• Journal of Korean Neurosurgical Society
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• v.29 no.7
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• pp.861-867
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• 2000

Purpose : Neuronal migration disorder(NMD) is a major underlying pathology of patients with intractable epilepsy. The role of NMD on seizure susceptibility or epileptogenecity, however, has not been documented. Methods : External irradiation of total amount of 250 cGY was performed to the fetal rats on days 16(E16) and 17(E17) of gestation. After delivery, the rats of 230-260g were decapitated for the histopathologic study. Epileptog-enecity of the NMD was studied by observing electroclinical events after intraperitoneal kainic acid(KA) injection in the control rats and NMD rats. Results : Histopathologic findings revealed focal and/or diffuse cortical dysplasia consisting of dyslamination of the cerebral cortex and appearance of the cytomegalic neurons, neuronal heterotopia in the periventricular white matter, dispersion of the pyramidal layer and the dentate gyrus of the hippocampus, and agenesis of the corpus callosum. Abnormal expression of neurofilaments protein(NF-M/H) was characteristically observed in the dysplastic neurons of the neocortex and hippocampus. Early ictal onset and prolonged ictal activity on EEG and clinical seizures were observed from the NMD rats unlike with the control rats. Conclusions : Exteranl irradiation on the fetal rats produced NMD. And the rats with NMD were highly susceptible to kainic acid provoked seizures. This animal model would be useful to study the pathophysiology of clinically relevant NMDs.

• Archives of Plastic Surgery
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• v.33 no.2
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• pp.259-262
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• 2006

Fibrous dysplasia(FD) of the bone is a slowly progressive, benign disease of unknown cause where normal architectures are replaced with fibrous and osteoid tissue. FD of the maxilla usually manifests as a bony enlargement with painless swelling and bone deformity, contouring to facial asymmetry. The lesion may involve the nasal fossae, orbits, or alveolus bone, causing diverse functional disturbance. Treatment options range from shaving to total maxillectomy and reconstruction depending on the presenting symptoms. Shaving, partial maxillectomy and maxillary sinus formation was performed in 5 patients with fibrous dysplasia in the past 2 years. Follow up period ranged from 1 month to 11 months. Aesthetic appearance, CT findings, and relief from symptoms were compared. In all patients, facial asymmetry was restored to symmetry and nasal obstructive symptoms were improved. With this procedure, expansion of the lesion will be controlled until puberty, preventing the development of new functional disturbances. After puberty, no further treatment can be anticipated due to the growth arrest inherent to the disease.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.25 no.1
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• pp.159-170
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• 1995

Few cases of florid osseous dysplasia has been described as a condition that characteristically affects the jaws. It usually manifests as multiple radiopaque masses distributed throughout the jaws. Confusion exists about the relationship of florid osseous dysplasia, gigantiform cementoma, chronic sclersing osteomyelitis, sclerosing osteitis or multiple enostosis. Authors experienced a case of florid osseous dysplasia of the jaws in 52-year-old female on the basis of clinical, radiographic and histopathologic findings. The characteristic features are as follows : 1. In clinical examination, there was no clinical sign and symptoms except extracted area. And there was no facial asymmetry. 2. Radiograms show round or lobular dense radiopaque masses surrounded by radiolucent bands in lower molar teeth area bilaterally. And slight increased radiopacities in maxillary molar teeth area bilaterally. There was no expansion or thinning of buccal and lingual cortical bones. There is no displacement or resorption of involved teeth. In right side of mandible, mandibular canal is displaced inferiorly due to mass. 3. Photomicrograms show densely mineralized sclerotic acellular masses with empty lacunae. Pattern is suggestive of cementum, although it could be considered sclerotic bone. In the periphery, lesion consisting of moderately cellular fibrous tissue in which globular calcified products are deposited.

• Journal of Korean Academy of Oral and Maxillofacial Radiology
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• v.29 no.1
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• pp.55-63
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• 1999

The author observed and compared the radiographic features of 49 cases of the fibrous dysplasia and 14 cases of the ossifying fibroma in the osteoblastic or mature stage radiologically and histopathologically. The obtained results were as follows: 1. Fibrous dysplasia occurred most frequently in the 2nd decade, but ossifying fibroma in the 3rd and 4th decades, and both lesions occurred with slight predilection in females. 2. In most cases, chief complaints were painless facial swelling. And 61.1％ of fibrous dysplasia occurred in the maxilla, 92.9％ of ossifying fibroma in the mandible, and most of these lesions occurred in the premolar-molar region. 3. In the mandibular lesions, ossifying fibroma was shown more oval and round shape. but fibrous dysplasia was shown fusiform shape. 4. Fibrous dysplasia was shown homogeneously distributed. complete radiopaque shadow at 63％, and ossifying fibroma was shown concentric. mixed appearance of radiolucent and radiopaque shadow at 92.9％. 5. Fibrous dysplasia was entirely shown poorly outlined and blended to normal surrounding bone, but ossifying fibroma was shown well-defined border. 6. Cortical thinning and expansion were observed in these lesions. but degree of cortical expansion was more severe in ossifying fibroma than fibrous dysplasia. 7. Loss of lamina dura. tooth displacement. and displacement of mandibular canal were observed in both lesions. but root resorption was observed in ossifying fibroma only.

