• Tuberculosis and Respiratory Diseases
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• v.64 no.3
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• pp.224-229
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• 2008

Erdheim-Chester disease (ECD) is a rare disease that is characterized by multi-organ involvement of foamy histiocytes. It causes systemic inflammation, and also demonstrates various clinical manifestations and has a poor prognosis. We encountered a case of ECD in a patient that had been treated for underlying polycythemia vera. As far as we know, this is the first reported case worldwide where ECD developed in association with polycythemia vera. A 59-year-old man visited our hospital due to pleuric pain at the right side of the chest. Pleural tissue that was obtained following a thoracoscopic biopsy showed non-Langerhan's cell histiocytosis, suggesting the presence of ECD. The histiocytes stained positively for CD68, but were negative for S-100 and CD1a. The patient also complained of pain at both hips and the right shoulder area. An X-ray and magnetic resonance image demonstrated that the lesion showed sclerosis and osteolysis in both the proximal femur and right humerus. Treatment was started with predinisolone, and subsequently cyclophosphamide was added. ECD is a very rare multi-systemic disease, and its cause and therapeutic options have not yet been defined. ECD has a poor prognosis. Therefore, we believe that additional case studies are needed prior to the determination of a novel therapy for ECD.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.30 no.6
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• pp.572-576
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• 2008

Gorham-Stout disease is a rare skeletal disease which is progressive and spontaneous and idiopathic resorption of whole body bone. In the initial stage, the osteolytic site is replaced by histologically benign vascular proliferation without new bone formation and finally by dense fibrous tissues. We encountered with a patient involving the mandible with attention by the clinical, radiographic, scintigraphic, angiographic, and computed tomography. We resected the mandibular lesion which was reconstructed with a fibular free flap. We report literature review with a mandibular lesion involved with Gorham-Stout disease.

• The Journal of Korean Obstetrics and Gynecology
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• v.18 no.1
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• pp.55-63
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• 2005

Purpose : 자하거(Hominis Placenta; HP)는 건강한 사람의 태반을 홍제(烘製)하여 건조한 것으로 한의학에서는 기혈(氣血)을 대보(大補)하고 신정(腎精)을 보익(補益)시켜 구병(久病)으로 인한 신체허약(身體虛弱)이나 혹은 체질허약(體質虛弱)과 혈기부족(氣血不足) 및 신허정휴(腎虛精虧) 등 등(證)을 치료(治療)하는데 단미(單味) 또는 복방(複方)에 배오(配伍)하여 쓰여왔다. 또한 자하거는 면역학적으로 골대사 활성이 있는 것으로 알려져 있어 본 연구에서는 자하거의 항골다공증 활성을 분자세포생물학적으로 검정하고자 하였다. Methods : Osteoblast cells에서 자하거가 COX-2 mRNA의 발현과 $PGE_2$ 생합성을 억제시키는지를 관찰하기 위해 먼저 TNF-${\alpha}$, IL-${\beta}$ 와 IL-6를 처리한 후 $PGE_2$의 생합성과 더불어 COX-2 mRNA의 발현을 확인하였다. 그 후 TGF-${\beta}$, 자하거(紫河車)와 이 둘의 조합인 자하거+TGF-${\beta}$가 COX-2 mRNA 발현과 $PGE_2$ 생합성을 저해시키는지 관찰하였다. 또한 자하거가 IL-1${\beta}$로 유발된 흰쥐의 과칼슘혈증을 감소시키는지를 확인하였다. Results : IL-6, IL-1${\beta}$와 TNF-${\alpha}$를 동시에 처리하면 이것을 단독으로 처리한 것과 비교해 볼 때 $PGE_2$의 생합성과 더불어 COX-2 mRNA의 수치가 상승작용을 일으키며 증가하였다. TGF-${\beta}$, 자하거와 이 둘의 조합인 자하거+TGF-${\beta}$은 COX-2 mRNA 발현, $PGE_2$ 생합성 및 골재흡수를 감소시켰다. 자하거(紫河車)는 IL-1${\beta}$, TNF-${\alpha}$와 IL-6 각각 또는 이들의 조합으로 인해 증가하는 COX-2 mRNA 발현과 $PGE_2$ 생성을 감소시키는 반면 COX-1 mRNA 발현에는 유의성 있는 영향을 미치지 않았다. 한편 자하거는 농도의존적으로 IL-1${\beta}$로 유발된 흰쥐의 과칼슘혈증을 감소시켰다. 이러한 결과는 흰쥐의 두개골 골아세포에서 $PGE_2$ 생산에 대한 IL-${\beta}$, TNF-${\alpha}$, IL-6의 상승작용이 COX-2의 유전자 발현 증가에 기인함을 보여주었다. Conclusions : 이러한 결과들로부터 자하거가 골대사과정중 골재흡수를 억제하는데 효과적임을 밝히게 되었으며, 자하거의 골다공증의 억제기전이 골재흡수관련 단백질들의 전사조절에 있음을 최초로 해명하게 되었다.

