Gaucher disease is a glycosphingolipid storage disease caused by deficiency of glucocerebrosidase, resulting in the accumulation of glucosylceramide in lysosomes. The neuronopathic forms of this disease are associated with neuronal loss and neurodegeneration. However, the pathophysiological mechanisms leading to prenatal and neonatal death remain uncharacterized. To investigate brain dysfunction in Gaucher disease, we studied the effects of neurotrophic factors during development in a mouse model of Gaucher disease. The expression of brain-derived neurotrophic factor and nerve growth factor was reduced in the cerebral cortex, brainstem, and cerebellum of Gaucher mice, compared with that in wild-type mice. Extracellular signal-regulated kinase (ERK) 1/2 expression was downregulated in neurons from Gaucher mice and correlated with a decreased number of neurons. These results suggest that a reduction in neurotrophic factors could be involved in neuronal loss in Gaucher disease.
Signal Transduction ; Neurons/*metabolism ; Nerve Growth Factors/*metabolism ; Models, Animal ; Mice, Inbred C57BL ; Mice ; MAP Kinase Signaling System/physiology ; Gaucher Disease/*metabolism/pathology ; Down-Regulation ; Cells, Cultured ; Cell Survival ; Brain/*metabolism ; Animals

BACKGROUND AND OBJECTIVES: Ten to twenty percent of children with Kawasaki disease (KD) do not respond to initial intravenous immunoglobulin (IVIG) treatment. If untreated, approximately 15% to 25% of KD patients have complications. The aim of this study was to find useful predictors of responsiveness to initial IVIG treatment in KD. SUBJECTS AND METHODS: We retrospectively reviewed medical records of 91 children diagnosed with KD at Myong Ji Hospital from March 2012 to April 2014. Before and after (24 hours to 36 hours) IVIG treatment, the following laboratory data were obtained: hemoglobin (Hb) level, white blood cell count, proportion of neutrophil, lymphocyte and eosinophil, platelet count, erythrocyte sedimentation rate (ERS), C-reactive protein (CRP), creatine kinase (CK), creatine kinase MB (CK-MB), and N-terminal pro-brain natriuretic peptide (NT-proBNP). Subjects were then divided into two groups: IVIG-responsive or IVIG-resistant. RESULTS: Of 91 patients, 11 (12%) required retreatment. By univariate analysis, before-IVIG laboratory parameters of white blood cell count, % neutrophil, ERS, CRP, sodium, CK, CK-MB, and NT-proBNP were significantly different between IVIG-responsive and IVIG-resistant patient groups. In the after-IVIG laboratory parameters, Hb level, white blood cell count, % neutrophil, % lymphocyte, CRP, CK, CK-MB, and NT-pro-BNP were significantly different between the two groups. While the mean-differences were not statistically significant, fractional change (FC)-CRP and FC-% neutrophil showed significant difference. By multivariate analysis, FC-CRP was confirmed to be an independent predictor for initial IVIG resistance. CONCLUSION: Fractional change-C-reactive protein might be a useful and important value for predicting initial IVIG resistance in KD patients.
Blood Sedimentation ; C-Reactive Protein ; Child ; Creatine Kinase ; Eosinophils ; Humans ; Immunization, Passive* ; Immunoglobulins ; Immunoglobulins, Intravenous ; Leukocyte Count ; Lymphocytes ; Medical Records ; Mucocutaneous Lymph Node Syndrome* ; Multivariate Analysis ; Neutrophils ; Platelet Count ; Retreatment ; Retrospective Studies ; Risk Factors ; Sodium

Diphyllobothrium latum and Diphyllobothrium nihonkaiense are morphologically similar to each other, and only genetic method can differentiate clearly between the 2 species. A strobila of diphyllobothriid tapeworm discharged from a 7-year-old boy was analyzed to identify the species by mitochondrial cytochrome c oxidase subunit 1 (cox1) gene sequencing. He and his family (total 4 persons) ate slices of 3 kinds of raw fish 16 days before visiting our outpatient clinic. All family members complained of abdominal pain and watery diarrhea. They all expelled tapeworm strobilae in their stools. They were treated with a single oral dose of praziquantel and then complained of no more symptoms. The cox1 gene sequencing of the strobila from the boy revealed 99.9% (687/688 bp) similarity with D. nihonkaiense and only 93.2% (641/688 bp) similarity with D. latum. Thus, we assigned this tapeworm as D. nihonkaiense. This is the first report of D. nihonkaiense infection in a family in Korea, and this report includes the 8th pediatric case in Korea. The current report is meaningful because D. nihonkaiense infection within a family is rare.
Animals ; Anthelmintics/therapeutic use ; Child ; Diphyllobothriasis/*diagnosis/drug therapy/*parasitology ; Diphyllobothrium/*classification/enzymology/genetics/*isolation & purification ; Electron Transport Complex IV/genetics ; *Family Health ; Humans ; Korea ; Male ; Praziquantel/therapeutic use ; Sequence Analysis, DNA ; Treatment Outcome

