• Journal of The Korean Dental Society of Anesthesiology
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• v.13 no.4
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• pp.209-214
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• 2013

Multiple caries in a pediatric patient often requires dental treatment under general anesthesia, especially when the patient is suffering from a systemic disease. The patient was a 6-year-old boy with Marfan syndrome and needed extensive dental treatment. Marfan syndrome is an inherited disorder resulting from mutations in Fibrillin-1 gene. Patients are known to have mainly cardiovascular, ocular, and musculoskeletal problems. Although clinical symptoms of the syndrome are age-realted, thus hindering early diagnosis of the disease in young children, our patient had been confirmed by a gene study at a younger age. Medical history of the patient revealed moderate to severe mitral regurgitation and aortic root dilatation, which required mitral valve replacement surgery with a mechanical valve. As a result, the patient was taking warfarin post-operatively and changes in medication had to be made before the dental treatment. Also, prophyalctic antibiotics had to be given before the treatment for prevention of (to prevent the) infective endocarditis. With careful control of the medications and bleeding tendency, general anesthesia and the treatment were done successfully without any complications.

• Journal of Cardiovascular Imaging
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• v.26 no.4
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• pp.201-213
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• 2018

BACKGROUND: Children with significant adenotonsillar hypertrophy (ATH) may show right ventricular (RV) dysfunction. We aimed to evaluate RV dysfunction in such children before adenotonsillectomy by evaluating peak longitudinal right atrial (RA) strain (PLRAS) in systole. PLRAS, electrocardiogram (ECG) and conventional echocardiographic parameters were compared to distinguish children with significant ATH with sleep-related breathing disorder (ATH-SRBD) from controls. METHODS: Fifty-six children (23 controls and 33 children with ATH-SRBD without symptoms of heart failure) were retrospectively studied. Preoperative echocardiograms and ECGs of children with ATH-SRBD who underwent adenotonsillectomy were compared to those of controls. Available postoperative ECGs and echocardiograms were also analyzed. RESULTS: Preoperatively, prolonged maximum P-wave duration (Pmax) and P-wave dispersion (PWD), decreased PLRAS, and increased tricuspid annulus E/E' were found in children with ATH-SRBD compared to those of controls. From the receiver operating characteristic curves, PLRAS was not inferior compared to tricuspid annulus E/E', Pmax, and PWD in differentiating children with ATH-SRBD from controls; however, the discriminative abilities of all four parameters were poor. In children who underwent adenotonsillectomy, echocardiograms $1.2{\pm}0.4$ years after adenotonsillectomy showed no difference in postoperative PLRAS and tricuspid annulus E/E' when compared with those of the preoperative period. CONCLUSIONS: Impaired RA deformation was reflected as decreased PLRAS in children with ATH-SRBD before adenotonsillectomy. Decreased PLRAS in these children may indicate subtle RV dysfunction and increased proarrhythmic risk. However, usefulness of PLRAS as an individual parameter in differentiating preoperative children with ATH-SRBD from controls was limited, similar to those of tricuspid annulus E/E', Pmax, and PWD.

• Korean Journal of Clinical Pharmacy
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• v.31 no.1
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• pp.21-26
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• 2021

Background: Pharmacogenomics is the study of how genetic mutations in patients affect their response to drugs. Pharmacogenomic studies aim to maximize drug effects and minimize adverse drug events. The Food and Drug Administration and the European Medicine Agency published guidelines for pharmacogenetics in 2005 and 2006, respectively; the Korean Ministry of Food and Drug Safety followed suit in 2015. Methods: This study analyzed pharmacogenomic information in the Korean Ministry of Food and Drug Safety's integrated drug information system to evaluate whether domestic pharmaceutical products reflect the current research on pharmacogenomic differences. Results: In June 2020, the Korean pharmacogenomic database contained genomic data on 90 compounds. Of these, 45 compounds were classified as "Antineoplastic and immunomodulating agents." The other 45 non-antineoplastic agents were in the following categories: Anti-infectives, Mental & behavior disorder, Hormone & metabolism related diseases, Cardiovascular system, Skin & subcutaneous tissue disease, Genito-urinary system and sex hormones, Blood and blood forming organs, Nervous system, Alimentary tract and metabolism, Musculo-skeletal system, and Other conditions including the respiratory system. In addition, 30 additives unrelated to the main ingredient were associated with genetic precautions. Conclusion: This study showed that antineoplastic and immunomodulating agents accounted for half the drugs associated with pharmacogenetic information. For antitumor and immunomodulatory drugs, genomic tests were recommended depending on the indication; this was in contrast to genomic testing recommendations for non-antineoplastic medications. Genomic tests were rarely requested or recommended for non-antineoplastic medications because the relationships between genotype and efficacy among those drugs were relatively weak.

