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Purpose:In contrast with traditional time-cycled, pressure-limited ventilation, during volume-controlled ventilation, a nearly constant tidal volume is delivered with reducing volutrauma and the episodes of hypoxemia. The aim of this study was to compare the efficacy of pressure-regulated, volume controlled ventilation (PRVC) to Synchronized intermittent mandatory ventilation (SIMV) in VLBW infants with respiratory distress syndrome (RDS). Methods : 34 very low birth... |
| Transient tachypnea of the newborn (TTN), respiratory distress syndrome (RDS), and persistent pulmonary hypertension (PPHN) are the three most common disorders that cause respiratory distress after birth. An understanding of the pathophysiology of these disorders and the development of effective therapeutic strategies is required to control these conditions. Here, we review recent papers on the pathogenesis and treatment of neonatal... |
| Poland syndrome is characterized by unilateral absence or hypoplasia of the pectoralis muscle and variable degree of ipsilateral hand anomalies. Möbius syndrome is a congenital neurological disorder characterized by complete or partial facial paralysis. Although the pathogeneses of these diseases are not well-characterized, diminished blood flow to the affected side is thought to play a role. A male infant weighing... |
| Purpose : Idiopathic nephrotic syndrome (NS) can be clinically classified as steroid-sensitive and steroid-resistant. The detailed mechanism of glucocorticoid action in NS is currently unknown. Methods : In this study, we investigated 3 known single nucleotide polymorphisms (SNPs) (ER22/23EK, N363S, and BclI) of the glucocorticoid receptor gene (the NR3C1 gene) in 190 children with NS using polymerase chain reaction-restriction fragment... |
| We report a case of a full-term neonate with persistent pulmonary hypertension who developed asphyxia after birth and was treated with iloprost. The neonate had persistent hypoxia and did not respond to supportive treatment. Because inhaled nitric oxide (iNO) was not available in our hospital, inhaled iloprost was administered via an endotracheal tube. This resulted in an immediate elevation of... |
| Edwards syndrome, also called trisomy 18, is one of the most common autosomal anomalies. The survival rate of patients with Edwards syndrome is very low and its characteristic findings include cardiac malformations, mental retardation, growth retardation, specific craniofacial anomalies, clenched hands, rocker-bottom feet, and omphalocele. Compared with the classic Edwards syndrome, the symptom of partial duplication of chromosome 18 is... |
| Purpose : Metabolic syndrome (MS), characterized by obesity and insulin resistance, elicits risk factors such as hyperlipidemia, hypertension, and glucose intolerance with additive effects on atherosclerosis, leading to cardiovascular diseases. The purposes of this study were to evaluate the prevalence of MS among overweight and obese adolescents and to investigate the impact of obesity on the cardiovascular system. Methods : In... |
| Two adolescent cases of Fitz-Hugh-Curtis syndrome, which has not been previously reported in Korean girls, presenting with right upper-quadrant abdominal pain, nausea, vomiting, and fever are reported here. A careful and thorough inquiry into the sexual history of the first patient, which was not done upon admission, led to a careful reassessment of the dynamic abdominal computed tomography scan revealing... |
| Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology that affects children. There are few reports that describe the Epstein-Barr virus (EBV) as the possible infectious agent of KD. Here, we describe a case of KD in a 15- year-old boy complicated with giant coronary artery aneurysms, pericardial effusion, and splenic infarction. The clinical course of KD was... |
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Purpose:We evaluated the effectiveness of treatment and cardiac complications of replacing a high dose of aspirin with a high dose of ibuprofen for children in acute phase of Kawasaki disease. We also analyzed the possibility of replacing a high dose of aspirin with a high dose of ibuprofen to prevent complications such as Reye s syndrome caused by aspirin. Methods:One hundred eight children... |
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Purpose:The aim was to investigate the clinical characteristics and responses to growth hormone (GH) therapy in children with attenuated growth who showed normal GH responses to GH stimulation tests (GHST). Methods:The study included 39 patients with height velocity (HV) of less than 4 cm/yr and normal GHST results. Clinical characteristics of patients were analyzed retrospectively. Results:Eleven were born as small for gestational age (SGA)... |
| Metabolic syndrome (MS) in children and adolescents has become a global public health problem. MS among Korean children and adolescents has been increasing significantly over the past decade. This paper aims to provide the currently available information on the MS in children and adolescents. This review focuses on the definition, diagnostic criteria, epidemiology and pathophysiology which may be helpful for... |
| SmithMagenis syndrome (SMS) is a rare disorder with multiple congenital anomalies caused by a heterozygous interstitial deletion involving chromosome 17p11.2, where the retinoic acid-induced 1 (RAI1) gene is located, or by a mutation of RAI1. Approximately 90% of the patients with SMS have a detectable 17p11.2 microdeletion on fluorescence in-situ hybridization (FISH). SMS is characterized by mental retardation, distinctive behavioral... |
| Blood eosinophilia can be classified as either familial or acquired. Familial eosinophilia is a rare autosomal dominant disorder characterized by a stable eosinophil count. Acquired eosinophilia is classified further into a primary or secondary phenomenon depending on whether eosinophils are considered integral to the underlying disease. Primary eosinophilia is considered clonal in the presence of either a cytogenetic abnormality or... |
