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( Min Jung Kim ),( Yoon-jeong Oh ),( Yoon Ho Hong ),( Sung-hye Park ),( Ji Seon Oh ),( Min Jung Kim ),( Jong-hee Chae ),( Kichul Shin ) 대한류마티스학회 2021 대한류마티스학회지 Vol.28 No.2
Limb-Girdle Muscular Dystrophy 2B (LGMD2B) presents with proximal and/or distal muscle weakness and markedly high creatine kinase level. It is caused by the loss of dysferlin due to mutations in the DYSF gene. Due to its similar clinical features as inflammatory myopathy, it is often difficult to distinguish between the two. We present a case of a 48-year-old male who developed progressive proximal muscle weakness, papulosquamous lesions on the knuckles, elevated levels of muscle enzymes, and electromyogram abnormalities. Based on the clinical presentation, the initial impression was dermatomyositis, yet it was refractory to immunosuppressive therapy. Subsequently, dysferlin immunostaining and genetic analysis led to the final diagnosis of LGMD2B. This case shows that LGMD2B can present with extramuscular symptoms mimicking inflammatory myopathy in later stages of life. Dysferlin immunostaining and/or genetic analysis of the DYSF gene are essential for its diagnosis. (J Rheum Dis 2021;28:101-106)
Kichul Shin,Jinsu Kim,Seokyoung Yoon,Eung-Ho Cho,Changwon Jung,Hye Jin Kang 이화여자대학교 의과학연구소 2015 EMJ (Ewha medical journal) Vol.38 No.3
A 37-year-old woman underwent a total mastectomy and adjuvant chemotherapy for HER2-positive breast cancer (pT1N0M), and then recurred in the right lung followed by the pancreas. Lung lobectomy and pylorus-preserving pancreaticoduodenectomy were performed, and systemic chemotherapies including trastuzumab were sequentially administered. However, metastasis to the pancreatic tail was detected. She underwent image-guided radiation therapy, but this was not effective. Lapatinib plus capecitabine combination was administered as forth-line treatment and the metastatic lesion was disappeared. She is continuing this regimen with a complete response for 48 months until now.
Kichul Shin,Hyun Mi Kwon,Min Jung Kim,Myung Jae Yoon,Hyun Gyung Chai,Seong-Wook Kang,Won Park,Sung-Hwan Park,Chang-Hee Suh,Hyun Ah Kim,Seung-Geun Lee,Choong Ki Lee,배상철,Yong-Beom Park,Yeong Wook Song 대한내과학회 2022 The Korean Journal of Internal Medicine Vol.37 No.5
Background/Aims: The aim of this study was to investigate long-term post-discontinuation outcomes in patients with rheumatoid arthritis (RA) who had been treated with tumor necrosis factor-α inhibitors (TNF-αi) which was then discontinued. Methods: Sixty Korean patients with RA who participated in a 5-year GO-BEFORE and GO-FORWARD extension trials were included in this retrospective study. Golimumab was deliberately discontinued after the extension study (baseline). Patients were then followed by their rheumatologists. We reviewed their medical records for 2 years (max 28 months) following golimumab discontinuation. Patients were divided into a maintained benefit (MB) group and a loss-of-benefit (LB) group based on treatment pattern after golimumab discontinuation. The LB group included patients whose conventional disease-modifying antirheumatic drug(s) were stepped-up or added/switched (SC) and those who restarted biologic therapy (RB). Results: The mean age of patients at baseline was 56.5 years and 55 (91.7%) were females. At the end of follow-up, 23 (38.3%) patients remained in the MB group. In the LB group, 75.7% and 24.3% were assigned into SC and RB subgroups, respectively. Fifty percent of patients lost MB after 23.3 months. Demographics and clinical variables at baseline were comparable between MB and LB groups except for age, C-reactive protein level, and corticosteroid use. Restarting biologic therapy was associated with swollen joint count (adjusted hazard ratio [HR], 1.90; 95% confidence interval [CI], 1.01 to 3.55) and disease duration (adjusted HR, 1.12; 95% CI, 1.02 to 1.23) at baseline. Conclusions: Treatment strategies after discontinuing TNF-αi are needed to better maintain disease control and quality of life of patients with RA.
