This study aimed to investigate dental treatment patterns in pediatric patients with rare diseases at Yonsei University Dental Hospital over 3 years. Data of 786 pediatric patients diagnosed with rare diseases who received dental treatment between January 2020 and December 2022 were analyzed retrospectively. Patients were identified through electronic medical record screening for rare disease codes registered with the Korea Disease Control and Prevention Agency. Demographics, rare disease categories, and dental treatment methods and modalities were analyzed using chi-square tests, Fisher’s exact tests, t-tests, and logistic regression. Significant differences in dental treatment needs and methods were observed among categories. Patients with nervous system diseases were less likely to receive preventive or restorative treatments, such as sealants (p = 0.0062) and composite restoration (p = 0.0171), and had a higher prevalence of disabilities (p < 0.00001), leading to greater dental care challenges. Conversely, patients with congenital malformations required more extensive treatments, including stainless-steel crowns (p = 0.0118) and surgery (p = 0.0234), often under general anesthesia (p = 0.0337). The study underscores the importance of tailored dental care protocols and interdisciplinary collaboration to address the needs of pediatric patients with rare diseases, enhancing their oral health outcomes and quality of life.

This study aimed to investigate dental treatment patterns in pediatric patients with rare diseases at Yonsei University Dental Hospital over 3 years. Data of 786 pediatric patients diagnosed with rare diseases who received dental treatment between January 2020 and December 2022 were analyzed retrospectively. Patients were identified through electronic medical record screening for rare disease codes registered with the Korea Disease Control and Prevention Agency. Demographics, rare disease categories, and dental treatment methods and modalities were analyzed using chi-square tests, Fisher’s exact tests, t-tests, and logistic regression. Significant differences in dental treatment needs and methods were observed among categories. Patients with nervous system diseases were less likely to receive preventive or restorative treatments, such as sealants (p = 0.0062) and composite restoration (p = 0.0171), and had a higher prevalence of disabilities (p < 0.00001), leading to greater dental care challenges. Conversely, patients with congenital malformations required more extensive treatments, including stainless-steel crowns (p = 0.0118) and surgery (p = 0.0234), often under general anesthesia (p = 0.0337). The study underscores the importance of tailored dental care protocols and interdisciplinary collaboration to address the needs of pediatric patients with rare diseases, enhancing their oral health outcomes and quality of life.

This study aimed to investigate dental treatment patterns in pediatric patients with rare diseases at Yonsei University Dental Hospital over 3 years. Data of 786 pediatric patients diagnosed with rare diseases who received dental treatment between January 2020 and December 2022 were analyzed retrospectively. Patients were identified through electronic medical record screening for rare disease codes registered with the Korea Disease Control and Prevention Agency. Demographics, rare disease categories, and dental treatment methods and modalities were analyzed using chi-square tests, Fisher’s exact tests, t-tests, and logistic regression. Significant differences in dental treatment needs and methods were observed among categories. Patients with nervous system diseases were less likely to receive preventive or restorative treatments, such as sealants (p = 0.0062) and composite restoration (p = 0.0171), and had a higher prevalence of disabilities (p < 0.00001), leading to greater dental care challenges. Conversely, patients with congenital malformations required more extensive treatments, including stainless-steel crowns (p = 0.0118) and surgery (p = 0.0234), often under general anesthesia (p = 0.0337). The study underscores the importance of tailored dental care protocols and interdisciplinary collaboration to address the needs of pediatric patients with rare diseases, enhancing their oral health outcomes and quality of life.

Background: and PurposeImpulse-control disorder is an important nonmotor symptom of Parkinson's disease (PD) that can lead to financial and social problems, and be related to a poor quality of life. A nationwide multicenter prospective study was performed with the aim of validating the Korean Version of the Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease Rating Scale (K-QUIP-RS). Methods: The K-QUIP-RS was constructed using forward and backward translation, and pretesting of the prefinal version. PD patients on stable medical condition were recruited from 27 movement-disorder clinics. Participants were assessed using the K-QUIP-RS and evaluated for parkinsonian motor and nonmotor statuses and for PD-related quality of life using a predefined evaluation battery. The test–retest reliability of the K-QUIP-RS was assessed over an interval of 10–14 days, and correlations between the KQUIP-RS and other clinical scales were analyzed. Results: This study enrolled 136 patients. The internal consistency of the K-QUIP-RS was indicated by a Cronbach's α coefficient of 0.846, as was the test–retest reliability by a Guttman split-half coefficient of 0.808. The total K-QUIP-RS score was positively correlated with the scores for depression and motivation items on the Unified PD Rating Scale (UPDRS), Montgomery-Asberg Depression Scale, and Rapid-Eye-Movement Sleep-Behavior-Disorders Questionnaire. The total K-QUIP-RS score was also correlated with the scores on part II of the UPDRS and the PD Quality of Life-39 questionnaire, and the dopaminergic medication dose. Conclusions The K-QUIP-RS appears to be a reliable assessment tool for impulse-control and related behavioral disturbances in the Korean PD population.

