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Kim, Bo Eun,Koh, Kyung-Nam,Suh, Jin Kyung,Im, Ho Joon,Song, Joon Sup,Lee, Ji Won,Kang, Hyoung Jin,Park, Kyung Duck,Shin, Hee Young,Choi, Hyoung Soo,Lee, Soo Hyun,Yoo, Keon Hee,Sung, Ki Woong,Koo, Hong by Lippincott Williams Wilkins. 2014 Journal of pediatric hematology/oncology Vol.36 No.2
A nationwide survey was conducted to clarify the clinical features and outcomes of Korean children with Langerhans cell histiocytosis (LCH). Korea Histiocytosis Working Party analyzed the data of 603 patients who were diagnosed with LCH between 1986 and 2010 from 28 institutions in Korea. Median age at diagnosis was 65 months (range, 0 to 276 mo). Bone was the most frequently affected organ (79.6%) followed by skin (19.2%). Initially, 419 patients (69.5%) had single-system involvement (SS), 85 (14.1%) with multisystem (MS) disease without risk organ involvement (MS-RO), and 99 (16.4%) multisystem disease with risk organ involvement (MS-RO). The 5-year overall survival (OS) rates in the SS, MS-RO, and MS-RO groups were 99.8%, 98.4%, and 77.0%, respectively (P<0.001), and the 5-year reactivation rates were 17.9%, 33.5%, and 34.3%, respectively (P<0.001). The OS rate was lower in patients with RO involvement (P=0.025) and lack of response to initial treatment (P=0.001). MS involvement (P=0.036) was an independent risk factor for reactivation. Permanent consequences were documented in 99 patients (16.4%). Reactivation of disease, MS involvement, and age at diagnosis ≤2 years were associated with higher incidence of permanent consequences. This study emphasized that further efforts are required to improve survival of MS-RO patients and reduce reactivation in younger patients with MS involvement.
구자설(Ja Soul Koo),송치욱(Chi Wook Song),조영직(Young Gik Jo),고동욱(Dong Wook Koh),손수민(Soo Min Shon),김용식(Yong Sik Kim),정록선(Rok Son Choung),진윤태(Yoon Tae Jeen),전훈재(Hoon Jai Chun),김창덕(Chang Duck Kim),류호상(Ho Sang R 대한소화기기능성질환·운동학회 2001 Journal of Neurogastroenterology and Motility (JNM Vol.7 No.2
N/A Backgroud/Aims: The nonspecific esophageal motility disorder (NEMD) has been reported to be related to gastroesophageal reflux disease (GERD) in some cases. However, the pathophysiologic mechanism of NEMD has not been estabilished. The aim of this study was to assess the prevalence of esophagitis and gastric acid reflux following abnormal contractions in patients with NEMD. Methods: 122 NEMD patients were enrolled (76 male and 46 female) and their endoscopic findings and 24 hour esophageal Ph data were compared with 24 healthy subj ects. The abnormal contractions were classified as 1) non-transmitted con-traction, 2) low amplitude contraction, 3) non-transmitted and low amplitude contraction, and 4) others. Results: Among the 122 patients with NEMD, 62 patients (50.8%) had GERD, 53 patients (43.4%) had endoscopic reflux esophagitis, and 41patients (33.6%) had both. Acid exposed time studied by 24 hour Ph monitoring was more increased in NEMD patients than in healthy controls (7.48 ±10.68 vs 1.42 ±1.17), but there were no differences among abnormal contraction patterns. Moderate to severe reflux esophagitis were frequently seen in patients with combined abnormal contractions as the results of endoscopic findings. Pre-existing factors for the gastric acid reflux in NEMD patients were male and esophageal hiatal hernia. Conclusions: Large numbers of NEMD patients were found to have concurrent GERD in our study. However, the esophageal peristaltic dysfunction was more closely related to the severity of esophagitis rather than to the amount of refluexed gastric acid itself. (Korean Journal of Gastrointestinal Motility 2001;7:168-174)
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권순국,고덕구,Kwun Soon-kuk,Koh Duck-koo 한국관개배수위원회 1995 한국관개배수논문집 Vol.2 No.2
In spite of their high availability in the field of water resources, finite element models generally require large amount of input data in which the preparation of them consists of complicated procedures and time consuming works. In addition, in case of a
이혜순,김명환,서동진,이성구,최호순,민영일,고문수,송일한,한덕종,최승목 대한내과학회 1996 대한내과학회지 Vol.51 No.2
An epidermoid cyst which is a beign tumor with a stratified squamous epithelium that lacks hair or skin appendage have been described as the most frequent tumorous malformation of central nervous system. Also epidermoid cyst may develop at any other organ, i.e. spleen, pancreas, testis, scalp, skull, vaginal wall, or epiglottis. Almost of epidermoid cyst were diagnosed postoperatively because they have no specific radiologic finding and have difficulty in diagnosis by percutaneous needle aspiration. We report a case of epidermoid cyst which was diagnosed preoperatively as pancreatic head mass but was diagnosed postoperatively as epidermoid cyst at retroperitoneum separated from pancreas.
Mycobacterium abscessus Lung Disease in a Patient with Kartagener Syndrome
Kim, Jung Hoon,Song, Won Jun,Jun, Ji Eun,Ryu, Duck Hyun,Lee, Ji Eun,Jeong, Ho Jung,Jeong, Suk Hyeon,Kang, Hyung Koo,Kim, Jung Soo,Lee, Hyun,Chon, Hae Ri,Jeon, Kyeongman,Kim, Dohun,Kim, Jhingook,Koh, W The Korean Academy of Tuberculosis and Respiratory 2014 Tuberculosis and Respiratory Diseases Vol.77 No.3
Primary ciliary dyskinesia (PCD) is characterized by the congenital impairment of mucociliary clearance. When accompanied by situs inversus, chronic sinusitis and bronchiectasis, PCD is known as Kartagener syndrome. The main consequence of impaired ciliary function is a reduced mucus clearance from the lungs, and susceptibility to chronic respiratory infections due to opportunistic pathogens, including nontuberculous mycobacteria (NTM). There has been no report of NTM lung disease combined with Kartagener syndrome in Korea. Here, we report an adult patient with Kartagener syndrome complicated with Mycobacterium abscessus lung disease. A 37-year-old female presented to our hospital with chronic cough and sputum. She was ultimately diagnosed with M. abscessus lung disease and Kartagener syndrome. M. abscessus was repeatedly isolated from sputum specimens collected from the patient, despite prolonged antibiotic treatment. The patient's condition improved and negative sputum culture conversion was achieved after sequential bilateral pulmonary resection.