Background: The clinical implications of myelin oligodendrocyte glycoprotein autoantibodies (MOG-Abs) are increasing. Establishing MOG-Ab assays is essential for effectively treating patients with MOG-Abs. We established an in-house cell-based assay (CBA) to detect MOG-Abs to identify correlations with patients’ clinical characteristics. Methods: We established the CBA using HEK 293 cells transiently overexpressing fulllength human MOG, tested it against 166 samples from a multicenter registry of central nervous system (CNS) inflammatory disorders, and compared the results with those of the Oxford MOG-Ab-based CBA and a commercial MOG-Ab CBA kit. We recruited additional patients with MOG-Abs and compared the clinical characteristics of MOG-Ab-associated disease (MOGAD) with those of neuromyelitis optica spectrum disorder (NMOSD). Results: Of 166 samples tested, 10 tested positive for MOG-Abs, with optic neuritis (ON) being the most common manifestation (4/15, 26.7%). The in-house and Oxford MOG-Ab CBAs agreed for 164/166 (98.8%) samples (κ = 0.883, P < 0.001); two patients (2/166, 1.2%) were only positive in our in-house CBA, and the CBA scores of the two laboratories correlated well (r = 0.663, P < 0.001). The commercial MOG-Ab CBA kit showed one falsenegative and three false-positive results. The clinical presentation at disease onset differed between MOGAD and NMOSD; ON was the most frequent manifestation in MOGAD, and transverse myelitis was most frequent in NMOSD. Conclusions The in-house CBA for MOG-Abs demonstrated reliable results and can potentially be used to evaluate CNS inflammatory disorders. A comprehensive, long-term study with a large patient population would clarify the clinical significance of MOG-Abs.

Atypical thymic carcinoid is an extremely rare tumor with a poor prognosis. In addition to its known association with multiple endocrine neoplasia type 1, its hallmark characteristics include local invasion and early distant metastasis. In this report, we share our experience treating atypical thymic carcinoid in a patient with Zollinger-Ellison syndrome.

Atypical thymic carcinoid is an extremely rare tumor with a poor prognosis. In addition to its known association with multiple endocrine neoplasia type 1, its hallmark characteristics include local invasion and early distant metastasis. In this report, we share our experience treating atypical thymic carcinoid in a patient with Zollinger-Ellison syndrome.
Carcinoid Tumor ; Humans ; Multiple Endocrine Neoplasia Type 1 ; Neoplasm Metastasis ; Neuroendocrine Tumors ; Prognosis ; Zollinger-Ellison Syndrome

BACKGROUND/AIMS: There have been numerous efforts to reduce mother-to-child transmission (MTCT) of hepatitis B virus (HBV) with antiviral agents during pregnancy. However, there are limited data regarding the outcomes of pregnant women after delivery. This study was performed to evaluate the efficacy of antiviral agents in preventing MTCT of HBV and maternal long-term outcomes. METHODS: The HBV-infected pregnant women treated with antiviral agents to prevent MTCT were retrospectively reviewed. Forty-one pregnant women who received telbivudine or tenofovir during late pregnancy (28-34 week) were analyzed. Hepatitis B virus surface antibody (HBsAb) positivity was tested in 43 infants after 7 months of birth. Eleven mothers were followed >1 year after delivery. RESULTS: The mean HBV DNA titer before antiviral therapy was 8.67 (6.60–9.49) log copies/mL, and the median age at delivery was 32 years (range, 22–40). Eleven patients were treated with tenofovir and 30 with telbivudine. The median duration was 57 days (range, 23–100), and the median HBV DNA titer at birth was 5.06 log copies/mL (range, 2.06–6.50). Antiviral treatments were associated with significant HBV DNA reduction (P < 0.001). Among 43 infants (two cases of twins), HBsAb was not detected in two, subsequently confirmed to have HBV infection. Biochemical flare was observed in two of 11 mothers followed >12 months, and an antiviral agent was administered. CONCLUSIONS: Antiviral treatment during late pregnancy effectively reduced MTCT. Long-term follow-up should be required in such cases. In addition, given that maternal biochemical flare occurred in 18% of mothers, re-administration of antiviral agents might be required.
Antiviral Agents ; DNA ; Female ; Follow-Up Studies ; Hepatitis B virus* ; Hepatitis B* ; Hepatitis* ; Humans ; Infant ; Mothers ; Parturition ; Postpartum Period ; Pregnancy ; Pregnant Women ; Retrospective Studies ; Tenofovir

