• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.964-970
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• 2008

Purpose : Methylmalonic aciduria (MMA) and propionic aciduria (PA) are inborn errors in the catabolism of branched-chain amino acids. The study was undertaken to investigate the genotypes and clinical features of Korean patients with MMA and PA. Methods : This study examined 12 patients with MMA and eight with PA. We analyzed various clinical features, laboratory findings, treatments, and neuro-developmental outcomes. Diagnoses were based on the presence of characteristic compounds detected by amino acid analysis in serum and organic acid analysis in urine. Mutation analysis was performed in the genes of MUT, MMAA, MMAB, and MMACHC for MMA and PCCA and PCCB for PA. Results : Among the 20 patients, six patients were diagnosed before one month of age and nine patients were diagnosed after the newborn period. Five patients were diagnosed via a neonatal screening test. Patients with early-onset forms had more severe illness at presentation and generally poor outcomes. A favorable outcome was obtained in 55% patients; most of them were of a late-onset type or diagnosed by neonatal mass screening test without symptoms. Genotypes were confirmed in all patients with MMA. We detected 11 different mutations by MUT gene analysis in 10 patients, and three different mutations in MMACHC genes in two patients. PCCA and PCCB gene mutations were identified in 14 of the 16 alleles, in eight patients with PA. Conclusion : Organic aciduria is a fatal disease; however, better outcomes are expected whenever early diagnosis and prompt management are made possible. Mutation analysis is useful for confirming diagnoses and planning management strategies.

• Pediatric Infection and Vaccine
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• v.17 no.2
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• pp.122-129
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• 2010

Purpose : Enteroviral infection is a common viral illness in children. We undertook this study in attempt to comprehend the epidemiologic and clinical features of enteroviral infections, particularly EV71 in children. Methods : We enrolled 63 children with enteroviral infection at Severance Children's Hospital in Seoul between May and August 2009. Reverse transcriptase-polymerase chain reaction (RT-PCR) was performed from stool or cerebrospinal fluid samples, which were then tested for enteroviral infection. Viral isolation and serotype identification also were performed by RT-PCR. Results : A total of 63 patients with clinical diagnosis of enteroviral infections were enrolled; of those, 38 (60%) were positive for enterovirus. The mean age of the patients was 2 years and 7 months and the sex ratio of male to female was 0.9 :1. Their clincal manifestations included aseptic meningitis (21 cases, 55%), HFMD (16 cases, 42%), herpangina (5 cases, 13%), neonatal fever (2 cases, 5%), encephalitis (1 case, 3%), and myocarditis (1 case, 3%). Serotypes of isolated enteroviruses were EV71 (8 cases, 21%), coxsackievirus B1 (8 cases, 21%), coxsackievirus A16 (2 cases, 6%), coxsakievirus A2 (1 case, 3%), coxsakievirus A5 (1 case, 3%), and echovirus 9 (1 case, 3%). Clinical symptoms of EV71 infection included HFMD (5 cases, 63%), aseptic meningitis (3 cases, 38%), encephalitis (1 case, 13%), and myocarditis (1 case, 13%). A positive rate of C-reactive protein in EV71 was higher than those in other enterviral infections. However, there was no statistically significant difference in other laboratory findings. Conclusion : We reported on identified enteroviruses, including EV71, during a period of 3 months in the summer of 2009. In this study, EV71 infection frequently occurred in male and clinical manifestation caused by EV71 was a more severe disease than that due to other enterviral infections. There is a need for continuous surveillance of enteroviral infection and its clinical manifestations for diagnosis and treatment of enteroviral infection.

• Pediatric Infection and Vaccine
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• v.17 no.1
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• pp.36-48
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• 2010

Purpose : In children on anticancer chemotherapy, bloodstream infections (BSIs) are a major cause of morbidity and mortality. We investigated febrile episodes and bloodstream infections in pediatric cancer patients to guide proper selection of empiric antibiotics for febrile pediatric hemato-oncologic patients. Methods : All febrile episodes treated in the division of hematology-oncology, the department of pediatrics, Hanyang University Hospital, between July 2005 and June 2008 were reviewed. Episodes with and without bloodstream infections were compared. Results : Forty cases (18.9%, 25 patients) of BSI occurred in 212 febrile episodes (63 patients). Thirty-seven cases (23.6%, 22 patients) of BSI occurred in 157 febrile episodes with neutropenia (54 patients). Microorganisms identified in BSI corresponded to 23 gram-positive bacteria (51.2%), 20 gram-negative bacteria (44.5%), and 2 fungi (4.4%). Rates of BSI between those who had received umbilical cord blood transplantation and those who had received transplantation from other source were significantly different (55.0% vs. 7.7%, P =0.001). No differences in mortality rate were observed among organisms in BSI patients. For febrile episodes the rate of BSI was higher among those with Chemoport than those with Hickman catheter (P =0.029) and gram-positive pathogens were more likely to be associated with Chemoport (P =0.001). Conclusion : The study showed the rate of BSI, distribution of pathogens with regard to neutropenia, transplantation, central venous catheters, and antimicrobial susceptibility of pathogens in order to help guide in the choice of optimal empiric antibiotics in pediatric febrile neutropenic hemato-oncologic patients.

