DiGeorge syndrome is an immunodeficient disease associated with abnormal development of 3rd and 4th pharyngeal pouches. As a hemizygous deletion of chromosome 22q11.2 occurs, various clinical phenotypes are shown with a broad spectrum. Conotruncal cardiac anomalies, hypoplastic thymus, and hypocalcemia are the classic triad of DiGeorge syndrome. As this syndrome is characterized by hypoplastic or aplastic thymus, there are missing... |
Blood culture is the most important tool for detecting bacteremia in children with fever. However, blood culture contamination rates range from 0.6% to 6.0% in adults; rates for young children have been considered higher than these, although data are limited, especially in Korea. This study determined the contamination rate and risk factors in pediatric patients visiting the emergency room (ER)... |
Methods for quick and reliable detection of Nasopharyngeal (NP) secretions were obtained from 842 pediatric patients admitted with lower respiratory infections at Severance Children's Hospital... |
Juvenile idiopathic arthritis (JIA) is comprised of a heterogeneous group of several disease subtypes that are characterized by the onset of arthritis before the age of 16 years and has symptoms lasting at least 6 weeks. The previous classification of JIA included seven different categories, whereas its current classification was compiled by the International League of the Association for Rheumatology,... |
Tumor necrosis factor-α (TNF-α) is a major proinflammatory cytokine involved in the pathophysiology of juvenile rheumatoid arthritis. Etanercept is an effective inhibitor of TNF-α and has shown a beneficial effect in patients with JRA. However, the most important cause of concern related to etanercept administration is infection. We report a case of encephalitis in a JRA patient receiving long-term treatment... |
Purpose : Acute interstitial pneumonia (AIP) is a rare disease, but its prognosis is fatal because of lack of efficient treatment modality. Recently, it has been reported that there was epidemic AIP in Korea. This study aims to investigate the past and current status of AIP in Korea. Methods : We performed a nationwide survey and a prospective study. From August... |
Purpose : Surveillance for detecting and managing latent tuberculosis infection (LTBI) is a key component of tuberculosis control. The classic surveillance tool, the tuberculin skin test (TST), may have some limitations when used in the Bacillus Calmette-Guérin (BCG)-vaccinated population. The object was to perform a blood test QuantiFERON®-TB Gold In Tube (QFT-G IT) based on the detection of interferon-γ (IFN-γ)... |
Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from... |
The diagnosis of juvenile rheumatoid arthritis (JRA) is based on patient's age at disease onset, symptom duration, gender, and clinical manifestations. JRA is of unknown origin, begins under the age of 16, and persists for a minimum of 6 weeks. JRA is categorized into three principal types, systemic, oligoarticular and polyarticular. Infection, other connective tissue diseases, malignancy, trauma, and immunodeficiency... |
Fever has been recognized as a cardinal feature of disease since antiquity, but only recently has the pathophysiology of fever come to be understood. It became clear that the ultimate cause of fever is not a bacterial product (a so-called exogenous pyrogen) but a product of host inflammatory cells (i.e., an endogenous pyrogen). Many studies have demonstrated that mononuclear phagocytes... |
Purpose : Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. Methods : The data of 45 patients who were diagnosed as pediatric SLE in Severance... |
Purpose : The purpose of this study is to investigate the pathogens of central venous catheter-related blood stream infections and search for the association among the insertion site, the duration and the underlying conditions with the prevalence of central venous catheter-related blood stream infections under 15 years old. Methods : A retrospective study was performed from Jan, 2003 to Dec, 2003... |
Purpose : To find the risk factors associated with coronory artery lesions, non-responsiveness to intravenous immunoglobulin(IVIG) treatment, and recurrences in Kawasaki disease patients. Methods : We retrospectively analyzed 1,000 Kawasaki disease patients who were admitted to Yonsei University Medical Center from September 1990 to December 2003. We compared between responder and non-responder groups to IVIG treatment as well as between relapsed... |
Kawasaki disease is an acute systemic vasculitis of unknown origin. Giant coronary aneurysm is one of the most serious complications, although peripheral artery vasculitis can produce life-threatening events. Myocardial ischemia and infarction can be caused by coronary artery stenosis, aneurysm, and stagnation of blood flow in coronary arteries which triggers thromboembolism. Atypical presentation in young infants often interferes with prompt... |
Objective : Toll like receptor(TLR) is known to be involved in innate immunity. Many microbial antigens stimulate TLR, and as a result of intracellular signal transduction, they activate nuclear factor-kB which produces diverse inflammtory cytokines. Until now, many research topics in Kawasaki disease focused on cytokine increasement. In this study, we aim to reveal TLR increasement which might be associated... |
Objective : Besides the functions of histones in the nucleus of the cells, there is growing evidence that histones have many other extra-cellular or extra-nuclear functions, such as stabilizing axonemal microtubule of sea urchin sperm flagella. This microtule assembly function of the histone is similar to that of taxol, which has an effect of controlling joint inflammation. In this study,... |