BACKGROUND AND PURPOSE: Upper respiratory infection (URI), including influenza, may exacerbate the symptoms of myasthenia gravis (MG), which is an autoimmune disease that causes muscle weakness. There is also concern that the influenza vaccine may trigger or worsen autoimmune diseases. The objective of this study was to determine the impacts of influenza infection and vaccination on symptom severity in MG patients. METHODS: Patients diagnosed with MG were enrolled from 10 university-affiliated hospitals between March and August 2015. Subjects completed a questionnaire at the first routine follow-up visit after enrolling in the study. The patient history was obtained to determine whether a URI had been experienced during the previous winter, if an influenza vaccination had been administered before the previous winter, and whether their MG symptoms were exacerbated during or following either a URI or vaccination. Influenza-like illness (ILI) was defined and differentiated from the common cold as a fever of ≥38℃ accompanied by a cough and/or a sore throat. RESULTS: Of the 258 enrolled patients [aged 54.1±15.2 years (mean±SD), 112 men, and 185 with generalized MG], 133 (51.6%) had received an influenza vaccination and 121 (46.9%) had experienced a common cold (96 patients) or ILI (25 patients) during the analysis period. MG symptoms were aggravated in 10 (40%) patients after ILI, whereas only 2 (1.5%) experienced aggravation following influenza vaccination. The rate of symptom aggravation was significantly higher in patients experiencing an ILI (10/25, 40%) than in those with the common cold (15/96, 15.6%, p=0.006). CONCLUSIONS: The results of this study suggest that the potential risk of aggravating autoimmune disease is higher for ILI than for influenza vaccination, which further suggests that influenza vaccination can be offered to patients with MG.
Autoimmune Diseases ; Common Cold ; Cough ; Fever ; Follow-Up Studies ; Humans ; Influenza Vaccines ; Influenza, Human* ; Male ; Muscle Weakness ; Myasthenia Gravis* ; Pharyngitis ; Vaccination*

BACKGROUND: Connexin 43-mediated gap junctional communication plays an important role in atherosclerosis. Numerous studies have demonstrated a correlation between mitral valve annular calcification and atherosclerotic disease. However, the relevance of connexin 43 to mitral valve disease remains unclear. We hypothesized that the mechanism contributing to mitral valve disease is associated with alterations in cell-to-cell communication mediated by changes in Connexin 43 expression. MATERIAL AND METHOD: Twenty male New Zealand rabbits were divided into two groups: animals in group 1 (n=10) were fed a normal chow diet, whilst those in group 2 (n=10) received a diet containing 1% cholesterol for 12 weeks. After sacrificing the animals, the mitral valves were excised and analyzed with immunohistochemical staining and Real-time Reverse Transcriptase polymerase chain reaction (real time RT-PCR). RESULT: Myofibroblasts and macrophages were found concentrated within the endothelial layer on the ventricular side of the leaflet in the cholesterol diet group. Immunohistochemial staining showed elevated expression of connexin43 in the cholesterol diet group. Real-time RT-PCR revealed increased connexin43 mRNA levels in mitral valves from hypercholesterolemic animals. CONCLUSION: Our finding that connexin43 expression is increased in mitral valves of hypercholesterolemic rabbits suggests that alterations in cell-to-cell communication via connexin43 containing gap junctions play a role in the development of mitral valve disease in hypercholesterolemia.
Animals ; Atherosclerosis ; Cholesterol ; Connexin 43 ; Diet ; Gap Junctions ; Humans ; Hypercholesterolemia ; Macrophages ; Male ; Mitral Valve ; Myofibroblasts ; Rabbits ; Reverse Transcriptase Polymerase Chain Reaction ; RNA, Messenger ; Up-Regulation

