• Journal of the korean academy of Pediatric Dentistry
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• v.39 no.2
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• pp.161-165
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• 2012

Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare genetic disorder involving eyes, kidney, brain and musculoskeletal system, and occurs predominantly in males. The patient with Lowe syndrome is characterized with congenital cataracts, glaucoma, prominent forehead, thin and sparse hair, mental and growth retardation, muscular hypotonia, renal dysfunction, and metabolic bone disease. We have experienced a 10-year-old boy with Lowe syndrome who had poor oral hygiene and trouble for teeth brushing. To manage his behavior and systemic metabolic disorder, sedation was performed during dental care. Excessive calculus formation in this patient is caused by both medication and lack of ability to maintain oral hygiene. The dental management of those patients has to be focused on prevention due to difficulties in dental treatment and dangers of general anesthesia for the Lowe syndrome.

• Anatomy and Cell Biology
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• v.56 no.3
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• pp.374-381
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• 2023

Although the epiglottis plays a vital role in deglutition, histological studies of the epiglottis and surrounding ligaments associated with swallowing dysfunction are limited. Therefore, we performed histological observations to clarify age-related changes in the morphological characteristics of the epiglottis and surrounding structures. Tissue samples comprising the epiglottis and surrounding structures were collected from corpses that were both orally fed and tubefed during their lifetimes. Following hematoxylin and eosin, Elastica Van Gieson, and immunohistochemical staining procedures, the chondrocytes, connective tissue, and glandular tissue were observed under the epiglottis epithelium, and intervening adipose tissue was observed in the surrounding area. Fatty degeneration of acinar cells was also observed in the glandular tissue, possibly because of aging. Bundles of elastic fibers were present around the vascular wall in the peri-epiglottic ligament, but some were reduced. Furthermore, large amounts of collagen fibers ran toward and through the cartilage, whereas the mesh-like elastic fibers stopped in front of the cartilage. Microfibrils considered to be oxytalan fibers, which are thinner and shorter than elastic fibers, were observed around the vascular wall and in the fiber bundles. Age-related changes included connective tissue fibrosis shown by the large amount of collagen fibers, atrophy of salivary glands, and an accompanying increase in adipose tissue. Regarding stretchability and elasticity, the elastic fibers may have an auxiliary function for laryngeal elevation during deglutition. This suggests that disuse atrophy of the laryngeal organs with or without oral intake might reduce the amount of elastic fiber in older adults.

• The Korean Journal of Pain
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• v.37 no.2
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• pp.164-177
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• 2024

Background: Neck and jaw pain is common and is associated with jaw functional limitations, postural stability, muscular endurance, and proprioception. This study aimed to investigate the effect of jaw and neck pain on cranio-cervico-mandibular functions and postural stability in patients with temporomandibular joint disorders (TMJDs). Methods: Fifty-two patients with TMJDs were included and assessed using Fonseca's Questionnaire and the Helkimo Clinical Dysfunction Index. An isometric strength test was performed for the TMJ depressor and cervical muscles. The TMJ position sense (TMJPS) test and cervical joint position error test (CJPET) were employed for proprioception. Total sway degree was obtained for the assessment of postural stability. Deep neck flexor endurance (DNFE) was assessed using the craniocervical flexion test. The mandibular function impairment questionnaire (MFIQ) was employed to assess mandibular function, and the craniovertebral angle (CVA) was measured for forward head posture. Results: Jaw and neck pain negatively affected CVA (R² = 0.130), TMJPS (R² = 0.286), DNFE (R² = 0.355), TMJ depressor (R² = 0.145), cervical flexor (R² = 0.144), and extensor (R² = 0.148) muscle strength. Jaw and neck pain also positively affected CJPET for flexion (R² = 0.116) and extension (R² = 0.146), as well as total sway degree (R² = 0.128) and MFIQ (R² = 0.230). Conclusions: Patients with painful TMJDs, could have impaired muscle strength and proprioception of the TMJ and cervical region. The jaw and neck pain could also affect postural stability, and the endurance of deep neck flexors as well as mandibular functions in TMJDs.

• Journal of Life Science
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• v.28 no.4
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• pp.454-464
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• 2018

Black soybeans are used as food sources as well as for traditional medicines because they contain an abundance of natural phenolic compounds. In this study, total phenolic contents (TPCs) of Korean black seed coat soybean varieties Socheongja (SCJ), Socheong 2 (SC2) and Cheongja 2 (CJ2) as well as their antioxidant capacities were investigated. Among them, TPCs were abundantly present in the order of CJ2$H_2O_2$-stimulated HaCaT human keratinocytes. Our results revealed that treatment with SCJ and SC2 prior to $H_2O_2$ exposure significantly increases the viability of HaCaT cells, indicating that the exposure of HaCaT cells to SCJ and SC2 conferred a protective effect against oxidative stress. SCJ and SC2 also effectively inhibited $H_2O_2$-induced apoptotic cell death through the blocking of mitochondrial dysfunction. SCJ and SC2 also attenuated the phosphorylation of Histone H2AX. Furthermore, they effectively induced the levels of thioredoxin reductase (TrxR) 1, a potent antioxidant enzyme, which is associated with the induction of nuclear transcription factor erythroid-2-like factor 2 (Nrf2); however, the protective effects of SCJ and SC2 were significantly reversed by Auranofin, a TrxR inhibitor. These results indicate that they have protective activity through the blocking of cellular damage related to oxidative stress via the Nrf2 signaling pathway. In conclusion, our study indicated that SCJ and SC2 might potentially serve as novel agents for the treatment and prevention of skin disorders caused by oxidative stress.