• Journal of Microbiology and Biotechnology
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• v.32 no.8
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• pp.1017-1025
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• 2022

Bone homeostasis is regulated by constant remodeling through osteogenesis by osteoblasts and osteolysis by osteoclasts and osteoporosis can be provoked when this balance is broken. Present pharmaceutical treatments for osteoporosis have harmful side effects and thus, our goal was to develop therapeutics from intrisincally safe natural products. Fucoidan is a polysaccharide extracted from many species of brown seaweed, with valuable pharmaceutical activities. To intensify the effect of fucoidan on bone homeostasis, we hydrolyzed fucoidan using AMG, Pectinex and Viscozyme. Of these, fucoidan biotransformed by Pectinex (Fu/Pec) powerfully inhibited the induction of tartrate-resistant acid phosphatase (TRAP) activity in osteoclasts differentiated from bone marrow macrophages (BMMs) by the receptor for activation of nuclear factor-κB ligand (RANKL). To investigate potential of lower molecular weight fucoidan it was separated into >300 kDa, 50-300 kDa, and <50 kDa Fu/Pec fractions by ultrafiltration system. The effects of these fractions on TRAP and alkaline phosphatase (ALP) activities were then examined in differentiated osteoclasts and MC3T3-E1 osteoblasts, respectively. Interestingly, 50-300 kDa Fu/Pec suppressed RANKL-induced osteoclasts differentiation from BMMs but did not synergistically enhance osteoblasts differentiation induced by osteogenic agents. In addition, this fraction inhibited the expressions of NFATc1, TRAP, OSCAR, and RANK, which are all key transcriptional factors involved in osteoclast differentiation, and those of Src, c-Fos and Mitf, as determined by RT-PCR. In conclusion, enzymatically low-molecularized 50-300 kDa Fu/Pec suppressed TRAP by downregulating RANKL-related signaling, contributing to the inhibition of osteoclasts differentiation, and represented a potential means of inducing bone remodeling in the background of osteoporosis.

• The Journal of the Korean bone and joint tumor society
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• v.20 no.1
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• pp.14-21
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• 2014

Purpose: To analyze the clinical features and treatment outcome of Langerhans' cell histocytosis. Materials and Methods: From August 1996 to June 2013, 28 patients who histologically proven with LCH were analyzed of medical records, radiography, pathologic character retrospectively. Results: A total of 28 cases of LCH including 22 child has been reported. Onset age was 0.6 to 51 years old, occurred in the average age was 14.8 years. Follow-up period was 6 months to 134 months average was 44.6 months. The M:F ratio was 2.5:1. The initial symptoms was pain in 18 cases, 5 cases of pathologic fracture, 3 case of palpable mass, 1 case of discovered by accident in radiography, 1 case of torticollis. In radiological examination osteolysis was seen all cases, 7 cases showed a periosteal reaction, 1 case showed soft tissue extension. Clinical type of all cases were eosinophilic granuloma. 25 cases were classified as unifocal disease and 3 cases were multifocal single systemic diseases. In all cases, incisional biopsy was performed. After histologic confirmed, 14 cases was treated with curettage or surgical excision of the lesion and the other 14 cases were followed up without treatment. There is no death during follow up period. 11 cases has no radiological improvement after 3-6 months observation, intralesional steroid injection was performed. Conclusion: Patients with LCH who has rapid systemic onset is very rare, so if you meet the young children who suspected LCH, you shoulder avoid the examination which cause excessive radiation exposure to the young patient. In order to confirm the diagnosis of disease, biopsy is needed. Close observation after confirmed by histological method will bring the satisfactory results. But the patients who had pathologic fracture or wide bone destruction already may need curettage and bone grafting to lesion or internal fixation. The lesion which has no radiological improvement after 3-6 months observation or appear with pain interferes daily life may need local steroid injection as a good treatment.

