Inflammatory bowel disease (IBD) is a chronic relapsing disorder of unknown etiology, which is believed to be multifactorial. Recently, the incidence of pediatric IBD has steeply increased in Korea since 2000. Poorly controlled disease activity can result in complications such as intestinal fistulae, abscess, and stricture, as well as growth retardation and delayed puberty in children. Because of a lack... |
Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is characterized by a severe idiosyncratic reaction including rash and fever, often with associated hepatitis, arthralgias, lymph node enlargement, or hematologic abnormalities. The mortality rate is approximately 10%, primarily owing to liver failure with massive or multiple disseminated focal necrosis. Here, we report a case of a 14-year-old girl treated with... |
Acute liver failure (ALF) is a very rare but devastating illness in children. Specific treatment to recovery is often not available, and the underlying cause of the liver failure is often unknown and diverse especially in children. Liver transplantation has increased the chance of survival; however it needs an optimal timing to reach the best result which is not familiar... |
Purpose : Alagille syndrome is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, vertebrae, and craniofacial region. The Jagged1(JAG1) gene, which encodes a ligand of Notch, has been found mutated in Alagille syndrome. The aim of the study was to investigate the mutation analysis of JAG1 gene in Korean patients with Alagille syndrome. Methods : Genomic DNA... |
Alagille syndrome is a autosomal dominant disorder characterized by intrahepatic bile duct paucity and resultant chronic cholestasis in combination with cardiac(mainly peripheral pulmonary stenosis), skeletal, ocular, and facial abnormalities. In addition to the pulmonary stenosis, in large series, anecdotal reports of vascular lesions have concerned the renal artery, aorta, hepatic artery, carotid artery, celiac artery or subclavian artery. Theses diffuse... |
Five to fifteen percent of patients affected by human immunodeficiency virus(HIV) are children in developing countries. In Korea, most of HIV infections in children have been transfusion-related, and cases of vertical transmission have been very rare so far and are usually suspected due to a maternal positive history. We experienced a case of vertical transmission of HIV in a 19... |
Purpose : Malnutrition and failure to thrive have long been recognized as common systemic consequences of congenital heart disease(CHD). But there is little data about the prevalence and severity of malnutrition with CHD in Korea. We designed this study to determine the prevalence of malnutrition among hospitalized children with CHD. Methods : We reviewed 100 hospitalized children with CHD who were... |
Purpose : Liver transplantation(LT) is regarded as an important management option for fulminant hepatitis(FH), which is associated with considerable mortality under conservative management. The aim of this study was to evaluate the outcome of children with FH according to management. Methods : We reviewed medical records of patients presented with FH from January 1994 until April 1999. The children were... |
Purpose : The efficacy and safety of interferon-alpha(IFN-α) therapy in children with chronic hepatitis B(CHB) is similar to that of adults. However, little information is available about the predictive factors of response to this therapy in children. The aim of this study was to determine the predictive factors for responsiveness to IFN-α therapy in children with CHB. Methods : The basal... |
Ornithine transcarbamylase deficiency(OTCD), the most common inborn error of the urea cycle, is inherited in X-linked manner. In affected hemizygote males, OTCD manifests hyperammonemic coma that often leads to death during the newborn period. Our patient was at high risk for inborn error of urea cycle metabolism, since his two elder brothers died a few days after birth due to... |
Ceftriaxone, a third-generation cephalosporin, is a very potent, broad spectrum antibiotic commonly used in patients with meningitis, osteomyelitis, pyelonephritis, Lyme disease and many other severe infectious diseases. Up to 46% of those receiving this antibiotic develop gallbladder sludge. Most of them are asymptomatic, but a small proportion may develop right upper quadrant pain, nausea, vomiting and even cholecystitis. We report... |
Purpose : This study was done to evaluate the clinical and pathologic aspects of allergic colitis. Methods : This study evaluated 19 infants who presented with fresh blood mixed stools. Limited colonoscopy and biopsy were performed. Among the 19 infants, 13 infants were diagnosed as allergic colitis by histological findings. We analyzed the clinical and histological characteristics of 13 infants. Results : Male and female... |
Purpose : Endoscopic retrograde cholangiopancreatography(ERCP) may provide more definite diagnosis, preoperative guidance, and endoscopic therapy in many children with known or suspected disorders of the pancreas and biliary tract. But the reports about the ERCP in children are rare, so this study is conducted to evaluate the indication, efficacy and safety of the ERCP in children. Methods : Twenty nine ERCPs in 19 children... |
Purpose : The aim of this study is to evaluate the influence of the feeding (fastingpostprandial) and the state of alertness(awake-asleep) on the amount of gastroesophageal reflux by using 24hour esophageal pH metry. Methods : From July 1994 to June 1995, 32 cases of 24 hour esophageal pH metry had been done at the Asan Medical Center. Twenty three children with normal pH monitoring and... |
Purpose : Kawasaki disease(KD) is multi-systemic vasculitis and coronary artery abnormalities can develop up to 30 percent in untreated patients. Several observations suggest that immune activation and the secretion of cytokines from immune effector cells contribute to the pathogenesis of this disease. Though high dose intravenous gamma globulin(IVGG,>1 gm/kg) is effective in reducing the prevalence of coronary artery aneurysms in KD,... |
Purpose : We have experienced frequently the ischemic hepatitis after cardiac operations which shows rapid striking rise of transaminase. But the ischemic hepatitis in children after cardiac operation is not defined yet in Korea. We examined the clinical characteristics and analyzed the risk factors and outcome in 20 children who showed typical clinical course of ischemic hepatitis after cardiac operation. Methods... |
Purpose : Several trials in children for chronic hepatitis B have shown the efficacy of alpha interferon in eliminating hepatitis B virus replication, accompanied by an improvement in the liver cell damage. But reports about the alpha interferon therapy are rare in Korean children and there is no report about histologic changes even with adult. So we tried to... |
Statistical analysis of outpatients and inpatients from 1985 to 1988 was assessed with aims to have an overal grasp of patterns and trends of childhood diseases at Seoul National University Children, s Hospital. 1) Number of outpatient visits were totally 458,390 with annual number of visits of 144,754 and average daily number of 461, including 241 visits in Department of Pediatrics. 2) Number of patients at Eemergency... |
Seventeen patients who were diagnosed as primary hypophosphatemic rickets at Department of Pediatrics, Seoul National University Children’s Hospital were analyzed to assess its clinical aspect and effect of treatment, especially on height and growth velosity. The average age of onset was 2 years, and their chief complaints were bowleg and short stature. Sex ratio was l.l:l(male: female). Familial hypophoshatemic rickets was known as X-linked... |
Achalasia is an espohageal motility disorder. It is characterized by the loss LES relaxation on swallowing and of esophageal peristalsis and results in dysphagia, regurgitation and substernal pain. Manometry is one of several diagnostic tests of achalasia and reveals 1) Complete absence of esophageal peristalsis, 2) hypertension of LES. 3) impaired sphincter relaxation on swallowing and 4) positive esophageal body pressure compared to intragastric pressure. We... |