The generation of a draft sequence of the human genome has lead to an opportunity to characterize human diversity, including the differences in host response to numerous pathogens. Host responses upon exposure to a pathogen can determine the wide spectrum of illness from subclinical or mild to severe diseases. Host factors, particularly genetic backgrounds, influence the pathogenesis of infectious diseases.... |
Objective : A probable diagnosis of Wiskott-Aldrich syndrome(WAS) should be considered in any boy presenting with unusual bleeding, congenital thrombocytopenia and small platelets. The definitive diagnosis of WAS is usually made by the detection of WASP gene mutation or a decrease or absence of the WAS protein(WASP) in blood cells using molecular genetic analysis. However, these methods are too time-consuming... |
Persistent pulmonary hypertension of the newborn(PPHN) is a disorder characterized by persistence of the pattern of fetal circulation after birth due to a sustained elevation of pulmonary vascular resistance. The two primary events in the pathophysiology of neonatal pulmonary hypertension are hypoxic pulmonary vasoconstriction(HPV) and hypoxic pulmonary vascular remodeling(HPR). Chronic hypoxemia may cause increased muscularity of the pulmonary arteries and... |
Purpose : Acute abdominal pain in an epidemic aseptic meningitis which is mostly an enterovirus as causative agent, is noted in 23-55% of patients. An enterovirus is also known as one of the causes of acute nonspecific mesenteric lymphadenitis(ANML). The purpose of this study was to see if ANML was associated with acute abdominal pain in epidemic aseptic meningitis. Methods :... |
In Kawasaki disease, arthritis may occur, generally affecting the hands, knees, ankles, or hips. Operations have not usually been needed. We report a case of Kawasaki Disease with multiple septic arthritis which was caused by S. pneumoniae and which needed athrotomy with drainage for both hips, both knees, and both ankle joints. A two year, ten-month-old girl was admitted to... |
Tuberous sclerosis(TS) is known to be associated with multi-organ involvements, such as neurologic, renal and cardiac abnormalities. Abdominal aortic aneurysms in children and young adults are uncommon, however, some have been observed in patients with TS. We present an 8 month-old female patient with TS who developed aortic aneurysm and underwent successful surgical management. Since an aortic aneurysm is potentially... |
Purpose : Kawasaki disease is a multisystemic inflammatory vasculitis and associated with neurologic features such as aseptic meningitis. High-dose intravenous immunoglobulin(IVIG) is used to treat Kawasaki disease and generally considered to be safe, but rare cases of aseptic meningitis with unknown etiology have been reported. The aseptic meningitis associated with Kawasaki disease was compared with meningitis as the adverse reaction... |
Purpose : This study was undertaken to find out a useful method that can predict the true leukocytosis in blood-contaminated cerebrospinal fluid(CSF) due to traumatic lumbar puncture(LP), using white and red cell counts of sanguineous CSF and peripheral blood. Methods : From May 2001 to December 2002, among 1,247 patients presenting symptoms of meningitis who had received LP at Chonnam Hospital,... |
Purpose : The cause of aseptic meningitis remains mostly unknown because viral culture and identification is difficult. Thus, we report a study on 123 children with aseptic meningitis in Gyeongju in 2002 to identify the causing virus and the relationship with the clinical manifestation. Methods : We prospectively investigated the patients, admitted to Dongguk University Hospital, into two groups between April... |
Purpose : To evaluate the accuracy of ultrasonography(USG) and abdominal computerized tomography(CT) and the possibility of reducing unnecessary appendectomy rate with improvement of diagnostic accuracy using optional CT with USG. Methods : From January 1999 to May 2003, 496 patients younger than 14 years received surgical intervention with preoperative impression of acute appendicitis. They were devided into three groups no... |
Purpose : Nephrocalcinosis in very low birth weight(VLBW) infants were known to be caused by a longer duration of furosemide use. However, etiologies, pathogenesis and risk factors remain unclear. Therefore, we examined the incidence and risk factors of nephrocalcinosis in VLBW infants retrospectively. Methods : Inborn babies of birth weights less than 1,500 gm were examined retrospectively. Data were reviewed on... |
Cutis verticis gyrata(CVG) is a morphologic syndrome of the scalp characterized by folds and furrows resembling the surface of brain. The primary form of cutis verticis gyrata is commonly associated with a neuropsychiatric condition and a secondary form occurs as a response of inflammatory or neoplastic process. The patient was a 1-day-old girl, who had a 15?1 cm sized, convoluted... |
Hemolytic uremic syndrome is the most common cause of acute renal failure in childhood. Atypical hemolytic uremic syndrome which is not usually associated with prodromal symptoms has a higher mortality rate and more tendency to progress to chronic renal failure. Children younger than two years of age are a more susceptible to pneumococcal infection and can develop more serious disease.... |
Purpose : To know the body handling properties and anti-proteinuric effect of cyclosporine A(CsA) in children with renal diseases, 34 patients with nephrotic syndrome or glomerular diseases were included to treatment trials and evaluated. Methods : Microemulsion formula CsA, 5 mg/kg/day was administered orally in two divided doses for 9.3?.6 months. Pharmacokinetic studies of CsA were done twice at beginning and... |
Purpose : There has been no exact criteria established for when to discontinue antiepileptic drugs (AEDs) in epileptic children who had been well controlled for a long period. This study was undertaken to evaluate the recurrence rate and predictive risk factors of relapse after discontinuation of AEDs in epileptic children who had been seizure-free. Methods : We retrospectively studied 294 children... |
