Thyroid rest is isolated deposit of normal thyroid tissue arising in the thyrothymic tract below the lower pole of thyroid gland. Malignant transformation of thyroid rest is very rare. We report an extremely rare case of papillary carcinoma arising from thyroid rest in a 56-year-old male. He presented with hoarseness due to vocal cord palsy. Paratracheal mass in the upper mediastinum was identified by the cause of vocal cord palsy on CT. During surgery, we identified that the mass invaded recurrent laryngeal nerve but had no connection to thyroid gland. Histopathologic examination revealed that the mass was primary papillary thyroid carcinoma and there was no evidence of malignancy in thyroid gland. The post-therapeutic I-131 whole body scan detected several focal hot uptake in lung and mediastinum, suggesting distant metastasis. We should have knowledge of developmental variations of thyroid gland such as thyroid rest and its malignant transformation.
Carcinoma, Papillary ; Hoarseness ; Humans ; Lung ; Male ; Mediastinum ; Middle Aged ; Neoplasm Metastasis ; Recurrent Laryngeal Nerve ; Thyroid Gland* ; Thyroid Neoplasms* ; Vocal Cord Paralysis* ; Vocal Cords* ; Whole Body Imaging

BACKGROUND AND OBJECTIVES: For idiopathic sudden sensorineural hearing loss (ISSHL) patients, steroids are one of the most important therapies which are used to reduce inflammation in the inner ear. However, dosage regimens of oral steroid therapy have not been well established. This study aims to investigate the progression in recovery from ISSHL, and the optimal dosage regimen with steroids. SUBJECTS AND METHOD: We undertook a retrospective study of 149 patients diagnosed with ISSHL at our institution. We compared various clinical parameters such as age, gender, vertigo, tinnitus, the interval between disease onset and initial treatment, and severity of hearing loss. The 149 patients were divided based on their steroid regimens into two groups: group 1 (which received 40 mg of prednisolone in the morning and 20 mg at night) and group 2 (which received 20 mg of prednisolone three times daily). We then compared the results of group 1 with that of group 2. RESULTS: Recovery rates in group 1 (58/90, 64.4%) were significantly higher in ISSHL than those in group 2 (27/59, 45.8%). In particular, the complete recovery rates of Siegel's criteria in group 1 (35/90, 38.9%) were higher than those in group 2 (12/59, 20.3%). Therapeutic results were affected by the presence of tinnitus. Of the patients with tinnitus, 64.3% in group 1, and 43.9% in group 2 recovered. CONCLUSION: In the group treated with twice-daily regimens, greater hearing improvements were observed compared with the group treated with three times-daily regimens. These findings suggest that higher dose for a single administration may be an important prognostic factor.
Ear, Inner ; Hearing ; Hearing Loss ; Hearing Loss, Sensorineural* ; Hearing Loss, Sudden ; Humans ; Inflammation ; Methods ; Prednisolone ; Retrospective Studies ; Steroids ; Tinnitus ; Vertigo

We report an unusual case of venous thrombosis complicated by pyogenic psoas muscle abscess in a patient with chronic biliary tract disease. A 64-year-old woman presented with high fever and progressive back and left flank pain. She had been admitted because of recurrent cholangitis and liver abscess 7 months ago. Both abscess and blood cultures had revealed Klebsiella pneumoniae and she had improved with treatment of antibiotics and percutaneous drainage of abscess. Computed tomography demonstrated psoas abscess and thrombosis of inferior vena cava and left iliac vein adjacent to abscess. Several days later, abscess culture showed Klebsiella pneumoniae. Because there was no evidence of deep vein thrombosis and the thrombosis was confined only to the vein adjacent to the abscess, we considered that the thrombosis would be associated with the abscess. She was immediately treated with percutaneous drainage of abscess and antibiotics. Follow-up computed tomography demonstrated complete disappearance of both psoas abscess and venous thrombosis.
Abscess* ; Anti-Bacterial Agents* ; Biliary Tract Diseases ; Cholangitis ; Drainage* ; Female ; Fever ; Flank Pain ; Follow-Up Studies ; Humans ; Iliac Vein ; Klebsiella pneumoniae ; Liver Abscess ; Middle Aged ; Psoas Abscess* ; Psoas Muscles ; Thrombosis ; Veins ; Vena Cava, Inferior ; Venous Thrombosis*

