• Childhood Kidney Diseases
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• v.24 no.1
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• pp.14-18
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• 2020

Chronic kidney disease-mineral bone disorder (CKD-MBD) is a systemic disorder of mineral and bone metabolism caused by CKD. Patients with early-stage CKD who present with disordered regulation of bone and mineral metabolism may be asymptomatic. However, if untreated, the condition can be a significant barrier in achieving optimal bone strength, linear growth, and cardiovascular health in pediatric patients with CKD. Thus, the current study evaluated the definition, pathogenesis, diagnosis, and management of pediatric CKD-MBD.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.42-47
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• 2014

Gaucher disease is a multisystemic disorder arising from a deficient activity of the lysosomal enzyme glucocerebrosidase, which leads to accumulation of glycosylceraide and other glycolipids in the regiculoendothelial system. The characteristics of Gaucher disease are anemia, thrombocytopenia, hepatosplenomegaly, and skeletal disease. Enzyme replacement therapy (ERT) has been proven to prevent progressive manifestations of Gaucher disease and effective in improving anemia, thrombocytopenia, bone markers and biomarkers. However, some patient needs still remain unmet because of the inaccessibility of certain sites including brain, bone and various organs. ERT could not Improve the irreversible lesion such as liver fibrosis, hepatopulmonary syndrome, and necrosis or infarction of bone and other organs. Adult patients with Gaucher disease should be screened for longterm complication such as bone disease, pulmonary hypertension, gallstone, and cancer, especially in patients with splenectomy. Parkinsonism and polyneuropathy was also reported among patients with type 1 Gaucher disease, but ERT does not improve neurological function. We need to review the benefits and unmet needs of ERT in Gaucher disease.

• Journal of Bone Metabolism
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• v.25 no.4
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• pp.213-217
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• 2018

Patients with gastrointestinal disease (GI) are at risk for osteopenia or osteoporosis, which can lead to fractures. Although these patients may be at risk from a young age, gastroenterologists often overlook this fact in practice. There are well-known GI diseases associated with osteopenia and osteoporosis, such as the post-gastrectomy state, inflammatory bowel disease (IBD), and celiac disease. As there is an increase in the prevalence of IBD patients, newly diagnosed celiac disease in adulthood, and gastric cancer survivors following gastrectomy, bone disease in these patients becomes an important issue. Here, we have discussed osteoporosis and fractures in GI disease, especially in the postgastrectomy state, IBD, and celiac disease. Although the pathogenesis of bone loss in each disease has not been fully identified, we have confirmed that the prevalence of osteoporosis and fractures in each of these diseases is high. There are scarce studies comparing the prevalence of osteoporosis or osteoporotic fractures in GI disease patients with studies in postmenopausal women, and specific guidelines for their management in each disease have not been established. Intensive surveillance and management are needed to ensure that these patients attain peak bone mass for age and sex to prevent fractures.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.1-11
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• 2023

Inborn errors of metabolism encompass a wide variety of disorders, frequently affecting bone. This review presents a comprehensive retrospect on the primary involvement of bone in inborn errors of metabolism. Primary involvement of bone in inborn errors of metabolism includes entities that primarily affect the bone marrow, mineral component or cartilage. These include lysosomal storage disorders, hypophosphatasia, and hereditary hypophosphatemic rickets. In this review, we discuss the primary involvement of bone in inborn errors of metabolism (hypophosphatasia, X-linked hypophosphatemic rickets, Gaucher disease, and mucopolysaccharidoses) along with the therapeutic agents used in clinical settings, diagnostic strategies, and general management. With the development of disease-specific targeted therapies and supportive care, more number of patients with these disorders live longer and survive into adulthood. Moreover, skeletal symptoms have become a more prominent feature of these disorders. This makes the awareness of these skeletal symptoms more important.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.29 no.1
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• pp.33-41
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• 2007

Bone scan using radioactive isotope can be more effective than conventional X-ray radiograph for finding jaw lesion because it takes an image of the physiologic change of bone. This study is designed to show how available bone scan is able to diagnose jaw lesion better than simple X-ray and CT, as well as to determine a basis of diagnosis for jaw lesion using bone scan. The 77 patients, visiting the Oral & Maxillofacial Surgery, Department of Dankook University Hospital from January 2002. to August 2005. who were diagnosed histopathologically with postoperative malignant tumor, osteomyelitis, and bone infiltrative benign disease. Preoperative X-ray, CT, bone scan were taken and were compared with histopathologic finding. Also to compare specificty of each lesion in bone scan, bone density was measured to compare. The results were as follows. 1. Among the 25 cases of oral malignant tumor of bony invasion, a positive diagnosis associated with histopathologic evaluation, 22 cases(88%) in bone scan, 14 cases(56%) in CT image, and 10 cases40%) in simple X-ray. 2. Among the 31 cases of osteomyelitis, a positive diagnosis associated with histopathologic evaluation, 30 cases(97%) in bone scan, 23 cases(74%) in CT image, and 19 cases(61%) in simple X-ray. 3. Among the 11cases of bone infiltrative benign disease, a positive diagnosis associated with histopathologic evaluation, 11 cases(100%) in bone scan, 10 cases(91%) in CT image, and 6 cases(55%) in simple X-ray. 4. Measurement of bone density in each group showed no statistical significant difference between malignant tumor and osteomyelitis as well as benign bone disease. But, a statistical significance was seen between osteomyelitis and benign bone disease. From this results, bone scan are more sensitive than simple X-ray and CT image in jaw lesion diagnosis, but specificity shows no significant difference. Therefore, it should be suggested that evaluation of bone scan must be carrying out in reference to final histopathologic diagnosis.

