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Bone Transport for Reconstruction in Benign Bone Tumors
오창선,정성택,조용진,안영섭,나보람 대한정형외과학회 2015 Clinics in Orthopedic Surgery Vol.7 No.2
Background: The aim of this study was to assess the results of using the Ilizarov apparatus to transport bones in the treatment of benign bone tumors. Methods: Seven patients (six males and one female) with benign bone tumors were treated by bone transport with an Ilizarov apparatus at our institution. Their mean age at surgery was 14.4 years (range, 4.8 to 36.9 years). The histological diagnoses were osteofibrous dysplasia (4), giant-cell tumor (1), intraosseous cavernous hemangioma (1), and aneurysmal bone cyst (1). Three radiological indices were used for evaluating the results: an external fixation index, a distraction index, and a maturation index. The bone and functional results were evaluated according to the Association for the Study and Application of the Method of Ilizarov classification. Results: Five patients had bone union at the reconstructed site, one patient had a local recurrence, and the other had a nonunion at the docking site. The mean length of distraction was 7.3 cm (range, 5.1 to 12.1 cm). The mean external fixation index was 26.0 day/cm (range, 19.8 to 32.5 day/cm), the distraction index was 9.6 day/cm (range, 6.8 to 12.0 day/cm), and the maturation index was 14.9 day/cm (range, 8.0 to 22.5 day/cm). Ultimately, the bone and the functional results were rated excellent in six cases and good in one case. Conclusions: Bone transport using the Ilizarov apparatus is a good treatment option in patients with bone defects after the resection of an active or aggressive benign bone tumor.
Osteosarcoma with Adenocarcinoma of Lung in Li-Fraumeni Syndrome: A Case Report
오창선,이진호,정성택,나보람,Oh, Chang-Seon,Lee, Jin-Ho,Jung, Sung-Taek,Na, Bo-Ram The Korean Musculoskeletal Tumor Society 2014 대한골관절종양학회지 Vol.20 No.2
Li-Fraumeni syndrome은 소아나 청장년층에서 다양한 형태의 종양을 유발할 수 있는 상염색체 우성 유전 질병이다. 이는 종양억제 유전자인 TP53 의 변형에 의해 발생하게 된다. 이 질환은 매우 드물며, 진단되지 못하고 간과되는 경향이 많다. 이에 저자들은 17세, 폐선암을 동반한 근위 경골 골육종 환자에서 가족력을 통해 이 질환을 의심하고, 유전자 검사를 통하여 확진한 증례를 보고하고자 한다. Li-Fraumeni syndrome (LFS) is an autosomal dominant hereditary disorder characterised by a variety of different tumor types in children and young adults. That contains with a germline mutation in the tumor suppressor gene Tumor Protein p53 (TP53). That is extremely rare. Furthermore, this is sometimes overlooked. Here, we report a case of LFS which was confirmed by mutational analysis of the p53 gene. Also, literature review is intended to improve understanding of this disease entity.