• Korean Journal of Veterinary Research
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• v.46 no.3
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• pp.225-234
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• 2006

In the present study toxicity and immunostimulating activity of the lectin(KML-C), which was extracted from Korean mistletoe(Viscum album coloratum) were investigated in swine. To determine the toxicity, lectin was injected into thigh or cervical muscles of 4-week-old piglets(Landrace) and observed clinically and pathologically. For determination of the immnunostimulating activity, lectin($0.7{\mu}g/kg$ of body weight)-adjuvanted vaccine of Aujeszky's disease virus(ADV)(NYJ1-87) which was inactivated by 0.2% formalin was injected into the cervical muscle of antibody-negative piglets in the same age group. Subpopulation of the immune cells and serum neutralizing(SN) antibodies in the piglets were examined after vaccination, and resistance of the piglets against challenge by virulent NYJ1-87 was further examined. The results were also compared with those from piglets injected with aluminum hydroxide [$Al(OH)_3$]-adjuvanted vaccine of inactivated NYJ1-87 and NYJ1-87 vaccine without adjuvant, and the results are as follows. By injection of lectin with $30{\mu}g/kg$ of body weight to the thigh muscle, all of 12 piglets died after signs such as dyspnea, fever, systemic erythema and subcutaneous hemorrhages, and lesions pertaining to poisonous hepatitis and dysfunction of kidney were observed. By injection of lectin with $7{\mu}g/kg$ of body weight to the thigh muscle, all of 12 piglets showed signs such as edema and cutaneous hemorrhage in the injected area, lameness and depression, and lesions pertaining to poisonous hepatitis and dysfunction of kidney were observed. By injection of lectin with 1, 3 and $5{\mu}g/kg$ of body weight to the thigh muscle of each one piglet, signs such as congestion, induration and grayish coloration in the injected area, depression and inappetence were observed in all piglets. Toxic changes were also observed in the liver and kidney of piglets by lectin of 3 and $5{\mu}g$. By injection of lectin with 0.5 and $0.7{\mu}g/kg$ of body weight to the cervical muscle of each 9 piglets, all piglets were clinically normal and there were no significant changes in blood counts and chemistry values. Whereas, epithelial swelling and vacuolation of convoluted tubules were observed from one piglet injected with lectin of $0.7{\mu}g$, and necrosis and fibrosis of muscular fiber were observed in the muscle of one piglet injected with lectin of $0.5{\mu}g$. Only population of sIgM+ B lymphocytes increased among immune cells in all of 15 piglets immunized with lectin($0.7{\mu}g/kg$ of body weight)-adjuvanted vaccine, while compared to those in $Al(OH)_3$-adjuvanted vaccine and vaccine without adjuvant. No additional stimulation to the immune cells was recognized when lectin was added to $Al(OH)_3$-adjuvanted vaccine. In piglets immunized with lectin-adjuvanted vaccine, SN titers in reciprocal values for loge were 1.3-4.0 at 1-4 weeks after vaccination, which was similar to those with 1.0-3.3 by vaccine without adjuvant but lower than those with 2.0-5.7 by $Al(OH)_3$-adjuvanted vaccine. Also, no additional increase in the SN titers was recognized when lectin was added to $Al(OH)_3$-adjuvanted vaccine. Piglets immunized with lectin-adjuvanted vaccine were resistant to challenge by the virulent NYJ1-87 at 4 weeks after vaccination, and the SN titers reached to 5.0 one week after challenge, which was higher than those with 4.0 by vaccine without adjuvant but somewhat lower than those with 7.7 by $Al(OH)_3$-adjuvanted vaccine.

• Journal of Nutrition and Health
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• v.56 no.6
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• pp.602-614
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• 2023

