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김기수(Ki Soo Kim),정철구(Chul Ku Jung),김윤미(Yoon-Mee Kim),고경옥(Kyong-Og Ko),이영혁(Young Hyuk Lee) 대한소아신경학회 2004 대한소아신경학회지 Vol.12 No.2
중추신경계의 비전형 기형/횡문 종양은 주로 영유아기에 많이 발생하는 매우 희귀한 종양으로 구토, 기면, 보챔 등의 비특이적 증상이나 두통, 뇌신경마비등의 증상이 나타날 수 있다. 진단은 대뇌전산화 다층촬영과 핵자기 공명 영상을 통하여 할 수 있고 조직검사를 통해 확진할 수 있다. 이 종양은 원시 신경 외피 종양-수모세포종과 매우 유사하여 흔히 잘못 지단되는 경우가 많으나 비전형 기영/횡문 종양이 좀더 어린 연령에서 발생하고 소뇌에 발생한느 경우가 더 적으며 치료에 대한 반응이 매우 나쁘다. 치료는 수술, 항암 화학요법, 방사선치료 등으로 구성되어 있고 치료에 대한 예후는 매우 나쁘다. 저자들은 중추형 안면 신경마비를 주소로 내원한 2세 된 여아에서 비전형 기형/횡문 종양 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Primary central nervous system atypical teratoid/rhabdoid tumors are rare and extremely aggressive malignancies of early childhood. These tumors are most common in infants less than 2 years of age. Diagnosis is based on distinctive light microscopic and immunohistochemical findings, coupled with a molecular genetic analysis. A histologic features of these tumors are epithelial and/or mesenchymal components in addition to rhabdoid, with or without neuroepithelial fields. The expression of the epithelial membrane antigen, vimentin, and the smooth muscle actin are characteristic of these tumors. Treatment includes surgery, chemotherapy, and radiotherapy. Prognosis is poor despite of an aggressive therapy. We report one case of an atypical teratoid/rhabdoid tumor in a young child. She is an 18 month-old-girl who presented with central type facial palsy. After extensive surgery she improved transiently but relapsed immediately. Her condition was not permitted to receive radiotherapy or chemotherapy. Thereafter, with phrenic nerve palsy, she suffered from recurrent episodes of pneumonia and respiratory difficulties. Finaly, she was expired three months after the diagnosis and treatment.
월슨병 환아에서 D-penicillamine 사용 중 조기 발병한 미세변화형 신증후군 1례
김기수,임재우,고경옥,김윤미,천은정,Kim Ki-Soo,Lim Jae-Woo,Ko Kyong-Og,Kim Yoon-Mee,Cheon Eun-Jung 대한소아신장학회 2004 Childhood kidney diseases Vol.8 No.2
월슨병은 구리 대사 이상으로 간, 뇌, 각막, 신 및 건혈구에 구리가 침착되어 생기는 상염색체 열성 유전성 질환이다. D-penicillamine이 주된 치료로서 10% 정도의 환자에서 가역적인 부작용이 발생 할 수 있다 저자는 3세된 여아에서 D-penicillamine치료 3주만에 미세변화형 신증후군이 발생하였고 투약 중단만으로 완전 관해된 증례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Wilson's disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson's disease. However, nephrotoxicity can occur after D-penlcillamlne treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syn,frome in a 3-year-old'girl with Wilson's disease 3 weeks after initiation of D-penicillamine.
이태환,김헌주,허철,한용표,홍순기,변진수,김명순,조미연,김윤미 대한신경외과학회 1996 Journal of Korean neurosurgical society Vol.25 No.11
in recent times, central nervous system(CNS) tuberculosis has been rare and the prevalence of the focal form, the tuberculoma, varies from 1 per 200 to 1 per 1000. CNS tuberculosis occurs as a result of hematogenous spread from a primary focus. mostly pulmonary tuberculosis. It can be diffuse exudative leptomeningitis or a localized tuberculoma ; the former is more common. We report a case of miliary cerebral tuberculosis in a 24-year-old female who had been having headaches for 4 months. Magnetic resonance imaging showed numerous small round Gadolinium-enhanced supratentorial and infratentorial lesions scattered throughout the brain. Histologic examination confirmed well defined tuberculous granulomas with central caseous necrosis in open biopsy, containing several acid-fast bacilli, the patient was treated with isoniazid, rifampin, ethambutol, pyrazinamide in combination with prednisolone.