PURPOSE: Infantile Marfan syndrome (MFS) is a rare congenital inheritable connective tissue disorder with poor prognosis. This study aimed to evaluate the cardiovascular manifestations and overall prognosis of infantile MFS diagnosed in a tertiary referral center in Korea. METHODS: Eight patients diagnosed with infantile MFS between 2004 and 2014 were retrospectively evaluated. RESULTS: Their median age at the time of diagnosis was 2.5 months (range, 0-20 months). The median follow-up period was 25.5 months (range, 0-94 months). The median length at birth was 50.0 cm (range, 48-53 cm); however, height became more prominent over time, and the patients were taller than the 97th percentile at the time of the study. None of the patients had any relevant family history. Four of the 5 patients who underwent DNA sequencing had a fibrillin 1 gene mutation. All the patients with echocardiographic data of the aortic root had a z score of >2. All had mitral and tricuspid valve prolapse, and various degrees of mitral and tricuspid regurgitation. Five patients underwent open-heart surgery, including mitral valve replacement, of whom two required multiple operations. The median age at mitral valve replacement was 28.5 months (range, 5-69 months). Seven patients showed congestive heart failure before surgery or during follow-up, and required multiple anti-heart failure medications. Four patients died of heart failure at a median age of 12 months. CONCLUSION: The prognosis of infantile MFS is poor; thus, early diagnosis and timely cautious treatment are essential to prevent further morbidity and mortality.
Connective Tissue ; Diagnosis ; Early Diagnosis ; Echocardiography ; Follow-Up Studies ; Heart Failure ; Humans ; Infant, Newborn ; Korea ; Marfan Syndrome* ; Mitral Valve ; Mitral Valve Insufficiency ; Mortality ; Parturition ; Prognosis ; Retrospective Studies ; Sequence Analysis, DNA ; Tertiary Care Centers* ; Tricuspid Valve Insufficiency ; Tricuspid Valve Prolapse

BACKGROUND: Thromboelastography (TEG) has recently become popular for assessment of whole blood coagulation in the operating room. Ketorolac, a potent injectable nonsteroidal anti-inflammatory drug (NSAID), is commonly used for postoperative analgesia. NSAID inhibit platelet aggregation in coagulation process. This study was designed to determine whether ketorolac used for postoperative analgesia can affect hemostatic function using a TEG. METHODS: Seventy-four female patients, ASA physical status 1 or 2, scheduled for an elective gynecologic surgery were randomly allocated into one of four groups (Group 1: n = 10, control without patient-controlled analgesia (PCA); Group 2: n = 21, PCA with morphine 60 mg; Group 3: n = 20, PCA with morphine 30 mg + ketorolac 90 mg; Group 4: n = 23, PCA with ketorolac 180 mg). Blood samples were obtained for TEG analysis preoperatively and 24, 48 and 72 h after surgery. Cumulative drug dosage, visual analog pain scale, satisfaction degree and side effects were measured at 24, 48 and 72 h after surgery. RESULTS: There were no significant differences in TEG parameters among the four groups at each time. There were no significant differences in visual analog pain scales and satisfaction degrees among the three groups using PCA for postoperative analgesia. Among the three groups using PCA for postoperative analgesia, Group 2 experienced more side effects. CONCLUSIONS: Ketorolac does not affect hemostatic function for 3 days after surgery when administrated as a PCA drug.
Analgesia* ; Analgesia, Patient-Controlled ; Blood Coagulation ; Female ; Gynecologic Surgical Procedures ; Hemostasis ; Humans ; Ketorolac* ; Morphine ; Operating Rooms ; Pain Measurement ; Passive Cutaneous Anaphylaxis ; Platelet Aggregation ; Thrombelastography

PURPOSE: To report our experiences of transarterial embolization for acute intercostal artery bleeding. MATERIALS AND METHODS: A retrospectively analysis of the causes, clinical manifestations, angiographic findings and transarterial embolization technique in 8 patients with acute intercostal artery bleeding, with a review of the anatomical basis. RESULTS:The causes of intercostal artery bleeding were iatrogenic and traumatic in 88 and 12% of cases, respectively. Active bleeding from the collateral intercostal or posterior intercostal arteries was angiographically demonstrated in 75 and 25% of cases, respectively. Transarterial embolization successfully achieved hemostasis in all cases. However, two patient with hypovolemic shock expired due to a massive hemothorax, despite successful transarterial embolization. CONCLUSION: Intercostal access should be performed through the middle of the intercostal space to avoid injury to the collateral intercostal artery. Transarterial embolization is an effective method for the control of intercostal artery bleeding.
Arteries* ; Hemorrhage* ; Hemostasis ; Hemothorax ; Humans ; Retrospective Studies ; Shock

PURPOSE: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. METHODS: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. RESULTS: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. CONCLUSION: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.
Abnormalities, Multiple ; Aortic Coarctation ; Aortic Valve Stenosis ; Cause of Death ; Counseling ; Diagnosis ; Early Diagnosis ; Follow-Up Studies ; Genetic Testing ; Heart Defects, Congenital ; Heart Diseases ; Heart* ; Humans ; Hypoplastic Left Heart Syndrome ; Intellectual Disability ; Mitral Valve Stenosis ; Prevalence ; Retrospective Studies