Advanced hepatocellular carcinoma (HCC) with tumor thrombus extending through the hepatic veins, inferior vena cava, and right atrium (RA) is very rare. However, whether active treatments such as radiation, transcatheter arterial chemoembolization, and sorafenib in advanced HCC with RA involvement prolong survival is uncertain. We present a rare case of advanced HCC with tumor thrombus nearly occupying the entire RA that was treated with sorafenib. The patient received 400 mg sorafenib twice daily. However, her liver enzyme levels continued to increase and abdominal computed tomography showed an increase in the tumor size in the liver and RA. In the present case, active treatment with sorafenib was ineffective; thus, palliative care may be more beneficial in advanced HCC with extensive RA involvement.
Carcinoma, Hepatocellular ; Heart Atria ; Hepatic Veins ; Humans ; Liver ; Palliative Care ; Thrombosis ; Vena Cava, Inferior

BACKGROUND AND OBJECTIVES: Obstructive sleep apnea (OSA) is a common yet an under-diagnosed sleep related breathing disorder associated with many adverse health outcomes. OSA is commonly associated with cardiovascular disorders that include coronary artery disease, heart failure, hypertension, cardiac arrhythmias and stroke. The aim of this study was to investigate the physical characteristics of OSA patients as a screening test for sleep apnea associated cardiovascular disease. SUBJECTS AND METHOD: One hundred twenty-six adult OSA patients were enrolled in this study. All subjects underwent an overnight polysomnography (PSG) and were determined body mass index (BMI), neck circumstance, waist circumstance, tonsil size, etc. Cardiovascular events, such as hypertension, cardiovascular accident, myocardial infarction, and angina were analyzed. The correlation and multiple regression analysis were conducted to find influential and predictive factors of cardiovascular complications of OSA. RESULTS: PSG findings, apnea-hypopnea index (AHI), minimal oxygen saturation, and oxygen desaturation index were associated with BMI, neck circumstance, and waist circumstance. Tonsil size was also associated with AHI and oxygen desaturation index. However, PSG results were not associated with cardiovascular accidents of OSA patients. Hypertension was associated with tonsil size, angina was associated with Mallampati score and cardiovascular accident was associated with tonsil size. CONCLUSION: Although we cannot find the statistical relation between PSG results and cardiovascular disease, tonsil size and Mallampati score influenced the risk of cardiovascular accident.
Adult ; Arrhythmias, Cardiac ; Body Mass Index ; Cardiovascular Diseases ; Coronary Artery Disease ; Heart Failure ; Humans ; Hypertension ; Mass Screening ; Myocardial Infarction ; Neck ; Oxygen ; Palatine Tonsil ; Polysomnography ; Respiration ; Sleep Apnea Syndromes ; Sleep Apnea, Obstructive* ; Stroke

This study was attempted to explore the effect of laughter therapy on Hansen people's Interpersonal Relations, certify the effect, and use that as a useful means to improve Hansen people's interpersonal ability. The research method, Hansen people receiving outpatient and admission medical examination and treatment at the research institute of Korea Hansen Welfare Association located in Euiwang-si, Gyeonggi-do, and Hansen People residing at Razaro Village, and divided 60 people approving participation at research into 30 as an experimental group and 30 as a control group, and conducted a questionnaire survey. The sex was distributed to 30 males, and 30 females, and this study conducted a laughter therapy to 30 of the experimental group over 8 times in total by 60 minutes for 2 weeks, and for the collected data, analyzed changes to interpersonal relations revealed before and after conducting the laughter therapy program using IBM/SPSS 20.0 program. The major findings of this study are as follows. Verification of effects of a laughter therapy on Hansen people' interpersonal relations indicated that the interpersonal scores of the experimental group statistically came out high, so the laughter therapy is effective in improving Hansen people' interpersonal relations. {Pre-test (M = 3.21) and post-test (M = 3.79) of the results showed a statistically significant difference (p<.001)}. It clarified that the laughter therapy is effective improving males' and females' interpersonal relations, where it has a higher effect on female Hansen people than male people. {(males : pretest (M = 2.93) and post-test (M = 3.48), females : pre-test (M = 3.45) and post-test (M = 4.07}
Academies and Institutes ; Female ; Gyeonggi-do ; Humans ; Interpersonal Relations* ; Korea ; Laughter Therapy* ; Laughter* ; Leprosy* ; Male ; Outpatients ; Surveys and Questionnaires

