Autoimmune enteropathy in Schimke immunoosseous dysplasia

The clinical phenotype of Schimke immunoosseous dysplasia (SID) is characterized by growth retardation, renal failure, recurrent infections, cerebral infarcts, and skin pigmentation beginning in childhood. We report here on a 4-year-old male child who had all characteristic symptoms of SID, and, in addition, vomiting and prolonged diarrhea. The study results suggest that malabsorption, demonstrated as increased serum immunoglobulin A anti-gliadin antibody, steatorrhea and partial villous atrophy of the jejunal small bowel, is a previously unrecognized feature of SID. Am. J. Med. Genet. 77:427–430, 1998. © 1998 Wiley-Liss, Inc.     

慢性肝病甲胎蛋白升高与原发性肝癌相关研究

目的 探讨慢性肝病甲胎蛋白升高与原发性肝癌的相关性。方法 对144例慢性肝病患者甲胎蛋白进行定量监测,前2m内每15d检查1次,2m后,2～6m进行肝功能、甲胎蛋白、肝胆B超检查,必要时行肝脏CT检查,随访6年。结果 108例慢性肝炎患者中2例(1．85％)出现恶变,30例肝硬化患者中4例(13．33％)出现恶变;甲胎蛋白动态观察显示：甲胎蛋白-过性升高和持续性低浓度水平无恶变发生,持续性升高恶变率100％(4／4),反复波动恶变率11．11％(2／18)。结论 监测慢性肝病甲胎蛋白动态变化,尤其是持续性升高或反复波动者对早期发现原发性肝癌有积极帮助。     

Immunoglobulin G4 (IgG4)-related autoimmune hepatitis and IgG4-hepatopathy: A histopathological and clinical perspective

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a chronic inflammatory disease that simultaneously or consecutively involves multiple organs of the body. It is characterized by elevated serum IgG4 levels and massive infiltration of IgG4⁺ plasma cells in the damaged tissues. IgG4-related autoimmune hepatitis (IgG4-AIH) and IgG4-hepatopathy are relatively new entities that have been proposed as a phenotype of IgG4-RD in the liver. Immunoglobulin G4-AIH is defined as a disorder with serological, histopathological, and clinical features of both IgG4-RD and AIH, simultaneously satisfying the diagnostic criteria of both classical AIH and IgG4-RD. Although there are several case reports and studies of IgG4-AIH among the published works, no consensus regarding the histopathological characteristics of IgG4-AIH has been established, and its clinical implications remain obscure. Immunoglobulin G4-hepatopathy is defined as a comorbidity of IgG4-RD in the liver, and patients not meeting the diagnostic criteria of classical AIH could be diagnosed with IgG4-hepatopathy. Numerous issues regarding these diseases, especially their epidemiology, histopathological and clinical characteristics, and treatment response to corticosteroids, remain unsolved, and need to be determined to establish the disease concepts of IgG4-AIH and IgG4-hepathopathy.     

慢性肝病中医证候规律分布特征

目的 探讨慢性肝病的中医症候及中医证型分布特点与疾病预后的关系。方法 对2008年9月至2011年1月各种慢性肝炎、肝硬化患者1025例进行回顾性调查。结果 慢性肝病患者中出现症候频率较高的依次为倦怠、急躁易怒、烦躁、神疲、口干、困重、太息。证型分布依次为肝郁脾虚>肝气郁结>肝胆湿热>血瘀阻络>肝胃不和>脾肾阳虚>肝肾不足>气滞湿阻>寒湿。结论 肝郁脾虚贯穿于慢性肝病的整个病程中,气机的不畅、正气的不足是肝病进展的关键。     

血吸虫性阑尾病变的超声声像特征探讨

目的探讨血吸虫性阑尾病变的超声声像特征。方法在2012年1月-2015年12月湖州市中心医院经彩超发现的血吸虫性肝病患者中,选择有明确血吸虫病病史及治疗史的患者50例作为血吸虫性肝病组,并选取50例未发现血吸虫性肝病,且来自非血吸虫病流行区、无血吸虫病病史的普通人群作为对照组,对两组行右下腹阑尾超声扫查。采集两组阑尾最大直径、阑尾壁最厚厚度,并观察阑尾壁回声是否均匀等。结果血吸虫性肝病组阑尾腔最小内径为(2.090±0.790)mm,小于对照组;阑尾壁最厚厚度为(1.332±0.313)mm,大于对照组,差异均有统计学意义(t=2.647、-4.526,P均0.05)。血吸虫性肝病组阑尾壁回声欠均匀、结构欠清晰、不均匀增厚者比例以及阑尾腔内有肠内容物者比例均高于对照组(χ~2=12.000、18.537、24.008、4.244,P均0.05)。结论血吸虫性阑尾病变在超声下具有明显的声像特征,超声在判断血吸虫病患者阑尾是否遭受累及,及早发现阑尾病变中有很大价值。     

