Neurofibromatosis type 1 is the most common tumor predisposition syndrome inherited in an autosomal dominant (100% penetrance) fashion with a wide variety of expressivity. From the perspective of plastic surgery, the most significant clinical symptoms, including disfiguration, peripheral neurologic symptoms, and skeletal abnormalities, are caused by various tumors originating from the affected nerves. Surgical removal is the standard of care for these tumors. However, the outcome is frequently unsatisfactory, facilitating the search for additional therapeutic adjuvants. Current trials of molecularly targeted therapies are promising.Abbreviations: CALMs, café-au-lait macules; CNs, cutaneous neurofibromas; FDG, 18F-fluoro-deoxy-glucose; MAPK, mitogen-activated protein kinase; MPNSTs, malignant peripheral nerve sheath tumors; MRI, magnetic resonance imaging; NF1, neurofibromatosis type 1; NIH, National Institutes of Health; PET, positron emission tomography; PN, plexiform neurofibromas; TME, tumor microenvironment

Tracheal invasion is an uncommon complication of thyroid cancer, but it can cause respiratory failure. A rigid bronchoscope may be used to help relieve airway obstruction, but general anesthesia is usually required. Tracheal balloon dilatation and stent insertion can be performed without general anesthesia, but complete airway obstruction during balloon inflation may be dangerous in some patients. Additionally, placement of the stent adjacent to the vocal cords can be technically challenging. An 86-year-old female patient with tracheal invasion resulting from thyroid cancer was admitted to our hospital because of worsening dyspnea. Due to the patient's refusal of general anesthesia and the interventional radiologist's difficulty in completing endotracheal stenting, we performed endotracheal tube balloon dilatation and argon plasma coagulation. We have successfully treated tracheal obstruction in the patient with thyroid cancer by using endotracheal tube balloon inflation and a flexible bronchoscope without general anesthesia or airway obstruction during balloon inflation.
Aged, 80 and over ; Airway Obstruction ; Anesthesia, General ; Argon Plasma Coagulation ; Bronchoscopes ; Bronchoscopy ; Dilatation ; Disulfiram ; Dyspnea ; Female ; Humans ; Inflation, Economic* ; Intubation, Intratracheal ; Neoplasm Invasiveness ; Respiratory Insufficiency ; Stents ; Thyroid Neoplasms* ; Trachea ; Vocal Cords

Rhythm control therapy is used in atrial fibrillation (AF) management to improve AF-related symptoms along with rate control. AF catheter ablation is effective in maintaining sinus rhythm and has an acceptable complication rate. Compared with antiarrhythmic drugs, AF catheter ablation is superior with respect to arrhythmia-free survival and improvement in the quality of life. Therefore, AF ablation is recommended for rhythm control after the failure of antiarrhythmic drugs and is sometimes considered a first-line therapy for AF patients. Radiofrequency and cryoballoon ablation show similar efficacy, with slightly different complication profiles. Surgery for AF is also an effective rhythm control therapy and should be considered in patients undergoing cardiac surgery or in those with failed catheter ablation. For patients undergoing AF catheter ablation, performing ablation under uninterrupted warfarin or non-vitamin K oral anticoagulant treatment is recommended for periprocedural stroke risk management. Here, we review existing data and discuss the general principles of AF catheter and surgical ablation in patients with AF.

Tube thoracostomy is known to cause complications such as bleeding or infection, but the incidence of chylothorax secondary to tube thoracostomy is under-reported, and therefore, we report this case. A patient was diagnosed as systemic lupus erythematosus with pleural and pericardial involvement. During repeated therapeutic thoracentesis, which were performed because of poor response to steroids and cylophosphamide, hemothorax developed and we therefore inserted a chest tube. The pleural effusion changed from red to milky color in several hours and we diagnosed the pleural effusion as chylothorax. Total parenteral nutrition based on medium-chain triglycerides was supplied to this patient and chylothorax was improved after 4 days.
Chest Tubes ; Chylothorax ; Hemorrhage ; Hemothorax ; Humans ; Incidence ; Lupus Erythematosus, Systemic ; Parenteral Nutrition, Total ; Pleural Effusion ; Steroids ; Thoracostomy ; Triglycerides

BACKGROUND AND OBJECTIVES: Angiotensin-II receptor blockers (ARBs) are known to reduce the development of atrial fibrillation (AF) through reverse-remodeling. However, the effect of ARBs on thrombogenicity in AF remains unknown. MATERIALS AND METHODS: Twelve dogs were assigned to control (n=4), ARB (candesartan cilexitil 10 mg/kg/day p.o., 12 weeks; n=4), or sham (n=4) groups. Sustained AF was induced by rapid atrial pacing. Both arterial and venous serum levels of tissue inhibitor of matrix metalloproteinase-1, von Willebrand factor, P-selectin, and vascular cell adhesion molecule-1 (VCAM-1) were measured at baseline and during AF (0, 4, and 12 weeks) with enzyme-linked immunosorbent assay. Biopsies from both atria including the appendages were performed to semi-quantitatively assess endocardial and myocardial fibrosis after 12 weeks. RESULTS: The serum levels of bio-markers were not significantly different at baseline or during AF between the control and the candesartan groups. The levels were not significantly different over time, but there was a trend toward a decrease in arterial VCAM-1 from 4 to 12 weeks in the candesartan group compared to the control group. The grades of endocardial fibrosis after 12 weeks but not those of myocardial fibrosis were slightly reduced in the candesartan group compared to the control group. CONCLUSION: This study did not show that the ARB candesartan significantly reverses thrombogenicity or fibrosis during AF. Future studies using a larger number of subjects are warranted to determine the therapeutic effect of renin-angiotensin-aldosterone system blockade on prothrombogenic processes in AF.
Angiotensin II ; Animals ; Atrial Fibrillation* ; Biomarkers ; Biopsy ; Dogs ; Enzyme-Linked Immunosorbent Assay ; Fibrosis ; Matrix Metalloproteinase 1 ; P-Selectin ; Renin-Angiotensin System ; Thromboembolism ; Vascular Cell Adhesion Molecule-1 ; von Willebrand Factor

