1.Genetic Variants and Clinical Phenotypes in Korean Patients With Hereditary Hemorrhagic Telangiectasia
Bo-Gyeong KIM ; Joo-hyun JUNG ; Mi-Jung KIM ; Eun-Hye MOON ; Jae-Hwan OH ; Jung-Woo PARK ; Heung-Eog CHA ; Ju-Hyun KIM ; Yoon-Jae KIM ; Jun-Won CHUNG ; Ki-Baik HAHM ; Hong-Ryul JIN ; Yong-Ju JANG ; Sung Wan KIM ; Seung-Kyu CHUNG ; Dae-Woo KIM ; Young Jae LEE ; Seon-Tae KIM
Clinical and Experimental Otorhinolaryngology 2021;14(4):399-406
Objectives:
. Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disorder characterized by recurrent epistaxis, telangiectasia, and visceral arteriovenous malformations (AVMs). Activin A receptor-like type 1 (ACVRL1/ALK1) and endoglin (ENG) are the principal genes whose mutations cause HHT. No multicenter study has yet investigated correlations between genetic variations and clinical outcomes in Korean HHT patients.
Methods:
. Seventy-two members from 40 families suspected to have HHT based on symptoms were genetically screened for pathogenic variants of ACVRL1 and ENG. Patients with genetically diagnosed HHT were also evaluated.
Results:
. In the HHT genetic screening, 42 patients from 24 of the 40 families had genetic variants that met the pathogenic criteria (pathogenic very strong, pathogenic strong, pathogenic moderate, or pathogenic supporting) based on the American College of Medical Genetics and Genomics Standards and Guidelines for either ENG or ACVRL1: 26 from 12 families (50%) for ENG, and 16 from 12 families (50%) for ACVRL1. Diagnostic screening of 42 genetically positive HHT patients based on the Curaçao criteria revealed that 24 patients (57%) were classified as having definite HHT, 17 (41%) as having probable HHT, and 1 (2%) as unlikely to have HHT. Epistaxis was the most common clinical presentation (38/42, 90%), followed by visceral AVMs (24/42, 57%) and telangiectasia (21/42, 50%). Five patients (12%) did not have a family history of HHT clinical symptoms.
Conclusion
. Only approximately half of patients with ACVRL1 or ENG genetic variants could be clinically diagnosed as having definite HHT, suggesting that genetic screening is important to confirm the diagnosis.
2.Clinical Endoscopy as One of Leading Journals in Gastrointestinal Endoscopy.
Kwang An KWON ; Il Ju CHOI ; Ji Kon RYU ; Eun Young KIM ; Ki Baik HAHM
Clinical Endoscopy 2015;48(4):312-316
Clinical Endoscopy (CE) is an official open access journal published bimonthly by the Korean Society of Gastrointestinal Endoscopy (KSGE, http://www.gie.or.kr) and is listed on PMC, PubMed and SCOPUS. The KSGE was established on August 14, 1976, and the journal of the KSGE was published in Korean for the first time in November 1981. The journal was then titled the "Korean Journal of Gastrointestinal Endoscopy" and was published in Korean untill the July 2011 issue. The journal was published in English from the September 2011 issue under the official title of CE. In this review, the past and present of CE are discussed and future perspectives are introduced. In addition, the efforts to progress to a "first come, first served journal" in the field of gastrointestinal endoscopy and to be indexed in Science Citation Index will be described.
Endoscopy*
;
Endoscopy, Gastrointestinal*
3.Highlights of International Digestive Endoscopy Network 2013.
Kwang An KWON ; Il Ju CHOI ; Eun Young KIM ; Seok Ho DONG ; Ki Baik HAHM
Clinical Endoscopy 2013;46(5):425-435
Rapid advances in the technology of gastrointestinal endoscopy as well as the evolution of science have made it necessary for us to continue update in either various endoscopic techniques or state of art lectures relevant to endoscopy. International Digestive Endoscopy Network (IDEN) 2013 was held in conjunction with Korea-Japan Joint Symposium on Gastrointestinal Endoscopy (KJSGE) during June 8 to 9, 2013 at Seoul, Korea. Two days of impressive scientific program dealt with a wide variety of basic concerns from upper gastrointestine (GI), lower GI, pancreaticobiliary endoscopy to advanced knowledge including endoscopic submucosal dissection forum. IDEN seems to be an excellent opportunity to exchange advanced information of the latest issues on endoscopy with experts from around the world. In this special issue of Clinical Endoscopy, we prepared state of art review articles from contributing authors and the current highlights will skillfully deal with very hot spots of each KJSGE, upper GI, lower GI, and pancreaticobiliary sessions by associated editors of Clinical Endoscopy.
Endoscopy
;
Endoscopy, Gastrointestinal
;
Joints
;
Korea
;
Lectures
4.Highlights of the 48th Seminar of Korean Society of Gastrointestinal Endoscopy.