Diphyllobothrium latum and Diphyllobothrium nihonkaiense are morphologically similar to each other, and only genetic method can differentiate clearly between the 2 species. A strobila of diphyllobothriid tapeworm discharged from a 7-year-old boy was analyzed to identify the species by mitochondrial cytochrome c oxidase subunit 1 (cox1) gene sequencing. He and his family (total 4 persons) ate slices of 3 kinds of raw fish 16 days before visiting our outpatient clinic. All family members complained of abdominal pain and watery diarrhea. They all expelled tapeworm strobilae in their stools. They were treated with a single oral dose of praziquantel and then complained of no more symptoms. The cox1 gene sequencing of the strobila from the boy revealed 99.9% (687/688 bp) similarity with D. nihonkaiense and only 93.2% (641/688 bp) similarity with D. latum. Thus, we assigned this tapeworm as D. nihonkaiense. This is the first report of D. nihonkaiense infection in a family in Korea, and this report includes the 8th pediatric case in Korea. The current report is meaningful because D. nihonkaiense infection within a family is rare.
Animals ; Anthelmintics/therapeutic use ; Child ; Diphyllobothriasis/*diagnosis/drug therapy/*parasitology ; Diphyllobothrium/*classification/enzymology/genetics/*isolation & purification ; Electron Transport Complex IV/genetics ; *Family Health ; Humans ; Korea ; Male ; Praziquantel/therapeutic use ; Sequence Analysis, DNA ; Treatment Outcome

Objectives: This study aims to define the role of the local dental clinic for people with disabilities and the appropriate role of the dental hygienist to resolve oral health inequality with people with disabilities. Methods: From September 12, 2022 to November 11, 2022, 16 experts who voluntarily participated in the research, among the experts recommended by the related institutes, were selected through stratification analysis. The results of this study and improvement plans are as follows. Results: First, as a result of measuring the importance of the role of a regional oral care center for people with disabilities in the analytic hierarchy process (AHP) middle category, check-up was found to be the most important (0.364, C.I=0.01). For examination, a facility visit checkup and regular checkups were required. Second, as a result of measuring the importance of the role of a dedicated dental hygienist in the AHP middle category, the institutional-linked activity was found to be the most important (0.417, C.I=0.02). As for institutional-linked activities, linkage with local community organizations, patient management, and public relations activities were required. Conclusions Considering these results, the role of a regional oral care center for people with disabilities is to perform examinations and preventive treatments, primary treatments (simple), education and counseling, and establish a smooth medical return system to serve as a base for dental treatment for people with disabilities. Education and counseling are provided through professional patient response and the arrangement of a dedicated dental hygienist, and oral health care is provided to people with disabilities through professional treatment assistance for patients and treatment support and screening, linkage with local community institutions, and patient management. Specialized dental hygienists should play a role in supporting the operation of oral health care for people with disabilities.

The elderly population with dementia is rapidly growing in South Korea. The aim of this study was to investigate the relationship between dementia and oral health in 197 subjects aged ≥65 years. The questionnaire included questions on subjective health status, subjective health concern, subjective oral health status and behavior, mastication ability, Oral Health Impact Profile-14 (OHIP-14), and Korean Dementia Screening Questionnaires (KDSQ). Oral examination was conducted by a single dentist to evaluate upper or lower denture use, and determine the numbers of remaining and functioning teeth, including implant and fixed prosthesis. The subjects who required a dementia test (KDSQ-C [cognition] of ≥6) had significant differences in systemic disease prevalence rate, subjective health status, subjective health concern, KDSQ-V (vascular disease) score, KDSQ-D (depression) score, subjective oral treatment need, key food mastication ability and OHIP-14 score compared to the healthy subjects. The proportion of denture wearers, total remaining teeth, total functioning teeth, toothbrushing frequency, oral pain severity, symptoms of periodontal disease, subjective oral health status, and subjective oral health concern showed no significant differences between the two groups. KDSQ-C and OHIP-14 scores showed a strong positive relationship, while KDSQ-C score and total remaining teeth or key food mastication ability showed a weak negative relationship. In the multiple regression analysis, the KDSQ-D, KDSQ-V, and OHIP-14 scores influenced the KDSQ-C scores. We suggest a relationship between oral health and cognitive impairment.
Aged* ; Cognition Disorders ; Dementia* ; Dental Restoration Wear ; Dentists ; Dentures ; Depression ; Diagnosis, Oral ; Diagnostic Self Evaluation ; Healthy Volunteers ; Humans ; Korea ; Mass Screening ; Mastication ; Oral Health* ; Periodontal Diseases ; Prevalence ; Prostheses and Implants ; Quality of Life ; Tooth ; Toothbrushing

