Background: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by abnormal dilation of the trachea and main bronchi. MKS can be easily missed on chest X-rays, making diagnosis difficult. Under general anesthesia, challenges such as airway leakage or collapse during mechanical ventilation may prevent the achievement of adequate tidal volumes in patients with MKS.Case: A 94-year-old woman requiring emergency hemiarthroplasty of the hip under general anesthesia was admitted. Preoperative chest X-rays revealed dilation of the trachea and main bronchi, but the patient exhibited no respiratory symptoms. We diagnosed her with MKS and opted for an 8.0-mm-inner-diameter reinforced tracheal tube. We positioned the cuff in the subglottic area, inflating it while monitoring for air leakage. Throughout the surgery, we ensured that adequate air leak and tidal volume were maintained. Conclusions Anesthesiologists must conduct a comprehensive evaluation of patients with MKS, including a review of chest radiographs, and establish a meticulous anesthesia plan prior to surgery.

Objective: The Scales for Outcomes in Parkinson’s Disease–Cognition (SCOPA-Cog) was developed to assess cognition in patients with Parkinson’s disease (PD). In this study, we aimed to evaluate the validity and reliability of the Korean version of the SCOPACog (K-SCOPA-Cog). Methods: We enrolled 129 PD patients with movement disorders from 31 clinics in South Korea. The original version of the SCOPA-Cog was translated into Korean using the translation-retranslation method. The test–retest method with an intraclass correlation coefficient (ICC) and Cronbach’s alpha coefficient were used to assess reliability. Spearman’s rank correlation analysis with the Montreal Cognitive Assessment-Korean version (MOCA-K) and the Korean Mini-Mental State Examination (K-MMSE) were used to assess concurrent validity. Results: The Cronbach’s alpha coefficient was 0.797, and the ICC was 0.887. Spearman’s rank correlation analysis revealed a significant correlation with the K-MMSE and MOCA-K scores (r = 0.546 and r = 0.683, respectively). Conclusion Our results demonstrate that the K-SCOPA-Cog has good reliability and validity.

Voglibose, an α-glucosidase inhibitor, inhibits breakdown of complex carbohydrates into simple sugar units in intestine. Studies showed that voglibose metabolism in the liver might be negligible due to its poor intestinal absorption. Numerous microorganisms live in intestine and have several roles in metabolism and detoxification of various xenobiotics. Due to the limited information, the possible metabolism of voglibose by intestinal microbiota was investigated in vitro and in vivo.

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.

BACKGROUND: AND PURPOSE: This study aimed to determine the clinimetric properties of the Korean version of Parkinson's Disease Sleep Scale-2 (K-PDSS-2) and whether distinct subtypes of sleep disturbance can be empirically identified in patients with Parkinson's disease (PD) using the cross-culturally validated K-PDSS-2. METHODS: The internal consistency, test–retest reliability, scale precision, and convergent validity of K-PDSS-2 were assessed in a nationwide, multicenter study of 122 patients with PD. Latent class analysis (LCA) was used to derive subgroups of patients who experienced similar patterns of sleep-related problems and nocturnal disabilities. RESULTS: The total K-PDSS-2 score was 11.67±9.87 (mean±standard deviation) at baseline and 12.61±11.17 at the retest. Cronbach's α coefficients of the total K-PDSS-2 scores at baseline and follow-up were 0.851 and 0.880, respectively. The intraclass correlation coefficients over the 2-week study period ranged from 0.672 to 0.848. The total K-PDSS-2 score was strongly correlated with health-related quality of life measures and other corresponding nonmotor scales. LCA revealed three distinct subtypes of sleep disturbance in the study patients: “less-troubled sleepers,”“PD-related nocturnal difficulties,” and “disturbed sleepers.” CONCLUSIONS K-PDSS-2 showed good clinimetric attributes in accordance with previous studies that employed the original version of the PDSS-2, therefore confirming the cross-cultural usefulness of the scale. This study has further documented the first application of an LCA approach for identifying subtypes of sleep disturbance in patients with PD.