Dynamic Changes in Helicobacter pylori Status Following Gastric Cancer Surgery
( Kichul Yoon ),( Nayoung Kim ),( Jaeyeon Kim ),( Jung Won Lee ),( Hye Seung Lee ),( Jong-chan Lee ),( Hyuk Yoon ),( Cheol Min Shin ),( Young Soo Park ),( Sang-hoon Ahn ),( Do Joong Park ),( Hyung Ho 대한소화기학회 2017 Gut and Liver Vol.11 No.2
Background/Aims: Helicobacter pylori eradication is recom-mended in patients with early gastric cancer. However, the possibility of spontaneous regression raises a question for clinicians about the need for “retesting” postoperative H. py-lori status. Methods: Patients who underwent curative gas-trectomy at Seoul National University Bundang Hospital and had a positive H. pylori status without eradication therapy at the time of gastric cancer diagnosis were prospectively enrolled in this study. H. pylori status and atrophic gastritis (AG) and intestinal metaplasia (IM) histologic status were as-sessed pre- and postoperatively. Results: One hundred forty patients (mean age, 59.0 years; 60.7% male) underwent subtotal gastrectomy with B-I (65.0%), B-II (27.1%), Roux-en-Y (4.3%), jejunal interposition (0.7%), or proximal gastrectomy (4.3%). Preoperative presence of AG (62.9%) and IM (72.9%) was confirmed. The mean period between surgery and the last endoscopic follow-up was 38.0±25.6 months. Of the 140 patients, 80 (57.1%) were found to be persistently posi-tive for H. pylori, and 60 (42.9%) showed spontaneous nega-tive conversion at least once during follow-up. Of these 60 patients, eight (13.3%) showed more complex postoperative dynamic changes between negative and positive results. The spontaneous negative conversion group showed a trend of having more postoperative IM compared to the persistent H. pylori group. Conclusions: A high percentage of spontaneous regression and complex dynamic changes in H. pylori status were observed after partial gastrectomy, especially in individ-uals with postoperative histological IM. It is better to consider postoperative eradication therapy after retesting for H. pylori. (Gut Liver 2017;11:209-215)
( Kichul Shin ),( Sung Soo Kim ),( Sang-heon Lee ),( Seung-jae Hong ),( Sung Jae Choi ),( Jung-yoon Choe ),( Seung-geun Lee ),( Hoon-suk Cha ),( Eun Young Lee ),( Sung-hwan Park ),( Jin-wuk Hur ),( Su 대한내과학회 2020 The Korean Journal of Internal Medicine Vol.35 No.1
Background/Aims: The objective of this study was to compare changes in the simplified disease activity index (SDAI) between biologic (b) and conventional (c) disease-modifying antirheumatic drugs (DMARD) users with seropositive rheumatoid arthritis (RA) in daily clinical practice. Methods: This was a nationwide multicenter observational study. Patients who had three or more active joint counts and abnormal inf lammatory marker in blood test were enrolled. The selection of DMARDs was determined by the attending rheumatologist. Clinical parameters, laboratory findings, and Health Assessment Questionnaire (HAQ) scores were obtained at baseline and at 6 and 12 months. Serial SDAI changes and clinical remission rate at 6 and 12 months were assessed. Results: A total of 850 patients participated in this study. The mean baseline SDAI score in bDMARD group was higher than that in cDMARD group (32.08 ± 12.98 vs 25.69 ± 10.97, p < 0.0001). Mean change of SDAI at 12 months was -19.0 in the bDMARD group and -12.6 in the cDMARD group (p < 0.0001). Clinical remission rates at 12 months in bDMARD and cDMARD groups were 15.4% and 14.6%, respectively. Patient global assessment and HAQ at 12 months were also significantly improved in both groups. Multivariate logistic regression showed that baseline HAQ score was the most notable factor associated with remission. Conclusions: There was a significant reduction in SDAI within 12 months after receiving DMARDs in Korean seropositive RA patients irrespective of bDMARD or cDMARD use in real-world practice. Clinical remission was achieved in those with lower baseline HAQ scores.
