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무증상으로 발견된 담낭-담관 누공을 동반한 Mirizzi 증후군 1예
홍진희,전태주,서지영,서동대,오태훈,신원창,최원충,신은아,김기환 인제대학교 2009 仁濟醫學 Vol.30 No.-
Mirizzi's syndrome is a rare complication of the gallstone disease, characterized by narrowing of the common bile duct (CBD) due to chronic extrinsic compression of an impacted gallstone in the cystic duct or the neck of the gallbladder. The impacted gallstone may erode into the bile duct, causing cholecystocholedochal fistula. The patients generally have history of repeated attacks of jaundice and abdominal pain. But there is no report about asymptomatic Mirizzi syndrome with cholecystocholedochal fistula which were found incidentally. So herein we present our clinical experience with a case of asymptomatic Mirizzi syndrome with cholecystocholedochal fistula in a young female.
Choi Yu Hyeon,Jhang Won Kyoung,Park Seong Jong,Choi Hee Joung,Oh Min-su,Kwon Jung Eun,Kim Beom Joon,Shin Ju Ae,Lee In Kyung,Park June Dong,Lee Bongjin,Chung Hyun,Na Jae Yoon,Choi Ah Young,Cho Joongbum 대한의학회 2024 Journal of Korean medical science Vol.39 No.3
Background: Over the last decade, extracorporeal membrane oxygenation (ECMO) use in critically ill children has increased and is associated with favorable outcomes. Our study aims to evaluate the current status of pediatric ECMO in Korea, with a specific focus on its volume and changes in survival rates based on diagnostic indications. Methods: This multicenter study retrospectively analyzed the indications and outcomes of pediatric ECMO over 10 years in patients at 14 hospitals in Korea from January 2012 to December 2021. Four diagnostic categories (neonatal respiratory, pediatric respiratory, postcardiotomy, and cardiac-medical) and trends were compared between periods 1 (2012–2016) and 2 (2017–2021). Results: Overall, 1065 ECMO runs were performed on 1032 patients, with the annual number of cases remaining unchanged over the 10 years. ECMO was most frequently used for post-cardiotomy (42.4%), cardiac-medical (31.8%), pediatric respiratory (17.5%), and neonatal respiratory (8.2%) cases. A 3.7% increase and 6.1% decrease in pediatric respiratory and post-cardiotomy cases, respectively, were noted between periods 1 and 2. Among the four groups, the cardiac-medical group had the highest survival rate (51.2%), followed by the pediatric respiratory (46.4%), post-cardiotomy (36.5%), and neonatal respiratory (29.4%) groups. A consistent improvement was noted in patient survival over the 10 years, with a significant increase between the two periods from 38.2% to 47.1% (P = 0.004). Improvement in survival was evident in post-cardiotomy cases (30–45%, P = 0.002). Significant associations with mortality were observed in neonates, patients requiring dialysis, and those treated with extracorporeal cardiopulmonary resuscitation (P < 0.001). In pediatric respiratory ECMO, immunocompromised patients also showed a significant correlation with mortality (P < 0.001). Conclusion: Pediatric ECMO demonstrated a steady increase in overall survival in Korea; however, further efforts are needed since the outcomes remain suboptimal compared with global outcomes.
Effect of genetic background differences between FVB and C57BL/6 mice in SARS-CoV-2 infection
Ah-Reum Kang,Hyun Ah Noh,Jae Hyung Son,Sun-Min Seo,Ji-Hun Lee,Na-Won Kim,Eun-Seon Yoo,Han-Bi Jeong,Da In On,Ji Yun Jang,Jun-Won Yun,Jun Won Park,Kang-Seuk Choi,Ho-Young Lee,Jun-Young Seo,Ki Taek Nam,J 한국실험동물학회 2022 한국실험동물학회 학술발표대회 논문집 Vol.2022 No.7
Indian Hedgehog signalling triggers Nkx3.2 protein degradation during chondrocyte maturation.
