• Journal of Genetic Medicine
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• v.13 no.2
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• pp.59-64
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• 2016

Gaucher disease type 1 (GD1) is an inherited lysosomal storage disorder caused by deficiency of acid ${\beta}$-glucosidase. The diminished enzyme activity leads to the accumulation of substrates and results in multi-systemic manifestations including hepatosplenomegaly, anemia, thrombocytopenia, and bone diseases. Enzyme replacement therapy (ERT) by infusion of recombinant protein has been the standard treatment for over 20 years. Despite the successful long-term treatment with ERT, several unmet needs remain in the treatment of GD1 such as severe pulmonary and skeletal manifestations. Substrate reduction therapy (SRT) reduces the accumulation of substrates by inhibiting their biosynthesis. Eliglustat, a new oral SRT, was approved in United States and Europe as a first-line therapy for treating adult patients with GD1 who have compatible CYP2D6 metabolism phenotypes. Although eliglustat is not yet available in Korea, introduction and summary of this new treatment modality are provided in this paper by review of literatures. Despite the fact that there are only limited studies to draw resolute conclusions, the current data demonstrated that eliglustat is not inferior to ERT in terms of its clinical efficacy. The approval of eligustat enables eligible adult GD1 patients to have the option of oral therapy although it still needs further studies on long-term outcomes. The individual patient should be assessed carefully for the choice of treatment modality when eliglustat becomes available in Korea. Furthermore, the clinical guidelines for Korean patients with GD1 regarding the use of eliglustat needs to be developed in near future.

• Archives of Plastic Surgery
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• v.34 no.1
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• pp.111-114
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• 2007

Purpose: Dystrophic calcification occurs in damaged or devitalized tissues in the presence of a normal calcium and phosphorus metabolism. There are many reports on dystrophic calcification caused by injections of various types of drugs. The aim of this report is to highlight the fact that dystrophic calcification can be caused by the injection of a foreign body for aesthetic augmentation. Methods: This case report describes a patient presenting with dystrophic calcification caused by an injection of an unknown foreign body approximately 50 years ago. An 80-year-old man had localized cellulitis with swelling and ulceration on the dorsum of the left hand. The radiographs demonstrated a $5{\times}3.5{\times}1.7cm$ lesion between the first and second metacarpal bones and a $5{\times}2.5{\times}1.5cm$ lesion in the hypothenar region. The laboratory data and physical examinations were generally within the normal limits. The microscopic examination revealed dead bone fragments and dense collagenous tissue with dystrophic calcification. Results: After surgically removing the masses, the resulting defects were treated with an abdominal flap. The result was satisfactory in terms of symptoms and appearance. Conclusion: This case suggests that dystrophic calcification can be caused by an injection of a foreign body for aesthetic augmentation.

• Allergy, Asthma & Respiratory Disease
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• v.6 no.6
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• pp.284-289
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• 2018

Purpose: Vitamin D plays an important role in calcium homeostasis and bone metabolism. It is associated with various diseases such as cardiovascular, immune, allergic and infectious disease. The aim of this study was to investigate the difference in clinical manifestations according to the concentration of vitamin D in mild bronchiolitis. Methods: We performed a retrospective review of medical records of patients with mild bronchiolitis from November 2016 to April 2017 in Daegu Fatima Hospital. Mild bronchiolitis was classified by the modified Tal's score method. Patients were divided into 2 groups according to a 25-hydroxyvitamin D level of 20 ng/mL. We analyzed the clinical characteristics and laboratory data from the 2 groups. Results: Of the 64 patients, 19 were included in the deficiency group and 45 in the normal group. Vitamin D levels were $11.7{\pm}4.9ng/mL$ in the deficiency group and $28.8{\pm}5.0ng/mL$ in the normal group. There were no differences in clinical features between both groups. However, the vitamin D deficiency group had significantly longer hospitalization than the normal group ($6.78{\pm}2.74$ days vs. $5.3{\pm}1.7$ days, P=0.045). In the deficiency group, the incidence of previous respiratory diseases was significantly higher (P=0.001). No significant difference in blood and respiratory virus tests was observed. Conclusion: Low vitamin D levels in mild bronchiolitis were associated with longer hospitalization and prior respiratory disease. Vitamin D may affect the course of mild bronchiolitis.