PURPOSE: To analyze the growth response to growth hormone (GH) therapy in prepubertal patients with Noonan syndrome (NS) harboring different genetic mutations. METHODS: Twenty-three patients with prepubertal NS treated at Pusan National University Children’s Hospital between March 2009 and July 2017 were enrolled. According to the disease-causing genes identified, the patients with NS were divided into 4 groups. Three groups were positive for mutations of the PTPN11, RAF1, and SOS1 genes. The five genes undetected (FGU) group was negative for PTPN11, RAF1, SOS1, KRAS, and BRAF gene mutations. The influence of genotype was retrospectively analyzed by comparing the growth parameters after GH therapy. RESULTS: The mean chronological age at the start of GH treatment was 5.85±2.67 years. At the beginning of the GH treatment, the height standard deviation score (SDS), growth velocity (GV), and lower levels of insulin-like growth factor-1 (IGF)-1 levels were not statistically different among the groups. All the 23 NS patients had significantly increased height SDS and serum IGF-1 level during the 3 years of treatment. GV was highest during the first year of treatment. During the 3 years of GH therapy, the PTPN11, RAF1, and SOS1 groups showed less improvement in height SDS, IGF-1 SDS, and GV, and less increase in bone age-to-chronological age ratio than the FGU group. CONCLUSION: The 3-year GH therapy in the 23 prepubertal patients with NS was effective in improving height SDS, GV, and serum IGF-1 levels. The FGU group showed a better response to recombinant human GH therapy than the PTPN11, RAF1, and SOS1 groups.
Busan ; Child ; Genotype ; Growth Hormone ; Humans ; Insulin-Like Growth Factor I ; Noonan Syndrome ; Retrospective Studies

PURPOSE: In our previous study, we demonstrated that both titrated extract of Centella asiatica (TECA) and astaxanthin (AST) have anti-inflammatory effects in a 5% phthalic anhydride (PA) mouse model of atopic dermatitis (AD). The increasing prevalence of AD demands new therapeutic approaches for treating the disease. We investigated the therapeutic efficacy of the ointment form of TECA, AST and a TECA + AST combination in a mouse model of AD to see whether a combination of the reduced doses of 2 compounds could have a synergistic effect. METHODS: An AD-like lesion was induced by the topical application of 5% PA to the dorsal ear and back skin of an Hos:HR-1 mouse. After AD induction, TECA (0.5%), AST (0.5%) and the TECA (0.25%) + AST (0.25%) combination ointment (20 μg/cm2) were spread on the dorsum of the ear or back skin 3 times a week for 4 weeks. We evaluated dermatitis severity, histopathological changes and changes in protein expression by Western blotting for inducible nitric oxide synthase (iNOS), cyclocxygenase (COX)-2, and nuclear factor (NF)-κB activity. We also measured the concentrations of tumor necrosis factor (TNF)-α, interleukin (IL)-6 and immunoglobulin E (IgE) in the blood of AD mice by enzyme-linked immunosorbent assay (ELISA). RESULTS: PA-induced skin morphological changes and ear thickness were significantly reduced by TECA, AST and TECA + AST treatments, but these inhibiting effects were more pronounced in the TECA + AST treatment. TECA, AST and the TECA+AST reatments inhibited the expression of iNOS and COX-2; NF-κB activity; and the release of TNF-α, IL-6 and IgE. However, the TECA+AST treatment showed additive or synergistic effects on AD. CONCLUSIONS: Our results demonstrate that the combination of TECA and AST could be a promising therapeutic agent for AD by inhibiting NF-κB signaling.
Animals ; Blotting, Western ; Centella ; Dermatitis ; Dermatitis, Atopic ; Ear ; Enzyme-Linked Immunosorbent Assay ; Immunoglobulin E ; Immunoglobulins ; Inflammation ; Interleukin-6 ; Interleukins ; Mice ; Nitric Oxide Synthase Type II ; Prevalence ; Skin ; Tumor Necrosis Factor-alpha

