• 한국임상수의학회지
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• 제26권3호
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• pp.259-262
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• 2009

Focal adnexal dysplasia(FAD) is a rare non-neoplastic lesion of adnexal appendages that occurs in dogs and has good prognosis. We report a case in which FAD diagnosis was favored by its histopathological features. A 8-year-old, castrated male American cocker spaniel dog presented with papillomatous masses on the distal phalanx of left forelimb. Histopathologically, the stroma was sharply demarcated from the adjacent dermis and subcutis. The adnexa was surrounded and interspersed with abundant, hyalinized collagen and the numerous sebaceous lobules distributed haphazardly around hair follicles, which is thickened, dilated and tortuous with abundant keratin. Epidermis was acanthotic. Proliferative lesions of the skin region are very common with similar gross features, thus the basic histopathological examination supported by additional diagnostic methods allow to establish the definitive diagnosis in most cases of cutaneous tumor or tumor-like lesions. And it is important that physicians and pathologists are aware of this unusual tumour-like nonneoplastic lesion.

• 대한치과교정학회지
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• 제30권6호
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• pp.739-745
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• 2000

외배엽 이형성증(ectodermal dysplasia)는 외배엽에서 분화되는 조직의 일부에 선천적인 이형성이 나타나는 유전적 질환으로 모낭 및 땀샘의 결여로 인한 성긴 모발과 눈썹, 눈과 입주위 피부의 착색 및 각화와 치아형성의 이상 등이 특징적으로 나타나는 질환이다. 구강내 소견으로 무치증 혹은 부분 무치증을 보이며 부분 무치증의 경우 치관이 원추형으로 나타나 결과적으로 전반적인 치아간극 및 수직고경의 감소로 저작 및 발음기능의 저하 뿐 아니라 심미적이지 못한 안모로 사회적 및 정서적 우울감에 빠지기 쉬우므로 조기치료가 요구된다. 바람직한 보철물 제작 및 안모의 개선을 위해서는 교정과 및 타 전문과와의 협진이 요구되며, 본 증례에서는 ectopic dysplasia로 진단된 10세 남아를 교정의사와 보철의사의 긴밀한 협진으로 저작 및 발음기능의 회복과 안면고경의 증가로 심미성이 개선된 좋은 결과를 얻었기에 보고하고자 한다.

• Imaging Science in Dentistry
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• 제52권4호
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• pp.421-427
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• 2022

McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by a clinical triad of polyostotic fibrous dysplasia (FD), skin pigmentation, and hyperfunctioning endocrinopathies. A 42-year-old man visited our medical hospital for the treatment of intermittent headaches and was diagnosed with MAS with acromegaly. This patient showed various clinical features of MAS, including pituitary adenoma, polyostotic FD, and hypogonadotropic hypogonadism. The FD lesions showed characteristic radiographic features, such as widespread, sclerotic bony lesions in the cranial bones, mixed radiolucent-radiopaque multilocular lesions in the mandible, and radiolucent lesions in the axial and appendicular skeleton. Over the years, the patient had been hospitalized multiple times due to accidental bony fractures associated with the fragile bony state of FD. This report presents a retrospective description of a case of MAS, with a review of the relevant literature.

• Journal of Oral Medicine and Pain
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• 제31권2호
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• pp.121-126
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• 2006

저한성 외배엽 이형성증은 감모증, 치아의 수 또는 형태이상과 땀샘의 이상이 특징으로 나타나는 질환이다. 이중 치아 이상은 유치열, 영구치열 모두 영향을 받을 수 있으며 완전 무치증, 부분무치증이 특징이며 치아의 형태는 원추형으로 비정상적으로 나타난다. 이에 저자는 완전무치증을 가진 저한성 외배엽 이형성증 경험하였기에 이에 대한 증례보고와 함께 문헌고찰을 하고자 한다.

• Imaging Science in Dentistry
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• 제30권2호
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• pp.117-122
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• 2000

Fibrous dysplasia is a benign disorder of bone consisting of intramedullary proliferation of fibrous tissue and irregularly distributed, poorly developed bone. The disease manifests itself in the monostotic form in which only one bone is involved and the polyostotic form in which multiple bones at different sites are affected. We reported a extensive case of polyostotic fibrous dysplasia with involvement of craniofacial bones, mandible, ribs and extremities. A 18-year-old man showed remarkable right facial swelling who had been treated on right femur 3 years ago with a bone graft for pathologic fracture and he recognized facial swelling 5 years ago. Extraoral radiograms and computed tomogram showed diffuse sclerosis with a ground glass appearance of the most cranial bones, facial bones. The right mandibular lesion showed very expansile lesion with mottled appearance. Bone scans showed mutifocal increased uptakes in craniofacial bones, right mandible, bilaterally in ribs, humerus, femur, tibia and characteristic varus deformity of right femur (shepherd's crook defomity). This case showed exceptionally bilateral, extensive nature of bone lesion and didn't show any features of skin pigmentation and endocrine disturbances.

• Tuberculosis and Respiratory Diseases
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• 제45권1호
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• pp.227-233
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• 1998

장티푸스에 합병되는 호흡기 질환은 약 1% 정도로 매우 드물다. 저자들은 호흡기 증상이 첫 증상이었던 장티푸스 환자에서 경기관지 내시경검사상 기관지염과 기관지 이형성 소견이 관찰되었고 항생제 투여로 기관지 병변이 호전되었던 환자 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제44권6호
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• pp.1414-1418
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• 1997

장티푸스에 합병되는 호흡기 질환은 약 1% 정도로 매우 드물다. 저자들은 호흡기 증상이 첫 증상이었던 장티푸스 환자에서 경기관지 내시경검사상 기관지염과 기관지 이형성 소견이 관찰되었고 항생제 투여로 기관지 병변이 호전되었던 환자 1례를 경험하였기에 고찰과 함께 보고하는 바이다.

