Langerhans cell histiocytosis (LCH) is characterized by clonal proliferation and accumulation of abnormal dendritic (Langerhans) cells in various organs. Pulmonary involvement, although rare in children, has been reported in 20%–50% of childhood cases of multisystem LCH. Isolated pulmonary LCH in children, especially in infants, is still rarer, but should be suspected in those with cystic lung disease. We report a case of a 10-month-old boy who presented with chronic dyspnea and whose chest computed tomography (CT) scan demonstrated cystic lesions. Lung biopsy established the diagnosis of LCH; microscopy revealed a background of lymphocytes and eosinophils with kidney-shaped abnormal cells. These abnormal cells were positive for S-100, CD207 (Langerin), and CD1a on immunohistochemical staining. Chemotherapy was administered using a cytotoxic agent (vinblastine) and a steroid. After 12 weeks of induction chemotherapy, although no significant change in cyst size was noted on chest CT, clinical symptoms improved. Consolidation chemotherapy was then administered for 1 year. Thereafter, chest CT findings demonstrated a significant decrease in cyst size and a significant increase in the volume of normal lung parenchyma. Therefore, aggressive treatment of isolated pulmonary LCH in infants with severe tissue destruction and symptoms seems warranted.
Biopsy ; Child ; Consolidation Chemotherapy ; Diagnosis ; Drug Therapy ; Dyspnea ; Eosinophils ; Histiocytosis, Langerhans-Cell* ; Humans ; Induction Chemotherapy ; Infant* ; Lung ; Lung Diseases ; Lymphocytes ; Male ; Microscopy ; Thorax ; Tomography, X-Ray Computed

The homeostasis of microenvironment in central nervous system, essential for normal function, is maintained by blood-brain barrier (BBB). ZO-1 in tight junctions (TJs) plays an important role in maintaining BBB endothelial ion and solute barriers. Malfunction of BBB by reactive oxygen species has been attributed to disruption of TJs. This study examined H2 O2 effects on paracellular permeability and changes in TJ protein ZO-1 using primary culture of bovine brain microvessel endothelial cells. The BBB permeability,measured as TER, increased in a dose-and time-dependent manner when treated with H2O2 (0.01, 0.1, 1.0 mM). Cytotoxicity test revealed that H2O2 did not cause cell death below 1 mM H2 O2 within 4 hr. H2O2 caused intermittent disruption and loss of ZO-1 at tight junctions, but ZO-1 maintained steady state levels of expression. In conclusion, we report that H2O2 induces increased paracellular permeability of BBB that is accompanied with alterations in localization of ZO-1.
Blood-Brain Barrier ; Brain ; Cell Death ; Central Nervous System ; Endothelial Cells ; Homeostasis ; Microvessels ; Oxidative Stress* ; Permeability ; Reactive Oxygen Species ; Tight Junctions*

Flare phenomenon refers to increased radiotracer uptake in bones despite clinical findings showing a positive response to treatment. Flare phenomena are most often observed in patients with breast or prostate cancer. Here, we present a case of bone flare in a 54-year-old male who had advanced gastric cancer with bone metastases. After three cycles of chemotherapy, a bone scan showed increased intensity, but the patient's bone pain was alleviated and abdominal computed tomography revealed a decrease in the size of the primary mass and metastatic lymph nodes. We therefore continued chemotherapy using the same regimen, and a follow-up bone scan revealed decreased intensity. A flare phenomenon after treatment is rare in cases of gastric cancer with bone metastasis. Although flare phenomena are not common, they should be considered in patients with gastric cancer when the clinical results are inconsistent with bone-scan findings.
Breast ; Diagnostic Imaging ; Drug Therapy ; Follow-Up Studies ; Humans ; Lymph Nodes ; Male ; Middle Aged ; Neoplasm Metastasis* ; Prostatic Neoplasms ; Stomach Neoplasms*

Pulmonary vein thrombosis is a rare disease related to a lung malignancy or complication after lung surgery. Generally, it is caused by tumor invasion or localized stenosis of a vein anastomosis site after an operation. Here we report a case of pulmonary vein thrombosis that occurred in a patient with local thrombophlebitis due to lobar pneumonia.
Constriction, Pathologic ; Humans ; Lung ; Pneumonia* ; Pulmonary Veins* ; Rare Diseases ; Thrombophlebitis ; Thrombosis* ; Veins