The objective of this study was to analyze the influence of socio-demographic factors on food consumption frequencies among adolescents in Korea. Data were obtained from the Seventh Korea Youth Risk Behavior Web-based Survey (2011 KYRBS), a nationwide representative sample of 75,643 (37,873 males and 37,770 females) middle and high school students. It was carried out as a self-administered on-line survey. The frequency of eating breakfast was 4.8 times per week for middle school students and 4.6 times per week for high school students (p < 0.001). Higher levels of perceived household economic status, family affluence scale (FAS) and education attainment of mother were associated with more frequent breakfast eating. The frequencies of consumption of vegetables and milk were higher in males than in females (p < 0.001). The frequency of consumption fruits was higher in females than in males (p < 0.01). Higher levels of perceived household economic status, FAS and education attainment of mother were associated with more frequent consumption of vegetable, fruits and milk. The frequencies of consumption of soda, fast food and instant noodls were higher in males than in females (p < 0.001). The frequency of consumption of snacks was higher in females than in males. Adolescents with lower levels of FAS and education attainment of mother were at risk for skipping breakfast and consuming of soda, fast food and instant noodls more frequently. Whereas, adolescents with higher levels of FAS, education attainment of mother were more likely to be frequent consumers of vegetable, fruits and milk. These findings demonstrated that being high school students and belonging to lower level of socio-economic status (SES) were associated with undesirable food habits.
Adolescent ; Breakfast ; Eating ; Family Characteristics ; Fast Foods ; Female ; Food Habits ; Fruit ; Humans ; Korea ; Male ; Milk ; Mothers ; Risk-Taking ; Snacks ; Vegetables

No abstract available.
Lymphoma, Large-Cell, Anaplastic

OBJECTIVES: The aim of this study was to investigate the expression of 7 different sirtuin genes (SIRT1-SIRT7) in human dental pulp cells (HDPCs), and to determine the role of SIRTs in the odontoblastic differentiation potential of HDPCs. MATERIALS AND METHODS: HDPCs were isolated from freshly extracted third molar teeth of healthy patients and cultulred in odontoblastic differentiation inducing media. Osteocalcin (OCN) and dentin sialophosphoprotein (DSPP) expression was analyzed to evaluate the odontoblastic differentiation of HDPCs by reverse transcription-polymerase chain reaction (RT-PCR), while alizarin red staining was used for the mineralization assay. To investigate the expression of SIRTs during odontoblastic differentiation of HDPCs, real time PCR was also performed with RT-PCR. RESULTS: During the culture of HDPCs in the differentiation inducing media, OCN, and DSPP mRNA expressions were increased. Mineralized nodule formation was also increased in the 14 days culture. All seven SIRT genes were expressed during the odontogenic induction period. SIRT4 expression was increased in a time-dependent manner. CONCLUSIONS: Our study identified the expression of seven different SIRT genes in HDPCs, and revealed that SIRT4 could exert an influence on the odontoblast differentiation process. Further studies are needed to determine the effects of other SIRTs on the odontogenic potential of HDPCs.
Dental Pulp* ; Dentin ; Gene Expression* ; Humans* ; Molar, Third ; Odontoblasts* ; Osteocalcin ; Real-Time Polymerase Chain Reaction ; RNA, Messenger ; Tooth

BACKGROUND: Research into the Baumann skin type (BST) has recently expanded, with growing interest in the development of an efficient and effective skin type classification system for better understanding of this skin condition. OBJECTIVE: We aimed to identify male-specific skin type characteristics with investigation into the distribution of BST by age and region in the Korean male population and to determine the intrinsic and extrinsic factors related to skin type. METHODS: A questionnaire was administered to collect information about age, region, working behavior, drinking behavior, smoking behavior, usual habit of sun protection, medical history, and the BST which consisted of four parameters; oily (O) or dry (D), sensitive (S) or resistant (R), pigmented (P) or non-pigmented (N), and wrinkled (W) or tight (T). RESULTS: We surveyed 1,000 Korean males aged between 20 and 60 years who were divided equally by age and region. Of the total respondents, OSNW type accounted for the largest percentage and ORPW type the lowest. In terms of Baumann parameters, O type was 53.5%, S type was 56.1%, N type was 84.4% and W type was 57.5%. Several behavioral factors were found to have various relationships with the skin type. CONCLUSION: The predominant skin type in the Korean male respondents was OSNW type, and the distribution of skin types with regards to age and region was reported to be distinct. Therefore, skin care should be customized based on detailed skin types considering the various environmental factors.
Classification ; Drinking Behavior ; Humans ; Male ; Skin Care ; Skin ; Smoke ; Smoking ; Solar System ; Surveys and Questionnaires