Comparative study on the inhibitory effects of antioxidant vitamins and radon on carbon tetrachloride-induced hepatopathy

We have previously reported that radon inhalation activates anti-oxidative functions and inhibits carbon tetrachloride (CCl₄)-induced hepatopathy. It has also been reported that antioxidant vitamins can inhibit CCl₄-induced hepatopathy. In the current study, we examined the comparative efficacy of treatment with radon, ascorbic acid and α-tocopherol on CCl₄-induced hepatopathy. Mice were subjected to intraperitoneal injection of CCl₄ after inhaling approximately 1000 or 2000 Bq/m³ radon for 24 h, or immediately after intraperitoneal injection of ascorbic acid (100, 300, or 500 mg/kg bodyweight) or α-tocopherol (100, 300, or 500 mg/kg bodyweight). We estimated the inhibitory effects on CCl₄-induced hepatopathy based on hepatic function-associated parameters, oxidative damage-associated parameters and histological changes. The results revealed that the therapeutic effects of radon inhalation were almost equivalent to treatment with ascorbic acid at a dose of 500 mg/kg or α-tocopherol at a dose of 300 mg/kg. The activities of superoxide dismutase, catalase, and glutathione peroxidase in the liver were significantly higher in mice exposed to radon than in mice treated with CCl₄ alone. These findings suggest that radon inhalation has an anti-oxidative effect against CCl₄-induced hepatopathy similar to the anti-oxidative effects of ascorbic acid or α-tocopherol due to the induction of anti-oxidative functions.     

Specificity variants in monoclonal antibodies reactive with peptide epitopes of the ring-infected erythrocyte surface antigen (RESA) of Plasmodium falciparum

It has been suggested that repeat sequence antigens of Plasmodium falciparum may serve the parasite in immune evasion by modifying the host antibody response and impairing the development of protective immunity. According to this proposal networks of cross-reactive, repeat sequence malarial antigens have the ability to stimulate a high proportion of all somatically mutated B cells with altered antibody specificity, and thus to hinder the normal process of antibody affinity maturation. To determine the rate at which immunoglobulin mutations produce new reactivities with repeat sequence antigens, hybridoma cell lines specific for the ring-infected erythrocyte surface antigen (RESA) were examined for the incidence of specificity variants that arose naturally or as a result of treatment with the chemical mutagen ethylmethane sulphonate (EMS). From one of the cell lines variants were readily isolated having reactivity towards a very closely related repeat sequence epitope within the same RESA antigen. However, the other hybridoma/antigen combinations revealed no variants. In general, mutations giving rise to antibodies with altered specificity for related repetitive antigens were not readily induced and only limited support of the hypothesis was obtained.     

Recent Advances in the Concept and Pathogenesis of IgG4-Related Disease in the Hepato-Bilio-Pancreatic System

Recent studies have proposed nomenclatures of type 1 autoimmune pancreatitis (AIP) (IgG4-related pancreatitis), IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-related cholecystitis, and IgG4-related hepatopathy as IgG4-related disease (IgG4-RD) in the hepato-bilio-pancreatic system. In IgG4-related hepatopathy, a novel concept of IgG4-related autoimmune hepatitis (AIH) with the same histopathological features as AIH has been proposed. Among organs involved in IgG4-RD, associations with pancreatic and biliary lesions are most frequently observed, supporting the novel concept of “biliary diseases with pancreatic counterparts.” Targets of type 1 AIP and IgG4-SC may be periductal glands around the bile and pancreatic ducts. Based on genetic backgrounds, innate and acquired immunity, Th2-dominant immune status, regulatory T (Treg) or B cells, and complement activation via a classical pathway may be involved in the development of IgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 from Treg cells and by B cell activating factor from monocytes/basophils with stimulation of toll-like receptors/nucleotide-binding oligomerization domain-like receptors. Based on these findings, we have proposed a hypothesis for the development of IgG4-RD in the hepato-bilio-pancreatic system. Further studies are necessary to clarify the pathogenic mechanism of IgG4-RD.