Purpose : In 1970, the Berlin-Frankfurt-Munster(BFM) group introduced an intensification therapy after remission induction to reduce relapse of acute lymphoblastic leukemia(ALL) in childhood. Delayed intensification(DI) phase has been included for treatment of ALL in our hospital since the mid-1990s. The purpose of this study is to evaluate the outcome with vs. without DI phase and the outcome with two vs.... |
Purpose : We assessed the dynamic characteristics of 16 nebulizer/compressor combinations currently available in Korea. Methods : The 16 nebulizer/compressor combinations(Pariboy Type 38/Long life, Pariboy Type N/ Long life, Pariboy Type N/Salter 8900, Pariboy Type N/LC, Devilbiss pulmoaid-LT/Hudson, Devilbiss pulmoaid/Hudson, Mesmed neb-300/Own, San-up 3040/Hudson, Midas(Basic)/Own, AirJolie 2/ Hudson, Thomas 1127/Salter 8900, Noel NE-2000/Salter 8900, Omron CX3/Hudson, Chang Woo CWN-100/Salter 8900, Voyage/Mefar,... |
Human parvovirus(HPV) B19 infection causes erythema infectiosum in children, sometimes red cell aplastic crisis with hemolytic anemia and chronic bone marrow failure in immunocompromised hosts. HPV B19 is directly cytotoxic for erythroid progenitor cells and inhibits erythropoiesis. Infrequently, HPV B19 inhibits hematopoiesis of three cell lineages and causes transient pancytopenia in patients with hemolytic disorders. We report three patients with... |
Purpose : Children with acute colonic diverticulitis(ACD), can be misdiagnosed with acute appendicitis. Methods : We reviewed 15 cases of ACD during five years, from January 1998 to June 2002 retrospectively. Results : Most patients(80%) with ACD in children presented with right lower quadrant pain. The primary diagnosis on admission was mostly acute appendicitis(87%), and all ACD in children occurred in... |
Hemophagocytic lymphohistiocytosis is a reactive disorder characterized by a generalized non-malignant histiocytic proliferation with prominent hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems resulting in pancytopenia and liver dysfunction. Several diseases including infection, malignancy and autoimmune disease are known to be causative disorders. This case demonstrated histiocytic hemophagocytosis in the bone marrow, resulting in pancytopenia during treatment... |
Canavan disease, also known as van Bogaert-Bertrand disease, is a rare autosomal recessive disorder characterized by early an onset and a progressive spongyform degeneration of the brain, associated with an edema of the central nerve system, intramyelinic swelling and neurologic symptoms. This disorder is most prevalent in people of Ashkenazi Jewish descent but has been observed in other ethnic groups.... |
Purpose : To determine the clinical association of diagnostic criteria and the prevalence of autoantibodies in newly diagnosed children with juvenile dermatomyositis(JDM). Methods : Thirty-two children with JDM were identified at Seoul National University Children's Hospital from March 1985 to March 1999 by retrospective review. The diagnosis of JDM was based on the criteria proposed by Bohan and Peter. We investigated... |
Purpose : Benign childhood epilepsy with centrotemporal spikes(BECT) is characterized by an excellent prognosis. Therefore, the necessity for the use of antiepileptic drugs is controversial. The object of this study is to know about the long-term follow-up of BECT, comparing daily treatment with antiepileptic drug(AED) versus no medication. Methods : We retrospectively studied 56 cases of BECTs, examined at Pediatric Neurology... |
Purpose : This study aimed to evaluate risk factors of the first year relapse in children with nephrotic syndrome(NS) without the need for biopsy. Methods : We reviewed, retrospectively, 78 children diagnosed with steroid responsive nephrotic syndrome between July 1997 and June 2002. Median years to follow up were 4.4 years(range : 1-5 years). We divided the patients into two groups(group... |
Purpose : The etiology of hemolytic anemia can be classified as either cellular or extracellular defects of red blood cells. The aim of this study was to investigate the clinical and laboratory findings of hemolytic anemia concerning its etiological classification. Methods : Clinical and laboratory findings of the patients with hemolytic anemia treated from January 1987 to May 2002 at Severance... |
Purpose : There was a outbreak of aseptic meningitis in Busan, 2002. We report the clinical features and causative viruses. Methods : Two hundred seventy six children with aseptic meningitis who were admitted to the Department of Pediatrics, Maryknoll Hospital between January and December, 2002 were included. CSF, stool and throat swab viral cultures were done in 244 of these... |
ARCI syndrome consists of arthrogryposis, renal tubular acidosis, cholestatic jaundice and icthyosis. We experienced an ARCI syndrome case with corpus callosum hypoplasia and atrial septal defect. This case had oral feeding difficulty, multiple joint contracture, renal tubular acidosis and neurogenic muscular atrophy at neonatal period. At two months of age, icthyosis and cholestatic jaundice were diagnosed. The case was hospitalized... |
Purpose : The aim of this study was to evaluate the effect of inhaled nitric oxide(iNO) on gas exchange, hemodynamics and pulmonary inflammation in newborn piglets with E. coli induced septic lung. Methods : Twenty three instrumented and ventilated piglets were randomized into three groups : CON(n=6), PCON(n=9), and PNO(n=8). In the piglets of the PCON and PNO groups, E. coli... |
The isoimmune hemolytic disease of newborn due to the incompatibility of minor blood groups is characterized by progressive neonatal hyperbilirubinemia and anemia caused by the IgG antibody transmitted from the mother to the fetus. Recently we had a case of hemolytic disease in a newborn due to anti-Jkb. There were no ABO and Rh(D) incompatibilities between mother and baby. The... |