Kwang An KWON ; Il Ju CHOI ; Eun Young KIM ; Seok Ho DONG ; Ki Baik HAHM
Clinical Endoscopy 2013;46(3):203-211
This special May issue of Clinical Endoscopy discusses the tutorial contents dealing with either the diagnostic or therapeutic gastrointestinal (GI) endoscopy that contain very fundamental and essential points in this filed. The seminar of Korean Society of Gastrointestinal Endoscopy (KSGE) had positioned as one of prime educational seminars covering the very beginner to advanced experts of GI endoscopy. Besides of four rooms allocated for each lecture, two additional rooms were open for either live demonstration or hands-on course, covering totally 20 sessions including one special lecture. Among these prestigious lectures, 12 lectures were selected for the current review articles in this special issue of Clinical Endoscopy journal. Basic course for beginner to advanced tips to expert were all covered in this seminar. This introductory review prepared by four associated editors of Clinical Endoscopy contained core contents divided into four sessions-upper gut, lower gut, pancreaticobiliary, and specialized topic session part-to enhance understandings not covered by enlisted review articles in this issue.
Endoscopy
;
Endoscopy, Gastrointestinal
;
Lectures
5.Huge Liposarcoma of Esophagus Resected by Endoscopic Submucosal Dissection: Case Report with Video.
Inku YO ; Jun Won CHUNG ; Myung Ho JEONG ; Jong Joon LEE ; Jungsuk AN ; Kwang An KWON ; Min Young RIM ; Ki Baik HAHM
Clinical Endoscopy 2013;46(3):297-300
Liposarcoma is one of the most common soft tissue sarcomas occurring in adults, but it rarely occurs in the gastrointestinal tract and more uncommonly in the esophagus. To the best of our knowledge, there are only 19 reported cases of esophageal liposarcoma in the literature published in English language up to the year 2008, and they were all treated by surgical methods. Here, we report a case of primary liposarcoma of the esophagus which was treated with endoscopic submucosal dissection (ESD). ESD was well tolerated in this patient, suggesting that it may be a therapeutic option for primary esophageal sarcomas.
Adult
;
Endoscopy
;
Esophageal Neoplasms
;
Esophagus
;
Gastrointestinal Tract
;
Humans
;
Liposarcoma
;
Sarcoma
6.International Digestive Endoscopy Network 2012: A Patchwork of Networks for the Future.
Kwang An KWON ; Il Ju CHOI ; Eun Young KIM ; Seok Ho DONG ; Ki Baik HAHM
Clinical Endoscopy 2012;45(3):209-210
This special September issue of Clinical Endoscopy will discuss various aspects of diagnostic and therapeutic advancement of gastrointestinal (GI) endoscopy, explaining what is new in digestive endoscopy and why international network should be organized. We proposed an integrated model of international conference based on the putative occurrence of Digestive Endoscopy Networks. In International Digestive Endoscopy Network (IDEN) 2012, role of endoscopy in gastroesophageal reflux disease and Barrett's esophagus, endoscopy beyond submucosa, endoscopic treatment for stricture and leakage in upper GI, how to estimate the invasion depth of early GI cancers, colonoscopy in inflammatory bowel disease (IBD), a look into the bowel beyond colon in IBD, management of complications in therapeutic colonoscopy, revival of endoscopic papllirary balloon dilation, evaluation and tissue acquisition for indeterminate biliopancreatic stricture, updates in the evaluation of pancreatic cystic lesions, issues for tailored endoscopic submucosal dissection (ESD), endoluminal stents, management of upper GI bleeding, endoscopic management of frustrating situations, small bowel exploration, colorectal ESD, valuable tips for frustrating situations in colonoscopy, choosing the right stents for endoscopic stenting of biliary strictures, advanced techniques for pancreaticobiliary visualization, endoscopic ultrasound-guided biliopancreatic drainage, and how we can overcome the obstacles were deeply touched. We hope that IDEN 2012, as the very prestigious endoscopy networks, served as an opportunity to gain some clues for further understanding of endoscopic technologies and to enhance up-and-coming knowledge and their clinical implications from selected 25 peer reviewed articles and 112 invited lectures.
Barrett Esophagus
;
Colon
;
Colonoscopy
;
Constriction, Pathologic
;
Drainage
;
Endoscopy
;
Gastroesophageal Reflux
;
Hemorrhage
;
Inflammatory Bowel Diseases
;
Lectures
;
Pancreatic Cyst
;
Peer Review
;
Stents
7.Erratum: International Digestive Endoscopy Network 2012: A Patchwork of Networks for the Future.
Kwang An KWON ; Il Ju CHOI ; Eun Young KIM ; Seok Ho DONG ; Ki Baik HAHM
Clinical Endoscopy 2012;45(4):454-454
The name of the IDEN 2011 member should be Prof. Young-Tae Bak (Korea University Guro Hospital, Seoul, Korea) instead of Prof. Young-Tae Kim (Korea University Guro Hospital, Seoul, Korea).
8.The Radial Artery Superficial Palmar (RASP) Branch Free Flap for Finger Soft Tissue Reconstruction.
