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Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3. We were unable to identify the cause for the HLH. He remained symptom-free until the development of back pain and right ankle joint pain with swelling at 15 years of age. He was diagnosed with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom aggravation despite taking naproxen and methotrexate, and the symptoms improved with etanercept. On admission, his laboratory data showed leukopenia with high ferritin and triglyceride levels. Bone marrow biopsy examination showed histiocytic hyperplasia with hemophagocytosis. There was no evidence of infection. He received naproxen alone, and his symptoms and laboratory data improved without any other immunomodulatory medications. Genetic study revealed no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune disease should have been considered as the cause of recurrent MAS in the young patient once primary HLH was excluded.

PurposeMycoplasma pneumoniae (MP) pneumonia epidemics have occurred in 3- to 4-year cycles in Korea. We evaluated the epidemiologic characteristics of MP pneumonia in Daejeon, Korea, from 2003 to 2012.MethodsWe retrospectively analyzed 779 medical records of children (0-15 years of old) with MP pneumonia admitted to our institution and compared the data from 3 recent epidemics.ResultsIn 779 patients, the mean age and male-to-female ratio were 5.0±2.2 years and 1:1, and most cases were observed in autumn. There were three epidemics during the study period, in 2003, 2006-2007, and 2011. In our comparison of the three epidemics, we found no differences in mean age, the male-to-female ratio, hospital stay, or the rate of seroconverters during hospitalization. All three epidemics began in early summer and peaked in September 2003 and 2011 and in October 2006 and then gradually decreased until the next year's spring season, although the 2006 epidemic extended further into 2007. The peak age groups in the children in 2003 and 2006 were 3-6 year-olds (57.5% and 56%, respectively), but in the 2011 epidemic, the peak group was 1-4 year-olds (46.5%). The proportion of the 10 years of age group was 5.2%, 13.8%, and 14.8% of total patients, respectively.ConclusionMP pneumonia outbreaks occurred every 3-4 years. The pattern of 3 recent epidemics was similar in demographic characteristics and seasonality with some variations in each outbreak.

Hemorrhagic cystitis is a common stem cell transplantation-related complication. The incidence of early-onset hemorrhagic cystitis, which is related to the pretransplant conditioning regimen, has decreased with the concomitant use of mesna and hyperhydration. However, late-onset hemorrhagic cystitis, which is usually caused by the BK virus, continues to develop. Although the BK virus is the most common pathogenic microorganism of poststem cell transplantation late-onset hemorrhagic cystitis, pediatricians outside the hemato-oncology and nephrology specialties tend to be unfamiliar with hemorrhagic cystitis and the BK virus. Moreover, no standard guidelines for the early diagnosis and treatment of BK virus-associated hemorrhagic cystitis after stem cell transplantation have been established. Here, we briefly introduce poststem cell transplantation BK virus-associated hemorrhagic cystitis.

PURPOSE Serum level of 25-hydroxyvitamin D (25-OHD) is considered as the most appropriate marker of vitamin D status. However, only a few studies have investigated the relationship between 25-OHD and parathyroid hormone (PTH) in children. To this end, this study was aimed at evaluating the lowest 25-OHD level that suppresses the production of parathyroid hormone in children. METHODS A retrospective record review was performed for children aged 0.2 to 18 years (n=193; 106 boys and 87 girls) who underwent simultaneous measurements of serum 25-OHD and PTH levels between January 2010 and June 2014. RESULTS The inflection point of serum 25-OHD level for maximal suppression of PTH was at 18.0 ng/mL (95% confidence interval, 14.3-21.7 ng/mL). The median PTH level of the children with 25-OHD levels of

Kikuchi-Fujimoto disease (KFD) is a benign self-limiting disease characterized by fever and lymphadenitis. The etiology and pathogenesis of KFD is unclear. However, two hypotheses have been suggested: a viral infection hypothesis and an autoimmune hypothesis. Several KFD patients with various types of autoimmune diseases have been reported, and these reports support the hypothesis for autoimmune pathogenesis of KFD. Here, we report the case of a 17-year-old female patient diagnosed with KFD and autoimmune thyroiditis. This case serves as additional evidence that the etiology of KFD is autoimmune origin.

