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임재욱 ( Jae Uk Im ),김은혜 ( Eun Hye Kim ),전홍길 ( Hong Gil Jun ),오지선 ( Ji Seon Oh ),최승원 ( Seung Won Choi ),강병성 ( Byeong Seong Kang ) 대한내과학회 2013 대한내과학회지 Vol.84 No.6
The gastrointestinal tract is commonly involved in patients with systemic sclerosis. The manifestations include motility disorder, pseudo-obstruction, malabsorption, bacterial overgrowth, diverticuli, and, less commonly, pneumatosis cystoides intestinalis (PCI). PCI is characterized by the presence of air in the submucosal or subserosal layer of the bowel wall and is often accompanied by pneumoperitoneum. Although PCI is a benign condition that often responds to conservative management, it is a poor prognostic factor of systemic sclerosis. We report a case of PCI in a patient with systemic sclerosis. The chest and abdominal radiographic findings comprised pneumoperitoneum, marked dilation of the bowels, and intramural air, compatible with PCI. The patient`s symptoms improved spontaneously. It is important to recognize PCI as a gastrointestinal manifestation of systemic sclerosis, and physicians should differentiate it from serious complications-such as intestinal perforation-based on clinical manifestations. (Korean J Med 2013;84:868-872)
Alemtuzumab을 이용한 이차성 순적혈구 빈혈의 치료
박혜민 ( Hye Min Park ),임재욱 ( Jae Uk Im ),박주환 ( Ju Hwan Park ),김은혜 ( En Hye Kim ),전홍길 ( Hong Gil Jun ),박재후 ( Jae Hoo Park ),김혁 ( Hawk Kim ) 대한내과학회 2013 대한내과학회지 Vol.85 No.2
Pure red cell aplasia (PRCA) in adults is usually idiopathic, although some underlying conditions can cause PRCA. Immunosuppressive therapy (IST) is used to treat PRCA, but IST has side effects and may fail. The anti-CD52 monoclonal antibody alemtuzumab (ALM) was recently used to successfully treat therapy-resistant PRCA. We herein report successful treatment of secondary PRCA after erythropoietin therapy using ALM and cyclosporin A (CsA) in one patient. The total dose of ALM was 60 mg over 3 days (10, 20, and 30 mg, respectively) plus CsA for at least 6 months. The patient achieved a complete response 18 months after ALM-CsA treatment and his treatment could be changed to a different erythropoietin stimulating agent. (Korean J Med 2013;85:214-217)