| Kawasaki disease (KD) causes multisystemic vasculitis but rarely manifests with pulmonary symptoms. As its etiology is still unknown, there are no specific diagnostic tools available, and KD can be diagnosed only by the symptom pattern. The presence of unusual clinical manifestations often leads to delayed diagnosis. Here, we report two cases of KD with an initial presentation of pneumonia. KD... |
| Purpose : To evaluate the clinical features and characteristics of childhood periodic syndromes (CPS) in Korea using the new criteria of the International Classification of Headache Disorders (ICHD)-II. Methods : The study was conducted at pediatric neurology clinics of five urban tertiary-care medical centers in Korea from January 2006 to December 2007. Patients (44 consecutive children and adolescents) were divided into... |
| Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of... |
| Purpose : The purpose of this study was to investigate the polymorphisms of the TNF-alpha promotor gene, its susceptibility to Kawasaki disease (KD) and to assess whether the TNF-alpha promotor gene polymorphism was related the risk of coronary artery lesions (CALs). Methods : From January 2003 to January 2007, 51 children (30 boys and 21 girls) with KD and 48... |
| AHS는 항경련제에 의한 부작용으로 드물게 발생하지만 생명을 위협할 수 있는 지연형 면역반응이다. 보통 항경련제 복용 후 1-8주 사이에 고열, 피부발진, 림프절병증의 임상 증세를 나타내고, 혈액, 간, 신장, 또는 폐 등의 내부 장기를 침범한다. AHS는 항경련제에 의한 부작용으로 증상이 전신적으로 다양하게 나타날 수 있다. 그리고 전염성 단핵구증이나 가와사키병과 같은 전신적 질환들과도 유사한 임상증상들을 보이기 때문에 이런 질환들과 감별하는 것이 중요하고 사망률도... |
| Purpose : The risk of metabolic syndrome (MS) and cardiovascular disease in Turner syndrome (TS) patients is high. We analyzed metabolic factors in adults with TS and evaluated the metabolic risk of insulin resistance. Methods : Forty-three adults with TS were enrolled. The frequency of MS and the values of the metabolic factors were analyzed. Patients were divided into insulin... |
| Purpose : Clinical and laboratory findings predict a severe outcome for mucocutaneous lymph node syndrome. This study aimed to define the clinical characteristics of Kawasaki disease (KD) patients with hyponatremia and to determine the factors associated with its development. Methods : Retrospective studies were performed on 114 KD patients who received an initial high-dose intravenous immunoglobulin (IVIG, single 2 g/kg/dose)... |
| Purpose : Pneumothorax is an important factor responsible for increased mortality and morbidity in neonates. Here, we compared the clinical findings and prognosis for neonatal pneumothorax induced by respiratory distress syndrome (RDS) and pneumonia. Methods : Between January 2001 and December 2005, 80 patients with neonatal pneumothorax induced by RDS and pneumonia and admitted to the NICU of Dongsan Medical... |
| Kawasaki disease is an acute systemic inflammatory disorder, the etiology of which has not yet been established. The clinical manifestations are non-specific and are common to many pediatric infectious and immunologic diseases. In 2 cases presenting fever, cervical lymphadenopathy, and retropharyngeal abscess-like lesions on the neck shown in a computerized tomography (CT) scan, the diagnosis of Kawasaki disease was delayed.... |
| Stevens-Johnson syndrome (SJS) presents with widespread blisters, erythematous or purpuric macules, and one or more mucous membrane erosions. Various etiologic factors, including infection, vaccination, drug administration, systemic diseases, physical agents, and food have been implicated as causes of SJS. Mycoplasma pneumoniae is the most common infectious agent to cause SJS in children. In recent literature, M. pneumoniae-induced SJS with mucositis... |
| Purpose : We investigated the relationship between thyroid hormone and serum tumor necrosis factor (TNF-α), interleukin (IL-6) and N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP) in patients with Kawasaki disease (KD). Methods : Serum levels of thyroid hormone, TNF-α, IL-6, and NT-proBNP were measured in 52 KD patients in the acute and subacute phase and 10 patients with acute... |
| Reduced fetal growth is independently associated with increased risk of health problems in later life, particularly type 2 diabetes and cardiovascular diseases. Insulin resistance appears to be a key component underlying these metabolic complications. It is suggested that detrimental fetal environment may program insulin resistance syndrome. An insulin-resistant genotype may also result in both low birth weight and insulin resistance... |
| Renal cell carcinoma (RCC) arising from epithelial cells of the renal tubules is a highly aggressive and malignant tumor in all ages; however, it rarely occurs in children. the standard treatment for RCC is radical nephrectomy with lymph node dissection when the tumor is localized and can be completely resected. Adjuvant chemotherapy, radiotherapy, and immunotherapy are used for pediatric patients... |
| Purpose : Many gene polymorphisms are associated with coronary artery abnormalities in Kawasaki disease. Catechol-O- methyltransferase (COMT) plays an important role in the metabolism of catecholamines, catechol estrogen, and catechol drugs. Polymorphisms of the COMT gene are reported to be associated with myocardial infarction and coronary artery abnormalities. The aim of this study was to evaluate the relationship between COMT... |
| Wilms` tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome is caused by deletion of chromosome 11p13, including the Wilms` tumor (WT1) and aniridia gene (PAX6) loci. Here, we report the first case of WAGR syndrome in Korea; the patient was a 2-year-old girl with bilateral aniridia from birth who presented with abdominal distention and mental retardation. Cytogenetically, she had... |
| Purpose : The aim of this study was to investigate the epidemiologic status of Kawasaki disease (KD) in infants ≤6 months of age. Methods : For the epidemiologic study of KD in Korea, data from 22,674 KD patients were collected from 1997 to 2005 on a 3-year basis by a retrospective survey. From this survey, data of 1,739 KD patients ≤6... |
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