PurposeWe phylogenetically analyzed the Escherichia coli strains isolated from children with urinary tract infection (UTI) in 2 regions of Korea. Virulence factors (VFs) and antibiotic resistance of the strains were also determined to compare the possible differences.MethodsA total of 138 E. coli strains were collected from the 2 regions; Gyeongin (78 strains) and Gyeongnam (60 strains). The phylogenetic groups were determined using the triplex polymerase chain reaction (PCR) method and multiplex PCRs were used to detect 7 VFs genes (fimH, papC, iutA, hlyA, sfa/focDE, afa/draBC, and kpsMT II). We also tested for antibiotic resistance.ResultsPhylogenetic groups, B2 (61.6%) and D (26.8%), comprised the majority of all isolated strains. Regional comparisons revealed that more B2 strains and fewer non-B2 (A+B1+D) strains were found in Gyeongnam, than in the Gyeongin region (P=0.033), and certain VFs were predominantly detected in Gyeongnam (P

Purpose Enterovirus 71, one of the enteroviruses that are responsible for both hand-foot-and-mouth disease and herpangina, can cause neural injury. During periods of endemic spread of hand-foot-andmouth disease caused by enterovirus 71, CNS infections are also frequently diagnosed and may lead to increased complications from neural injury, as well as death. We present the results of our epidemiologic research on the clinical manifestations of children with CNS infections caused by enterovirus 71. Methods The study group consisted of 42 patients admitted for CNS infection by enterovirus 71 between April 2009 and October 2009 at the Department of Pediatrics of 5 major hospitals affiliated with the Catholic University of Korea. We retrospectively reviewed initial symptoms and laboratory findings on admission, the specimen from which enterovirus 71 was isolated, fever duration, admission period, treatment and progress, and complications. We compared aseptic meningitis patients with encephalitis patients. Results Of the 42 patients (23 men, 19 women), hand-foot-and-mouth disease was most prevalent (n=39), followed by herpangina (n=3), upon initial clinical diagnosis. Among the 42 patients, 15 (35.7%) were classified as severe, while 27 (64.3%) were classified as mild. Factors such as age, fever duration, presence of seizure, and use of intravenous immunoglobulin (IVIG) were statistically different between the 2 groups. Conclusion Our results indicate that patients with severe infection caused by enterovirus 71 tended to be less than 3 years old, presented with at least 3 days of fever as well as seizure activity, and received IVIG treatment.

Listeria monocytogenes is a facultative anaerobic, gram-positive bacillus that is isolated from the soil, vegetables, and wild or domestic animals. Listeria occurs predominantly in the elderly, immunocompromised patients, pregnant women and newborns. Infections by this microorganism are rare in healthy infants and children. L. monocytogenes may cause meningitis, meningoencephalitis, brain abscess, pyogenic arthritis, osteomyelitis, and liver abscesses in children. The course of meningoencephalitis by listeria is often severe and even fatal. Acute hydrocephalus can develop as a complication and the mortality associated with listeriosis is significantly high. We present a case of meningoencephalitis caused by L. monocytogenes in a previously healthy 7-year-old girl.

Listeria monocytogenes is a facultative anaerobic, gram-positive bacillus that is isolated from the soil, vegetables, and wild or domestic animals. Listeria occurs predominantly in the elderly, immunocompromised patients, pregnant women and newborns. Infections by this microorganism are rare in healthy infants and children. L. monocytogenes may cause meningitis, meningoencephalitis, brain abscess, pyogenic arthritis, osteomyelitis, and liver abscesses in children. The course of meningoencephalitis by listeria is often severe and even fatal. Acute hydrocephalus can develop as a complication and the mortality associated with listeriosis is significantly high. We present a case of meningoencephalitis caused by L. monocytogenes in a previously healthy 7-year-old girl.

