Mixed connective tissue disease (MCTD) is characterized by diverse symptoms including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, and dermatomyositis, associated with high titers of antibodies to extractable nuclear antigen (ENA), especially anti-ribonucleoprotein (anti-RNP) antibody. Since the first report of 25 cases with MCTD in adults, there have been only a few cases of MCTD reported in children. Here, we report... |
The economic recession of North Korea has been prolonged, the need for humanitarian assistance for the women and children of DPRK has been raised. In March 2006, South Korean government signed MOU with World Health Organization (WHO) to financially support "Improving Women's and Children's Health in DPRK (IWCH)" project. The assistance projects through UNICEF and the non-government organizations of South... |
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... |
Purpose : Allergic proctocolitis is a major cause of bloody stool in early infancy. This study was aimed at ascertaining the clinical courses, sigmoidoscopic and histologic findings of allergic proctocolitis. We also analyzed the relationship between peripheral eosinophilia, the age at symptom onset, and sigmoidoscopic and histologic findings. Methods : We reviewed 25 infants retrospectively who had sigmoidoscopy and biopsy... |
The mission of National Health Screening Program for Infant and Children is to promote and improve the health, education, and well-being of infants, children, families, and communities. Although the term 'diagnosis' usually relates to pathology, a similar diagnostic approach applies to the child seen primarily for health supervision. In the case of health, diagnosis determines the selection of appropriate health... |
Purpose : It has been known that breast milk cause prolonged unconjugated hyperbilirubinemia. UGT1A1 is a important gene of uridine diphosphate glucuronosyltransferase (UGT) which has a major role of bilirubin metabolism. These findings suggest that there is a relationship between UGT1A1 gene mutation and prolonged jaundice of breast feeding infant. The aim of study was to investigate whether a polymorphism... |
of microvilli, but also separated the intercellular gaps and linear ZO-1. PAN induced oxidative stresses in time and dose dependent manners and increases of intercellular permeability in anti-oxidants inhibitable manners. High concentration of PAN induced not only actin polymerization and disorganization, but also the conglomerulation and internal dislocation of α-actinin protein. The intensities of fluorescences of ZO-1 protein were diminished... |
Purpose : The serum levels of total insulin-like growth factor (IGF)-I and IGF binding protein (IGFBP)- 3 reflect endogenous growth hormone (GH) secretion in healthy children. Free form of IGF-I which is suggested to have more potent biological action than complex form of IGF-I. The aim of this study is to investigate the serum levels of free IGF-I and its... |
Purpose : Apolipoprotein E (Apo E) plays a major role in lipoprotein metabolism and lipid transport. Many investigators have described that Apo E polymorphisms is one of the most important genetic determinants for cardiovascular disease. The purpose of this study was to evaluate the association between Apo E polymorphisms and serum lipid profiles in obese adolescent. Methods : We measured... |
Purpose : Juvenile dermatomyositis (JDM) is the most common of the idiopathic inflammatory myopathies in children. The purpose of this study is to observe demographic, initial presentations, duration of time between disease onset and diagnosis, clinical manifestations and laboratory findings at diagnosis of patients with JDM. Methods : Forty seven patients identified at Seoul National University Children's Hospital from January 1986... |
Purpose : Since 1998, school urinary screening tests have been performed on Korean school children. We could detect and treat so many asymptomatic chronic renal disease in early stage. We investigated the efficacy of school urinary screening tests from children with membranoproliferative glomerulonephritis (MPGN) type I. Methods : We analyzed the characteristics and prognosis of 18 patients with MPGN type I... |
Purpose : Acute symptomatic seizure is defined as a temporary seizure together with acute systemic, metabolic, or toxic insult in association with an acute central nervous system insult. And unprovoked seizure is defined as seizure without provocating factors. We studied the risk factors of unprovoked seizures after acute symptomatic seizure in children. Methods : We retrospectively reviewed the records of... |
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,... |
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 : 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... |
We report on two premature infants who developed nosocomial infection caused by Chryseobacterium meningosepticum in a neonatal intensive care unit (NICU). One premature infant developed sepsis, meningitis, and hydrocephalus, and was treated successfully with ciprofloxacin plus trimethoprim-sulfamethoxazole combination therapy for 4 weeks and with a ventriculoperitoneal shunt. The other premature infant, who was in a chronically debilitated state, had infection... |
