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정욱진 ( Wook Jin Chung ),최승원 ( Seung Won Choi ),이승근 ( Seung Keun Lee ),이상길 ( Sang Gil Lee ),권진욱 ( Jin Wuk Kwon ),최승혁 ( Seung Hyuk Choi ),송정식 ( Jung Sik Song ),이찬희 ( Chan Hee Lee ),송창호 ( Chang Ho Song ) 대한류마티스학회 1998 대한류마티스학회지 Vol.5 No.1
Sacroiliitis is a prototyical feature of the seronegative spondyloarthropathies. It has been reported rarely in patients with systemic lupus erythematosus. We report a case of bilateral sacroiliitis in a 26 year-old female who presented 5 out of 11 ARA criteria for systemic lupus erythematosus. She complained of right low back pain since 1 week after delivery. Plain roentgenogram, computed tomogram and magnetic resonance imaging revealed no evidence of subluxation or pyogenic infection that may be possibly related to delivery, but bilateral sacroiliitis especially more severe in the right side that showed concordantly increased uptake on bone scan. Also she had neither HLA DR-3 nor B-27 antigens and had no other clinical findings of limitation of motion of spine and chest expansion, which suggest that there is a less likelihood of coexistent ankylosing spondylitis. In this case, we suggested that sacroiliitis may be a infrequent manifestation of systemic lupus erythematosus.
정욱진 ( Wook Jin Chung ) 대한내과학회 2010 대한내과학회지 Vol.78 No.1
폐고혈압의 치료에서 비-폐동맥 고혈압에서는 그 원인 질환에 맞는 치료를 한다. 폐고혈압 환자에서 제일 많은 폐정맥 고혈압(PVH)을 일으키는 좌측 심장질환의 경우 일부에서 경중도에 비해 많이 높은 폐동맥압을 갖는 `과잉(disproportionate)` PH인 경우는 PAH와 같은 약물 치료를 시도하는 연구도 있다. 폐동맥 고혈압(PAH) 환자의 고식적 치료는 산소, 항응고제, 이뇨제, 디곡신 치료를 하고, 급성 약물 반응 검사를 통해 칼슘차단제 사용 유무를 결정한다. PAH 치료에 있어 `targeted therapy`로 우리나라에서 사용할 수 있는 약제들은 WHO 기능분류 II, III에서 endothelin 수용체 길항제인 경구용 bosentan과 ambrisentan, phosphodiesterase-5 저해제인 경구용 sildenafil이 있고, WHO 기능분류 III, IV에서는 흡입용 iloprost 등이 있다. PAH는 희귀질환이지만 치명적인 질환으로, 특별한 이유 없이 운동 시에 숨이 찰 때 감별진단이 필요하다. 의심되면 심초음파 검사 등의 스크리닝 검사를 시행하여 조기에 발견하고 진단하는 것이 가장 중요한 치료의 첫걸음이다. Most commonly encountered pulmonary hypertension is pulmonary venous hypertension (PVH) accompanying elevated left ventricular filling pressure. So, the treatment for the underlying left heart disease should be mainstay of PVH treatment. On the contrary, pulmonary arterial hypertension (PAH) is an `orphan` disease, which means a very rare one. To the date, PAH have been one of diseases showing high mortality rate. But, recently, `targeted therapies` were introduced and several novel treatment guidelines for PAH were recommended. This review discusses new insights into the recent trend focusing on novel therapies and the necessity of specialized referral centers for PAH in Korea. (Korean J Med 78:28-35, 2010)
김진호 ( Jin Ho Kim ),이승준 ( Seung Jun Lee ),이한철 ( Han Cheol Lee ),이진영 ( Jin Young Lee ),이영재 ( Young Jae Lee ),정욱진 ( Wook Jin Chung ),장혁재 ( Hyuk Jae Chang ) 대한내과학회 2012 대한내과학회지 Vol.83 No.4
Pulmonary arterial hypertension is a rare life-threatening disorder. Inherited cases of this disorder are known as heritable pulmonary arterial hypertension. Familial cases of heritable pulmonary arterial hypertension are caused by germline mutations of the bone morphogenetic protein receptor type 2 gene (BMPR), a type II receptor of the TGF-beta superfamily. This has not been reported in Korea. We report the first case of familial hereditable pulmonary arterial hypertension in Korea. The family has a mutation in exon12 of the BMPR2 gene (c.2695C > T, p.R899X). (Korean J Med 2012;83:494-497)