PURPOSE: To explore trends of women's health in nursing research by analyzing articles on women aged 13 years or older that were published in Korean Journal for Women Health Nursing from 2010 to 2015. METHODS: Seven focus areas were identified and modified to reflect integrative conceptual models of women's health: maternity care, menstrual concerns, health problems in women, gender influences on health risks, social influences on women's health, women and health care policy, and sexual health and violence against women. A total of 383 studies were analyzed according to these seven focus areas. RESULTS: Health problems in women, maternity care, and societal influences on women's health were the most widely studied topics in Korean women's health. There was increased attention to societal influences on women's health and gender influences on health risk. However, these areas are still limited in nursing research. Only 1% of these studies were in area of women's health policy. CONCLUSION: More studies in area of sexual health and violence against women are needed. Studies in area of women and health care policy are also needed to improve women's health in Korea.
Delivery of Health Care ; Female ; Humans ; Korea ; Nursing Research ; Nursing* ; Reproductive Health ; Violence ; Women's Health*

PURPOSE: Overgrowth syndromes are conditions that involve generalized or localized areas of excess growth. In this study, the clinical, molecular, and genetic characteristics of Korean patients with overgrowth syndrome were analyzed. MATERIALS AND METHODS: We recruited 13 patients who presented with overgrowth syndrome. All patients fulfilled inclusion criteria of overgrowth syndrome. Analysis of the clinical and molecular investigations of patients with overgrowth syndrome was performed retrospectively. RESULTS: Among the 13 patients with overgrowth syndrome, 9 patients (69.2%) were found to have molecular and genetic causes. Among the seven patients with Sotos syndrome (SS), two had a 5q35microdeletion that was confirmed by fluorescent in situ hybridization. In two patients with SS, intragenic mutations including a novel mutation, c.5993T>A (p.M1998L), were found by Sanger sequencing. One patient had one copy deletion of NDS1 gene which was confirmed by multiplex ligation-dependent probe amplification. Among five patients with Beckwith-Wiedemann syndrome, three had aberrant imprinting control regions; 2 hypermethylation of the differentially methylated region of H19, 1 hypomethylation of the differentially methylated region of Kv. In one patient displaying overlapping clinical features of SS, a de novo heterozygous deletion in the chromosomal region 7q22.1-22.3 was found by single nucleotide polymorphism-based microarray. CONCLUSION: Considering high detection rate of molecular and genetic abnormalities in this study, rigorous investigations of overgrowth syndrome may be an important tool for the early diagnosis and genetic counseling. A detailed molecular analysis of the rearranged regions may supply the clues for the identification of genes involved in growth regulation.
Beckwith-Wiedemann Syndrome ; Early Diagnosis ; Genetic Counseling ; Humans ; In Situ Hybridization, Fluorescence ; Multiplex Polymerase Chain Reaction ; Retrospective Studies ; Sotos Syndrome

OBJECTIVES: This study was conducted to assess the current management status of the vestibular function test laboratories in Korea. METHODS: Questionnaire about the management status of the vestibular function test laboratories was sent by email to the entire members of the Korean Balance Society. The contents of questionnaire included situation of employees who perform the tests, the types of vestibular function tests and equipment, frequency of the test and types of dizziness related questionnaires. RESULTS: Forty-nine hospitals and clinics responded. All the 49 respondents answered that they have videonystagmography. Spontaneous nystagmus analysis by videonystagmogrphy was the most frequently tests for patients with dizziness. Questionnaires for dizziness were used by 27 respondents (55.1%) for initial evaluation of the dizziness patients. The Korean version of dizziness handicap inventory was the most frequently used dizziness related questionnaire. CONCLUSIONS: We analyzed the current management status of vestibular function test laboratories to comprehend the present condition of the vestibular function test. We think that these results will help to provide a standard for laboratory operations and prepare for the education, focusing on high-demand tests.
Dizziness ; Education ; Electronic Mail ; Humans ; Korea ; Surveys and Questionnaires ; Vertigo ; Vestibular Function Tests