• Proceedings of the Korean Society of Computer Information Conference
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• 2017.01a
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• pp.263-265
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• 2017

본 논문에서는 대사증후군관련 의료 빅데이터 표준화 시스템의 환경과 소프트웨어 환경을 설계한 사항에 대해서 기술한다. 이를 위해 임상데이터를 기반으로 의료 빅데이터를 수집하고 국제 표준화인 공통 데이터 모델로 수집된 데이터를 ETL하여 통합 데이터베이스에 저장하였다. 본 연구를 통해 구축된 의료 빅데이터 표준화 시스템은 향후 의사결정 보조시스템 개발과 연계하여 효과적인 검색과 다양한 통계 분석을 지원할 계획이다. 또한 병원의 다양한 임상 연구를 지원하기 위한 주요 시스템으로 자리매김할 것으로 기대한다.

• Journal of Physiology & Pathology in Korean Medicine
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• v.28 no.1
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• pp.29-34
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• 2014

Bone homeostasis is maintained by balance between bone resorbing-osteoclasts and bone forming-osteoblasts. Excessive osteoclastic bone resorption plays a critical role in bone destruction in pathological bone diseases such as osteoporosis, rheumatoid arthritis, and periodontal disease. Many compounds derived from natural products have pharmacological applications and have therapeutic value for treating or preventing several bone diseases characterized by excessive bone resorption. To discover new compounds that can act as anti-resorptive agents, we screened for natural compounds that regulate osteclast differentiation, and found that water extract of Ziziphus Jujuba Mill (WEZJ) has inhibitory effects on osteoclast differentiation. In this study, WEZJ clearly inhibits the osteoclast differentiation in the presence of receptor activator of nuclear factor kB (RANKL), macrophage colony-stimulating factor (M-CSF) without cytoxicity by blocking activation of nuclear factor of activated T cells (NFAT)c1, and c-Fos. In signaling pathway, the phosphorylation of Akt, p38, c-Jun N-terminal kinases (JNK), extracellular signal-regulated kinases (ERK) and the expression of osteoclast-associated receptor (OSCAR), tartrate-resistant acid phosphates (TRAP), Integrin av, Integrin b3, Cathepsin K are suppressed, too. These result suggest that WEZJ may have therapeutic value for treating or preventing several bone diseases characterized by excessive bone destruction.

• Asian Pacific Journal of Cancer Prevention
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• v.13 no.9
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• pp.4369-4371
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• 2012

Aim: Metastatic tumor of bone is the most common malignancy involving bone and is an important predictor of prognosis in advanced cancers. The prognosis depends upon the primary site of origin and the extent of disease. In current study, we present the pattern and distribution of metastatic bone disease seen in the leading cancer care center of Pakistan, Shaukat Khanum Cancer Hospital & Research Center (SKMCH & RC), Lahore. Materials & Methods: All cases of bony metastatic disease were included that presented in the Pathology Department, from Jan 2005 to July 2011. Patients of all ages and both sexes were included. Primary bone tumors, lymphomas, sarcomas and other malignancies were excluded. The data were recorded and analyzed with SPSS 16.0. Results: A total of 146 cases of metastatic bone disease were included in the study. Out of the total cases, 79 were male and 67 were female. Age range 25-82 years (median 52). Hip bone was the most frequent bone involved, with femur and vertebrae as second and third in the list. The commonest bone involved in males was vertebrae with 23 cases and in females was hip bone with 22 cases. Regarding primary site, cancers of breast, prostate and gastrointestinal tract were at the top of the list with prostate and breast being the most frequent primary sites of metastasis in males and females respectively. Conclusion: Bone metastasis is an important entity to consider in the differential diagnosis whenever a bony tumor especially carcinoma present in older age. Our data are comparable with international findings and the literature available regarding the site and distribution of skeletal metastatic lesions. A slight deviation noted was more common bony metastatic lesions with ovarian primaries in females and gastrointestinal tract cancers in males in our study.

• Journal of muscle and joint health
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• v.19 no.3
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• pp.350-361
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• 2012

Purpose: The purpose of this study is to explore the level of bone mineral densities of the femur neck and to identify factors affecting bone mineral density of the femur in Parkinson's disease (PD) patients. Methods: Participants were 121 PD patients visiting the outpatient clinic of D University Hospital in B City. Bone mineral density was measured at the femur neck by using a dual-energy x-ray absorptiometry. The serum vitamin D level, the amounts of milk intake, caffeine intake, cigaret smoking, and alcohol consumption, and the number of steps taken daily were measured. Past and present disease history, the medication history, and duration of the disease were also collected. The level of disability was obtained by neurologists using the Hoehn and Yahr stage and the Unified Parkinson's Disease Rating Scale. Results: Among PD patients, 57% had osteopenia and 15.7% had osteoporosis. The bone mineral density of the femur was associated with weight, serum vitamin D level, age, and working status. Conclusion: The identification of weight and serum vitamin D level as important predictive factors emphasizes the importance of balanced nutrition for PD patients.

• Proceedings of the Korea Information Processing Society Conference
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• 2015.10a
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• pp.1446-1447
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• 2015

In this paper, we developed the quantification software for evaluating the voxel-based cellular heterogeneity of gadoxetic acid-enhanced magnetic resonance imaging (MRI) in the liver. Our software is clinically applied to accurately quantify and interpret the alterations of liver functions in patients with hepatocellular carcinoma.