Purpose: The balance between synthesis and degradation of proteins plays a critical role in the maintenance of skeletal muscle mass. Mitochondrial dysfunction has been closely associated with skeletal muscle atrophy caused by aging, cancer, and chemotherapy. Polygalacin D is a saponin derivative isolated from Platycodon grandiflorum (Jacq.) A. DC. This study aimed to investigate the effects of polygalacin D on myoblast differentiation and muscle atrophy in association with mitochondrial function in in vitro and in zebrafish models in vivo. Methods: C2C12 myoblasts were cultured in differentiation media containing different concentrations of polygalacin D, followed by the immunostaining of the myotubes with myosin heavy chain (MHC). The mRNA expression of markers related to myogenesis, muscle atrophy, and mitochondrial function was determined by real-time quantitative reverse transcription polymerase chain reaction. Wild type AB^* zebrafish (Danio rerio) embryos were treated with 5-fluorouracil, leucovorin, and irinotecan (FOLFIRI) with or without polygalacin D, and immunostained to detect slow and fast types of muscle fibers. The Tg(Xla.Eef1a1:mitoEGFP) zebrafish expressing mitochondria-targeted green fluorescent protein was used to monitor mitochondrial morphology. Results: The exposure of C2C12 myotubes to 0.1 ng/mL of polygalacin D increased the formation of MHC-positive multinucleated myotubes (≥ 8 nuclei) compared with the control. Polygalacin D significantly increased the expression of MHC isoforms (Myh1, Myh2, Myh4, and Myh7) involved in myoblast differentiation while it decreased the expression of atrophic markers including muscle RING-finger protein-1 (MuRF1), mothers against decapentaplegic homolog (Smad)2, and Smad3. In addition, polygalacin D promoted peroxisome proliferator-activated receptor-gamma coactivator (Pgc1α) expression and reduced the level of mitochondrial fission regulators such as dynamin-1-like protein (Drp1) and mitochondrial fission 1 (Fis1). In a zebrafish model of FOLFIRI-induced muscle atrophy, polygalacin D improved not only mitochondrial dysfunction but also slow and fast muscle fiber atrophy. Conclusion: These results demonstrated that polygalacin D promotes myogenesis and alleviates chemotherapy-induced muscle atrophy by improving mitochondrial function. Thus, polygalacin D could be useful as nutrition support to prevent and ameliorate muscle wasting and weakness.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.869-875
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• 1997

Perimembranous ventri ular septal defects(PMVSDS) are the most common type of ventricular septal defects(VSDs) and consist morphologically of deficiency of the membranous septum and variable portions of the adjacent muscular septum. Repair of VSD has begun via a right ventriculotomy. Even with this exposure, however, it mght lead to ventricular dysfunction. Transatrial exposure of VSDs is luiown to a versatile approach to PMVSDS and even malaligunent defects can be repaired by this method. Although transatrial exposure can be improved by taking down'the atrioventricular valve at the annulus, surgeons have been hesitant to do so because of concern for valvular competence. Therefore, this study was undertaken to clarity the effects of transamlular approach of tricuspid valve (TATV) at operation of PMVSD. During last 5 years, twenty eight cases from 96 patients of PMVSD were closed by TATV and follow up study was done from 3 months to 33 months and results were obtained as follows. 1. Age at operation was fr m 4 months to 38 years and most patients(17, 62%) were above 5 years. 2. Preoperative pulmonary-systemic flow ratio(QPIQS) was ranged from 1 to 2.8 and 22 patients(79%) were less than 2. 3. Peak systolic pulmonary artery pressure was below 30mmHg in 8, 30-50mmHg in 17, above 50mmHg in 3 patients and 25 patients(89%) were less than 50mmHg. 4. Preoperative tricuspid regurgitation(TR) is none in 12, trivial in 6, mild in 3, moderate in 5, severe in 2 patients but postoperative TR was none in 18, trivial in 6, mild in 4 patients, so TR in most patients had decreased or not. 5. Indications for operation were based on the presence of a significant shunt. However, in patients with small shunts, indications for operation were included additional factors, tricuspid valve pouch, RVOT obstruction(right ventricular outflow tract obstruction), subacute bacterial endocarditis and associated anomalies. 6. There were no hospital deaths and residual shunts in postoperative echocardiography. Therefore TATV is especially a good method in PMVSn where patients have trcuspid valve pouch. And it is a safe and effective technique that improves exposure for PMVSD repair and does not adversely affect tricuspid valvular competence.

• Journal of Life Science
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• v.29 no.2
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• pp.215-222
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• 2019

Muscle dysfunction may arise from skeletal muscle atrophy caused by aging, injury, oxidative stress, and hereditary disease. Powdered heat-killed Enterococcus faecalis (EF-2001) has anti-allergy, anti-inflammatory, and anti-tumor effects. However, its antioxidant and anti-atrophy effects are poorly characterized. In this study, we examined the effects of EF-2001 on muscle atrophy. To determine the protective effect of EF-2001 on oxidative stress, C2C12 myoblasts were treated with $H_2O_2$ to induce oxidative stress. This induced cell damage, which was reduced by treatment with EF-2001. The mechanism of EF-2001's effect was examined in response to oxidative stress. Treatment with EF-2001 reversed the expression of HSP70 and SOD1 proteins. Also, mRNA levels of Atrogin-1/MAFbx and MuRF1 increased under oxidative stress conditions but decreased following EF-2001 treatment. To evaluate muscle volume, two and three dimensional models of the muscles were analyzed using micro-CT. As expected, muscle volume decreased after sciatic denervation and recovered after oral administration of EF-2001. Therefore, EF-2001 is a candidate for the treatment of muscular atrophy, and future discovery of the additional effects of EF-2001 may yield further applications as a functional food with useful activities in various fields.