Pyogenic liver abscess (PLA) is rare in healthy children. We report a case of PLA in an immunocom­ petent 12­year­old boy. Percutaneous catheter drainage was performed for the abscess. In addition, parenteral antibiotics were administered for 3 weeks. Klebsiella pneumoniae was detected in the culture of blood and drained fluid. Here, we present this case and a brief review of the literature on this subject.

Purpose Mycoplasma pneumoniae (MP) pneumonia epidemics have occurred in 3- to 4-year cycles in Korea. We evaluated the epidemiologic characteristics of MP pneumonia in Daejeon, Korea, from 2003 to 2012. Methods We retrospectively analyzed 779 medical records of children (0-15 years of old) with MP pneumonia admitted to our institution and compared the data from 3 recent epidemics. Results In 779 patients, the mean age and male-to-female ratio were 5.0±2.2 years and 1:1, and most cases were observed in autumn. There were three epidemics during the study period, in 2003, 2006-2007, and 2011. In our comparison of the three epidemics, we found no differences in mean age, the male-to-female ratio, hospital stay, or the rate of seroconverters during hospitalization. All three epidemics began in early summer and peaked in September 2003 and 2011 and in October 2006 and then gradually decreased until the next year's spring season, although the 2006 epidemic extended further into 2007. The peak age groups in the children in 2003 and 2006 were 3-6 year-olds (57.5% and 56%, respectively), but in the 2011 epidemic, the peak group was 1-4 year-olds (46.5%). The proportion of the 10 years of age group was 5.2%, 13.8%, and 14.8% of total patients, respectively. Conclusion MP pneumonia outbreaks occurred every 3-4 years. The pattern of 3 recent epidemics was similar in demographic characteristics and seasonality with some variations in each outbreak.

Purpose We phylogenetically analyzed the Escherichia coli strains isolated from children with urinary tract infection (UTI) in 2 regions of Korea. Virulence factors (VFs) and antibiotic resistance of the strains were also determined to compare the possible differences. Methods A total of 138 E. coli strains were collected from the 2 regions; Gyeongin (78 strains) and Gyeongnam (60 strains). The phylogenetic groups were determined using the triplex polymerase chain reaction (PCR) method and multiplex PCRs were used to detect 7 VFs genes (fimH, papC, iutA, hlyA, sfa/focDE, afa/draBC, and kpsMT II). We also tested for antibiotic resistance. Results Phylogenetic groups, B2 (61.6%) and D (26.8%), comprised the majority of all isolated strains. Regional comparisons revealed that more B2 strains and fewer non-B2 (A+B1+D) strains were found in Gyeongnam, than in the Gyeongin region (P=0.033), and certain VFs were predominantly detected in Gyeongnam (P

Purpose : Recently, we have noticed an increase in the number of patients with mumps in Incheon, Korea. The aim of this study is to estimate the regional trend in mumps incidence and to evaluate the factors related to the recent increase. Methods : We reviewed the medical records of 66 patients with mumps who had been admitted to Incheon St. Mary’s Hospital from July 1999 to June 2009. We compared the differences in records between “outbreak, 2009” and “nonoutbreak, 2000–2008.” Results : Of the 66 patients, 35 (53%) were admitted in 2009, and 31 (47%) were admitted between 2000 and 2008. Most of the patients admitted during the outbreak were over 15 years of age (80.0%), were born before 1993 (77.1%), and had received a single dose of mumps vaccine (62.9%). There were no significant differences in contact history, present address, clinical manifestations, and complications, except for orchitis, between the two groups. Conclusion : There was a sharp increase in the number of inpatients with mumps who were born before 1993 and who were over 15 years of age. We recommend that a booster vaccination be considered for adolescents and young adults born before 1993 who are susceptible to mumps infection due to their reduced opportunities for receiving two doses of mumps vaccine.