• Nutrition Research and Practice
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• v.15 no.5
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• pp.579-590
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• 2021

BACKGROUND/OBJECTIVES: Petasites japonicus Maxim (P. japonicus) has been used as an edible and medicinal plant and contains many bioactive compounds. The purpose of this study is to investigate the effect of P. japonicus on osteogenesis. MATERIALS/METHODS: The leaves and stems of P. japonicus were separated and extracted with hot water or ethanol, respectively. The total phenolic compound and total polyphenol contents of each extract were measured, and alkaline phosphatase (ALP) activity of each extract was evaluated to determine their effect on bone metabolism. To investigate the effect on osteoblast differentiation of the aqueous extract of P. japonicus leaves (AL), which produced the highest ALP activity among the tested extracts, collagen content was measured using the Sirius Red staining method, mineralization using the Alizarin Red S staining method, and osteocalcin production through enzyme-linked immunosorbent assay analysis. Also, real-time reverse transcription polymerase chain reaction was performed to investigate the mRNA expression levels of Runt-related transcriptional factor 2 (Runx2) and Osterix. RESULTS: Among the 4 P. japonicus extracts, AL had the highest values in all of the following measures: total phenolic compounds, total polyphenols, and ALP activity, which is a major biomarker of osteoblast differentiation. The AL-treated MC3T3-E1 cells showed significant increases in induced osteoblast differentiation, collagen synthesis, mineralization, and osteocalcin production. In addition, mRNA expressions of Runx2 and Osterix, transcription factors that regulate osteoblast differentiation, were significantly increased. CONCLUSIONS: These results suggest that AL can regulate osteoblasts differentiation, at least in part through Runx2 and Osterix. Therefore, it is highly likely that P. japonicus will be useful as an alternate therapeutic for the prevention and treatment of osteoporosis.

• Archives of Plastic Surgery
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• v.46 no.4
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• pp.365-370
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• 2019

The syndrome of the trephined is a neurologic phenomenon that manifests as sudden decline in cognition, behavior, and sensorimotor function due to loss of intracranial domain. This scenario typically occurs in the setting of large craniectomy defects, resulting from trauma, infection, and/or oncologic extirpation. Cranioplasty has been shown to reverse these symptoms by normalizing cerebral hemodynamics and metabolism. However, successful reconstruction may be difficult in patients with complex and/or hostile calvarial defects. We present the case of a 48-year-old male with a large cranial bone defect, who failed autologous cranioplasty secondary to infection, and developed rapid neurologic deterioration leading to a near-vegetative state. Following debridement and antibiotic therapy, delayed cranioplasty was accomplished using a polyetheretherketone (PEEK) implant with free chimeric latissimus dorsi/serratus anterior myocutaneous flap transfer for vascularized resurfacing. Significant improvements in cognition and motor skill were noted in the early postoperative period. At 6-month follow-up, the patient had regained the ability to speak, ambulate and self-feed-correlating with evidence of cerebral/ventricular re-expansion on computed tomography. Based on our findings, we advocate delayed alloplastic implantation with total vascularized soft tissue coverage as a viable alternative for reconstructing extensive, hostile calvarial defects in patients with the syndrome of the trephined.

• Journal of the Korean Applied Science and Technology
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• v.36 no.1
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• pp.189-199
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• 2019

The aim of this study was to investigate the effect of combine exercise on the fall injury related index and myokine in elderly women. Sarcopenia leads to a loss of strength, alter on to a decreased functional status, impaired mobility, a higher risk of falls, and eventually an increased risk of mortality. Aerobic exercise characterized by rhythmic and repetitive movements of large muscles, for sustained periods that depends primarily on the use of oxygen to meet energy demands through aerobic metabolism, and that is structured and intended to generate improvements in cardiopulmonary fitness, body composition, and cardiorespiratory health. Resistance training has performance in the elderly. As combined exercise therapy can be used to enhance muscle functions and cardiopulmonary functions, it is being highlighted as an effective health management methods for the aged. The myokine has been regarded an important factor of exercise how muscle communicate adipose tissue, bone and muscle to exert beneficial effects at the whole body level.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.45 no.2
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• pp.108-115
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• 2019

Objectives: Medication-related osteonecrosis of the jaw (MRONJ) is a well-known side effect of certain drugs that are used to influence bone metabolism to treat osteometabolic disease or cancers. The purpose of our study was to investigate how high-concentration and low-concentration bisphosphonate (BP) intake affects the disease severity. Materials and Methods: Data collected from the medical records of 52 patients treated with BPs, antiresorptive, antiangiogenic drugs and diagnosed with MRONJ were included in this study. Age, sex, type of systemic disease, type of drug, duration of drug treatment, jaw area with MRONJ, drug administration protocol, and MRONJ clinical and radiological findings were obtained. Patients were divided into two groups: anti-neoplastic (Group I, n=23) and anti-osteoporotic (Group II, n=29). Statistical evaluations were performed using the IBM SPSS ver. 21.0 program. Results: In both groups, more females had MRONJ. MRONJ was found in the mandibles of 30 patients (Group I, n=14; Group II, n=16). When we classified patients according to the American Association of Oral and Maxillofacial Surgeons staging system, significant differences were seen between groups (${\chi}^2=12.23$, P<0.01). More patients with advanced stage (stage 2-3) MRONJ were found in Group I (60.9%). Conclusion: According to our results, high-concentration BP intake, age and duration of drug intake increased disease severity.

