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항생제 치료로 유발된 혈액응고 제V인자에 대한 후천적 억제인자
임두호 ( Doo Ho Lim ),김태오 ( Tae Oh Kim ),정유문 ( Yumun Jeong ),김원장 ( Won Jang Kim ),박승정 ( Seung Jung Park ),이제환 ( Je Hwan Lee ),장성수 ( Sung Soo Jang ) 대한내과학회 2014 대한내과학회지 Vol.87 No.1
Acquired factor V inhibitor is a rare condition with a variety of clinical manifestations that range from no bleeding symptoms to life-threatening hemorrhage or thromboembolic events. Treatment is determined by the clinical course and focuses on controlling the hemorrhagic event and decreasing the antibody titer if bleeding symptoms are present. We report herein a case involving a 70-year-old man who developed acquired factor V inhibitor after antibiotic administration (11-day course of ceftriaxone and successive 5-day course of piperacillin-tazobactam) for pneumonia. His condition was characterized by elevated prothrombin and activated partial thromboplastin times without bleeding events. Coagulation factor assays revealed undetectable factor V activity and a factor V inhibitor level of 3.29 Bethesda units. After cessation of the antibiotics, both the prothrombin and activated partial thromboplastin times gradually normalized. (Korean J Med 2014;87:105-109)
낭종성 폐병변으로 내원하여 진단된 Birt-Hogg-Dube 증후군 2예
서명숙 ( Myeong Sook Seo ),임두호 ( Doo Ho Lim ),송준선 ( Joon Seon Song ),박찬식 ( Chan Sik Park ),채은진 ( Eun Jin Chae ),송진우 ( Jin Woo Song ) 대한내과학회 2014 대한내과학회지 Vol.87 No.4
Birt-Hogg-Dube (BHD) syndrome is a rare autosomal-dominant disease caused by germline folliculin (FLCN) mutations, characterized by fibrofolliculoma or trichodiscoma, renal tumors, and multiple lung cysts with or without spontaneous pneumothorax. Here, we report two cases of BHD syndrome that presented with bilateral pulmonary cysts. One patient was a 39-year-old woman who had a history of pneumothorax, multiple papules on her cheeks, and a family history of the same skin lesions and renal cell carcinoma in her father. BHD syndrome was confirmed by molecular tests that revealed a missense mutation in FLCN gene (exon 4, c.31T > C). The other patient was a 56-year-old man who showed FCLN gene polymorphism and typical radiopathological features of multiple cysts in the lung, but apparently no other manifestation. (Korean J Med 2014;87:477-483)
6년간의 자연 경과를 관찰할 수 있었던 IgG4 연관 경화성 담관염
김태오 ( Tae Oh Kim ),김명환 ( Myung Hwan Kim ),최준혁 ( Joon Hyuk Choi ),임두호 ( Doo Ho Lim ),박상우 ( Sang Woo Park ),최준호 ( Jun Ho Choi ),김진희 ( Jin Hee Kim ) 대한내과학회 2014 대한내과학회지 Vol.87 No.2
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a rare disease characterized by increased serum levels of IgG4 and dense infiltration of IgG4-positive plasma cells with fibrosis in the bile duct wall. IgG4-SC is frequently associated with autoimmune pancreatitis (AIP) and typically shows an excellent response to steroid therapy. Despite recent progress in understanding the clinical presentation of IgG4-SC, its diagnosis still remains a challenge, particularly if it manifests as an isolated hilar stricture with normal serum IgG4 concentrations. In this article, we report the case of a 75-year-old man with IgG4-SC in whom it was difficult to distinguish hilar cholangiocarcinoma due to the normal serum IgG4 concentration and no accompanying AIP. He had an indolent clinical course and showed slow progression of a bile duct lesion over 6 years. (Korean J Med 2014;87:182-186)
박수헌,김재광,정규원,최황,조성원,한준열,선희식,박두호,고승현,문건웅,임현선 대한소화기내시경학회 1997 Clinical Endoscopy Vol.17 No.6
Eosinophilic gastroenteritis is a rare disorder of the stomach, small intestine and colon, characterized by variable eosinophilic infiltration of the bowel wall and peripheral blood eosinophilia, abnormal gastrointestinal symptoms and signs. The pathogenesis of this dis- ease still remains unknown, but several studies support allergic or immunologic etiology. Considerable variability in clinical presentation depends on the site of the gastrointestinal tract and the depth of bowel wall involvement. Herein we experienced a case of eosinophilic gastroenteritis confined to small intestine presenting progressive weight loss, abdominal pain and diarrhea. Enteroscopic findings showed diffuse multiple nodularity of mucosal folds in small bowel, especially duodenum and proximal jejunum. Jejunal and duodenal biopsy specimen demonstrated marked eosinophilic infiltration. These symptoms and signs were improved within 3 weeks after short course of prednisone therapy.