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          Monilethrix 1 예

          김진택,김신규 대한피부과학회 1979 大韓皮膚科學會誌 Vol.17 No.4

          Monilethrix is a rare hereditary disorder of hair first described by Walter G. Smith in 1879, and usually transmitted as autosomal dominant trait. It is characterized hy bead like enlargement af the affected hair, alapecia resulting from ha.ir breakage and keratosis pilaris. This 3 year old female child patient has been suffered from easy destrurtion of beaded scalp hair and follicular hyperkeratosis of the occipital region since infancy. past hiatory was ncecontributory and the pe,digree of family showed autosomal dominant trait. Physical examinatior was not rernarkable except skin lesian and laboratory findings were within normal limit. Microscopic finding of the affected hair showed alternating constrictians and node. The scalp bivpsy findings showed mild hyperkeratosis, keratotic plug, mild cellular infiltration of papillary derrnia and perifollicular area.

        • SCOPUSKCI등재

          신경종성 (神經腫性) 상피증 1 예

          김태진,김진택,양순균,김신규 대한피부과학회 1980 大韓皮膚科學會誌 Vol.18 No.6

          Elephantiasis neuromatosa is one of 3 types of cutaneous tumors in Von Recklinghausen's disease. This 28 year-old male patient visited for adult-fist sized tumor associated with hairy pigmented patch on right antecubital fossa, and various sized tumors and cafe-au-lait spots on whole body of about 27 years duration. Past history was noncontributory. Family history revealed that his father and son had similar manifestations. Laboratory findings were noncontributory. Histopathology of cafe-au-lait spot and hairy pigmented patch revealed increase of melain in basal cell layer of the epidermis and increase in the concentratiton and activity of the melanocytes in basal cell layer of the epidermis. Histopathology of the mass revealed slightly thin, wavy fibrs lying in loosely textured strands extending in varying directions and a fairly large number of nuclei that are ovel to spindle-shaped and fairly uniform in size.

        • SCOPUSKCI등재

          성인의 두피에 발생된 황색육아종 1예

          오영진,이유신,김신규,안규중,천영일 대한피부과학회 1985 大韓皮膚科學會誌 Vol.23 No.3

          Juvenile xanthogranuloma is characterized by one, or oftener many, yellow, re3dish yellow, or, brown 4-to-20 mm papules or nodules on the face, scalp, trunk, and mostly, extensor surfaces. It may involve internal organ besides the skin such as eye, gastrointestinal and genitourinary tracts. anthogranuloma in adult is usually solitary and dose not involve organs other than skin, and generally dose ncit resolve without treatment. Histopathologically, characteristic Touton giant cells usually appear numerously. We report a case of juvenile xanthogranulorna which occured on scalp of 49 year-old male showing typical clinical and histopathological findings.

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          이상뇌피소견 (異常腦彼所見) 을 동반한 결절성 경화증 (硬化症) 의 1 예

          조성호,김진택,양순균,김신규 대한피부과학회 1980 大韓皮膚科學會誌 Vol.18 No.2

          Tuberous Sclrosis is a rare hereditary disease first described by Bourneville in 1880, and usually transmitted as autosomal dominant trait, and is characterized by mental retardation, seizares, and adenoma sebaceum. This 21 year old male patient visited for multiple pin head to pea siaed yelhwish red waxy papules on the face and gingival papillema af 10 years dunatio. Attacks of grandmal seisure eccr oacurred times in the past 8 yeara. Past hiatory and family history were noncountributory. There was a shagreen patch on left lower lumbo-sacral area. Hiatopathology disclosed an adenoma sebaceum showing dilatation of the capillaries, proliferation of collagen, mild cellular infiltration of papillary dermis and perivascular areas. EEG revealed asymmetrieal slow waves on right side of the brain, predommantly in anterior part, and intermittent sharp waves on right anterior frontotemporal area of the brain.

        • KCI등재

          골에 발생한 거대세포종의 재발 인자

          김한식(Han Sik Kim), 김성환(Sung Hwan Kim), 신규호(Kyoo-Ho Shin), 한수봉(Soo Bong Hahn) 대한정형외과학회 2005 대한정형외과학회지 Vol.40 No.1