Idiopathic pulmonary hemosiderosis (IPH) is a rare disease affecting mostly children. This disorder is characterized by recurrent episodes of hemoptysis, bilateral diffuse pulmonary infiltrates, and iron-deficiency anemia. An acute fulminant alveolar hemorrhage can be fatal due to respiratory failure, while chronic hemorrhage leads to hemosiderin-laden macrophages and pulmonary fibrosis. Genetic, autoimmune, allergic, environmental, and metabolic mechanisms of pathogenesis have been suggested, but the etiology of IPH remains unknown. We report on a 9-year-old girl with idiopathic pulmonary hemosiderosis who showed seasonal recurrences without cause.

Purpose : To examine the effect of bosentan, a dual endothelin receptor (ER) antagonist, on the development of monocrotaline (MCT)-induced pulmonary hypertension in rats by especially focusing on the pulmonary vascular morphology changes. Methods : Sprague-Dawley rats were treated as follows: controls received a subcutaneous saline injection, MCT-treated rats received a subcutaneous MCT injection, and bosentan-treated rats received a MCT injection followed by treatment with bosentan (20 mg/kg/day). To assess the effects of ER blockade on the time course, the animals were exsanguinated, and their hearts and lungs were dissected after 7, 14, or 28 days. Results : The mean body weights of the MCT- and bosentan-treated rats were significantly lower than that of the control rats on days 7, 14, and 28. Bosentan administration significantly inhibited the progression of right ventricular hypertrophy on day 28 (right ventricle/[left ventricle＋septum]: 0.710.10 in MCT-treated rats vs. 0.490.09 in bosentan-treated rats; P＜0.05). Quantitative analysis of peripheral pulmonary arteries revealed that the increase in medial wall thickness after MCT injection was significantly attenuated in the bosentan-treated rats on day 28 (49.9610.06% in MCT-treated rats vs. 47.0910.48% in bosentan-treated rats; P＜0.05). In addition, the increase in the number of intra-acinar muscular arteries after MCT injection was reduced by bosentan on days 14 and 28. Conclusion : Bosentan administration in intermediate doses exerts inhibitory effects on lung vascular hypertrophy and right ventricular hypertrophy during the development of MCT-induced pulmonary hypertension in rats.

Purpose : To investigate the discriminative ability of pediatric index of mortality 2 (PIM2) and pediatric risk of mortality III (PRISM III) in predicting mortality in children admitted into the intensive care unit (ICU). Methods : We retrospectively analyzed variables of PIM2 and PRISM III based on medical records with children cared for in a single hospital ICU from January 2003 to December 2007. Exclusions were children who died within 2 h of admission into ICU or hopeless discharge. We used Students t test and ANOVA for general characteristics and for correlation between survivors and non-survivors for variables of PIM2 and PRISM III. In addition, we performed multiple logistic regression analysis for Hosmer-Lemeshow goodness-of-fit, receiver operating characteristic curve (ROC) for discrimination, and calculated standardized mortality ratio (SMR) for estimation of prediction. Results : We collected 193 medical records but analyzed 190 events because three children died within 2 h of ICU admission. The variables of PIM2 correlated with survival, except for the presence of post-procedure and low risk. In PRISM III, there was a significant correlation for cardiovascular/neurologic signs, arterial blood gas analysis but not for biochemical and hematologic data. Discriminatory performance by ROC showed an area under the curve 0.858 (95% confidence interval; 0.779-0.938) for PIM2, 0.798 (95% CI; 0.686-0.891) for PRISM III, respectively. Further, SMR was calculated approximately as 1 for the 2 systems, and multiple logistic regression analysis showed χ2 (13)=14.986, P=0.308 for PIM2, χ2 (13)= 12.899, P=0.456 for PRISM III in Hosmer-Lemeshow goodness-of-fit. However, PIM2 was significant for PRISM III in the likelihood ratio test (χ2 (4)=55.3, P