• Journal of Korean Dental Science
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• 제16권1호
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• pp.99-103
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• 2023

McCune-Albright syndrome (MAS) is a disease with clinical features such as fibrous dysplasia in which normal bone tissue is replaced with abnormal fibrous tissue, abnormalities in the endocrine system, and cafe-au-lait spots on the skin. Although MAS patients are generally known to have reduced bone healing ability, postoperative healing after invasive surgical extraction is still not clearly known due to its relatively rare occurrence. In this report, a 25-year-old female patient, who had been diagnosed with MAS and had a history of abnormal bone healing after fractures of her extremities, underwent surgical extraction of the mandibular third molar with surrounding bone removal. Postoperatively, the patient showed favorable soft tissue and bone healing at the surgical site without abnormal findings such as newly developed fibro-osseous lesions, postoperative infection, or osteomyelitis.

• 한국환경성돌연변이발암원학회지
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• 제19권1호
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• pp.46-55
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• 1999

To develop a chemopreventive strategy based on the different stages of premalignant lesions, we hypothesized that the inhibitory effect of chemopreventive agents is influenced by different promotion stages during carcinogenesis. DMBA-TPA-induced skin carcinogenesis was used with ICR mice and chlorophyllin (CHL) was applied as a chemopreventive agent. In vitro assay was performed with Salmonella typhi. TA100 to observe any anti-mutagenic activity of CHL against DMBA. Pre-initiation and pre-promotion effects of CHL were observed by CHL treatment before initiation and before promotion. To evaluate the inhibitory effect at different promotion stages, each group was divided into three subgroups after TPA promotion for 6, 18 and 24 weeks, respectively ; the first subgroup was immediately sacrificed after termination of TPA, the second subgroup was treated with CHL, and the third subgroup was sacrificed 8 weeks after termination of TPA without CHL treatment. The degrees of epithelial dysplasia, papilloma formation, and invasive carcinoma were observed histologically, and GST-Pi expression was observed immunohistochemically. ODC mRNA level was analyzed by reverse transcriptase-polymerase chain reaction. Results showed : CHL dose-dependently inhibited the mutation of Salmonella typhi. TA100; the incidence of epithelial dysplasia and papilloma formation was lower in pre-initiation and pre-promotion CHL-treated mice than DMBA-TPA-treated mice; no invasive carcinoma developed in pre-initiation CHL-treated groups, while 67% of DMBA-TPA treated mice had carcinomas; GST-Pi expression decreased when CHL was treated before initiation and before promotion; and when CHL was treated after termination of TPA application at 18 and 24- week-TPA promotion stages, respectively, the incidence of epithelial dysplasia and papilloma was markedly reduced compared to non-treated groups. When comparing the incidence of epithelial dysplasia and papilloma between the immediately-sacrificed subgroup and the non-treated group with a waiting period, we speculated that the 18-week-TPA promotion stage might belong to the promoter-independent progression stage. At the 18-week-TPA promotion stage, the level of ODC mRNA in the CHL-treated group was clearly reduced to the level of normal tissue. Taking these results together, CHL showed both anti-initiation and anti-promotion effects, while the inhibitory effect of CHL was prominent in the 18-week-TPA promotion stage. However, CHL seems to be incapable of completely blocking the progression in the 24-week-TPA promotion stage.

• 대한골관절종양학회지
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• 제2권1호
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• pp.78-87
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• 1996

The results of treatment of eighteen lesions of fibrous dysplasia which of them seventeen lesions were treated with surgery were reviewed at the Department of Orthopedic Surgery, College of Medicine, Hanyang University Hospital. We studied to evaluate the functional clinical results and the recurrence according to the type of disease, grafted bone, methods of treatment and location of lesion. We treated sixteen patients(five males and eleven females) and their mean age was 22.6 years. There was no association with skin pigmentation or dysfunction of endocrine system. Twelve patients had a monostotic pattern and four patients had a polyostotic pattern. Twelve lesions were treated with curettage and bone grafting and four lesions in the proximal femur were treated by internal fixation with curettage and bone grafting. One lesion was treated by en-bloc resection. There were eleven satisfactory results in twelve monostotic lesions and there were four satisfactory results in five polyostotic lesions, but the recurrence were four cases, respectively. The two unsatisfactory results were seen in two patients treated by autografting, but there were three recurrence of four lesions in autografting only, one of five in autografting and allografting, four of eight in allgrafting or xenografting. Four of six lesions in upper extremity were recurred after curettage and bone grafting and five of them showed satisfactory results. In pelvic and lower extremity lesions, the recurrence were occurred in two lesions after curettage and bone grafting and in two lesions after internal fixation and bone grafting. The recurrence does not always provide an unsatisfactory functional results and the recurrence showed higher rate in radiologic pattern of ground glass appearance with-out marginal sclerotic rim, but the recurrence according to grafted bone showed similar rates. Curettage and bone grafting is adequate for a symptomatic lesion and firm internal fixation is needed for a lesion in proximal femur. In use of grafted bone, xenograft or allograft may be preferable to autograft because of the disadvantage of autografting like a increased blood loss, prolonged operation times, etc.