A mixed infection of Mycobacterium abscessus subsp. abscessus (Mab) and Mycobacterium tuberculosis (MTB) in the lung is an unusual clinical manifestation and has not yet been reported. A 61-year-old woman had been treated for Mab lung disease and concomitant pneumonia, and was diagnosed with pulmonary tuberculosis (PTB). Despite both anti-PTB and anti-Mab therapy, her entire left lung was destroyed and collapsed. She underwent left pneumonectomy and received medical therapy. We were able to successfully treat her mixed infection by pneumonectomy followed by inhaled amikacin therapy. To the best of our knowledge, thus far, this is the first description of a mixed Mab and MTB lung infection.
Amikacin ; Coinfection* ; Female ; Humans ; Lung Diseases ; Lung* ; Middle Aged ; Mycobacterium tuberculosis* ; Mycobacterium* ; Pneumonectomy ; Pneumonia ; Tuberculosis, Pulmonary

Congenital cardiovascular anomalies, such as dextrocardia, persistent left superior vena cava (SVC), and pulmonary artery (PA) sling, are rare disorders. These congenital anomalies can occur alone, or coincide with other congenital malformations. In the majority of cases, congenital anomalies are detected early in life by certain signs and symptoms. A 56-year-old man with no previous medical history was admitted due to recurrent wide QRS complex tachycardia with hemodynamic collapse. A chest radiograph showed dextrocardia. After synchronized cardioversion, an electrocardiogram revealed Wolff-Parkinson-White (WPW) syndrome. Persistent left SVC, PA sling, and right tracheal bronchus were also detected by a chest computed tomography (CT) scan. He was diagnosed with paroxysmal supraventricular tachycardia (PSVT) associated with WPW syndrome, and underwent radiofrequency ablation. We reported the first case of situs solitus dextrocardia coexisting with persistent left SVC, PA sling and right tracheal bronchus presented with WPW and PSVT in a middle-aged adult. In patients with a cardiovascular anomaly, clinicians should consider thorough evaluation of possibly combined cardiovascular and airway malformations and cardiac dysrhythmia.
Adult* ; Arrhythmias, Cardiac ; Bronchi ; Catheter Ablation ; Dextrocardia ; Electric Countershock ; Electrocardiography ; Hemodynamics ; Humans ; Middle Aged ; Pulmonary Artery ; Radiography, Thoracic ; Tachycardia ; Tachycardia, Supraventricular ; Thorax ; Vena Cava, Superior ; Wolff-Parkinson-White Syndrome*

PURPOSE: The modified Yale Preoperative Anxiety Scale (mYPAS) was developed for evaluating the level of preoperative anxiety in children. The purpose of this study was to develop a Korean version of the mYPAS (K-mYPAS) and to establish its validity and reliability based on the Korean preoperative pediatric patients. METHODS: K-mYPAS was made through stringent back-translation procedure. Total enrolled 102 patients answered questionnaires of Korean version of State-Trait Anxiety Inventory for Children (K-STAIC), and were videotaped for 2 to 5 minutes before induction of anesthesia. Three observers of experienced psychiatrist, surgeon, and nurse analyzed videotape with K-mYPAS comparing to K-STAIC. The inter- and intraobservers reliability, concurrent and construct validity, sensitivity, specificity, and predictive value were analyzed. RESULTS: The value of Cronbach alpha for interobservers reliability was 0.939 and intraobserver reliability was statistically significant (P < 0.001). Concurrent and construct validity were also statistically significant (P < 0.001 and P < 0.001, respectively). Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 81.3%, 91.4%, 81.3%, 91.4%, and 88.2%, respectively. CONCLUSION: The K-mYPAS had good psychometric properties and can be used as a reliable and valid instrument for the assessment of preoperative anxiety in children.
Anesthesia ; Anxiety* ; Child ; Humans ; Psychiatry ; Psychometrics ; Reproducibility of Results* ; Sensitivity and Specificity ; Videotape Recording