Purpose : This study was undertaken to evaluate the immunogenicity and reactogenicity of Td booster immunization in early preadolescents of Korea. Methods : Healthy preadolescents, who had been vaccinated with 4 or 5 doses of DTaP vaccines until 6 years old age, were enrolled in this study from August 2006 to April 2007. Diphtheria and tetanus anti-toxoid antibodies in sera were measured by ELISA just before vaccination and 4 weeks after vaccination to evaluate immunogenicity. Local and systemic adverse reactions observed for 4 weeks after vaccination to access reactogenicity. Results : 183 preadolescents were enrolled and mean age was 11.40±0.51 years old. All subjects achieved seroprotective diphtheria and tetanus anti-toxoid antibodies (titers ≥0.1 IU/mL) after Td booster vaccination. Among 183 vaccinees, 73.8% showed local adverse reactions and 37.2% systemic adverse reactions. Pain at injection site (66.1%) was the most common local reaction, and the most commonly shown systemic reaction was myalgia (17.5%). The adverse reactions were spontaneously relieved within three days after vaccination. Conclusion : Td vaccine in this study was high immunogenic and showed an acceptable tolerance in Korean preadolescents. Td booster vaccination at 11-12 years old is the most effective method to increase compliance of the vaccination and to decrease the incidence of diphtheria and tetanus.

Purpose : While pediatric observation units (POU) have become a common practice in hospitals throughout developed countries, there has been no report about POUs in Korea so far. The aims of this study were to analyze our one-year's experience of the POU and to decide which disease entities are suitable for the POU.Methods : All children admitted from March 2006 to February 2007 to the POU at the Department of Pediatrics in Our Lady of Mercy Hospital were included in this study. Data were collected from retrospective reviews of their medical records. Results : There were a total of 1,076 POU admissions. Median age of patients was 2.4 years and median length of hospital stay 14.0 hours. The most common diagnoses were gastroenteritis (42.7%), pharyngotonsillitis (19.1%), bronchiolitis (7.8%), pneumonia (5.5%) and febrile seizure (5.2%). Overall, 7.5% of the POU patients required subsequent inpatient admissions due to hospital stays of longer than 48 hours. The disease entities that were most likely to require inpatient admission were pneumonia (17.0%), febrile seizure (12.5%) and asthma (11.5%). Diseases that allowed successful discharge from the POU were gastroenteritis (4.6%), upper respiratory tract infection (5.8%), such as otitis media and pharygnotonsillitis and seizure disorder (6.4%). Compared with the previous year when the POU was not in operation, there was a statistically significant reduction in the average length of hospital stays (from 4.69 to 3.75 days), as well as a rise in the bed turnover rate (from 78.8 to 98.2 patients/bed). Conclusion : Our study shows that the POU is efficient for the management of children with certain acute illnesses. Based on this study, we suggest that the POU be used as a new modality which links between the outpatient, inpatient, and emergency departments in the field of pediatrics in Korea.

Patients with febrile illness and skin rashes need full and immediate attention. In general, these diseases show mild manifestations and good prognosis. However, causalities of some diseases with fever and rash may be life threatening or trivial. So, the differential diagnosis for those patients is extensive. A through history, a careful physical examination and close observation of clinical progress are very helpful and essential to confirming the diagnosis. Histories of recent travel, drug or specific food ingestion, exposure to human or an animal source of infection may be useful to discover the cause. Although laboratory tests can be useful in making the diagnosis, laboratory results usually are not available immediately. Knowledge and experiences of such diseases may be helpful to reduce the differential diagnosis to a few major possibilities. Rashes can be categorized as petechial, maculopapular, vesicular, urticarial and erythematous. Potential causes include infectious pathogens such as virus, bacteria, rickettsiae, spirohetes, connective tissue diseases, allergic diseases and heamto-oncologic diseases. Because the severity of these diseases can vary mild to life threatening, physicians must perform prompt management decisions regarding empirical therapies. In this article, the differential etiological diagnosis of each type rash is reviewed and discussed, and with emphasis on intensive care of life threatening febrile diseases with rashes that are seen in our country.