Yong Jin KIM ; Young Suk SUH ; Sang Hyun LEE ; Dong Gil HAHM
Journal of the Korean Microsurgical Society 2012;21(1):21-26
The radial artery superficial palmar branch free flap is based on the perforators of the superficial palmar branch of the radial artery and its venae comitantes. This flap can be used as a sensible flap including palmar cutaneous branch of the median nerve. Forty radial artery superficial palmar branch free flaps were performed at Centum Institute during October 2010 to December 2011. There were 32 males and 8 females and their mean age were 48 years (range 30 to 66 years). The thumb injured in 13 patients, the index finger in 16 patients, the middle finger in 4 patients, the ring finger in 2 patients, and the little finger in 5 patients. The mean size of the flap was 2.5x3.5 cm(range 2x2.5 to 3x7 cm). The donor site was always closed primarily. The overall survival rate was 90.2 percent. The flaps showed well-padded tissue with glabrous skin. All patients have touch sensation and showed 12 mm two point discrimination in an average(range 8 to 15 mm). Donor site morbidity was conspicuous. One patient showed unsightly scar. Early postoperative range of motion of the affected thumb showed slightly limited radial and palmar abduction. But it improved after postoperative 2 months, and patients did not complaint limitation of motion. In conclusion, the radial artery superficial palmar branch free flap can be used as an option for soft tissue reconstruction of finger defects where local or island flaps are unsuitable.
Cicatrix
;
Discrimination (Psychology)
;
Female
;
Fingers
;
Free Tissue Flaps
;
Humans
;
Male
;
Median Nerve
;
Radial Artery
;
Range of Motion, Articular
;
Sensation
;
Skin
;
Surgical Flaps
;
Survival Rate
;
Thumb
;
Tissue Donors
9.Factors Predicting Recidivism within 6 Months after Release among Arrested Delinquent Adolescents.
Dong Hyuck SUH ; Sun Ju CHUNG ; Chang Ho SOHN ; Won Sik KIM ; Seung Hee KOH ; Bong Jin HAHM ; Sung Jin CHO ; Young Ki KIM ; Joong Je LEE
Journal of Korean Neuropsychiatric Association 2001;40(3):463-476
OBJECTIVES: This study was performed to investigate the factors predicting recidivism within 6 months after release in arrested juvenile delinquents for proper screening and intervention of high-risk subjects. METHODS: The subjects consisted of 125 arrested male adolescents who were released after legal procedure in prosecutor office and court. The socio-demographic and crime-related characteristics were evaluated by police records, and the familial, psychopathology and personality characteristics, and the level of moral development measured by the Conflict Tactics Scale(CTS), Family Environment Scale(FES), Parental Behavioral Inventory, Symptom Checklist-90-Revised(SCL-90-R), Beck Depression Inventory, Beck Anxiety Inventory, Korean form of Moral Developmental Inventory for adolescents, and Personality Disorder Questionnaire-Revised(PDQ-R). RESULTS: The recidivism rate within 6 months after release was 44% of the initial sample. Recidivists were younger and had higher scores on Anger-hostility scale of SCL-90-R and lower principle morality score than nonrecidivists. More recidivists had history of physical abuse and antisocial personality disorder on PDQ-R than nonrecidivists. They also showed the crimerelated characteristics of lower percent of violence crime, higher incidence of previous conviction, younger age of the first crime. Among those variables, logistic regression showed that age of first crime, antisocial personality disorder, and history of physical abuse best predicted the recidivism within 6months after release. CONCLUSION: These results of present study suggest that the recidivism rate of arrested male adolescents is very high, and the age of first crime, antisocial personality disorder and history of physical abuse were key factors predicting recidivism. To prevent repeated crime of delinquent adolescents, the development of programs for those high-risk groups to help rehabilitation and adaptation in community would be essential.
Adolescent*
;
Antisocial Personality Disorder
;
Anxiety
;
Crime
;
Depression
;
Humans
;
Incidence
;
Logistic Models
;
Mass Screening
;
Moral Development
;
Morals
;
Parents
;
Personality Disorders
;
Police
;
Psychopathology
;
Rehabilitation
;
Violence
10.Chronic Recurrent Multifocal Osteomyelitis
Duck Yun CHO ; Young Gil HAHM ; Jong Woo KIM
The Journal of the Korean Orthopaedic Association 1996;31(4):920-927
Chronic recurrent multifocal osteomyelitis (CRMO) is a rare and recently recognized disease of unknown etiology, characterized by remission and exacerbation of multiple bone lesions which radiologically and pathologically have the appearance of hematogenous osteomyelitis. The natural history appears to be slow and spontaneous resolution of the osseous lesions without specific treatment. And antimicrobial agents seem to have no beneficial effect. In proper clinical setting, CRMO should be considered, because recognition of this entity would help us to avoid costly and potentially harmful diagnostic and therapeutic interventions. We report a case of a 48-year-old adult who had chronic recurrent multifocal osteomyelitis.
Adult
;
Anti-Infective Agents
;
Humans
;
Middle Aged
;
Natural History
;
Osteomyelitis

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