Hereditary hemorrhagic telagiectasia (HHT), which is characterized by the classic triad of mucocutaneous telangiectases, arteriovenous malformations (AVMs) and inheritance, is an autosomal dominant disorder. The characteristic manifestations of HHT are all due to abnormalities of the vascular structure. This report deals with the case of a 14-year-old girl with typical features of HHT that include recurrent epistaxis, mucocutanous telangiectases, pulmonary... |
Purpose : We tried to assess the optimal conditions to improve low transduction efficiency and their effect on target cells. Methods : Cultured NIH 3T3 cells were incubated with retroviral vectors bearing an enhanced green fluorescent protein (eGFP) gene. We varied the ratio of viral vectors to target cells (1:1-1:8) and the number of transfections (X1, X2), and compared transduction... |
Purpose : To determine the optimal time of high dose intravenous immune globulin (IVIG) treatment, we analysed the clinical characteristics and progress of a group of Kawasaki disease patients who had early treatment with IVIG. Method : A retrospective study was conducted of 188 patients with Kawasaki disease who were admitted to Yeungnam University Medical Center from January 2000 to December... |
Purpose : Kawasaki disease is a systemic vasculitis, leading cause of pediatric acquired heart disease. Vascular endothelial growth factor (VEGF) has functions as vascular permeability factor, plays an important role in coronary artery lesion (CAL). We studied the clinical significance of serum VEGF in Kawasaki disease. Methods : Kawasaki group was 49 patients, and control group was 15 patients. Diagnosis followed... |
Purpose : This study investigated the clinical significance of AN in children and adolescents with obesity induced metabolic complications. Methods : Forty-nine patients who had obesity induced metabolic complications were participated in this cross-sectional study. Obesity induced metabolic complications are as follows: hypertension, dyslipidemia, impaired fasting glucose (IFG), impaired glucose tolerance (IGT), nonalcoholic steatohepatitis (NASH), homeostasis model assessment of insulin... |
Purpose : Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. Methods : Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels... |
Purpose : Aortic valve or aortic root (AoRo) replacement is occasionally required because of AoRo dilatation and aortic regurgitation (AR) in repaired tetralogy of Fallot (TOF). We evaluated AoRo size and possible factors associated with its hemodynamic nature in patients with repaired TOF. Methods : We investigated 130 repaired TOF patients more than 15 years of age who followed-up by... |
Purpose : Accurate measurement of defect size is important in transcatheter closure of atrial septal defect (ASD). We performed this study to analyze the difference between the measured ASD size and balloon occlusive diameter (BOD) by transthoracic (TTE) or transesophageal echocardiography (TEE). Methods : We investigated 78 patients who underwent transcatheter closure of ASD. The defect size and the distance between... |
Purpose : Measurement of antistreptolysin O (ASO) is often necessary to confirm a clinical diagnosis of recent streptococcal infection, especially in patients suspected of rheumatic fever and acute glomerulonephritis. Standard normal ranges for ASO should be established locally for each age group. We analyzed ASO to determine the upper limit of normal (ULN) ASO in children in the Gyeonggi-Incheon area. Methods... |
Purpose : The purpose was to investigate the clinical considerations of patients affected by meconium aspiration syndrome (MAS) and the effect of suctioning through endotracheal intubation immediately after delivery on the prognosis of MAS. Methods : A total of 44 inpatients diagnosed as MAS in the Neonatal Intensive Care Unit (NICU) at Cheil Hospital were selected from January 2004 to... |
Purpose : The purpose of this study was to identify factors influencing premature infants who are small for their gestational ago. Methods : The medical records of 1,010 premature infants of 26 to 35 weeks of gestational age born at Il-Sin Christian Hospital, Busan from January 2000 to August 2006 were reviewed. We collected data on gestational age, birth weight, infant... |
Acute renal failure is the generic term for an abrupt and sustained decrease in renal function resulting in retention of nitrogenous and non nitrogenous waste product. This may results in life threatening consequences including volume overload, hyperkalemia, and metabolic acidosis. Acute renal failure is both common and carries high mortality rate, but as it is often preventable, identification of patients... |
Many dialysis modalities such as peritoneal dialysis (PD), hemodialysis (HD) and continuous hemofiltration or hemodialysis (CRRT) are available for the management of pediatric patients with acute renal failure (ARF). PD is a relatively simple, inexpensive modality and can be used in hemodynamically unstable patients. But, it may not be the optimal therapy for patients with severe volume overload or life... |
The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however,... |
Pulmonary venous stenosis may be congenital or acquired. Regardless of its origin, the prognosis for patients affected with PVS remains poor. There have been many attempts to palliate PVS with little success. This report describes two patients with PVS which became evident after repair of total anomalous pulmonary venous connection. Intravascular stents were successfully implanted, but progressive restenoses in the... |
