Double aortic arch with an atretic left arch distal to the origin of left subclavian artery was diagnosed with multi-detector row computed tomography (MDCT) in two children with dysphagia. This rare type of complete vascular ring is clinically important because it may be confused with right aortic arch in mirror imaging. Anatomic details of this rare type of complete vascular ring demonstrated on MDCT facilitated appropriate surgical treatment.
Adolescent ; Aorta, Thoracic/*abnormalities/radiography/surgery ; Child, Preschool ; Deglutition Disorders/etiology/radiography/surgery ; Female ; Humans ; Multidetector Computed Tomography/*methods ; Subclavian Artery/*abnormalities/radiography/surgery ; Vascular Malformations/complications/*radiography/surgery ; Vascular Surgical Procedures

Malignant pleural mesothelioma(MPM) is an uncommon neoplasm which is originated from pleural mesothelial cells. The majority of MPM is associated with prior asbestos exposure. Patients often present with chest pain and dyspnea due to pleural effusion, which might be diagnosed with tuberculous pleurisy especially in Korea. MPM is well known for its poor prognosis with a median survival time of less than 12 months after diagnosis and no established standard treatment modality. We report 3 cases of MPM confirmed by video-assisted thoracoscopic biopsy first misdiagnosed as tuberculous pleurisy.
Asbestos ; Biopsy ; Chest Pain ; Diagnosis ; Dyspnea ; Humans ; Korea ; Mesothelioma* ; Pleural Effusion ; Prognosis ; Thoracoscopy ; Tuberculosis, Pleural*

PURPOSE: This study described the post-exposure prophylaxis (PEP) and secondary varicella infection in children inadvertently exposed to varicella zoster virus (VZV) in the hospital. METHODS: We retrospectively analyzed data from patients with VZV infection who were initially not properly isolated, as well as children exposed to VZV at the Seoul National University Children's Hospital between January 2010 and December 2015. The PEP measures were determined by the presence of immunity to VZV and immunocompromising conditions. Patient clinical information was reviewed via medical records. RESULTS: Among 147 children hospitalized between 2010 and 2015, 13 inadvertent exposures were notified due to VZV infection. Five index children had a history of VZV vaccination. Eighty-six children were exposed in multi-occupancy rooms and 62.8% (54/86) were immune to VZV. The PEP measures administered to 27 exposed patients included varicella zoster immunoglobulin and VZV vaccination. Four children developed secondary varicella, which was linked to a single index patient, including one child who did not receive PEP and three of the 27 children who received PEP. The rates of secondary varicella and prophylaxis failure were 4.7% (4/85) and 11.1% (3/27), respectively. The secondary varicella rates were 1.9% (1/54) and 9.7% (3/31) among immunocompetent and immunocompromised children, respectively. CONCLUSIONS: Delayed diagnosis of VZV infection can lead to unexpected exposure and place susceptible children and immunocompromised patients at risk for developing varicella. The appropriateness of the current PEP strategy based on VZV immunity may require re-evaluation.
Chickenpox* ; Child ; Child, Hospitalized* ; Delayed Diagnosis ; Herpes Zoster ; Herpesvirus 3, Human* ; Humans ; Immunocompromised Host ; Immunoglobulins ; Medical Records ; Post-Exposure Prophylaxis* ; Retrospective Studies ; Seoul ; Vaccination

Tendon and ligament (T/L) have been known to be obviously different from each other in tissue level. However, due to the overlapping gene markers, distinction in cellular level has not been clearly verified yet. Recently, the use of nuclear magnetic resonance (NMR) spectroscopy has shown the potential to detect biological markers in cellular level. Therefore, in this study we applied a non-invasive technique based on NMR spectroscopy to establish biomarkers to distinguish between T/L fibroblasts. In addition the cellular morphologies and gene expression patterns were also investigated for comparison through optical microscopy and real-time polymerase chain reaction (PCR). No difference was observed from morphology and real-time PCR results, either as expected. However, we found clear differences in their metabolomic spectra using ¹H NMR spectroscopy. The calculated integral values of fatty acids (with chemical shifts at ~0.9, 1.26, 1.59, 2.05, 2.25, and 2.81 ppm), lactate (~1.33 ppm), and leucine (~2.72 ppm) were significantly different between the two types of fibroblasts. To be specific tendon group exhibited higher level of the metabolite than ligament group. In conclusion, in-cell metabolomic evaluation by NMR technique used in this study is believed to provide a promising tool in distinguishing cell types, especially T/L cells, which cannot be classified by conventional biological assays.
Biological Assay ; Biomarkers ; Fatty Acids ; Fibroblasts* ; Gene Expression ; Genes, Overlapping ; Lactic Acid ; Leucine ; Ligaments* ; Magnetic Resonance Spectroscopy* ; Metabolomics ; Microscopy ; Real-Time Polymerase Chain Reaction ; Spectrum Analysis* ; Tendons*