Bronchiolitis obliterans (BO) or bronchiolitis obliterans organizing pneumonia (BOOP) is one of manifestations of graft-versus-host disease (GVHD), a complication of hematopoietic stem cell transplantation (HSCT). Recently there are reports about thoracic air-leakage syndrome (TALS), but real incidence, clinical course, and implications of TALS remain unclear. Retrospective review of 18 TALS patients among 2,177 patients who received allogeneic HSCT between January 2000 to July 2007 was done. Clinical manifestations, treatments, and outcomes of TALS were reviewed. The incidence of TALS was 0.83% (18/2,177). The onset of TALS was mean 425.9+/-417.8 days (60-1,825 days) after HSCT, and the duration was mean 16.3+/-21 days (2-90 days). The most common types of TALS were spontaneous pneumothroax (n=14), followed by subcutaneous emphysema (n=6), pneumomediastinum (n=5), interstitial emphysema (n=2), and pneumopericardium (n=1). TALS persisted in six patients, who died during the same hospitalization. The 12 patients recovered from TALS, but only 2 survived, while others died due to aggravation of GVHD. TALS may complicate BO/BOOP and be an initial manifestation of BO/BOOP. TALS is hard to be resolved, and even after the recovery, patients die because of aggravation of GVHD. We suggest specifically in HSCT patients, when once developed, TALS seems hard to be cured, and as a result, be related to high fatality.
Adolescent ; Adult ; Comorbidity ; Female ; Graft vs Host Disease/*mortality/*surgery ; Hematopoietic Stem Cell Transplantation/*mortality ; Hemothorax/*mortality ; Humans ; Incidence ; Korea ; Male ; Middle Aged ; Pneumothorax/*mortality ; Prognosis ; Risk Assessment ; Risk Factors ; Survival Analysis ; Survival Rate ; Syndrome ; Treatment Outcome ; Young Adult

BACKGROUND: The treatment of tracheal stenosis includes less invasive bronchoscopic intervention and more invasive segmental resection & anastomosis. Depending on the patient's clinical features, sometimes all these methods are inappropriate. Silicone T-tube stenting has recently been used as an alternative, safe management of tracheal stenosis. We studied the short term and Intermediate term results of using T-tubes in patients with tracheal stenosis, and this tracheal stenosis was caused by various underlying diseases. MATERIAL AND METHOD: We retrospectively reviewed 57 patients with tracheal stenosis and who were treated with T-tubes between Jan 1997 and Apr 2007. Based on the patient's medical records and the imaging studies, we evaluated the clinical findings and status of T-tube removal. RESULT: There was no T-tube related morbidity or mortality in this series. On follow-up, one patient underwent sleeve resection and end-to-end anastomosis. The T-tube could be successfully removed from 13 patients (13/57, 22.8%) without additional interventions. For another four patients, a T-tube was again inserted after removal of the first T-tube due to tracheomalacia or recurrent stenosis. Four patients died of underlying disease and cancer. The patients' gender and previous tracheostomy significantly affected T-tube removal. By contrast, multiple logistic regression analysis identified gender as a predictor of successfully removing a T-tube. Gender (p=0.033) and previous tracheostomy (p=0.036) were the two factors for success or failure of T-tube removal. CONCLUSION: A T-tube provided reliable patency of a stenotic airway that was caused by any etiology. We have proven that using a T-tube is safe and effective therapy for patients with tracheal stenosis for the short term or the intermediate term.
Constriction, Pathologic ; Follow-Up Studies ; Humans ; Logistic Models ; Medical Records ; Retrospective Studies ; Silicones ; Stents ; Tracheal Stenosis ; Tracheomalacia ; Tracheostomy