Purpose : Breast milk contains several components that provide specific immunity and affect the maturation of the infant's immune system. Allergic disease (AD), including atopic eczema, asthma, allergic rhinitis, and food allergy is characterized by an imbalance between cytokines produced by distinct T-helper cell subtypes. The aim of the study was to investigate the concentrations of cytokines and chemokines that... |
Purpose : Ghrelin, being secreted from the stomach, stimulates growth hormone secretion and controls energy homeostasis by increasing appetite. Leptin, being secreted from the adipocytes, controls weight and energy homeostasis by decreasing appetite. Leptin concentration is reported to increase after childhood cancer therapy. This study was aimed to compare ghrelin and leptin concentrations in normal children and children who received... |
Purpose : Reversible posterior leukoencephalopathy syndrome is a complex disorder with characteristic clinical and radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. The purpose of this study was to determine its clinical and radiological characteristics. Methods : A total of 15 pateints were involved in the study. Their medical records and radiological features of brain MRI were... |
Purpose : The aim of this study was to investigate the speech problems in benign rolandic epilepsy (BRE) according to the seizure focus in EEG and semiology. Methods : Twenty three patients [right origin (13 patients) or left side (10 patients)] who met the BRE criteria by International League Against Epilepsy (ILAE) were prospectively enrolled. We excluded the patients who... |
Purpose : Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system and mostly develops after viral illness or vaccinations. We investigated the clinical differences and neurologic outcomes according to the distribution of the lesions on brain MRI. Methods : The study group was composed of 21 patients from January 1995 to August 2003 in Kyunghee University hospital.... |
Purpose : Preterm very low birth weight infant have high rate of adverse neurodevelopmental sequale. Recently, there have been lots of reports that human umbilical cord blood transplantation ameliorates functional deficits in animal models as hypoxic ischemic injury. This pilot study was undertaken to determine the clinical efficacy and safety of autologous umbilical cord blood cell transplantation for preventing neurodevelopmental... |
Purpose : Chromosome analysis is important in genetic study and genetic counseling. This study was performed to evaluate the type and incidence of chromosome abnormalities in a single hospital for 25 years. Methods : Chromosome analyses were performed on peripheral blood lymphocytes, obtained from 4,856 patients with suspected chromosomal aberrations, referred to cytogenetic laboratory in Department of Pediatrics, Kyungpook National... |
Purpose : We performed this study to investigate the perinatal and developmental features of the patients with congenital myotonic dystrophy (CDM) confirmed by the molecular genetic method and the clinical characteristics of their mother, and to identify the relation between the number of CTG repeats and the clinical severity. Methods : A retrospective review of the medical records and the results... |
Purpose : The impulse oscillometry (IOS) is applicable to young children because it requires minimal cooperation and a non-invasive method to measure the mechanics of respiratory system. This study aimed to develop the reference values in school-aged children in Korea, using IOS which is a modification of forced oscillation technique (FOT). Methods : Measurements were performed in 92 previously untrained healthy... |
Purpose : The most common causes of acute viral gastroenteritis in newborn period are rotavirus, astrovirus, norovirus and enteric adenovirus. This study was designed to investigate the clinical characteristics, clinical symptoms, isolation rate and distribution of these viruses in full-term neonates during neonatal period. We also studied the influence on the viral isolation rate by postnatal care place and feeding... |
Purpose : The present study examined the etiology and risk factors of the early breast-feeding jaundice and the usefulness of auditory brainstem response test as early predictor of kernicterus. Methods : Medical records of neonatal jaundice in newborn admitted to Daegu Fatima Hospital between September 2005 and May 2006 were analyzed prospectively. Infants were grouped according to feeding method : breast... |
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... |
Any infant noted to be jaundiced at 2 weeks of age should be evaluated for cholestasis with measurement of total and direct serum bilirubin. With the insight into the clinical phenotype and the genotypephenotype correlations, it is now possible to evaluate more precisely the neonate who presents with conjugated hyperbilirubinemia. Testing should be performed for the specific treatable causes of... |
Interferon (IFN) alpha has been the first line therapy of chronic hepatitis B in children, but HBeAg seroconversion occurred in 26% of treated children compared to 11% of controls in multinational randomized controlled study. Recently, lamivudine was shown to be a potent inhibitor of Hepatitis B virus (HBV) reproduction both in HBeAg positive and in HBeAg negative (the pre-core mutant... |
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