• Journal of Genetic Medicine
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• v.18 no.2
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• pp.142-146
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• 2021

Alagille syndrome (AGS) is a rare autosomal dominant inherited disorder, with major clinical manifestations of bile duct paucity, cholestasis, cardiovascular anomaly, ophthalmic abnormalities, butterfly vertebrae, and dysmorphic facial appearance. It is caused by heterozygous mutations in JAG1 or NOTCH of the Notch signaling pathway presenting with variable phenotypic penetrance and involving multiple organ systems. The following case report describes a unique case of a 16-year-old female with AGS who presented with the primary complaint of renovascular hypertension. She had a medical history of ventricular septal defect and polycystic ovary syndrome. The patient had a dysmorphic facial appearance including frontal bossing, bulbous tip of the nose, a pointed chin with prognathism, and deeply set eyes with mild hypertelorism. Stenoocclusive changes of both renal arteries, celiac artery, lower part of the abdominal aorta, and left intracranial artery, along with absence of the left internal carotid artery were found on examination. Whole exome sequencing was performed and revealed a pathologic mutation of JAG1, leading to the diagnosis of AGS. Reverse phenotyping detected butterfly vertebrae and normal structure and function of the liver and gallbladder. While the representative symptom of AGS in most scenarios is a hepatic problem, in this case, the presenting clinical features were the vascular anomalies. Clinical manifestations of AGS are diverse, and this case demonstrates that renovascular hypertension might be in some cases a presenting symptom of AGS.

• Investigative Magnetic Resonance Imaging
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• v.25 no.4
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• pp.332-337
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• 2021

Behçet's disease is a chronic inflammatory disorder involving vessels of various sizes and organs, including the skin, joints, gastrointestinal tract, lungs, and cardiovascular system. The etiology of Behçet's disease is unclear, and clinical diagnosis is important in the absence of definitive laboratory or pathological findings diagnostic of Behçet's disease. Cardiac involvement is rare but might present as endocarditis, myocarditis, pericarditis, or intracardiac thrombosis. This report presents a case of Behçet's disease involving the heart in a 22-year-old man with unusual manifestations of right ventricular fibrosis and intracardiac thrombosis. Cardiac magnetic resonance imaging revealed multiple intracardiac thrombi and delayed diffuse subendocardial enhancement involving the right ventricle. No peripheral eosinophilia was detected. Endomyocardial biopsy showed mixed inflammatory cell infiltrates. Based on the patient's clinical history of oral ulcer and arthritis, a diagnosis of Behçet's disease was made considering the clinical, radiological, and histological findings. Intracardiac thrombi and endomyocardial fibrosis are rare manifestations of Behçet's disease, and the diagnosis is often a clinical challenge. Early diagnosis is important for appropriate management. Behçet's disease should be considered in the differential diagnosis of patients with intracardiac thrombosis and endomyocardial fibrosis of the right chamber.