• Journal of Chest Surgery
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• v.36 no.12
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• pp.952-960
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• 2003

It has been reported that p53 regulates the G2-M checkpoint transition through cyclin Bl, and it has been suggested that p53 plays an important role in the development and progression of various malignancies. The aim of this study is to clarify the role of the cell cycle regulators, cyclin B1 and p53 in patients with esophageal squamous cell carcinoma (ESCC). Material and Method: Tissue samples from 46 patients with ESCC were included in this study. Expression levels of cyclin Bl and p53 in samples of normal squamous epithelium, dysplasia, and tumor cells from patients with ESCC were analyzed by immunohistochemical study Result: Several cells in the basement layer of normal epithelium expressed cyclin B1. The number of cyclin B1 positive cells tended to increase as the degree of dysplasia increased from low grade to high grade. More than 10% of tumor cells were cyclin B1 positive in 19 patients (41.3%). Several clinicopathologic parameters, including tumor stage (p<0.05), pathologic Iymph node status (p<0.05) and invasion of Iymphatic vessels (p<0.05), were correlated with the overexpression of cyclin B1. Elevated expression levels of cyclin B1 also correlated with a poor prognosis in patient with ESCC in univariate analysis (p<0.05) and multivariate analysis (p<0.05), In contrast, p53 expression exhibited significant correlation with the level of cyclin B1 expression, but was not associated with prognostic parameters in patients with ESCC. Conclusion: These findings suggest that cyclin B1 is involved in the pathogenesis of carcinoma of the esophagus and that elevated levels of cyclin B1 expression, but not p53 expression, may indicate a poor prognosis for patients with ESCC.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.596-602
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• 2002

Purpose : The purpose of this study was to analyze the association of peripheral neutrophil count with the development of respiratory failure in preterm infants. Methods : A retrospective study was conducted from January 1993 to December 1999 on 44 preterm infants, who were admitted to the neonatal intensive care unit of St. Francisco hospital. Preterm infants(birth weight 500 to 1,350 gm) who had a complete blood count obtained within 2 hours after delivery. Patients in the lowest of neutrophil count(early neutropenia, < $1.0{\times}10^9/L$) were compared with patients in the remaining group. Results : Low neutrophil count were transient in early neutropenia group. The concentration the circulating neutrophil count rose from $0.85{\pm}0.11{\times}10^9/L$ at average of 2 hours after delivery to $5.3{\pm}2.7{\times}10^9/L$ at 24 hours after delivery in the early neutropenia group and from $3.6{\pm}1.6{\times}10^9/L$ to $5.8{\pm}3.2{\times}10^9/L$ in the non-neutropenia group during the same time period. Compare to the non-neutropenia group, the neutropenia group had a lower birth weight($1,046.50{\pm}180.76gm$ Vs $1,156.70{\pm}124.99gm$), a lower Apgar score(1 min : $3.41{\pm}1.18$ Vs $4.30{\pm}1.46$, 5 min : $5.41{\pm}0.87$ Vs $6.15{\pm}0.95$), and a higher incidence of bronchopulmonary dysplasia(27.27% Vs 7.0%). Patients who had early neutropenia were more likely to require mechanical ventilation, supplemental oxygen and hospital stay. Also, main effect factors for the two groups were birth weight(Odds ratio=5.457, 95% CI=1.551-27.525), initial peripheral blood white cells(odds ratio=8.308, 95% CI=2.054-52.699), and bronchopulmonary dysplasia(odds ratio=0.099, 95% CI=0.017-0.397). Conclusion : A low count of neutrophil in the systemic circulation of premature infants within 2 hours of birth is associated with more severe respiratory distress.

• Archives of Craniofacial Surgery
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• v.11 no.2
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• pp.91-94
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• 2010

Purpose: For the best possible aesthetic reconstruction after craniofacial bone tumor resection, pasteurization has been adopted to devitalize neoplastic cells while maintaining osteoinductive properties and mechanical strength. This case report aims to demonstrate a long-term follow-up result of a monostotic fibrous dysplasia in frontal bone which was reconstructed by pasteurized tumoral autograft in situ. Methods: A 14-year-old girl presented with a hard, nontender, slowly growing mass of 6-year duration on her left supraorbital area. CT showed $5{\times}4{\times}3cm$ sized well defined bony mass confined to frontal bone with heterogeneous density. Tumor was excised completely through bicoronal approach and reimplanted to its original site after pasteurization at $60^{\circ}C$ for 30 minutes. The pathologic examination confirmed fibrous dysplasia. Results: She revisited our clinic 5 years later after suffering some assault on her face. On CT examination, pasteurized tumoral autograft was incorporated to host bone except the fractured upper orbital rim without any evidence of recurrence. She has been satisfied with the result. Conclusion: Pasteurization offers a simple, reliable, cosmetic, economic, and durable reconstruction method for craniofacial skeletal tumor. It has advantages of both biologic incorporation ability and mechanical strength without risk of recurrence. So, it should be considered as one of the primary options in benign as well as resectable malignant tumors of craniofacial skeleton.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.35 no.5
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• pp.304-309
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• 2009