BACKGROUND: Primary focal hyperhidrosis is characterized by overactivity of the sympathetic nervous function, and this has been effectively treated with endoscopic thoracic sympathetic denervation (ESD). The imbalance of sympathetic and parasympathetic nervous system that's created by ESD may affect the heart, lung and other thoracic organs. We analyzed the heart rate and ECG changes after performing ESD at our hospital, and this is the first such study that has been conducted on this. MATERIAL AND METHOD: Of the 263 patients who underwent ESD between October 1996 and October 2006, 130 had ECG before and after ESD, and they were classified into 3 groups according to the level of ESD: Group I (n=40) patients underwent ESD at the 2nd rib (T2ESD), Group II (n=80) at the 3rd rib (T3ESD) and Group III (n=10) at the 4th rib (T4ESD). RESULT: There was no mortality or major morbidity. Heart rate (HR) was significantly decreased from 71.6+/-10.6/min to 66.8+/-10.2/min after ESD (p<0.01); however, the PR (from 148.6+/-21.2 msec to 152.8+/-20.5 msec) and QTc (from 399.2+/-15.4 msec to 404.0+/-15.1 msec) intervals were significantly increased after ESD in the patients who suffered with primary hyperhidrosis (p<0.01). According to the level of ESD, there were significant changes in the HR and QTc interval in group I (T2ESD), the HR and PR interval in group II and the QTc interval in Group III. CONCLUSION: There were significant changes in the heart rate and ECG findings after ESD. The thoracic sympathetic denervation of T2, T3 and T4 affected the electrical activity of the heart at the resting state.
Electrocardiography ; Heart ; Heart Rate ; Humans ; Hyperhidrosis ; Lung ; Parasympathetic Nervous System ; Ribs ; Sympathectomy

BACKGROUND: Tracheal reconstruction after extended tracheal resection still remains as a major surgical challenge because good clinical outcomes are usually correlated with limited tracheal resection. Recent investigations with a using cryopreserved trachea for the reconstruction of a trachea have been carried out to overcome this problem. In this study, we analyzed viability of tracheas, which is an important determining factor for the success of transplanting a cryopreserved trachea and the development of post-transplantation tracheal stenosis, according to three different experimental factors: 1) the warm-ischemic time, 2) the cryopreservation solution and 3) the preserving temperature, to determine a better cryopreservation protocol and a better composition of the cryopreservation solution. MATERIAL AND METHOD: Rats tracheas were harvested for different warm-ischemic times (0 hr, 12 hrs, 24 hrs). The tracheas were treated with recombinant insulin growth factor-1 (IGF-1) and they were stored at three different temperatures (4 degreesC, -80 degreesC, -196 degreesC) for two weeks. After two weeks, we thawed the stored trachea and isolated the cells of the tracheas with using type II collagenase. We cultured the cells for seven days and then we compared the cellular viability by the MTT reduction assay. RESULT: Though cryopreservation is required to preserve a trachea for a longer time period, the viability of the tracheas stored at -80 degreesC and -196 degreesC was significantly reduced compared to that of the tracheas stored at 4 degreesC. The viability of the tracheas with warm-ischemic times of 12 hrs and 24 hrs was also reduced in comparison to the tracheas with a warm-ischemic time of 0 hrs.Department of Thoracic and Cardiovascular Surgery, St. Mary's Hospital, The Catholic University of Korea College of Medicine Our data showed that the warm ischemic time and the parameters of cryopreservation negatively affect on trachea viability. However, a cryopresrvation solution containing IGF-1 improved the cellular viability better than the existing cryopreservation solution. For the warm ischemic time group of 0 hr, the addition of IGF-1 improved the viability of trachea at all the preserving temperatures. CONCLUSION: These experiments demonstrate that the viability of a cryopreserved trachea can be improved by modifying the components of the cryopreservation solution with the addition of IGF-1 and reducing the warm-ischemic time.
Animals ; Collagenases ; Cryopreservation ; Insulin ; Insulin-Like Growth Factor I ; Korea ; Rats ; Trachea ; Tracheal Stenosis ; Transplants ; Warm Ischemia