Purpose : In this study, a possible suppressive effect of a flavon extracted from Artemisia absinthium on a mouse collagen-induced arthritis (CIA) model was investigated. Methods : DBA/1 mice were injected intradermally with emulsified chicken type II collagen. Three weeks after immunization, a flavon was introduced p.o. everyday. Clinical incidences of arthritis and arthritis index were measured. Measurement of anti-collagen antibodies... |
Purpose : Kawasaki disease is a systemic vasculitis observed in children under five years of age and the leading cause of pediatric acquired heart disease, but its pathophysiology is still not completely understood. With proteomics, the study of quality and quantity of proteins, having been developed dramatically, we tried to find a way to evaluate the etiology and pathogenesis of... |
Purpose : Hyper IgM syndrome(HIGM) is characterized by severe recurrent bacterial infections with decreased serum levels of IgG, IgA, and IgE but elevated IgM levels. Recently, it has been classified into three groups; HIGM1, HIGM2 and a rare form of HIGM. HIGM1 is a X-linked form of HIGM and has now been identified as a T-cell deficiency in which mutations... |
Purpose : The acute respiratory distress syndrome(ARDS), an acute form of severe alveolar-capillary injury evolving after a direct or indirect lung insult is thought to be a common cause of respiratory failure though not many clinical studies on the subject have been made yet. Methods : Between January 1992 and December 2001, we conducted a retrospective study on 33 children who... |
Polyarteritis nodosa(PAN) is a multisystemic disease primarily involving the small and medium- sized vessels. Prognosis depends on the presence and severity of visceral involvement. Gastrointestinal involvement in PAN may result in vague abdominal pain, nausea and melena. Angiography can be used to establish a diagnosis of PAN. We report a case of polyarteritis nodosa presenting with recurrent abdominal pain, melena... |
Purpose : Streptococcus pneumoniae is a normal flora and common pathogen of the upper respiratory tract. S. pneumoniae infections are estimated to cause not only localized infection such as pneumonia, and otitis media, but also systemic infections, for example meningitis, sepsis, and so on. Recently, the resistance of S. pneumoniae to penicillin and multidrug has been rapidly increasing in many... |
Purpose : To survive in an ecological environment, an individual must develop immunity to various antigens. Therefore, populations of peripheral blood mononuclear cells(PBMC) in humans change continuously with growth. The object of this study is to evaluate the apoptosis of peripheral blood mononuclear cells(PBMC) in normal children of different ages. Methods : PBMC were isolated from the study groups. Ten... |
Purpose : To evaluate apoptosis of cord blood mononuclear cells(CBMC) infected with enterovirus. Methods : Cord blood was obtained from 20 neonates which were delivered vaginally without any complications. Cord blood mononuclear cells(CBMC) were isolated, and after 24h and 48h incubation, cells were stained with Annexin V and Propidium Iodide and were analyzed with FACScan flowcytometer. CBMCs were also incubated with... |
Purposes : Rotaviruses are the main cause of infantile diarrheal diseases worldwide. The purpose of this study is to obtain epidemiologic data of rotavirus infections in Korea. Methods : Stool specimens were collected from 150 patients with acute diarrheal symptoms, who were admitted to Yonsei Medical Center and Chungbuk National University Hospital. After isolating the virus from the specimens, the viruses... |
Purpose : It is well known that the Acanthopanax koreanum extract has an anti-inflammatory action without any adverse effects reported. We conducted this study to see whether the Acanthopanax koreanum extract has a preventive effect on the development of collagen induced arthritis in DBA/1J mice. Methods : Three groups of BDA/1J mice were immunized by intradermal injection of 2mg/kg bovine type... |
Kawasaki disease is an acute illness of early childhood, especially less than 5 years of age, characterized by prolonged fever(> 5 days), changes of lips and oral cavity, changes of peripheral extremities, polymorphous skin rash, bilateral conjunctival congestion and acute nonpurulent cervical lymphadenopathy. Usually, the recurrence of Kawasaki disease is not frequent. It is thought that the factors in recurrence of Kawasaki disease was... |
Purpose : Neck masses are frequently encountered in pediatric practice, and have various underlying diseases. If the underlying diseases of the neck mass are not confirmed by its clinical characteristics, it can be done by neck mass biopsy. To define the necessity and the appropriate time of neck mass biopsy is very difficult and also important. Therefore the authers have conducted this study to... |
Purpose : Aseptic meningitis is an infection of CNS caused by virus and there are remarka-ble changes in endogenous cytokine level. Interleukin-6 is a pleiotropic and multi-functional cyt-okine and plays a central role in host defense mechanisms. We studied the IL--6 level in serum and CSF with the patients of aseptic meningitis and investigated the relationship of IL-6 with C... |
House dust mites, Dermatophagoides farinae and Dermatophagoides pteronyssius, have been identified as the main sources of house dust allergen and are considersed the most important causative allergen in the etiology of allergic respiaratory disease and allergic rhinitis. To evaluate the incidence of positive skin reactivity to house dust mites and relationship between total serum IgE levels and specific IgE antibody to... |