The PACS system built and used in hospitals nowadays has quite significant overload on the central server of it because of both treatment of very large data and full management of medical images. We suggest a distributed communication and management methodology using Peer to Peer multicasting strategy for efficient management of medical images produced by DICOM modalities. It is absolutely necessary for reducing strict degradation of PACS system due to large size of medical images and its very high transport rates. DICOM Peer to Peer component is composed a service manager to execute requested queries, a communication manager to take charge of file transmission, and a DICOM manager to manage stored data and system behavior. Each manager itself is a component to search for requested file by interaction or transmit the file to other Peers. Distributed management and transformation of medical information based on Peer to Peer multicasting methodology will enhance performance of central server and network capacity reducing overload on them.

BACKGROUND AND OBJECTIVES: This study is aimed at elucidating potential temporal associations between the occurrence of Kawasaki disease (KD) and various viral infections. SUBJECTS AND METHODS: We obtained monthly patterns of KD from the seventh nationwide survey and viral detection data from the Korea Centers for Disease Control and Prevention from 2009 to 2011 and evaluated temporal correlations between them for each month. The respiratory viruses detected using a multiplex real-time-polymerase chain reaction kit were influenza virus (A/H1N1, A/H3N2, A/H5N1, and B), adenovirus, parainfluenza virus (type 1, 2, 3), respiratory syncytial virus (type A, B), human rhinovirus, human coronavirus (OC43/229E, NL63), human bocavirus, and enterovirus. RESULTS: We obtained data from a total of 13031 patients who were treated for acute KD from 87 hospitals with pediatric residence programs. During this survey, KD showed highest overall incidence in summer and winter seasons and lowest incidence in February and October. We received viral detection data for a total of 14267 patients. Viral detection was highest during winter and spring seasons. The most commonly detected virus was human rhinovirus (32.6%), followed by influenza virus (26.8%). The monthly incidence of KD showed significant correlation with the monthly overall viral detection (p=0.022, r=0.382). In particular, human bocavirus and enterovirus have significant correlations with monthly patterns of KD occurrence (p=0.032 and p=0.007, respectively) and influenza virus correlated with KD occurrence with borderline significance (p=0.063). CONCLUSION: The temporal association between monthly occurrence of KD and viral detection suggests the etiologic importance of precedent infection in the development of KD.
Adenoviridae ; Centers for Disease Control and Prevention (U.S.) ; Coronavirus ; Enterovirus ; Human bocavirus ; Humans ; Incidence ; Korea ; Mucocutaneous Lymph Node Syndrome* ; Orthomyxoviridae ; Paramyxoviridae Infections ; Respiratory Syncytial Viruses ; Rhinovirus ; Seasons

Acute giant coronary aneurysm after Kawasaki disease (KD) is a catastrophic complication that can be fatal and very difficult to manage. However, no fixed consensus has been reached for the management of super-giant coronary aneurysms in the acute setting. Here, we report the successful management of young children with super-giant coronary aneurysms after KD. Based on our experience, hemodynamic stabilization to prevent further coronary dilation or rupture and strict anticoagulation to avoid thrombus formation are mandatory in the management of this condition.
Child ; Consensus ; Coronary Aneurysm* ; Coronary Artery Disease ; Coronary Thrombosis ; Hemodynamics ; Humans ; Mucocutaneous Lymph Node Syndrome* ; Rupture ; Thrombosis

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

BACKGROUND AND OBJECTIVES: In Duchenne and Becker muscular dystrophies, cardiac function deteriorates with time resulting in heart failure which is often fatal. We prospectively evaluated the effect of enalapril and carvedilol on left ventricular (LV) dysfunction in middle childhood and adolescent patients with muscular dystrophy. SUBJECTS AND METHODS: Twenty-three patients with LV dysfunction (22 with Duchenne muscular dystrophy, 1 with Becker muscular dystrophy) were enrolled. We prescribed enalapril (13 patients) or carvedilol (10 patients) randomly from July 2008 to August 2010 and followed up the patients until September 2011. The changes in LV function parameters before and after the treatment were evaluated by echocardiography. RESULTS: The mean age at the start of treatment with enalapril or carvedilol was 12.6+/-3.7 years (median 13 years), and mean follow-up duration was 20.1+/-8.9 months. In the enalapril group, LV fractional shortening (FS) increased from 25.8+/-2.1 to 26.6+/-3.0 (p=0.241). In the carvedilol group, LV FS increased from 26.4+/-1.1 to 28.6+/-4.2 (p=0.110). In all 23 patients, LV FS significantly increased from 26.1+/-1.7 (before) to 27.6+/-3.7 (after treatment) (p<0.046). Indexed LV dimension at end diastole and LV end-diastolic volume decreased slightly, but without statistical significance by tri-plane volumetry. LV diastolic functional parameters were maintained during follow-up period. CONCLUSION: Enalapril or carvedilol could improve LV systolic function in middle childhood and adolescent patients with muscular dystrophy without significant adverse effects.
Adolescent ; Carbazoles ; Cardiomyopathies ; Diastole ; Echocardiography ; Enalapril ; Follow-Up Studies ; Heart Failure ; Humans ; Muscular Dystrophies ; Muscular Dystrophy, Duchenne ; Propanolamines ; Prospective Studies ; Ventricular Dysfunction, Left