Purpose : The purpose of this study was to investigate the clinical features and outcome in newborns undergoing cardiac surgery. Methods : Eighty two neonates underwent heart surgery for congenital heart defect at Kyungpook National University Hospital between March 2000 and February 2006. Patient characteristics (sex, age, diagnosis), pre-operative conditions, operation type, postoperative complications and mortality were reviewed retrospectively. Results : In... |
Purpose : Fluconazole prophylaxis for very low birth weight (VLBW) infants has been shown to reduce invasive fungal infection and its mortality. This study aims to evaluate the effect of fluconazole prophylaxis in VLBW infants on the incidence and mortality of fungal infection. Methods : VLBW infants with endotracheal intubation and central vascular access admitted to the Neonatal Intensive Care... |
Purpose : The purpose of this study was to determine the incidence and potential predictors of weight gain in older children and teens treated with valproate (VPA) for epilepsy. Methods : Sixty-five subjects aged 8 to 17 years of age, who began VPA treatment between January 1, 2001, and December 31, 2004, and who had documented weight and height measurements... |
Purpose : To evaluate the potential prognostic value of the antithrombin-III (AT-III) level in the children with acute lung injury (ALI), we analyzed several early predictive factors of death including AT-III level at the onset of ALI and compared the relative risk of them for mortality. Methods : Over a 18-month period, a total of 198 children were admitted to our... |
Even though we drink and excrete water without recognition, the amount and the composition of body fluid remain constant everyday. Maintenance of a normal osmolality is under the control of water balance which is regulated by vasopressin despite sodium concentration is the dominant determinant of plasma osmolality. The increased plasma osmolality (hypernatremia) can be normalized by the concentration of urine,... |
Purpose : Perinatal hepatitis B viral infection is decreasing; however, 10% of babies to HBeAg positive mothers still become chronic carriers despite perinatal prophylaxis. Although, the cause of prophylaxis failure is still unclear, an importance of maternal HBV-DNA level at the delivery time has been suggested. This study was established to certify if it would be a useful predictable factor... |
Purpose : Bell's palsy is defined as an idiopathic facial nerve paralysis of sudden onset. In spite of intensive clinical and experimental investigation, there is still uncertainty in the incidence, etiology, and preferred mode of treatment in children. The objective of this study was to analyze clinical outcome and prognosis of children with Bell' palsy. Methods : We analyzed 61 cases... |
Purpose : Congenital muscular torticollis (CMT) is a common and benign congenital disorder of the musculoskeletal system in neonates and infants. The pathophysiology is that the sternocleidomastoid muscle (SCM) is shortened on the involved side by fibrosis, leading to ipsilateral tilt and contralateral rotation of the face and chin. In this study, we investigated the clinical features of CMT, the... |
Purpose : Clinicopathological features were investigated to clarify the outcome and prognostic indicators for patients with IgA nephropathy in Korean children. Methods : We reviewed the outcomes of 61 patients in whom IgA nephropathy was diagnosed before the age of 15 years from 1991 to 2005 and followed-up at least for one year. All patients were confirmed by renal biopsy. Results... |
Fever of unknown origin (FUO) has been a convenient term used to classify patients who warrant a particular systemic approach to diagnostic evaluation and management. The greatest clinical concern in evaluating FUO is identifying patients whose fever has a serious or life-threatening cause when a delay in diagnosis could jeopardize successful intervention. Thorough history and complete physical examination are critical... |
Purpose : The reticuloendothelial system is composed of sinusoidal capillaries, through which even large protein molecules are freely movable between plasma and interstitial space, including the lymphatic system. Therefore, high-dose intravenous immunoglobulin (IVIG) would cause a redistribution of proteins between two compartments. To investigate this hypothesis, we measured plasma protein and lipid levels in patients with Kawasaki disease before and... |
Purpose : This study was conducted to compare the efficacy and safety of ibuprofen and indomethacin in the treatment of patent ductus arteriosus (PDA) in preterm infants and to determine whether ibuprofen can be an alternative drug. Methods : A total of 32 preterm infants with symptomatic PDA were enrolled in the study. Twelve infants received intravenous ibuprofen 10 mg/kg, followed... |
Sideroblastic anemia is a rare, heterogeneous group of disorders characterized by hyperferremia, microcytic hypochromic anemia, and bone marrow erythroid hyperplasia with the presence of numerous ringed sideroblasts. We describe herewith the case of a rare coincidence of sideroblastic anemia and mitral valve prolapse with resultant regurgitation in a 2-year-old boy. In addition to the inherent propensity for the development of... |
Purpose : To evaluate various sepsis screening tests, individually and in combination, to formulate a guideline for the diagnosis of neonatal sepsis. Methods : The study was a retrospective cohort study. It took place at the neonatal intensive care unit of the Paediatric Department, Il Sin Christian Hospital, Busan, Korea, over a period of 68 months from 1st, April, 2001 to... |