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임연정 ( Yeun Jung Lim ),김정순 ( Jeong Soon Kim ),김인환 ( In Hwan Kim ),박상순 ( Sang Soon Park ),조성욱 ( Seong Wook Cho ),김동진 ( Dong Jin Kim ),조상균 ( Sang Kyoon Cho ) 대한내과학회 2007 대한내과학회지 Vol.73 No.1
단독 심실 비치밀화증은 태아기에 심근의 치밀화 과정이 정지됨으로서 과도한 육주(trabecula)가 형성되고 그 육주 사이에 깊은 함몰이 생기는 선천성 심근병증이다. 진단을 위해 심초음파는 가장 중요한 검사이며, 심초음파로 진단이 확실하지 않을 때에는 비침습적인 방법으로 다중단면 컴퓨터 단층촬영(MD CT), 자기공명단층촬영(MRI)을 통해 도움을 받을 수 있다. 임상양상은 매우 다양하여 무증상인 경우부터 심장이식이 필요하거나 사망할 수도 있는 전격성 심부전까지 나타날 수 있으며, 부정맥, 혈전 색전증이 발생할 수 있다. 저자들은 성인남자에서 심계항진 및 호흡곤란을 주소로 내원하여 단독 심실 비치밀화증으로 진단받고 심부전, 심방 세동 및 심첨부의 혈전에 대한 치료를 하였으나 결국 난치심부전 및 다발성 장기부전으로 사망한 1예를 경험하였기에 보고하는 바이다. Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy that is thought to be caused by arrest of normal endomyocardial embryogenesis. It is usually diagnosed via echocardiography. The three major clinical manifestations of noncompaction are heart failure, arrhythmia and embolic events. We report here on a case of isolated noncompaction of the ventricular myocardium, and the patient was admitted due to palpitation and dyspnea. Sadly, he died of intractable heart failure despite that we administered intensive medical treatment.(Korean J Med 73:96-102, 2007)
복통과 전신 피부 병변을 주소로 내원한 Degos병 1예
구본호 ( Bon Ho Ku ),임연정 ( Yeun Jung Lim ),최효경 ( Hyo Kyung Choi ),김영호 ( Young Ho Kim ),박종욱 ( Jongwook Park ),김치훈 ( Chi Hoon Kim ),장태훈 ( Tae Hoon Jang ),서용우 ( Yong Woo Seo ),조영철 ( Young Cheol Jo ),장기택 ( K 대한장연구학회 2010 Intestinal Research Vol.8 No.1
Malignant atrophic papulosis (MAP), also known as Degos’ disease, is a rare and often fatal occlusive thrombotic vasculopathy, with fewer than seven cases reported in Korea. MAP is characterized by porcelain-white, atrophic, papular skin lesions and multi-organ system involvement, especially the gastrointestinal (GI) tract and nervous system. Involvement of the GI tract is usually associated with a poor prognosis. To date, no treatment has been shown to be effective in the treatment of MAP. We describe a 52-year-old man who presented with a 5-month history of abdominal angina and a 2-year history of multiple skin lesions on the trunk and extremities. The skin lesions were papules, 4-6 mm in diameter, with a porcelain-white center and a slightly raised erythematous telangiectatic rim. A biopsy of a skin lesion showed a wedge-shaped degeneration of collagen in the dermis and atrophic epidermis. An explorative laparoscopy revealed multiple, yellow-white plaques scattered throughout the small bowel. A biopsy of the small bowel showed sclerotic vascular alterations containing intravascular fibrin thrombi. He was started on aspirin (100 mg daily) and has survived for 24 months since the onset of gastrointestinal symptoms. (Intest Res 2010;8:70-74)
황태준 ( Tae Jun Hwang ),김정순 ( Jeong Soon Kim ),권유석 ( Yoo Seok Kwon ),유현정 ( Hyun Jung Yu ),김현수 ( Hyun Soo Kim ),허선 ( Sun Huh ),최창규 ( Chang Kyu Choi ),임연정 ( Yeun Jung Lim ),김도훈 ( Do Hoon Kim ),최정희 ( Jeong 대한천식알레르기학회 2005 천식 및 알레르기 Vol.25 No.4
Eosinophilic mcningitis is defined as the presence of 10 or more eosinophils per microliter in the cerebrospinal fluid (CSF) or eosinophilis of at least 10% of the total CSF leukocyte count. Eosinophilic meningitis is a rare disease and the most common cause is the invasion of the central nervous system by helminthic parasites, particularly Angio-strongylus cantonensis. Toxocara canis, a common intestinal parasite of dog, has been also reported to cause eosinophilic meningitis. We report a case of eosinophilic meningitis which was proven to be a human toxocariasis by the serologic test. (Korean J Asthma Allergy Clin Immunol 2005;25:324-327)