BACKGROUND AND OBJECTIVES: Milrinone is often used in children to treat acute heart failure and prevent low cardiac output syndrome after cardiac surgery. Due to the lack of studies on the long-term milrinone use in children, the objective of this study was to assess the safety and efficacy of the current patterns of milrinone use for > or =3 days in infants and children with heart diseases. SUBJECTS AND METHODS: We retrospectively reviewed the medical records of patients aged <13 years who received milrinone for > or =3 days from January 2005 to December 2012. Patients' characteristics including age, sex, height, weight, and body surface area were recorded. The following parameters were analyzed to identify the clinical application of milrinone: initial infusion rate, maintenance continuous infusion rate, total duration of milrinone therapy, and concomitantly infused inotropes. The safety of milrinone was determined based on the occurrence of adverse events such as hypotension, arrhythmia, chest pain, headache, hypokalemia, and thrombocytopenia. RESULTS: We assessed 730 admissions (684 patients) during this period. Ventricular septal defects were the most common diagnosis (42.4%) in these patients. Milrinone was primarily used after cardiac surgery in 715 admissions (97.9%). The duration of milrinone treatment varied from 3 to 64.4 days (> or =7 days in 149 admissions). Ejection fraction and fractional shortening of the left ventricle improved in patients receiving milrinone after cardiac surgery. Dose reduction of milrinone due to hypotension occurred in only 4 admissions (0.5%). Although diverse arrhythmias occurred in 75 admissions (10.3%), modification of milrinone infusion to manage arrhythmia occurred in only 3 admissions (0.4%). Multivariate analysis indicated that the development of arrhythmia was not influenced by the pattern of milrinone use. CONCLUSION: Milrinone was generally administered for > or =3 days in children with heart diseases. The use of milrinone for > or =3 days was effective in preventing low cardiac output after cardiac surgery when combined with other inotropes, suggesting that milrinone could be safely employed in pediatric patients with heart diseases.
Arrhythmias, Cardiac ; Body Surface Area ; Cardiac Output, Low ; Chest Pain ; Child ; Diagnosis ; Headache ; Heart Diseases* ; Heart Failure ; Heart Septal Defects, Ventricular ; Heart Ventricles ; Humans ; Hypokalemia ; Hypotension ; Infant ; Medical Records ; Milrinone* ; Multivariate Analysis ; Off-Label Use* ; Retrospective Studies ; Thoracic Surgery ; Thrombocytopenia

Takayasu arteritis is a chronic inflammatory disease of unknown etiology primarily affecting the aorta and its major branches and usually occurring in the second or third decade of life. Here, we report a case of Takayasu arteritis in a 10-month-old patient. The infant presented with signs of congestive heart failure and severe aortic regurgitation. Echocardiography and computed tomography angiography showed an abnormally dilated thoracic and abdominal aorta. The infant was initially treated with prednisolone, followed by commissuroplasty of the aortic valve but neither approach ameliorated the heart failure. The patient was eventually treated with a mechanical aortic valve replacement surgery at the age of 12 months, and her condition stabilized. Although unusual, this case indicates that the diagnosis of Takayasu arteritis should be considered in children with unexplained systemic symptoms, aortic valve regurgitation, and heart failure. Because severe aortic regurgitation may be a fatal complication of Takayasu arteritis, early aortic valve replacement surgery should be considered, even in very young children.
Angiography ; Aorta ; Aorta, Abdominal ; Aortic Valve ; Aortic Valve Insufficiency ; Child ; Echocardiography ; Heart Failure ; Humans ; Infant ; Prednisolone ; Takayasu Arteritis

Unguarded tricuspid regurgitation (TR) due to a flail tricuspid leaflet is a rare condition of newborn cyanosis. A high perinatal mortality has been associated with this fatal condition. But, there are feasible surgical repairs to improve survival. We report the case of a male full-term neonate with intractable hypoxia. He had profound tricuspid insufficiency and leaflet prolapse caused by a ruptured papillary muscle supporting the anterior leaflet of the tricuspid valve. He presented with severe cyanosis and respiratory distress immediately after birth. Despite medical management, the pulmonary vascular resistance was not decreased and a low cardiac output persisted. Initial stabilization was accomplished with nitric oxide and extracorporeal membrane oxygenation. The tricuspid valve repair surgery was successfully performed subsequently. TR resulting from papillary muscle rupture is a potentially lethal condition. Timely diagnosis and proper surgical treatment can be lifesaving.
Anoxia ; Cardiac Output, Low ; Cyanosis ; Diagnosis ; Extracorporeal Membrane Oxygenation ; Female ; Humans ; Infant, Newborn* ; Male ; Nitric Oxide ; Papillary Muscles* ; Parturition ; Perinatal Mortality ; Persistent Fetal Circulation Syndrome* ; Prolapse ; Rupture ; Thoracic Surgery ; Tricuspid Valve Insufficiency ; Tricuspid Valve* ; Vascular Resistance

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

Timothy syndrome, long QT syndrome type 8, is highly malignant with ventricular tachyarrhythmia. A 30-month-old boy had sudden cardiac arrest during anesthesia induction before plastic surgery for bilateral cutaneous syndactyly. After successful resuscitation, prolonged QT interval (QTc, 0.58-0.60 sec) and T-wave alternans were found in his electrocardiogram. Starting beta-blocker to prevent further tachycardia and collapse event, then there were no more arrhythmic events. The genes KCNQ1, KCNH2, KCNE1 and 2, and SCN5A were negative for long QT syndrome. The mutation p.Gly406Arg was confirmed in CACNA1C, which maintains L-type calcium channel depolarization in the heart and other systems.
Anesthesia/*adverse effects ; Calcium Channels, L-Type/*genetics ; Death, Sudden, Cardiac/*etiology ; Electroencephalography ; Humans ; Infant ; Long QT Syndrome/*genetics ; Magnetic Resonance Imaging ; Male ; Methyl Ethers/adverse effects ; Nitric Oxide/adverse effects ; Polymorphism, Single Nucleotide ; Sequence Analysis, DNA ; Surgery, Plastic ; Syndactyly/diagnosis/*genetics/surgery