• Pediatric Infection and Vaccine
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• v.20 no.1
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• pp.9-16
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• 2013

Purpose : This study aims to investigate preexposure prophylaxis and postexposure prophylaxis of rabies that the National Medical Center (NMC) handled and to check whether appropriate measures were performed according to the recent domestic and overseas guidelines after animal bites. Methods : This study surveyed 41 people who were 18 years or under and received preexposure and postexposure prophylaxis of rabies at the NMC from November 2006 to December 2011. Their medical records were reviewed for their age, gender, the reason for preexposure prophylaxis, the body sites of animal bite, the kind of the animal that bit children, the region where the biting occurred and rabies vaccination and inoculation of immunoglobulin. Results : Eleven children took rabies vaccination for preexposure prophylaxis and 30 children received post exposure prophylaxis of rabies. Of patients who were bitten by unvaccinated animals including wild animals or by animals which were not certain to be vaccinated, 50% (13 of 26 children) received postexposure prophylaxis, while 75% (3 of 4 children) of patients who were bitten by vaccinated animals received postexposure prophylaxis of rabies. Ten of 30 bitten patients knew whether or not the biting animals had received rabies vaccination. Of them, four people (40%) were bitten by animals which had received rabies vaccination. Conclusion : To prevent the occurrence of rabies, people and health care providers need to correctly understand latest guideline for rabies preexposure and postexposure prophylaxis and the information for bitten patient, biting animal and area at bitten by animal should be accurately recorded.

• Clinical and Experimental Pediatrics
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• v.48 no.12
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• pp.1324-1329
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• 2005

Purpose : The aim of this study was to evaluate recent improvements in the survival rate of extremely low birth weight (ELBW) infants and to identify perinatal and management factors that are associated with improved survival. Methods : Two groups of ELBW infants who were admitted to our neonatal intensive care unit (NICU) during two distinct eras : November 1994-December 1999 (Period 1 : n=100) and January 2000-April 2004 (Period 2 : n=166) were retrospectively reviewed. Results : Despite the younger gestational age and smaller birth weight of the ELBW infants in period 2, not only did their survival rate increased to 75 percent from 60 percent in period 1, but their incidence of morbidities such as bronchopulmonary dysplasia, confimed sepsis and intraventricular hemorrhage (${\geq}$Grade III) also declined. Factors significantly associated with improved survival included the use of antenatal steroids, a longer duration of nasal continuous positive airway pressure and the absence of intraventricular hemorrhage (${\geq}$Grade III). Conclusion : We believe that optimized clinical practice, that emphasized less invasive care, contributed to the recent improvements in the survival rate of ELBW infants.

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.757-762
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• 2006

Purpose : Pneumonia is one of the most common infections in children who visit emergency departments(ED), but standard clinical guidelines for children with pneumonia in Korea have not been studied. This study was performed to collect and evaluate a data-base of children with pneumonia for establishing clinical guidelines in ED. Methods : This study reviewed 304 children who were diagnosed and treated for pneumonia in the ED at one tertiary hospital between January 2003 and December 2003 retrospectively by reviewing the charts and analyzing the clinical characteristics, laboratory findings, and radiologic findings between an admission group and a discharge group. Results : The 2 year-5 year age group was the top of age distribution and the peak incidence of monthly distribution was December. Two hundred forty seven(81.3 percent) children were hospitalized(admission group), and the mean length of hospitalization was $7.24{\pm}3.24$ days. The most common indications of admission were fever, tachypnea and an age of less than three months. There was statistical differences in the outpatient department follow-up between the two groups(85.8 percent in admission group vs 35.1 percent in discharge group). Conclusion : More prospective studies are needed to establish clinical standard guidelines for children with pneumonia. This will be helpful in ED management and will aid the prevention of pneumonia.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.42-50
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• 2003

Purpose : The acute respiratory distress syndrome(ARDS), an acute form of severe alveolar-capillary injury evolving after a direct or indirect lung insult is thought to be a common cause of respiratory failure though not many clinical studies on the subject have been made yet. Methods : Between January 1992 and December 2001, we conducted a retrospective study on 33 children who fulfilled the definition of the ARDS recommended by the American-European Consensus Conference in 1994. Results : A total of 33 patients(20 boys and 13 girls) were selected. Their age ranged from 4 months to 12 years with seven children younger than 1 year. The overall mortality rate was 78.8% and no significant difference was noted based on age or sex. Concerning seasonal variation, incidence of the ARDS increased in spring, especially in May(21.2%). Pneumonia(66.7%) was found to be the most common risk factor of the ARDS followed by sepsis(24.2%) and aspiration(3.0%). In immune compromised children(six cases), including a recipient of bone marrow transplantation, the mortality rate was 100%. Compared with children with multiple organ failure recording a 83.3% mortality rate, those with isolated respiratory failure, showed a lower mortality rate of 33.3%, although stastistically insignificant. Between survivor and non-survivor groups, significant differences were shown in hematocrit, $PaO_2$, $PaCO_2$, PEEP, and $PaO_2/FiO_2$ on the seventh day after the onset of the ARDS. Conclusion : According to our study, respiratory failure proved to have a great effect on mortality rate in the ARDS. More aggressive intervention and further studies on this subject should be done to improve the survival rate.