Purpose: Fibrous dysplasia (FD) is a fibro-osseous disease associated with activating missense mutations of the gene encoding the $\alpha$-subunit of stimulatory G protein. FD may affect a single bone (called monostotic form) or multiple bones (called polyostotic form). The extent of lesions reflects the onset time of mutation. In this study, cells from monostotic FD in maxilla of a patient were isolated and cultured in vitro for characterization. Materials and Methods: The single cells were released from FD lesion which was surgical specimen from 15 years-old boy. These isolated cells were cultured in vitro and tested their proliferation activity with MTT assay. In osteogenic media, these cells underwent differentiation process comparing with its normal counterpart i.e. bone marrow stromal cells. The proliferated FD cells were detached and transplanted into the dordsal pocket of nude mouse and harvested in 6 weeks and 12 weeks. Results and Summary: FD cells have an increased proliferation rate and poor differentiation. As a result, cells isolated from FD lesion decreased differentiation into osteoblast and increased proliferation capacity. MTT assay presented that proliferation rate of FD cells were higher than control. However, the mineral induction capacity of FD was lesser than that of control. Monostotic FD cells make fewer amounts of bone ossicles and most of them are woven bone rather than lamellar bone in vivo transplantation. In transplanted FD cells, hematopoietic marrow were not seen in the marrow space and filled with the organized fibrous tissue. Therefore, they were recapitulated to the original histological features of FD lesion. Collectively, these results indicated that the FD cells were shown that the increased proliferation and decreased differentiation potential. These in vitro and in vivo system can be useful to test FD cell's fate and possible.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.28 no.1
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• pp.80-86
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• 2006

Cleidocranial dysplasia is rare inherited skeletal dysplasia. It was first reported by Martin in 1765. Subsequently, Marie and Sainton independently documented the criteria of the disease. Cleidocranial dysplasia is a bone disorder caused by a defect in the CBFA1 gene of chromosome 6p21. This gene guides osteoblastic differentiation and appropriate bone formation. Patient with cleidocranial dysplasia has maxillary deficiency, high and narrow palate, prolonged retention of primary teeth, unerupted permanent teeth and supernumerary teeth. Therapeutic options in these patients include of autotransplantation of selected impacted teeth, forced eruption of permanent teeth, full denture, overdenture and implant-supported prosthesis. We report a patient with cleidocranial dysplasia. This patient was treated with implant supported bar overdenture. Despite of gene defect that affects osteoblastic activity, bone remodeling and osseointegration occurred in our patient. So, we report this case with review of literature.

• Journal of Chest Surgery
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• v.34 no.1
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• pp.35-40
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• 2001

배경: 혈역학적으로 문제가 되며 인도메타신 치료가 불가능한 미숙아 동맥관 개존증에서 동맥관 결찰술은 비교적 안전하고 효과적인 치료법으로 알려져 있다. 대상 및 방법: 1995년 1월부터 2000년 5월까지 동맥관 개존증을 가진 50명의 미숙아를 대상으로 인도메타신 치료와 동맥관 결찰술의 치료성적을 검토하였다. 결과: 50례의 미숙아 동맥관 개존증 중 28례에서 혈역학적으로 문제가 되어 치료가 요구되었고 그 중 5례에서는 인도메타신 치료를, 나머지 23례는 인도메타신 치료의 금기가 되어 동맥관 결찰술을 시행하였다. 제태기간과 출생시 체중은 치료를 시행하지 않았던 군(32.1$\pm$2.1주, 1731$\pm$450.9g)과 인도메타신 치료군(32.0$\pm$2.1주, 1830$\pm$165.5g)보다 동맥관 결찰술군(29.6$\pm$2.1주, 1435$\pm$431.0g)이 가장 짧았다(p<0.05). 치료를 시행한 28례에서 치료시 나이(8.6$\pm$5.5일, 7,3$\pm$4.4일)는 인도메타신 치료군과 동맥관 결찰술군 간에 차이가 없었으나, 체중(1670$\pm$43.6g, 1211$\pm$22.4g)은 동맥관 결찰술군에서 의의있게 적었다(p<0.05). 치료후 생존율은 100%와 73.9%로 인도메타신 치료군에서 높았고 술후 사망은 23.7$\pm$22.4일(6-68일)째 발생하였으며 사망원인은 패혈증 5례, 뇌실질내 출혈과 기관지폐이형성증이 각각 2례, 패혈증 쇼크와 기흉이 각각 1례로 수술과는 직접적인 관련이 없었다. 결론: 미숙아 동맥관 개존증에서 조기에 동맥관 결찰술을 시행하는 것은 비교적 안전하고 효과적이며, 특히 인도메타신 치료를 시행할 수 없는 경우와 초저체중의 미숙아에서도 안전하게 적용 될 수 있을 것으로 사료된다.