PURPOSE: We evaluated the hemodynamic statuses of patients after partial closure of atrial septal defects with fenestration due to pulmonary hypertension. MATERIALS AND METHODS: Seventeen adult patients underwent partial atrial septal defect closure and follow-up cardiac catheterization. We analyzed hemodynamic data and clinical parameters before and after closure. RESULTS: The median age at closure was 29 years old. The baseline Qp/Qs was 1.9+/-0.6. The median interval from the operation to the cardiac catheterization was 27 months. The CT ratio decreased from 0.55+/-0.07 to 0.48+/-0.06 (p<0.05). The mean pulmonary arterial pressure decreased from 50.0+/-11.5 mm Hg to 32.5+/-14.4 mm Hg (p<0.05), and the pulmonary resistance index decreased from 9.2+/-3.6 Wood units*m2 to 6.3+/-3.8 Wood units*m2 (p<0.05). Eleven patients (64.7%) continued to exhibit high pulmonary resistance (over 3.0 Wood units*m2) after closure. These patients had significantly higher pulmonary resistance indices and mean pulmonary arterial pressures based on oxygen testing before the partial closures (p<0.05). However, no significant predictors of post-closure pulmonary hypertension were identified. CONCLUSION: Despite improvement in symptoms and hemodynamics after partial closure of an atrial septal defect, pulmonary hypertension should be monitored carefully.
Adult ; Aged ; Cardiac Catheterization/*adverse effects ; Female ; Follow-Up Studies ; Heart Defects, Congenital/epidemiology/*physiopathology/*surgery ; Heart Septal Defects, Atrial/surgery ; Hemodynamics/*physiology ; Humans ; Hypertension, Pulmonary/diagnosis/epidemiology/*physiopathology ; Male ; Middle Aged ; Oxygen ; Postoperative Complications/diagnosis/epidemiology/*physiopathology ; Retrospective Studies ; Treatment Outcome ; Young Adult

Wilson's disease is a rare autosomal recessive metabolic disease. The ATB7B gene mutation results in a defect of biliary copper excretion and subsequent accumulation of copper in the liver, brain, and sclera. The usual clinical signs of Wilson's disease include hepatitis, liver cirrhosis, movement disorder, or a Kayser-Fleisher ring in the sclera, but patients occasionally present with hepatic failure or hemolytic anemia. Under such metabolic conditions, free copper induce chronic hemolysis with oxidative damage via free radical production, and chronic hemolysis, in turn, can cause secondary pigment bililary stone formation. Herein we report a case of Wilson's disease associated with cholelithiasis in a young female.
Anemia, Hemolytic ; Brain ; Cholelithiasis ; Copper ; Hemolysis ; Hepatitis ; Hepatolenticular Degeneration ; Humans ; Liver ; Liver Cirrhosis ; Liver Failure ; Metabolic Diseases ; Movement Disorders ; Sclera

Purpose: This study aimed to examine the relationships between participative decision-making, psychological empowerment, and organizational citizenship behavior of nurses working in hospitals; moreover, we verified the moderated mediating effect of power distance orientation in these relationships. Methods: A total of 201 nurses working at a university hospital in South Korea responded to the survey. Self-report surveys were used to gather data from July 15-August 15, 2021. SPSS 22.0, AMOS 22.0, and SPSS PROCESS macro were used for data analysis. Results: Psychological empowerment significantly mediated the relationship between participative decision making and organizational citizenship behavior. Moreover, the moderating effect of power distance orientation on the relationship between participative decision making and psychological empowerment was also significant when the power distance orientation was at a low and average levels. However, the moderated mediating effect of power distance orientation on the relationship between participative decision making, psychological empowerment, and organizational citizenship behavior was insignificant. Conclusion Involving nurses with lower power distance orientation in decision making is crucial to elicit positive work performance and different strategies are necessary for those with higher power distance orientation. Finally, further studies are needed to understand the power distance orientation and positive work outcomes of nurses.