• Clinical and Experimental Pediatrics
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• v.51 no.8
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• pp.834-841
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• 2008

Purpose : This study was perfomed to analyze in detail the viral etiology of acute lower respiratory tract infections (ALRI) in Cheunan, Korea by multiplex RT-PCR, including human rhinovirus (hRV) and newly identified viruses such as human metapneumovirus (hMPV) and human coronavirus (HCoV-OC43, HCoV-229E/NL63). Method : Nasopharyngeal aspirates (NPA) were collected from 863 hospitalized children with ALRI on the first day of admission at Soonchunhyang University Cheonan Hospital and analyzed by multiplex RT-PCR from December 2005 to November 2006. Results : Viral agents were detected from 474 subjects (54.9%). The identified viral pathogens were hRV 9.2%, hMPV 6.8%, HCoV-229E/NL63 1.4%, and HCoV-OC43 2.1%. Coinfections with ${\geq}2$ viruses were observed in 108 patients (22.8%). The major period of viral ALRI was the first year of life. Clinical diagnoses of viral ALRI were pneumonia (59.5%), bronchiolitis (24.7%), tracheobronchitis (11.4%), and croup (4%). The most common causes of bronchiolitis was respiratory syncytial virus B (RSV B), whereas hMPV, hRV, HCoV-229E/NL63, and HCoV-OC43 were commonly found in patients with pneumonia. The number of hMPV infections peaked between March and May 2006. HCoV-OC43 was prevalent from November to February 2006, whereas HCoV-229E and hRV were detected throughout the year. Conclusion : Although the study was confined to one year, hMPV was not detected during winter and peaked between March and April, which was not consistent with previous studies'. This present study indicates that HCoV is a less common respiratory pathogen in cases of ALRI in Korean children

• Clinical and Experimental Pediatrics
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• v.48 no.2
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• pp.178-185
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• 2005

Purpose : The purpose of this study was to evaluate the outcome of children with juvenile myelomonocytic leukemia(JMML) treated with allogeneic hematopoietic stem cell transplantation(allo-HSCT). Methods : Eleven JMML patients aged 8-39 months underwent allo-HSCT. The sources of grafts were unrelated donors(n=7), HLA-matched siblings(n=3) and an HLA 1-antigen mismatched familial donor. All patients had received chemotherapy ${\pm}13$-cis-retinoic acid(CRA) before transplant, and CRA was used, posttransplant, in six patients. Results : Only three patients were in complete remission(CR) at the time of transplantation. Initial chimeric status revealed complete donor chimerism(CC) in five patients, mixed chimerism(MC) in five and autologous recovery(AR) in one. One patient with MC having persistent splenomegaly eventually turned to CC and CR after rapid tapering of cyclosporine, combined with daily use of CRA. An AR case relapsed shortly after transplant but was rescued with second, unrelated cord blood transplantation. Ultimately, six patients are alive, event-free, with a median follow-up of 15.5 months posttransplant. All three deaths occurred in patients who failed to achieve CC, leading to disease progression. Conclusion : We suggest that graft-versus-leukemia effect play an important role and CRA a possible role in posttransplant leukemic involution in JMML. In patients whose leukemic burden is still high with MC after transplant, early tapering of immunosuppressants and introduction of CRA might provide a chance of a cure for some patients.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1332-1339
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• 2002

Purpose : Through a survey undertaken among doctors practicing pediatric patients in primary medical facilities on nutrition during infancy, the present study was done to determine whether these doctors were giving appropriate advice on infant nutrition. Methods : We visited pediatric specialists(group one) and non-pediatric specialists or general practitioners(group two) who were practicing in Daegu to ask them to fill out a questionaire on infant nutrition. Results : When 10 points were given to each question for a total of 100 points, the results of the evaluation showed that the score in group one was $73.0{\pm}15.8$ points and $45.1{\pm}18.4$ points in group two, showing a significant difference. No statistical difference was seen in the scores between non-pediatric specialists and general practitioners. Each answer to each question on the feeding period of breast milk, on loose stools during breast milk feeding, methods to deal with jaundice during breast milk feeding, choice of commercial baby food, criteria on directing soy milk, and criteria of guiding infant cereal was different between group one and two. Conclusion : The results of the present investigation showed that specialists in other fields or general practitioners were giving inappropriate advice on nutrition during the infant stage compared with pediatric specialists; thus, we need to confirm methods to prevent inappropriate consultations by specialists of other fields, together with continued education of pediatric specialists.