• BMB Reports
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• v.54 no.6
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• pp.323-328
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• 2021

Periodontal diseases have been reported to have a multidirectional association with metabolic disorders. We sought to investigate the correlation between periodontitis and diabetes or fatty liver disease using HFD-fed obese mice inoculated with P. gingivalis. Body weight, alveolar bone loss, serological biochemistry, and glucose level were determined to evaluate the pathophysiology of periodontitis and diabetes. For the evaluation of fatty liver disease, hepatic nonalcoholic steatohepatitis (NASH) was assessed by scoring steatosis, inflammation, hepatocyte ballooning and the crucial signaling pathways involved in liver metabolism were analyzed. The C-reactive protein (CRP) level and NASH score in P. gingivalis-infected obese mice were significantly elevated. Particularly, the extensive lobular inflammation was observed in the liver of obese mice infected with P. gingivalis. Moreover, the expression of metabolic regulatory factors, including peroxisome proliferator-activated receptor γ (Pparγ) and the fatty acid transporter Cd36, was up-regulated in the liver of P. gingivalis-infected obese mice. However, inoculation of P. gingivalis had no significant influence on glucose homeostasis, insulin resistance, and hepatic mTOR/AMPK signaling. In conclusion, our results indicate that P. gingivalis can induce the progression of fatty liver disease in HFD-fed mice through the upregulation of CD36-PPARγ axis.

• BMB Reports
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• v.54 no.5
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• pp.278-283
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• 2021

Our understanding of the differential effects between specific omega-3 fatty acids is incomplete. Here, we aimed to evaluate the effects of docosahexaenoic acid (DHA) and eicosapentaenoic acid (EPA) on T-helper type 1 (Th1) cell responses and identify the pathways associated with these responses. Naïve CD4⁺ T cells were co-cultured with bone marrow-derived dendritic cells (DCs) in the presence or absence of palmitate (PA), DHA, or EPA. DHA or EPA treatment lowered the number of differentiated IFN-γ-positive cells and inhibited the secretion of IFN-γ, whereas only DHA increased IL-2 and reduced TNF-α secretion. There was reduced expression of MHC II on DCs after DHA or EPA treatment. In the DC-independent model, DHA and EPA reduced Th1 cell differentiation and lowered the cell number. DHA and EPA markedly inhibited IFN-γ secretion, while only EPA reduced TNF-α secretion. Microarray analysis identified pathways involved in inflammation, immunity, metabolism, and cell proliferation. Moreover, DHA and EPA inhibited Th1 cells through the regulation of diverse pathways and genes, including Igf1 and Cpt1a. Our results showed that DHA and EPA had largely comparable inhibitory effects on Th1 cell differentiation. However, each of the fatty acids also had distinct effects on specific cytokine secretion, particularly according to the presence of DCs.

• Animal Bioscience
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• v.36 no.2
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• pp.167-174
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• 2023

Phosphorus (P) is a macro mineral needed for bone mineralization and cell membrane structure and P is also involved in several fundamental pathways of metabolism in the body. Because of the low concentration and digestibility of P in plant ingredients that are the main components of diets for poultry and pigs, feed phosphates are usually included in diets in addition to the P contributed by plant ingredients. The most widely used feed phosphates in poultry and swine diets are dicalcium phosphate (DCP) and monocalcium phosphate (MCP), but tricalcium phosphate (TCP), monosodium phosphate (MSP), and magnesium phosphate (MgP) may be used as well. Because feed phosphates are mostly produced from rock phosphate, feed phosphates have impurities that contain minerals other than P. Concentrations of P in feed phosphates range from 14.8% (MgP) to 25.7% (MSP). The standardized total tract digestibility (STTD) of P in pigs ranges from 71% (TCP) to 95% (MSP). The STTD of Ca and the standardized ileal digestibility (SID) of P and Ca in feed phosphates fed to pigs and poultry have been determined only in a few experiments. Available data indicate that the STTD of Ca and SID of P in MCP are greater than in DCP in both poultry and pigs, but the SID of Ca is similar between DCP and MCP fed to broilers. Information on mineral concentrations and digestibility values in feed phosphates is needed in diet formulation for pigs and poultry, but if diets are formulated to contain equal concentrations of digestible P and Ca, it is unlikely that animal performance will be impacted by the source of feed phosphates used in the diet.