          목적: 골의 거대세포종으로 확진된 환자들의 재발률을 확인하여 치료결과를 평가하고, 환자에 따른 인자, 종양의 성질, 수술 방법에 따른 재발과의 상관관계 유무를 확인하고자 한다. 대상 및 방법: 1992년 3월부터 2001년 3월까지 수술적 치료를 받고, 병리학적으로 골의 거대세포종으로 확진된 환자 중 최소 2년 이상 추적관찰이 가능하였던 52예를 후향적으로 검토하였다. 임상적 특성으로 연령, 성별, 발생 부위, 병적골절 유무, Campanacci 등에 의한 종양 등급을 확인하며 치료 방법, 재발 및 재발시기, 재발 후 치료방법, 합병증, 최종 추시 결과를 확인하였다. 정기적인 임상적, 방사선학적 검사를 통해 재발이 의심되는 경우 생검을 통한 병리학적 결과를 확인 하였고, 국소재발과 각 항목과의 상관관계는 Fisher's exact test를 사용하였으며 통계적 유의수준은 p<0.05로 하였다. 결과: 재발은 16예(31%)에서 발생하였고 재발 시기는 평균 17개월로 1예를 제외하고 모두 2년 내에 재발하였다. 성별, 발생부위, Campanacci 분류, 병적골절, 내원 시 종양의 재발여부, 절제연, 골이식, 금속내고정, 골시멘트 충전술 등 각 항목과 국소재발과는 통계적으로 상관관계를 보이지 않았다. 결론: 임상적 특성 및 치료방법과 국소 재발은 상관관계가 없었으며 재발은 대부분 수술 후 2-3년 내에 발생하므로 이 기간 내에 정기적 추시와 검사를 시행하고 이후에도 재발 및 폐 전이에 대한 장기적인 추시 관찰이 요구된다. Purpose: The aim of this study was to evaluate the recurrence rates of a giant cell tumor of the bone and determine what, if any, patient factors, tumor characteristics and surgical treatments correlate with the incidence of a local recurrence. Materials and Methods: The records of fifty two cases who were diagnosed with a giant cell tumor of the bone between March 1992 and March 2001 and were could be followed up for at least 2 years were analysed retrospectively. This study reviewed the clinical characters, and the treatment method, recurrence, complication, the result of the follow-up. A Fisher's exact test was carried out to analyse the statistics. Results: Sixteen patients (31%) had a local recurrence. The average time to recurrence was 17 months. All but case one recurred within 2 years after receiving surgical treatment. There was no significant differences in the recurrence rates based on the following variables: gender, tumor location, Campanacci grade, the presence of a pathologic fracture, primary versus recurrent tumor at presentation, resection margin, the use of a bone graft, internal fixation or polymethylmethacrylate. Conclusion: The clinical characters and methods of treatment were not correlated with a local recurrence. Most recurrences of a giant cell tumor can be expected within the first two to three years. Therefore the patient needs to be followed up in that period and longer for the a late recurrence and pulmonary metastasis.

        • 슬관절 주위 악성 및 침윤성 골종양의 분절 절제술 후 회전 성형술

          한수봉,박홍준,김형식,김성훈,신규호,Hahn,,Soo-Bong,Park,,Hong-Jun,Kim,,Hyoung-Sik,Kim,,Sung-Hun,Shin,,Kyoo-Ho 대한근골격종양학회 2001 대한골관절종양학회지 Vol.7 No.2

          목적 : 어린이나 혹은 성인에 생긴 대퇴부 절단술이 필요한 슬관절 주위의 악성 및 침윤성 골종양에서, 분절 절제 및 회전 성형술을 시행한 치험례를 분석하여 보고하는 바이다. 대상 및 방법 : 1988년 2월부터 1994년 6월까지 슬관절 부위의 악성 및 침윤성 골종양으로 하지 분절 절제 및 회전 성형술을 시행 받은 2 6명을 대상으로 하였으며, 추시 기간은 악성종양의 경우 평균 57(6~120)개월이었고, 평균 연령은 21.4(5~37)세였다. 26례 중 stage IIB 이상의 골육종이 18례, 활막 육종이 2례, 병적골절 또는 재발된 거대 세포종이 6례였다. 결과 : 기능적 결과는 사망 또는 절단술을 받은 4례를 제외한 22례에 대하여 Shriner's rating system에 따라 실시하였으며, 18례에서 우수, 3례에서 양호, 1례는 보통이었다. 족관절의 평균 운동범위는 $-11^{\circ}$(족배굴)~$80^{\circ}$(족저굴)였으며, 보조기 착용 후 일상 생활의 보행이 가능하였다. 국소 재발은 2례, 원격 전이는 10례에서 관찰되었다. 초기 합병증은 혈전증 3례와 패혈증 1례가 있었다. 후기 합병증은 불유합 및 지연유합이 6례, 부정 회전이 2례, 족관절의 운동제한이 1례 있었다. 결론 : 약 10세 미만의 소아에서 많은 하지 부동이나 성장에 따른 골수강의 확장으로 종양 삽입물의 해리가 예상되는 경우와 악성 및 침윤성 골종양으로 절단술이 불가피한 경우에, 기능적으로 우수한 회전 성형술은 부분 사지 구제술로써 유효한 방법으로 사료된다. Purpose : In patients having malignant and aggressive bone tumors around knee joint requiring amputation, segmental resection and rotationplasty were performed and the clinical results were analyzed. Materials and Methods : Twenty-six patients underwent segmental resection and rotationplasty between February 1988 and June 1994, because limb salvage with tumor prosthesis after removal of tumor was impossible. The mean follow-up of malignant tumors was 57(6~120) months and the average age of patients was 21.4(5~37) years old. Out of 26 patients, there were 18 osteosarcoma(${\geq}$stage IIB), 2 synovial sarcoma, and 6 giant cell tumor. Results : Clinical results were evaluated by the Shriner's rating system. Four patients were excluded due to death or amputation and remaining 22 patients were included for assessment. Eighteen patients had excellent result, 3 good, and 1 fair. Range of motion of ankle joint was -11(dorsiflexion)~80(plantarflexion) degrees and daily walking activity with prosthesis was possible. Local recurrence developed in 2 patients and distant metastasis in 10. Early complications had 3 thrombosis and 1 sepsis, and late complications had 6 nonunion, 2 malrotation and 1 stiffness of ankle joint. Conclusion : Rotationplasty which is functionally excellent may serve as an effective partial limb salvage procedure, especially in patients less than 10 years old that lower extremity discrepancy or loosening tumor prosthesis due to enlargement of medullary cavity are anticipated or amputation is inevitable for wide resection margin.

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