Cardiac lymphatic system in the remodeling after acute myocardial infarction (AMI) has been overlooked. We wanted to investigate the role of bone marrow-derived endothelial progenitor cells (EPCs) and their contribution to lymphatic distribution in myocardial remodeling after AMI. Mouse (C57bl/6J) MI models were created by ligation of the left anterior descending coronary artery and were treated with phosphate buffered saline (PBS) or EPCs. Real-time RT-PCR with 2- to 4-week myocardial tissue samples revealed that lymphangiogenetic factors such as vascular endothelial growth factor (VEGF)-C (8.5 fold, P < 0.05), VEGF-D (6.1 fold, P < 0.05), Lyve-1 (15 fold, P < 0.05), and Prox-1 (11 fold, P < 0.05) were expressed at significantly higher levels in the PBS group than the EPC group. The PBS group also showed a significantly higher density of lymphatic vessels in the peri-infarction area. Echocardiography showed that from 2 weeks after the treatment, left ventricle (LV) dimensions at both systole and diastole were significantly smaller in the EPC group than in the PBS group (P < 0.01) and LV fractional shortening was higher in the EPC group accordingly (P < 0.01). Lymphangiogenic markers increased in a mouse MI model. EPC transplantation decreased lymphangiogenesis and adverse ventricular remodeling after AMI. These novel findings suggest that new lymphatic vessels may be formed in severely damaged myocardium, and may be involved in adverse myocardial remodeling after AMI.
Animals ; Cell Transplantation ; Endothelial Cells/*cytology ; Homeodomain Proteins/genetics/metabolism ; Immunohistochemistry ; Lymphangiogenesis/genetics/*physiology ; Mice ; Mice, Inbred C57BL ; Mice, Transgenic ; Myocardial Infarction/metabolism/physiopathology/*therapy ; Real-Time Polymerase Chain Reaction ; *Stem Cell Transplantation ; Tumor Suppressor Proteins/genetics/metabolism ; Vascular Endothelial Growth Factor A/genetics/metabolism ; Vascular Endothelial Growth Factor D/genetics/metabolism

BACKGROUND AND OBJECTIVES: It was reported that atorvastatin co-administered with clopidogrel for 8 months did not affect the anti-platelet potency of clopidogrel in Korean patients with acute coronary syndrome, but not in patients with stable angina. We investigated whether co-administration of statins with clopidogrel affected the anti-platelet efficacy of clopidogrel in Korean patients with stable angina. SUBJECTS AND METHODS: This was a randomized, open-label and two-period crossover design study conducted at two centers. Two hundreds thirty three patients with stable angina scheduled for coronary stenting were randomized into two groups. In Group A, 119 patients first received atorvastatin (10 mg) followed by fluvastatin (80 mg) for 12 weeks per treatment. In Group B, 114 patients received the same treatments in reverse order. RESULTS: Baseline adenosine diphosphate (ADP, 10 micromol/L)-induced platelet aggregation was 54.4+/-9.1% in Group A and 53.8+/-9.0% in Group B (p=0.44), and significant differences were noted after each treatment period (p<0.001). Inhibition of platelet aggregation was similar between Group A and Group B at 24 hours following clopidogrel loading (29.2+/-11.0% vs. 30.4+/-12.7%; p=0.42). The two treatment least square means of 12-week ADP (10 mol/L)-induced platelet aggregation [29.50+/-0.79 {standard error (SE)}% on the atorvastatin treatment group vs. 28.16+/-0.70 (SE)% in the fluvastatin treatment group] in a 2x2 cross-over study were not significantly different (p=0.204). CONCLUSION: Statin and clopidogrel co-administration for 12 weeks is not associated with attenuated anti-platelet activity of clopidogrel in Korean patients with stable angina after coronary stenting, in support of the findings of similar studies conducted in Caucasian populations.
Acute Coronary Syndrome ; Adenosine Diphosphate ; Angina, Stable ; Cardiovascular Diseases ; Cross-Over Studies ; Cytochrome P-450 CYP3A ; Fatty Acids, Monounsaturated ; Heptanoic Acids ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Indoles ; Platelet Aggregation ; Pyrroles ; Stents ; Ticlopidine ; Atorvastatin Calcium

Hepatic portal venous gas (HPVG), a rare radiologic finding, is associated in some cases with severe or lethal conditions requiring urgent surgical intervention. Computed tomography has recently demonstrated a wider range of clinical conditions associated with HPVG, some of which are benign and do not necessarily require surgery. However, HPVG remains an ominous sign in cases of bowel ischemia or necrosis. We report on a case of massive HPVG caused by a fatal intestinal infarction, which showed rapid disease progression, eventually resulting in death due to septic shock. The HPVG in this case was huge and could therefore be confused with an air-biliarygram.
Colitis, Ischemic ; Disease Progression ; Infarction ; Ischemia ; Mesenteric Veins ; Necrosis ; Portal Vein ; Shock, Septic