BACKGROUND AND OBJECTIVES: Cardiac catheterization is used to diagnose structural heart disease (SHD) and perform transcatheter treatment. This study aimed to evaluate complications of cardiac catheterization and the associated risk factors in a tertiary center over 10 years. SUBJECTS AND METHODS: Total 2071 cardiac catheterizations performed at the Seoul National University Children's Hospital from January 2004 to December 2013 were included in this retrospective study. RESULTS: The overall complication, severe complication, and mortality rates were 16.2%, 1.15%, and 0.19%, respectively. The factors that significantly increased the risk of overall and severe complications were anticoagulant use before procedure (odds ratio [OR] 1.83, p=0.012 and OR 6.45, p<0.001, respectively), prothrombin time (OR 2.30, p<0.001 and OR 5.99, p<0.001, respectively), general anesthesia use during procedure (OR 1.84, p=0.014 and OR 5.31, p=0.015, respectively), and total procedure time (OR 1.01, p<0.001 and OR 1.02, p<0.001, respectively). Low body weight (OR 0.99, p=0.003), severe SHD (OR 1.37, p=0.012), repetitive procedures (OR 1.7, p=0.009), and total fluoroscopy time (OR 1.01, p=0.005) significantly increased the overall complication risk. High activated partial thromboplastin time (OR 1.04, p=0.001), intensive care unit admission state (OR 14.03, p<0.001), and concomitant electrophysiological study during procedure (OR 3.41, p=0.016) significantly increased severe complication risk. CONCLUSION: Currently, the use of cardiac catheterization in SHD is increasing and becoming more complex; this could cause complications despite the preventive efforts. Careful patient selection for therapeutic catheterization and improved technique and management during the peri-procedural period are required to reduce complications.
Anesthesia, General ; Body Weight ; Cardiac Catheterization* ; Cardiac Catheters* ; Catheterization ; Catheters ; Fluoroscopy ; Heart Diseases* ; Heart* ; Intensive Care Units ; Mortality ; Partial Thromboplastin Time ; Patient Selection ; Prothrombin Time ; Retrospective Studies ; Risk Factors ; Seoul

Two isolates of Tricholoma matsutake T-008 and T-034, preserved in Entomopathogenic Fungal Culture Collection (EFCC) of Korea, were used in the present study. The isolates had 100% Bootstrap homology with Tricholoma matsutake U62964 and T. matsutake AB188557 and AF309538 preserved in Gene Bank of NCBI. Mycelial growth of T. matsutake was highest in TMM and MYA at 25degrees C. The highest dry wt. of mycelium was obtained after 65 days of culture, when 6 mycelial discs were inoculated in 100 ml of broth in 250 ml shaking flask. Mycelial mats were observed in clumped condition at the inoculation sites of pine forest after two weeks of inoculation. After 5 months of inoculation, mycelia mats were observed growing inside soil and walls of a few inoculation sites, while mycelial mats growth up to 5~8 cm were observed in the roots of pine tree after 6 months. The survival rate of the inoculum was about 40% of the total inoculation sites. The survival rate was found below 20% when the mycelium was inoculated in the summer. The reasons for low survival rates of the mycelium were mainly due to dry season and the soil-borne small animals such as earthworm and mole. After one year of inoculation, no external difference was observed between the artificially inoculated mycelia and the naturally existing mycelia of T. matsutake. The present study showed that fruiting bodies of T. matsutake could be produced by artificial inoculation under the appropriate environmental conditions.
Animals ; Fruit ; Korea ; Mya ; Mycelium ; Oligochaeta ; Pinus ; Seasons ; Soil ; Survival Rate ; Trees* ; Tricholoma*

Background and Objectives: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, RYR2, the major CPVTcausative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT. Methods: We retrospectively reviewed 23 patients diagnosed with CPVT before 19 years of age. Genetic analysis, history of neuropsychiatric manifestations, changes in ventricular arrhythmia burden before and after treatment, occurrence of cardiac events, and overall survival (OS) were investigated. Results: RYR2 variants were identified in 17 patients, and 14 were classified as pathogenic or likely pathogenic. Neuropsychiatric manifestations, including intellectual disability and attention deficit hyperactivity disorder, were identified in 10 patients (43.5%). The 5-year cardiac event-free survival rate was 31.2%, and the 10-year OS rate was 73.1%. Patients diagnosed since 2009 had a higher cardiac event-free survival rate than those diagnosed before 2009 (p=0.0028).Combined beta-blocker and flecainide therapy demonstrated a lower risk of cardiac events than beta-blocker monotherapy (hazard ratio [HR], 0.08; 95% confidence interval [CI], 0.02–0.38;p=0.002). Left cardiac sympathetic denervation (LCSD) reduced the ventricular arrhythmia burden in Holter monitoring. Occurrence of near-fatal cardiac events after diagnosis was an independent predictor of death (HR, 33.40; 95% CI, 6.23–179.95; p<0.001). Conclusions Neuropsychiatric manifestations are common in children with CPVT. Flecainide and/or LCSD, when added to beta-blocker therapy, reduce the ventricular arrhythmia burden and cardiac events, thereby improving treatment outcomes in recent years.