Cho, Kichul,Wencheng, Li,Takeshita, Satoshi,Seo, Jung-Kil,Chung, Young-Ho,Kim, Daekyung,Oda, Tatsuya Elsevier 2017 Aquatic toxicology Vol.189 No.-
<P><B>Abstract</B></P> <P> <I>Heterocapsa circularisquama</I>, a harmful dinoflagellate, has multiple haemolytic toxins that are considered to be involved in the toxic mechanism against shellfish and certain species of zooplankton. To evaluate the further nature of the toxins of <I>H. circularisquama</I>, we investigated its effects on several species of bacteria. By colony formation assay, we found that <I>H. circularisquama</I> had antibacterial activity toward the marine bacterium <I>Vibrio alginolyticus</I> in a cell density-dependent manner. When the inoculated bacterial cells were co-cultured with <I>H. circularisquama</I> under dinoflagellate cell culture conditions, the bacterial growth was significantly suppressed, whereas the number of live bacterial cells increased when cultured in the medium alone. Since the cell-free culture supernatant and the ruptured dinoflagellate cell suspension showed no toxic effects on <I>V. alginolyticus</I>, it is speculated that direct cell-to-cell contact mediated by the live dinoflagellate cells may be the major toxic mechanism. The decrease in bactericidal activity of theca-removed dinoflagellate cells may further support this speculation. <I>H. circularisquama</I> also showed bactericidal activities towards <I>Escherichia coli</I> and <I>Staphylococcus aureus</I>. In the dinoflagellate/bacteria co-culture system, the number of live bacterial cells declined with increasing incubation time. Light-dependent antibacterial activity of the ruptured dinoflagellate cells against <I>S. aureus</I> was observed, whereas no such activity was detected against <I>E. coli</I>. These results suggest that intracellular photosensitising bactericidal toxins, which were previously found to be porphyrin derivatives, may have specificity towards gram-positive bacteria. Based on these results together with previous studies, it is obvious that <I>H. circularisquama</I> possesses antibacterial activity, which may be mediated through toxins located on its cell surface. It is likely that such toxins play a role in the defence mechanism against predators and infectious bacteria. Although the exact biological significance of intracellular photosensitising toxins is still unclear, such toxins may have potential to be developed as novel photo-controllable antibiotics.</P> <P><B>Highlights</B></P> <P> <UL> <LI> <I>H. circularisquama</I> (HC) exhibits anti-bacterial activity toward <I>V. alginolyticus</I>. </LI> <LI> Direct cell contact mediated by the live HC cells may be the major toxic mechanism. </LI> <LI> HC also shows bactericidal activities towards <I>E. coli</I> and <I>S. aureus</I>. </LI> <LI> HC has intracellular photosensitizing agents toxic to <I>S. aureus</I>. </LI> </UL> </P>
( Min Jung Kim ),( Kichul Shin ) 대한결핵 및 호흡기학회 2021 Tuberculosis and Respiratory Diseases Vol.84 No.4
Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.
Utilization of PEST questionnaire to detect early psoriatic arthritis in daily clinical practice
( You-jung Ha ),( So Yeon Cho ),( Sang Woong Youn ),( Kichul Shin ) 대한내과학회 2015 대한내과학회 추계학술발표논문집 Vol.2015 No.1
Background: Psoriatic arthritis (PsA) is a systemic inflammatory condition mostly preceded by long-term psoriasis(PsO). The prevalence of PsA in Koreans is regarded to be lower than Caucasians. PsA is still can be a serious rheumatic disease causing articular and extra-articular problems irrespective of ethnicity or region. Several questionnaires have been developed to help identify PsA among PsO patients, but there is currently no validated screening tool in Korea yet. Methods: The Psoriasis Epidemiology Screening Test, or PEST was translated into Korean and then back-translated to English for comparison; a simple,five questionnaire that could be used without assistance. This PEST form was tested on PsO patients visiting the Dermatology clinic at 5 medical facilities in urban areas. Clinical information including body surface area (BSA), psoriasis area severity index (PASI), nail changes were also obtained. Patients who checked ‘yes’ to 2 or more questions were referred to Rheumatology for further evaluation. Joint exams, followed by laboratory tests and plain imaging were performed. Patients meeting the CASAPR criteria were confirmed to have PsA. Results: Data of 80 PsO patients from 2 centers were analyzed. The mean age was 46.1 years, and gender ratio was near 1: 1. Mean BSA and PASI score was 7.4%, 5.9, respectively. Nail Psoriasis was discovered in 29 (36.3%) patients. Among the 20 (25%) patients checked ‘yes’ to ≥2 questions, 11 (55%) met the CASPAR criteria. For those who checked ‘yes’ to ≥3 questions, 5 (46%) were diagnosed as PsA. When comparing baseline characteristics between PsA and PsO (only) patients, nail psoriasis was highly prevalent in PsA patients (63.6 vs. 31.9%, p=0.047). BSA and PASI were also higher in PsA patients, but did not reach statistical significance. Conclusions: This pilot study supports that PsO patients with musculoskeletal symptoms and nail psoriasis should be carefully evaluated for PsA. A validated, well-adapted PsA screening tool is warranted to help both patients and physicians better detect PsA. This investigator-intitiated study was supported by Abbvie.