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신경학적 증상 없이 통풍성 관절염만으로 발현된 Lesch-Nyhan Syndrome 1례
여유미 ( Yoo Mi Yeo ),최은영 ( Eun Young Choi ),윤혜진 ( Hyae Jin Yoon ),정소담 ( So Dam Jung ),김담 ( Dam Kim ),이승훈 ( Seung Hun Lee ),주경빈 ( Kyung Bin Joo ),전재범 ( Jae Bum Jun ) 대한류마티스학회 2014 대한류마티스학회지 Vol.21 No.4
Deficiency of hypoxanthine-guanine phosphoribosyltrans-ferase is a purine nucleotide disorder and is the most common genetic cause of uric acid overproduction. This disease has a wide range of spectrum with regard to neurological features depending on the extent of the enzymatic deficiency. Complete deficiency of hypoxanthine-guanine phosphoribosyltransferase, called Lesch-Nyhan syndrome, is presented with hyperuricemia and characteristic neurological manifestation and self-mutilation. Partial hypoxanthine-guanine phosphoribosyltransferase--deficient patients are presented with a various intensities of the aforementioned symptoms, from almost normal neurologic manifestation to a severe form along with hyperuricemia. We report a twenty-year-old man with complete hypoxanthine- guanine phosphoribosyltrans-ferase mutation and Lesch-Nyhan sydrome, who manifested gouty arthritis without neurologic symptom.
김지연 ( Ji Yeoun Kim ),유교상 ( Kyo Sang Yoo ),오영하 ( Young Ha Oh ),최종민 ( Jong Min Choi ),이상기 ( Sang Ki Lee ),여유미 ( Yoo Mi Yeo ),황순우 ( Soon Woo Hwang ),윤혜진 ( Hye Jin Yoon ) 대한췌장담도학회 2014 대한췌담도학회지 Vol.19 No.1
Autoimmune pancreatitis or IgG4-related sclerosing cholangitis often involves the liver. Most common lesion involving the liver is shown as mass or masses often referred as inflammatory pseudotumor. Inflammatory pseudotumor usually needs to be discriminated with malignancy. Here we report a case of IgG4-related sclerosing cholangitis with liver involvement presented as a mass. It was proven by biopsy and did not show any evidence of autoimmune pancreatitis. The mass infiltrated around the portal tract and portal vein thrombosis was also present. Korean J Pancreatobiliary 2014;19(1):26-30
췌장외 점액누출을 동반한 췌관내 유두상 점액종양에 의한 복막 가성점액종 1예
김해수 ( Hae Su Kim ),원영웅 ( Young Woong Won ),최정혜 ( Jung Hye Choi ),유교상 ( Kyo Sang Yoo ),여유미 ( Yoo Mi Yeo ),이지영 ( Ji Young Yhi ),표주연 ( Ju Yeon Pyo ),박훤겸 ( Hwon Kyum Park ) 대한췌담도학회 2013 대한췌담도학회지 Vol.18 No.4
복막 가성점액종은 원발병소에 관계없이 복막표면과 대망 등에 점액성 물질이 침착되면서 점액성 복수가 복강 내에 축적되는 상태를 말하는 임상적 질환이다. 원인질환으로 매우 드물지만 췌관내 유두상 점액종양이 보고되고 있으며, 시술과 관련된 점액의 누출, IPMN 파열 및 샛길 형성에 의한 복강내 점액누출 등이 유발기전으로 제시되고있다. 저자들은 췌장의 악성 IPMN 점액누출에 의해 발생한 복막 가성점액종 1예를 경험하였기에 문헌고찰과 함께보고하는 바이다. Pseudomyxoma peritonei (PMP) is a rare clinical entity characterized by gelatinous tumor implants within the peritoneal cavity and a large amount of mucinous ascites. Although most PMP cases originate in the appendix, few are associated with pancreatic intraductal papillary mucinous neoplasm (IPMN). IPMNs secrete thick mucin; therefore, they usually present with pancreatic duct dilatation. The presence of IPMN with extra-pancreatic mucin could be a marker of the eventual seeding of tumor outside the primary site. PMP associated with mucin leakage from pancreatic IPMN has been rarely reported. Here, we report the case of a patient with PMP that originated from pancreatic IPMN.