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강주형 ( Joo Hyung Kang ),조문균 ( Moon Kyun Cho ),최보라 ( Bo Ra Choi ),황규왕 ( Kyu Yang Whang ),노영석 ( Young Suck ),이창우 ( Chang Woo Lee ) 대한피부과학회 2008 대한피부과학회지 Vol.46 No.8
Background: Heat shock proteins (HSP), especially the HSP 70 family, may play certain roles in the immunophysiology of some skin diseases such as psoriasis, pemphigus, and lichen planus. HSPs generally induce down-regulation of the process of apoptosis that is considered to be one of the acantholysis-producing pathways in pemphigus. Objective: We planned to examine possible roles of HSPs 70/105 in the blistering process in pemphigus vulgaris (PV) and pemphigus foliaceus (PF), in connection with the detection results of apoptosis in local tissue specimens. Methods: Immunohistochemical stainings and Western blot analysis were performed for the detection and semiquantitation of HSPs 70/105 in skin specimens from lesional, nonlesional, and normal control sites. Hoechst 33342 staining was simultaneously carried out to examine features of apoptosis in lesional skin specimens. Results: The findings on expression of HSP were as follows. In PV, the expression of HSP 70 was minimum or negative; however, in PF, the expression was obvious and recognizable in lesional and perilesional normal skin. In contrast, HSP 105 was not detected in all cases of PV and PF. The features of apoptosis were evident at the lesional skin of all cases of pemphigus with acantholytic changes. Conclusion: PV and PF had different relative intensities of HSPs in lesional tissue stainings, especially in cases with HSP 70. This suggests that there may be subtle differences in the mechanisms causing acantholysis between PV and PF. (Korean J Dermatol 2008;46(8):1003~1010)
이용주,강주형,이수진,박호진,신충호,정해일,Lee Yong Joo,Kang Ju Hyung,Lee Soo Jin,Park Ho Jin,Shin Choong Ho,Cheong Hae Il 대한소아신장학회 2005 Childhood kidney diseases Vol.9 No.2
표피모반증후군은 중추신경계, 안구, 골격계, 심장 또는 요로생식기의 기형을 동반하는 선천성 표피 모반을 특징으로 하는 질환으로 보행장애를 주소로 내원한 8세 여아에서 저인산혈증성 구루병을 동반한 표피모반증후군 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Epidermal nevus syndrome is a rare disease consisting of epidermal nevus and multisystem pathologic conditions associated with anomalies in the central nervous system, bone, eye, heart, vasculature and genito-urinary system. Hypophosphatemic rickets has been rarely observed in association with this syndrome. We report a case of hypophosphatemic rickets as sociated with epidermal nevus syndrome with review of the literature. (J Korean Soc Pediatr Nephrol 2005;9:263-268)
최연주(Yeun Joo Choi),강주형(Joo Hyung Kang),김성환(Sung Hwan Kim) 대한소아신경학회 2002 대한소아신경학회지 Vol.10 No.2
목 적: 소아 허혈성 뇌졸중의 위험인자를 체계적으로 분석하고, 성인에서 치료방침을 세우기 위해 사용되는 뇌졸중 위험인자에 대한 TOAST 분류법이 소아에서도 적용될 수 있는지를 밝히고자 하였다. 방 법:1995년 3월부터 2002년 2월까지 아주대학교병원에 뇌졸중으로 입원한 16세 이하 소아 환자 87명의 의무기록을 토대로 후향적 연구를 하였다. 허혈성 뇌졸중에 관계되는 위험인자, 연령에 따른 위험인자와 위험인자에 따른 뇌졸중의 유형을 분석하였으며, 성인에서 사용되는 TOAST 분류체계를 소아에 적용하여 뇌졸중의 위험인자를 다시 분류하였다. 결 과: 총 87명의 환자 중 허혈성 뇌혈관 질환은 64명(73.6%), 출혈성 뇌혈관 질환은 23명(26.4%)이었다. 소아 뇌혈관 질환은 1세 이하 영아 13명(14.9%), 5-8세 26명(26.9%), 9-12세 22명(25.3%)으로 세 연령군에 호발하였다. 57명(89.1%)에서 허혈성 뇌혈관 질환을 초래하는 위험인자를 규명할 수 있었고, 이중 뇌혈관 병변이 64명 중 30명(46.9%)으로 가장 많았고, 고혈압성 뇌증 8명(12.5%), 감염질환,유전대사질환이 각 5명(7.8%), 혈액질환 4명(6.3%) 순이었다. 30명의 뇌혈관 병변 환자 중 모야모야병이 26명(86.7%)으로 가장 많았으며 혈관염 2명, Raynaud 증후군, 경동맥 박리가 각 1명 있었다. 유전대사질환 5명은 MELAS 3명, Leigh disease와 호모시스틴뇨증이 각 1명이었다. 뇌혈관 병변에 의한 뇌졸중 환자 30명 중 27명(90.0%), 유전대사 질환 5명 중 4명, 고혈압성 뇌중 8명은 5세 이후에 뇌졸중이 발생하였다. 이에 반해 감염질환에 의한 뇌졸중은 모두 4세 이하에서 발생하였다. 전체 환자에서 허혈성 뇌졸중의 유형은 고정성 뇌졸중이 46례 중 24례(52.2%)로 가장 많았으나, 모야모야병 환자는 TIA가 25례 중 20례(80.0%)로 고정성 뇌졸중 5례 보다 많았다. TOAST 분류체계는 허혈성 뇌졸중의 원인이 규명된 57명의 환자중 2명에서 소혈관 질환으로 분류할 수 있었고, 나머지 환자는 위험인자들이 다양해서 분류할 수 없었다. 결 론: 소아 허혈성 뇌졸중 환자의 90%에서 뇌졸중을 초래하는 위험인자를 찾을 수 있었고 원인은 다양하였으나 모야모야병을 포함한 뇌혈관 병변이 가장 많았다. 특히 5세 이상 소아는 뇌혈관 병변과 더불어 유전대사질환과 고혈압성 뇌증이 중요한 원인이었으며 혈액응고질환도 일부 환자에서 발견되기 때문에 위험인자에 대한 적극적 검사가 필요하다. 