Candida species is indigenous fungus of healthy individuals, and frequently found in sputum culture. Candida isolation from the respiratory tract is not generally considered as a marker of lung infection, and definitive diagnosis of Candida pneumonia is confirmed by tissue biopsy. A few cases of Candida pneumonia pathologically confirmed by transthoracic needle aspiration of mycetoma have been reported. In Korea, a case of Candida pneumonia diagnosed by bronchial washing and blood culture was reported, but there is no case report diagnosed by biopsy. We report a case of Candida pneumonia diagnosed by endobronchial biopsy, and antifungal therapy resulted in successful resolution of the pneumonia.
Biopsy* ; Candida albicans* ; Candida* ; Diagnosis ; Fungi ; Korea ; Lung ; Mycetoma ; Needles ; Pneumonia* ; Respiratory System ; Sputum

BACKGROUND AND OBJECTIVES: Individuals with similar degrees of hearing impairment frequently show significantly different speech understanding in noise stimulation. A Korean version of the Hearing in Noise Test (KHINT) has been developed to assess the ability to recognize speech in noise. However, sentences in KHINT have been designed for subjects older than 16 years old and there have been no reports on KHINT related to the effect of age in the children younger than that age. The objective of this study was to evaluate the result of KHINT in children. SUBJECTS AND METHOD: One hundred one children between 7 and 16 years with no otologic history and normal hearing were included in this study. KHINT was conducted under the following four different conditions: speech in the quiet environment, with noise in the front, with noise in the right or in the left. The mean reception threshold for speech (RTS) in the quiet condition and signal to noise ratio (SNR) in the noisy condition were measured under four different conditions. RESULTS: There was no correlation between age and RTS. However, SNR with noise in the front, left and right conditions, as well as composite SNR, showed significant decrease with the decreasing age of children. CONCLUSION: According to KHINT, children showed decreased speech with decreasing age. Therefore, a separate KHINT with correlation factors to assess children's functional hearing ability in noise should be developed.
Child ; Hearing ; Hearing Loss ; Hearing Tests ; Humans ; Noise ; Signal-To-Noise Ratio

Cinnabar is the mineral with mercury in combination with sulfur, and it has been used to make charms in China and Korea. If cinnabar is overheated, mercury vapor that is extremely hazardous or sometimes fatal can be released. We experienced 5 patients of a family who were exposed to mercury vapor when they burnt charms. One of them developed severe acute respiratory failure and the patient needed mechanical ventilation and extracorporeal membrane oxygenation (ECMO). Despite treatment with cortiocosteroid, D-penicillamine, ECMO and plasmapheresis, the radiologic findings of a patient worsened and he died.
Burns ; China ; Extracorporeal Membrane Oxygenation ; Humans ; Inhalation ; Korea ; Mercury Compounds ; Penicillamine ; Plasmapheresis ; Respiration, Artificial ; Respiratory Insufficiency ; Sulfur

The choice of an adequate antithrombotic regimen for atrial fibrillation patients undergoing emergent or elective percutaneous coronary intervention (PCI) should be based on the ischemic event and on the risk of bleeding. Recent randomized controlled trials have consistently demonstrated that dual antithrombotic therapeutic regimens, using non-vitamin K anticoagulants and clopidogrel, are superior to triple or dual therapy with warfarin and aspirin. This report incorporates findings of recent notable studies to provide concrete, clinically useful details and recommendations for bleeding risk assessment and optimal antithrombotic therapeutic strategies after PCI. In addition, we introduce guidelines for antithrombotic management after structural heart disease intervention.
Anticoagulants ; Aspirin ; Atrial Fibrillation ; Heart Diseases ; Heart ; Hemorrhage ; Humans ; Percutaneous Coronary Intervention ; Risk Assessment ; Warfarin

The choice of an adequate antithrombotic regimen for atrial fibrillation patients undergoing emergent or elective percutaneous coronary intervention (PCI) should be based on the ischemic event and on the risk of bleeding. Recent randomized controlled trials have consistently demonstrated that dual antithrombotic therapeutic regimens, using non-vitamin K anticoagulants and clopidogrel, are superior to triple or dual therapy with warfarin and aspirin. This report incorporates findings of recent notable studies to provide concrete, clinically useful details and recommendations for bleeding risk assessment and optimal antithrombotic therapeutic strategies after PCI. In addition, we introduce guidelines for antithrombotic management after structural heart disease intervention.