• Fisheries and Aquatic Sciences
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• v.26 no.6
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• pp.406-422
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• 2023

Sarcopenia is an age-related, progressive skeletal muscle disorder involving the loss of muscle mass and strength. Previous studies have shown that γ-aminobutyric acid (GABA) from fermented oysters aids in regulatory T cells (Tregs) cell expansion and function by enhancing autophagy, and concomitantly mediate muscle regeneration by modulating muscle inflammation and satellite cell function. The fermentation process of oysters not only increases the GABA content but also enhances the content of branched amino acids and free amino acids that aid the level of protein absorption and muscle strength, mass, and repair. In this study, the effect of GABA-enriched fermented sarco oyster extract (FSO) on reduced muscle mass and functions via Treg modulation and enhanced autophagy in aged mice was investigated. Results showed that FSO enhanced the expression of autophagy markers (autophagy-related gene 5 [ATG5] and GABA receptor-associated protein [GABARAP]), forkhead box protein 3 (FoxP3) expression, and levels of anti-inflammatory cytokines (interleukin [IL]-10 and transforming growth factor [TGF]-β) secreted by Tregs while reducing pro-inflammatory cytokine levels (IL-17A and interferon [IFN]-γ). Furthermore, FSO increased the expression of IL-33 and its receptor IL-1 receptor-like 1 (ST2); well-known signaling pathways that increase amphiregulin (Areg) secretion and expression of myogenesis markers (myogenic factor 5, myoblast determination protein 1, and myogenin). Muscle mass and function were also enhanced via FSO. Overall, the current study suggests that FSO increased autophagy, which enhanced Treg accumulation and function, decreased muscle inflammation, and increased satellite cell function for muscle regeneration and therefore could decrease the loss of muscle mass and function with aging.

• Clinical Nutrition Research
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• v.13 no.1
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• pp.22-32
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• 2024

Rheumatoid arthritis (RA) is a systemic inflammatory autoimmune disorder with widespread synovitis. Isoflavones, the main active component of soy, have been reported to have potent anti-inflammatory effects; the previous RA animal models showed the promising effect of soy supplementation. We aimed to evaluate the effect of soy bread on inflammatory markers and lipid profiles in RA patients. The present study was designed as a randomized controlled trial. RA patients were randomly allocated to obtain soy bread (n = 22) or placebo bread (n = 22) for 8 weeks. Fasting serum levels of lipid profile, total antioxidant capacity (TAC), tumor necrosis factor-α (TNF-α), C-reactive protein (CRP), and DAS28 were checked. Findings showed that there were no significant differences between the two groups in physical activity and dietary intake at the beginning of the study and the end of the study. There were no significant differences between the two groups in measured lipid profile markers, including high-density lipoprotein, low-density lipoprotein, total cholesterol, triglyceride, and very low-density lipoprotein, at the end of the trial. In addition, TAC and CRP also were not significant at the end of the trial between the 2 groups (0.66 and 0.12, respectively). However, the serum levels of TNF-α reduced significantly in the soy bread group at the end of the intervention (p < 0.000) and compared with the control group (p < 0.019). Soy bread consumption only decreased circulating TNF-α serum concentration. Other outcome measures were not changed following supplementation. Future long-term, well-designed studies are needed to confirm these findings.

• Korean Journal of Psychosomatic Medicine
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• v.18 no.1
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• pp.19-29
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• 2010