• The Journal of the Korean bone and joint tumor society
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• v.6 no.4
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• pp.152-162
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• 2000

Purpose : To verify radiological changes of limb salvage operation using tumor prosthesis. Materials and Methods : Sixty-nine cases which used tumor prosthesis were reviewed. They have been followed up for average four years and nine months. We evaluated radiographs by ISOLS(International Symposium On Limb Salvage) radiological implants evaluation system immediate postoperatively, at postoperative 1 year and last follow-up. After converting 'excellent, good, fair, poor' to '4, 3, 2, 1' for stastical analysis, data were analyzed according to the use of cement, anatomical sites, diagnosis implant designs and age (<20 year vs. ${\geq}$20 year) using t-test and ANOVA. Results : The outcomes of cemented type prostheses were superior in remodelling and interface but inferior in anchorage. The scores of proximal femur and distal tibia were worse in bone remodelling. Osteosarcoma group had lower scores in anchorage and implant articular problem. There were no significant differences according to four different implant designs and age. Conclusion : In the intermediate term follow-up radiological evaluation, cemented type prosthesis were better in the aspects of bony resorption and osteolysis, and cementless one in anchorage. Distal femur and proximal tibia have higher scores in bony resorption, and osteosarcoma was worse tumor entity in anchorage and articular problem, and chemotherapy may be the cause.

• The Journal of the Korean bone and joint tumor society
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• v.11 no.1
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• pp.46-53
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• 2005

Purpose: Bisphosphonates (BPs) are the analogues of endogenous pyrophosphates: they have been used in the treatment of skeletal diseases such as Paget's disease, osteoporosis, and tumorinducing ostelysis, and are used in treatment of osteolytic metastasis of breast cancer recently. They are also used as one of the therapeutic agents for metastasis of prostatic cancer of which metastasis makes the mixed nature of osteolysis and ostegenesis. Although the action mechanism of BPs are well known for diseases with excessive osteoclastic bone resorption, the direct effect of BPs has not been known yet. This study was intended to see the tumor suppression capability of Zoledronic acid(ZOL) using nude mouse with osteosarcoma. Materials and Methods: MG-63 and HOS osteosarcoma cell lines were used and the transforemed MG-63-GFP and HOS-GFP cells, which were made for detection under fluorescent light, were subcutaneously injected to make osteosarcoma. The five 6-week male mice were used for the experiment at each group. After the injection, mice were cultivated until tumor pieces grow up to $3{\times}3{\times}3$ $mm^3$ and ZOL of 120 ug/kg was subcutaneously injected twice a week. Sizes of tumor were measured twice a week and photographed under fluorescent light. Results: In in vivo test with HOS osteosarcoma cell lines, mean size of tumors was 2,520 $mm^3$ in control group and was 131 $mm^3$ in ZOL group, which showed 94% of reduction comparing with the control ; with MG-63 osteosarcoma cell lines, mean size of tumors was 2,866 $mm^3$ in control group and was 209 $mm^3$ in test group with 72% of reduction (p<0.05). Conclusion: In in vivo tests with nude mice, we suggest that ZOL has direct effect on osteosarcoma cells and it would be used as one of the therapeutic agents for osteosarcoma, especially to ZOL-sensitive osteosarcoma cells.