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Purpose: The radiographic assessment of lung edema (RALE) score enables objective quantification of lung edema and is a valuable prognostic marker of adult acute respiratory distress syndrome (ARDS). We aimed to evaluate the validity of RALE score in children with ARDS. Materials and Methods: The RALE score was measured for its reliability and correlation to other ARDS severity indices. ARDSspecific mortality was defined as death from severe pulmonary dysfunction or the need for extracorporeal membrane oxygenation therapy. The C-index of the RALE score and other ARDS severity indices were compared via survival analyses. Results: Among 296 children with ARDS, 88 did not survive, and there were 70 ARDS-specific non-survivors. The RALE score showed good reliability with an intraclass correlation coefficient of 0.809 [95% confidence interval (CI), 0.760-0.848]. In univariable analysis, the RALE score had a hazard ratio (HR) of 1.19 (95% CI, 1.18-3.11), and the significance was maintained in multivariable analysis adjusting with age, ARDS etiology, and comorbidity, with an HR of 1.77 (95% CI, 1.05-2.91). The RALE score was a good predictor of ARDS-specific mortality, with a C-index of 0.607 (95% CI, 0.519-0.695). Conclusion: The RALE score is a reliable measure for ARDS severity and a useful prognostic marker of mortality in children, especially for ARDS-specific mortality. This score provides information that clinicians can use to decide the proper time of aggressive therapy targeting severe lung injury and to appropriately manage the fluid balance of children with ARDS. [ABSTRACT FROM AUTHOR]

Background: Spirometry is an unrivalled tool for determining asthma and asthma severity. The ratio of forced expiratory volume (FEV) in 1 second (FEV1) to forced vital capacity (FVC) and the forced expiratory flow between 25% and 75% of FVC (FEF25-75) are well-known markers of airway obstruction, but they are limited by low reproducibility, particularly in children. In this study, we defined terminal expiration volume (TEV) as FEV in 3 seconds forced expiratory volume in 3 seconds (FEV3) minus forced expiratory volume in 1 seconds (FEV1) and investigate whether TEV/FEV3 can function as a coherent marker to compensate for existing markers. Methods: This retrospective study comprised 980 children ages ≤ 18 years who underwent spirometry and the bronchial provocation testing. TEV/FEV3 was compared with regard to asthma presence and severity. The findings were verified with an external validation group (n = 105). Results: FEV3 was obtained in 837 children (85.4%). TEV/FEV3 was significantly higher in patients with asthma than in patients who did not have asthma (17.1 ± 5.5 versus 12.0 ± 4.4, p < 0.001). External validation with 73 patients showed similar results (18.0 ± 5.9 in asthma versus 10.2 ± 5.1 in non-asthma, p < 0.001). The discriminatory power of TEV/FEV3 for asthma was comparable with that of FEF25-75 (p = 0.804). TEV/FEV3 significantly increased with asthma severity (mild, 16.1 ± 5.4; moderate, 17.7 ± 5.4; severe, 22.0 ± 5.3; p < 0.001). For patients who could not achieve FEV3, FEF25-75 demonstrated no significant difference between mild and moderate asthma, and could not discriminate asthma or asthma severity. Conclusion: TEV/FEV3 is a new metric that may help diagnose and determine asthma severity by using conventional spirometry by assessing small airway dysfunction. TEV/FEV3 promotes a reassessment of the reliability of other spirometric parameters, particularly in young children. Caution is needed in interpreting the result of spirometry in children who cannot achieve FEV3. [ABSTRACT FROM AUTHOR]

The article analyzes morphologic differences in facial soft tissue of subjects with bulbar amyotrophic lateral sclerosis (ALS) requiring noninvasive ventilation (NIV). An NIV mask was virtually fitted to understand how the NIV mask interface affects the face in different postures. Topics discussed include facial pressure distribution, facial deformations in different postures, and anteroposterior directional difference of the lower lip area during transition from sitting to supine.

