involvement of multiple organs and space-occupying lesions. Initially, the patient was thought to have metastasis of a pancreatic tumor. However, the patient was diagnosed as pancreatic neuroendocrine tumors, liver perivascular epithelioid tumors, splenic hamartoma, and renal angiomyolipoma by pathological examination after surgery. We performed genetic mutation detection to identify that tuberous sclerosis complex 2 gene presented with a heterozygous variant. Tuberous sclerosis often presents with widespread tumors, but it is less common to present with pancreatic neuroendocrine tumors and liver perivascular tumors as highlighted in the case. So we analyzed the relationship between TSC gene mutations and related tumors. And we also reviewed the current molecular mechanisms and treatments for tuberous sclerosis complex.

目的 非酒精性脂肪性肝病(NAFLD)是目前全球最流行的慢性肝脏疾病,其主要发生机制包括胰岛素抵抗和氧化应激等.不同血清标志物与NAFLD发病机制密切相关,但尚无明确的定论.本文主要就NAFLD发生发展过程中不同血清标志物的作用作一综述.

Hepatocellular carcinoma (HCC) is a common cancer in the world, and its incidence is increasing yearly. Hepatitis B virus (HBV) infection and hepatitis C virus (HCV) infection are important causes of HCC. Liver cirrhosis, age, sex, smoking and drinking, and metabolic risk factors will increase the risk of cancer in HBV/HCV patients. And viral load, APRI, FIB-4, and liver stiffness can all predict the risk of HCC in patients with viral infection. In addition, effective prevention strategies are essential in reducing the risk of HCC. The prevention of HCC involves mainly tertiary prevention strategies, while the primary prevention is based on standardized vaccine injections to prevent the occurrence of HBV/HCV. Eliminating the route of transmission and vaccination will lead to a decrease in the incidence of HCC. Secondary prevention involves effective antiviral treatment of HBV/HCV to prevent the disease from progressing to HCC, and tertiary prevention is actively treating HCC to prevent its recurrence.

Clonorchis sinensis infection is still a major public health problem. It is estimated that more than 15 million people worldwide are infected, especially in Northeast China, Taiwan, South Korea, and North Vietnam. The detection of Clonorchis sinensis eggs in feces and bile is still the only gold standard for the diagnosis of Clonorchis sinensis infection, and new detection methods are needed to improve the detection rate. After Clonorchis sinensis invades the human body, it mainly parasitizes the hepatobiliary tract. Therefore, it is closely related to hepatobiliary diseases such as cholangitis, bile duct stones, liver fibrosis, and cholangiocarcinoma. The increase in immunoglobulin G4 (IgG4) caused by Clonorchis sinensis infection is rare and there are few reports about the relevant mechanism. It may be related to the inflammatory factors interleukin (IL)-4, IL-10, and IL-13 produced by human phagocytes, T cells, B cells, and other immune cells in the process of resisting the invasion of Clonorchis sinensis. However, this finding still needs further clarification and confirmation. This article reviews the epidemiology, clinical manifestations, serology, imaging, pathogenic mechanism, and control measures of Clonorchis sinensis infection to help establish the diagnostic process for Clonorchis sinensis. We report novel mechanisms of IgG4 elevation due to Clonorchis sinensis infection to provide more experience and a theoretical basis for clinical diagnosis and treatment of this infection.

放射疗法是多种恶性肿瘤治疗的主要手段,尽管非常有效且更具有针对性,但许多患者仍存在不良反应.放射性肠炎(radiation enteritis,RE)是腹盆腔恶性肿瘤放疗后最常见并发症之一,肠道症状如腹痛、腹胀、腹泻等极大地影响患者的生活质量,目前常用的药物治疗效果不佳.众多研究表明,肠道菌群在放疗引起的肠道损伤的发生发展中起关键作用,粪菌移植(fecal microbiota transplantation,FMT)2014年被美国克利夫兰医学院列入当年的十大医学创新,是目前治疗各种肠道疾病的重大医学突破.本文报道1例因宫颈癌行放疗导致RE的患者,常规内科治疗效果不佳,经供体FMT治疗获得显著缓解的病例.