또한 소아 뇌졸중의 원인은 성인과 달리 대단히 다양하기 때문에 치료방침을 결정하기 위해 성인에서 사용되는 TOAST 분류법은 소아 허혈성 뇌졸중 환자에서 적용할 수 없다. Purpose: This paper evaluate and classified the risk factors that could possibly cause ischemic stroke in children, and investigate whether TOAST classification could be applied to the ischemic stroke in children. Methods: From March 1995 to February 2002, we retrospectively reviewed the medical record of 87 patients under the age 16 who had been registered to the Ajou Stroke Registry. We evaluated the risk factors of ischemic stroke according to the Ajou Stroke Registry Protocol, and classified the risk factors into 6 main subgroups. In addition, the risk factors were investigated according to the age distribution. TOAST classification had been applied to children with ischemic stroke. Results: There was 64 ischemic stroke(73.6%) and 23 hemorrhagic stroke(26.4%) among the 87 patients. In children with ischemic stroke, there was three major peak age; 13 patients younger than 1 year old(15.3%), 26 patients in age 5 to 8 years old(40.6 %), 22 patients in 9 to 12 years old(34.3%). Vasculopathy(48.6%) was the most common risk factor of the ischemic stroke in this study. Other important risk factors in order of frequency were hypertensive encephalopathy(12.5%), infectious disease(7.8%), metabolic disease(7.8%), and hematologic disease(6.2%). Moyamoya disease was the most common cause of ischemic stroke due to vasculopathy. If we analyze the risk factors according to the age at the presentation of ischemic stroke, 90% of the ischemic stroke due to vasculopathy had been developed after 5 years of age. However, ischemic stroke due to infectious disease had been developed less than 4 years of age. Major stroke was the most common subtype of the ischemic stroke in children, but TIA had been found in 80% of the patients with ischemic stroke due to moyamoya disease. TOAST classification determined the subtype of the ischemic stroke only in 2 children. Risk factors of ischemic stroke in the remained 62 patients were extremely variable, and could not be applicable to the TOAST classification. Conclusion: Risk factor of ischemic stroke were found in the 90% of patients. Although risk factors of ischemic stroke were extremely variable in children, cerebral vasculopathy including moyamoya disease was the most common. However, inherited metabolic disorder, coagulopathy, and hypertensive encephalopathy were also relatively common risk factors of ischemic stroke especially in children older than 5 years old. Therefore, we must extensively evaluate all the possible risk factors of ischemic stroke in children. TOAST classification could not be applicable in children because the risk factors of ischemic stroke were extremely variable.
급성 감염후 사구체 신염이 동반된 성인 Henoch-Schonlein 자반증 1례
문경민 ( Kyoung Min Moon ),강주형 ( Joo Hyung Kang ),김동규 ( Dong Gyu Kim ),이웅철 ( Woong Chul Lee ),문유리 ( Yu Ri Moon ),주종은 ( Jong Eun Joo ),김용일 ( Yong Il Kim ),양승오 ( Seoung Oh Yang ),이영숙 ( Young Sook Lee ) 대한신장학회 2008 Kidney Research and Clinical Practice Vol.27 No.4
Henoch-Schonlein purpura (HSP) is a small-vessel vasculitis that are predominantly observed in children, affecting their skin, joints, gut and kidneys. The renal prognosis of HS nephritis is known to be worse in adults than in children. It is defined by tissue deposition of IgA. In most HSP patients, serum complement levels are usually normal. However, some authors reported abnormalities of serum complement levels in children-onset HSP. The authors report here a rare adult-onset case of HSP combined with post-infectious glomerulonephritis, especially hypocomplementemia and subepithelial `hump`-like electron dense deposits.
강주형,박정환,김정수,노영석 대한피부과학회 2004 大韓皮膚科學會誌 Vol.42 No.2
Vcrrucous cyst is a rare form of epidermal cyst showing verrucous changes of their stratified squamous epithelial lining, characterized by papillomatosis, acanthosis, and hypergranulosis with large, irregular keratohyaline granules. In addition, vacuolated keratinocytes, parakeratosis, and squamous cddies are often present. Herein, we describe a 45-year-old woman with a verrucous cyst, which occurred on left axilla three months ago, showing the above-mentioned characteristic histologic features. Both the immunohistochemical stain and polymcrase chain reaction for the common antigen for human papillomavirus and DNA chip study for human papillomavirus were negative. (Korean J Dermatol 2004;42(2):232~235)