Although noninvasive ventilation (NIV) is administered to manage respiratory failure due to various causes, safety and effectiveness issues associated with the use of NIV masks have been reported. The present article aims to provide health professionals with a comprehensive review of the ergonomic considerations of NIV masks in terms of design, evaluation, and personalization. Based on a review of 93 papers, we provide guidelines for mask selection and troubleshooting during mask use as well as ergonomic approaches including face anthropometry, sizing systems, mask design, evaluation, and personalization. The comprehensive information presented in this review provides ergonomic perspectives to identify and prevent safety and usability problems associated with the use of NIV masks. Ergonomic improvement and personalization are important goals in order to facilitate the success of NIV treatment. [ABSTRACT FROM AUTHOR]

GLUT1 deficiency is a rare neurometabolic disorder that can be effectively treated with ketogenic diet. However, this condition is underdiagnosed due to its nonspecific, overlapping, and evolving symptoms with age. We retrospectively reviewed the clinical course of nine patients diagnosed with GLUT1 deficiency, based on SLC2A1 mutations and/or glucose concentration in cerebrospinal fluid. The patients included eight boys and one girl who initially presented with seizures (44%, 4/9) or delayed development (44%, 4/9) before 2 years of age, except for one patient who presented with apnea as a neonate. Over the clinical course, all of the children developed seizures of the mixed type, including absence seizures and generalized tonic--clonic seizures. About half (56%, 5/9) showed movement disorders such as ataxia, dystonia, or dyskinesia. We observed an evolution of phenotype over time, although this was not uniform across all patients. Only one child had microcephaly. In five patients, ketogenic diet was effective in reducing seizures and movement symptoms, and the patients exhibited subjective improvement in cognitive function. Diagnosing GLUT1 deficiency can be challenging due to the phenotypic variability and evolution. A high index of clinical suspicion in pediatric and even older patients with epilepsy or movement disorders is key to the early diagnosis and treatment, which can improve the patient's quality of life. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate changes in the volume and cross-sectional area of the nasal airway before and 1 year after nonsurgical miniscrew-assisted rapid maxillary expansion (MARME) in young adults. Materials and Methods: Fourteen patients (mean age, 22.7 years; 10 women, four men) with a transverse discrepancy who underwent cone beam computed tomography before (T0), immediately after (T1), and 1 year after (T2) expansion were retrospectively included in this study. The volume of the nasal cavity and nasopharynx and the cross-sectional area of the anterior, middle, and posterior segments of the nasal airway were measured and compared among the three timepoints using paired t-tests. Results: The volume of the nasal cavity showed a significant increase at T1 and T2 (P<.05), while that of the nasopharynx increased only at T2 (P<.05). The anterior and middle cross-sectional areas significantly increased at T1 and T2 (P<.05), while the posterior cross-sectional area showed no significant change throughout the observation period (P>.05). Conclusions: The results demonstrate that the volume and cross-sectional area of the nasal cavity increased after MARME and were maintained at 1 year after expansion. Therefore, MARME may be helpful in expanding the nasal airway. [ABSTRACT FROM AUTHOR]

Objective To evaluate the effect of 3-month kale (Brassica oleracea acephala) juice supplementation on coronary artery disease risk factors among hypercholesterolemic men. Methods Thirty-two men with hypercholesterolemia (> 200 mg/dL) were recruited after annual health examinations among the faculty and staff at university. The subjects consumed 150 mL of kale juice per day for a 12-week intervention period. Dietary and anthropometric assessments were performed and blood samples were collected to evaluate biochemical profiles before and after supplementation. Results Serum concentrations of HDL-cholesterol, and HDL- to LDL-cholesterol ratio were significantly increased by 27% (P<0.0001) and 52% (P<0.0001), respectively. The LDL-cholesterol concentration and the atherogenic index were significantly reduced by 10% (P=0.0007) and 24.2% (P<0.0001), respectively without affecting body mass index, waist and hip circumferences, or nutrient intakes after three months of supplementation. While there was no difference in the concentration of malondialdehyde, significant increase in glutathione peroxidase activity (P=0.0005) were accompanied by a significant increase in the serum selenium level (P=0.0132). It was also found that the responses of these risk factors to kale juice administration were dependent on smoking status. Conclusion Regular meals supplementation with kale juice can favorably influence serum lipid profiles and antioxidant systems, and hence contribute to reduce the risks of coronary artery disease in male subjects with hyperlipidemia. [ABSTRACT FROM AUTHOR]