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나수진 ( Soo Jin Na ),이혜연 ( Hye Yeon Lee ),김현선 ( Hyun Seon Kim ),성현진 ( Hyeon Jin Seong ),전부석 ( Bu Seok Jeon ),전희경 ( Hui Kyung Jeon ) 대한내과학회 2013 대한내과학회지 Vol.85 No.5
Neurofibromatosis type I is a genetic disease caused by mutations in the neurofibromin 1 (NF1) gene. Although it is characterized by a number of distinct clinical features, including cafe au lait macules, freckling in the axillary or inguinal regions, neurofibromas, and Lisch nodules (iris harmartomas), it can affect all physiological systems in the body [1]. Neurofibromatosis-related pulmonary hypertension has also been reported, and some patients showed a poor prognosis despite having received proper medical treatment [2-4]. We herein describe a case of pulmonary hypertension in a patient with neurofibromatosis type I who had no identified risk factors of pulmonary hypertension. To our knowledge, this is the first such report in Korea. (Korean J Med 2013;85:521-525)
김일 ( Il Kim ),김진일 ( Jin Il Kim ),권의종 ( Eui Jong Kwon ),장재혁 ( Jae Hyuk Jang ),정성민 ( Sung Min Jung ),성현진 ( Hyeon Jin Seong ),정대영 ( Dae Young Cheung ),박수헌 ( Soo Hern Park ) 대한소화기학회 2016 대한소화기학회지 Vol.67 No.5
Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by multiple gas-filled cysts of varying size in the wall of gastrointestinal tract. PCI may idiopathic or secondary to various disorders. The etiology and pathogenesis of PCI are unclear. Treatment is usually conservative, and includes oxygen and antibiotics therapy. Surgery is reserved for cases of suspected inconvertible intestinal obstruction or perforation. Eleven patients who were diagnosed with PI between 2005 and 2015 were reviewed. We report three cases of PCI and describe causes and complications. The most important point in the treatment of PCI is to determine whether the patient needs surgery. Conservative care should be considered first if the patient is stable. If any complication is observed, such as ischemia in the intestine, surgery is needed. It is important to choose the best treatment based on prognostic factors and CT findings. (Korean J Gastroenterol 2016;67:262-266)