Choi, Seung-Won,Jeong, Da-Un,Kim, Jeong-Ah,Lee, Boyoung,Joeng, Kyu Sang,Long, Fanxin,Kim, Dae-Won Biochemical Society 2012 The Biochemical journal Vol.443 No.3
<P>The Ihh (Indian Hedgehog) pathway plays an essential role in facilitating chondrocyte hypertrophy and bone formation during skeletal development. Nkx3.2 (NK3 homeobox 2) is initially induced in chondrocyte precursor cells, maintained in early-stage chondrocytes and down-regulated in terminal-stage chondrocytes. Consistent with these expression patterns, Nkx3.2 has been shown to enhance chondrocyte differentiation and cell survival, while inhibiting chondrocyte hypertrophy and apoptosis. Thus, in the present study, we investigated whether Nkx3.2, an early-stage chondrogenic factor, can be regulated by Ihh, a key regulator for chondrocyte hypertrophy. We show that Ihh signalling can induce proteasomal degradation of Nkx3.2. In addition, we found that Ihh can suppress levels of Lrp (low-density-lipoprotein-receptor-related protein) (Wnt co-receptor) and Sfrp (secreted frizzled-related protein) (Wnt antagonist) expression, which, in turn, may selectively enhance Lrp-independent non-canonical Wnt pathways in chondrocytes. In agreement with these findings, Ihh-induced Nkx3.2 degradation requires Wnt5a, which is capable of triggering Nkx3.2 degradation. Finally, we found that Nkx3.2 protein levels in chondrocytes are remarkably elevated in mice defective in Ihh signalling by deletion of either Ihh or smoothened. Thus these results suggest that Ihh/Wnt5a signalling may play a role in negative regulation of Nkx3.2 for appropriate progression of chondrocyte hypertrophy during chondrogenesis.</P>
Molecular Genetic Analysis of MT-TL1 Gene in Patients with MELAS Syndrome
( Won Ah Choi ),( Min Young Park ),( Chang Do Hyun ),( Eun Sim Shin ) 대한임상병리사협회 2010 조직세포검사학회 발표자료집 Vol.2010 No.-
Background: MELAS (Mitochondrial Encephalomyopathy, Lactic acidosis, and Stroke like episodes, OMIM 540000) is one of the most frequent diseases caused by mitochondrial DNA (mtDNA) mutations and is a genetically heterogeneous mitochondrial disorder with variable clinical phenotypes. The diagnosis of MELAS is based on a combination of clinical findings, including stroke like episodes, encephalopathy with seizures, mitochondrial myopathy and lactic acidosis and molecular genetic testing of mtDNA. It has been reported that mutation of MT-TL1 gene of mtDNA encoding tRNA Leu(UUR) is associated with approximately 80% of MELAS cases. Most of mutation in MT-TL1 gene is an A-to-G transition at nucleotide 3243 (A3243G) (80%). Moreover, T3271C (7.5%), A3252G (<5%) mutations of MT-TL1 gene are also found as well as additional rare mutations. In the present study, MT-TL1 gene mutations, including A3243G, T3271C and A3252G, were analyzed by PCR-sequencing. Materials and Methods: Genomic DNA was isolated from peripheral blood sample by using G-DEX™ IIb DNA Extraction Kit (iNtRON, Korea), according to the manufacturer``s instructions. Extracted DNA was amplified by PCR with MT-TL1 gene specific primers. Amplified PCR product was analyzed by 2% agarose gel electrophoresis, and was sequenced on a 3130 Genetic Analyzer (Applied Biosystems, USA). The sequences were compared with those in GenBank using the BLAST program for detecting MT-TL1 gene mutations. Results: We developed PCR-sequencing to detect the mutation of MT-TL1 gene. The result showed clear sequencing data and mutations were not found in normal healthy control. Discussion: The MELAS syndrome is typically a multi systemic disorder presenting in childhood. However both clinical features, the appearance of symptoms and the phenotypic spectrum show great varieties. Therefore molecular genetic analysis is helpful for the diagnosis of MELAS syndrome. The PCR-sequencing technology used by this study, could be applicable for molecular genetic laboratories to find MT-TL1 gene mutations in patients with MELAS syndrome.
Choi, Jung Won,Son, Sung Min,Mook-Jung, Inhee,Moon, Youn Joo,Lee, Ji Yeoun,Wang, Kyu-Chang,Kang, Hyun-Seung,Phi, Ji Hoon,Choi, Seung Ah,Chong, Sangjoon,Byun, Jayoung,Kim, Seung-Ki Journal of Neurosurgery Publishing Group 2018 Journal of neurosurgery Vol.129 No.5
<B>OBJECTIVE</B><P>Moyamoya disease (MMD) is a unique cerebrovascular disorder characterized by the progressive occlusion of the bilateral internal carotid arteries. Endothelial colony-forming cells (ECFCs), previously termed “endothelial progenitor cells,” play an important role in the pathogenesis of MMD. In this study, the authors performed morphological and functional studies of the mitochondria of ECFCs from patients with MMD to present new insights into the pathogenesis of the disease.</P><B>METHODS</B><P>The morphology of ECFCs from 5 MMD patients and 5 healthy controls was examined under both a transmission electron microscope and a confocal laser scanning microscope. The oxygen consumption rates (OCRs), mitochondrial membrane potentials (MMPs), intracellular Ca<SUP>2+</SUP> concentrations, mitochondrial enzyme activities, and reactive oxygen species (ROS) levels were measured. Functional activity of the ECFCs was evaluated using a capillary tube formation assay.</P><B>RESULTS</B><P>The ECFCs from the MMD patients displayed a disrupted mitochondrial morphology, including a shorter and more circular shape. The ECFC mitochondria from the MMD patients exhibited functional abnormalities, which were assessed as a decreased OCR and an increased intracellular Ca<SUP>2+</SUP> concentration. Moreover, the ECFCs from MMD patients showed increased ROS levels. Interestingly, treatment with an ROS scavenger not only reversed the mitochondrial abnormalities but also restored the angiogenic activity of the ECFCs from the MMD patients.</P><B>CONCLUSIONS</B><P>The mitochondria of ECFCs from MMD patients, as compared with those from healthy patients, exhibited morphological and functional abnormalities. This finding suggests that the mitochondrial abnormalities may have a role in the pathogenesis of MMD.</P>