Objectives：Authors hypothesized that bipolar tendency of non-psychiatric person would be associated with stress vulnerability. To test the hypothesis, we compared perceived stress level, Type A Behavioral Pattern (TABP) and unhealthy lifestyle between person with and without bipolar tendency. Methods：The study cohort consisted of 1987 subjects without past and current psychiatric history. In this study, bipolar tendency was determined by MDQ response which requires endorsement of at least 7 of the 13 "yes or no" questions. We compared the scores of Perceived Stress Scale, A/B lifestyle questionnaire, and unhealthy lifestyle(alcohol, smoking, lack of exercise, irregular meal) between MDQ positive and negative respondents. Results：We identified the bipolar tendency group determined by the MDQ positive response were 329 subjects(16.6%). The overall score of the bipolar tendency group was significantly high on the A/B lifestyle questionnaire compared to the non-bipolar tendency group(125.4 vs. 115.9, p<0.001), and the rate of the subjects defined as TABP was also significantly high(41.3% vs. 30.1%, p<0.001). In comparison of subjectively perceived stress level, the bipolar tendency group was observed to be significantly higher than the non-bipolar tendency group (18.5% vs. 16.5%, p<0.001). The bipolar tendency group was significantly higher than the non-bipolar tendency group on the rate of answering：'irregular eating habit' (20.1% vs. 14.3%, p=0.002), '4-or-more-times alcohol intake per week' (29.8% vs. 16.5%, p<0.001), 'currently smoking' (41.9% vs. 23.0%, p<0.001), '2-or-less-times physical exercises per week' (63.2% vs. 55.1%, p=0.007). Conclusion：By clinical diagnosis, even a person who does not meet the criteria of bipolar disorder has a high risk of stress-related behavioral pattern, and perceived stress when possessing bipolar tendencies such as the (hypo) maniac tendency or affective instability. Such individual has significantly higher risk of having unhealthy life-style such as smoking, drinking, irregular eating habit and lack of exercise compared to non-bipolar tendency group. Since these risk factors lead to increase the risk of obesity, metabolic syndrome, diabetes, and cardiovascular diseases, the individual with the bipolar tendency requires a more effort to manage stress and to maintain healthy lifestyle.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.2
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• pp.94-98
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• 2010

Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by various ocular and extraocular malformations. The incidence of Axenfeld-Rieger syndrome has been estimated to be 1 per 200,000. The syndrome is characterized by short body stature, delayed bone age, and deficient or arrested development of neural crest cells involving the anterior chamber of the eyes, facial bones, teeth, periumbilical skin, and cardiovascular system. This is a case of a 6 year-old girl, who came to the department of the pediatric dentistry, Yonsei University Dental Hospital, for evaluation and treatment of multiple congenital missing permanent teeth. The patient presented typical dental, craniofacial, and systemic features of Axenfeld-Rieger syndrome, such as glaucoma, oval pupil, heterochromatic iris, umbilical hernia, and delayed bone age. On the panoramic view, 3 primary teeth were missing and 13 permanent tooth germs were absent. On the lateral cephalograph, underdevelopment of the maxilla and normal growth pattern of the mandible were confirmed. Periodic dental follow-up is planned for evaluation and interceptive treatment of her dental and craniofacial problems. Denture or removable space maintainer with a pontic is considered for esthetic and functional restoration. In addition, orthognathic surgery is also planned in future to prevent further midfacial skeletal deformation. Early diagnosis of Axenfeld-Rieger syndrome is very important to prevent exacerbation of complications, such as glaucoma and skeletal deformities.

• Environmental Analysis Health and Toxicology
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• v.31
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• pp.19.1-19.7
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• 2016

A large portion of the Korean population has been exposed to toxic humidifier disinfectants (HDs), and considering that the majority of the victims are infants, the magnitude of the damage is expected to be considerably larger than what has currently been revealed. The current victims are voicing problems caused by various diseases, including but not limited to lung, upper respiratory tract, cardiovascular, kidney, musculoskeletal, eye, and skin diseases, etc. However, there has been difficulty in gaining validation for these health problems and identifying causal relationships due to lack of evidence proving that toxic HD is the specific causes of extrapulmonary diseases such as allergic rhinitis. Furthermore, the victims and bereaved families of the HD case have not received any support for psychological distress such as post-traumatic stress disorder, depression, feelings of injustice, and anger caused by the trauma. In addition, because the underlying mechanisms of the toxic materials within the HDs such as polyhexamethylene guanidine phosphate, poly(oxyalkylene guanidine) hydrochloride, chloromethylisothiazolinone /methylisothiazolinone have yet to be determined, the demand for information regarding the HD issue is growing. The victims of the HD cases require support that goes beyond financial aid for medical costs and living expenses. There is a desperate need for government-led integrated support centers that provide individualized support through health screenings; in other words, we need an integrated facility that provides the appropriate social support to allow the victims to recover their physical and mental health, so as to well prepare them to return to a normal life. The implementation of such a plan requires not only the close cooperation between those departments already directly involved such as the Ministry of Environment and the Ministry of Health and Welfare, but also active support on a national scale from pan-governmental consultative bodies.