A Case of Familial Spondyloenchondrodysplasia with Immune Dysregulation Masquerading as Moyamoya Syndrome

No abstract available.

Mouse models for hepatitis B virus research / 한국실험동물학회지

Hepatitis B virus (HBV) infection remains a major global health problem; indeed, there are 250 million carriers worldwide. The host range of HBV is narrow; therefore, few primates are susceptible to HBV infection. However, ethical constraints, high cost, and large size limit the use of primates as suitable animal models. Thus, in vivo testing of therapies that target HBV has been hampered by the lack of an appropriate in vivo research model. To address this, mouse model systems of HBV are being developed and several are used for studying HBV in vivo. In this review, we summarize the currently available mouse models, including HBV transgenic mice, hydrodynamic injection-mediated HBV replicon delivery systems, adeno-associated virus-mediated HBV replicon delivery systems, and human liver chimeric mouse models. These developed (or being developed) mouse model systems are promising and should be useful tools for studying HBV.

Murine γδ T Cells Render B Cells Refractory to Commitment of IgA Isotype Switching

γδ T cells are abundant in the gut mucosa and play an important role in adaptive immunity as well as innate immunity. Although γδ T cells are supposed to be associated with the enhancement of Ab production, the status of γδ T cells, particularly in the synthesis of IgA isotype, remains unclear. We compared Ig expression in T cell receptor delta chain deficient (TCRδ⁻/⁻) mice with wild-type mice. The amount of IgA in fecal pellets was substantially elevated in TCRδ⁻/⁻ mice. This was paralleled by an increase in surface IgA expression and total IgA production by Peyer's patches (PPs) and mesenteric lymph node (MLN) cells. Likewise, the TCRδ⁻/⁻ mice produced much higher levels of serum IgA isotype. Here, surface IgA expression and number of IgA secreting cells were also elevated in the culture of spleen and bone marrow (BM) B cells. Germ-line α transcript, an indicator of IgA class switch recombination, higher in PP and MLN B cells from TCRδ⁻/⁻ mice, while it was not seen in inactivated B cells. Nevertheless, the frequency of IgA+ B cells was much higher in the spleen from TCRδ⁻/⁻ mice. These results suggest that γδ T cells control the early phase of B cells, in order to prevent unnecessary IgA isotype switching. Furthermore, this regulatory role of γδ T cells had lasting effects on the long-lived IgA-producing plasma cells in the BM.

NF-kappaB Activation in T Helper 17 Cell Differentiation

CD28/T cell receptor ligation activates the NF-kappaB signaling cascade during CD4 T cell activation. NF-kappaB activation is required for cytokine gene expression and activated T cell survival and proliferation. Recently, many reports showed that NF-kappaB activation is also involved in T helper (Th) cell differentiation including Th17 cell differentiation. In this review, we discuss the current literature on NF-kappaB activation pathway and its effect on Th17 cell differentiation.

Interleukin-17 in the Inflammatory Bowel Disease / 한양의대학술지

Inflammatory bowel diseases(IBD), including Crohn's disease and ulcerative colitis, are chronic inflammatory states of the intestinal tract. While the exact mechanisms inducing chronic inflammation are still unclear, it is hypothesized that the inflammation is caused in part by an inappropriate immune response to the intestinal microflora. Although inflammatory diseases are not directly linked to patient survival, symptoms of these diseases significantly decrease quality of life. The incidence rate is higher in western people than eastern people, but the incidence rate of IBD in eastern people, including Korean, is increasing. Recently, it has been reported that IL-17 is an important factor that appears to be involved in IBD induction and progression. This report reviews many recent papers reporting the relationship between IBD and IL-17, which may provide an understanding leading to new means of prevention and treatment for IBD.

A case of achalasia without dilatation of the esophageal body / 대한소화관운동학회지

Achalasia is a motility disorder of the esophagus consisting of abnormal relaxation of the lower esophageal sphincter and aperistalsis of the esophageal body. Esophageal dilatation and bird beak appearance are characteristic radiologic findings of achalasia, but achalasia patients do not always show typical findings on esophagography. We recently experienced a 38-year-old female patient who complained of chest pain and dysphagia. She showed no dilatation of the esophagus with delayed emptying of the contrast media in esophagography, but achalasia was diagnosed by typical manometric findings. The patient's symptoms improved after a balloon dilatation. Therefore, esophageal manometry should always be performed when the patient's history suggests the presence of achalasia without typical radiologic findings. We report this case with a review of the literature.

Changes in Antibody Titers of Measles, Mumps, Rubella, and Hepatitis B Virus AftEr Bone Marrow Transplantation in Korea: A Preliminary Report / 감염

BACKGROUND: Bone marrow transplantation (BMT) has become a significant treatment modality for hematopoietic and solid organ malignancy. Recipients of BMTs lose immunity to measles-mumps-rubella (MMR) and hepatitis B infections which are preventable with vaccination. There is no consensus regarding a vaccination schedule after BMT and time of vaccination is variable according to each institution. We analyzed sequential changes in antibody titers of MMR and hepatitis B during the first year after BMT in an attempt to identify the time, dose, and needs for revaccination. METHODS: Total 20 patients with hematologic malignancies were studied. Serum levels of IgG antibodies of MMR and hepatitis B virus (HBV) were determined every three months post-BMT by enzyme immunoassay (EIA), chemical luminescence immunoassay (CLIA) and immunofluorescence assay (IFA). RESULTS: IgG antibody levels of measles, rubella, HBV were 1:746, 80 85 IU/mL, 214 343 IU/L before BMT, declined to 1:633, 18 11 IU/mL, 4 6 IU/L one year after BMT, respectively. All the antibody levels were still above cut-off value for positive immunity. Mumps antibody titers were 1:62 before BMT, declined to 1:25 significantly from 6 months after BMT, but the antibody level was still above cut-off value. CONCLUSION: Antibody titers of MMR and hepatitis B decline during the first year after BMT, but the levels are still above cut-off value. Thus, the timing of revaccination should be after the first year post-transplantation. Long-term studies are needed to determine the optimal time for revaccination.

A Case of Boerhaave Syndrome with Empyema in both Lungs in Alcoholic Liver Cirrhosis / 대한소화기내시경학회지

Spontaneous esophageal perforation (Boerhaave Syndrome) is an unusual condition that frequently leads to fatal complications. It typically occurs with rigorous emesis after an unduly large meal or heavy drinking. Its diagnosis is often delayed in almost all cases due to nonspecific symptoms and signs, resulting in increased morbidity and mortality. Therefore early diagnosis and appropriate treatment are very important. Recently we experienced a case of esophagogastric junctional perforation accompanied by bilateral empyema and mediastinitis after heavy alcohol drinking in a 56-year-old male patient. He was presented with hematemesis and abdominal pain. We diagnosed him using esophagography and chest CT. Thus, we report a case with a brief review of related literatures.