Purpose : We conducted the study to evaluate the immunogenicity and safety of three component DTaP vaccine (Infanrix®) in a group of Korean healthy infants on a three-dose primary vaccination. And we compared the immunogenicity of this DTaP vaccine with two component DTaP vaccine which has been widely used in Korea. Methods : We enrolled one hundred fifty one healthy infants aged 8-9 weeks. These infants were vaccinated at age 2, 4 and 6 months of age with three component DTaP vaccine. Solicited adverse events were actively monitored for 72 hours following each vaccination, and all adverse events after each vaccination were observed for three weeks. Anti-diphtheria toxoid Ab., anti-tetanus toxoid Ab., anti-pertussis toxin Ab., anti-filamentous hemagglutinin Ab., and anti-pertactin Ab. were measured using ELISA for assessing immunogenicity of study vaccine in 60 infants. Immunogenicity analysis of two component DTaP vaccine was performed with same methods in 14 infants as control. Results : The seroconversion rates of anti-diphtheria toxoid Ab, anti-tetanus toxoid Ab. anti- filamentous hemagglutinin Ab. were 100% in both group. Seroconversion rate of anti-pertactin Ab in study group was 100%, but the rate in control group was 50%. However, geometric mean concentration of anti-pertussis toxin Ab. was higher in control group. Mild local and systemic reactions were observed within three days after vaccination, and no serious adverse events related study vaccine were happened during study period. Conclusion : Our study results suggest that three component DTaP vaccine (Infanrix®) is a well- tolerable and high immunogenic vaccine, especially anti-Pertactin Ab. of the study vaccine is very immunogenic. It can be available as routine DTaP vaccination in our infants.

Purpose:In this study, we created a questionnaire and collected answers concerning actual conditions of vaccination and parents' knowledge of vaccination issues, in order to find false knowledge of patients. We tried to give correct information and suggested the role of the pediatrician. Methods:We made questionnaires and collected answers from 466 parents from March 2004 to June 2004. Results:Places of vaccination were pediatric clinics(49.4 percent), health centers(27.7 percent), general/university hospitals(15.0 percent) and other clinics(4.9 percent). We found 38.8 percent of parents thought that the reason for vaccination at pediatric clinics was a belief of speciality, even though there is no difference in the vaccination itself. We also found 15.0 percent of parents thought that there were no differences between pediatricians and other physicians, but 52.0 percent of parents wanted to receive vaccination at pediatric clinics in the future. Our study also found that 62.4 percent of parents wanted to make out a preliminary questionnaire for vaccination. Many parents got vaccination information from vaccination record books(57.9 percent), and 52.6 percent of parents incorrectly believed that Hib vaccination could prevent all kinds of meningitis. Conclusion:Our study suggests that pediatricians need to make efforts to give out correct information. It is necessary to use preliminary questionnaires for vaccination and correct vaccination information should be written on the vaccination record book. Also, there needs to be counselling with parents about weaning, growth and development at the time of vaccination, and to point out the differences between pediatricians and other physicians.

Purpose:Urinary tract infection(UTI) is one of the most frequent infections in children. E. coli is the most frequent etiological micropathogen in pediatric community UTI, and E. coli has developed resistance to many antibiotics, highlighting the need for regular surveys of this organism resistant patterns in the community. The aim of this study was to determine the oral antibiotic susceptibility patterns of E. coli, isolated from pediatric patients with uncomplicated community acquired UTI. Methods:E. coli isolates, obtained from pediatric patients with uncomplicated community acquired UTI between October in 2004 to September in 2005. And minimal inhibitory concentrations(MICs) of oral aminopenicillins and beta-lactamase inhibnitors(ampicillin, amoxacillin, ampicillin-sulbactam), oral cephalosporins(cefaclor, cefixime) and sulfa drug(trimethoprime-sulfamethoxazole) were performed according to the National Committee for Clinical Laboratory Standards(NCCLS) guide line. Results:Total 211 organisms were isolated from pediatric out-patients with community UTI. E. coli was the most common organism(89 percent), followed by E. fecalis, Proteus species, S. aureus, M. morganii, and P. aeruginosa. The resistant rates of aminopenicillins and beta-lactamase inhibitors, cefaclor and sulfa drug to E. coli were very high. But, the resistant rate of cefixime was markedly low, and ESBL strains were isolated with small rates. Conclusion:Our study results suggest that aminopenicillins, cefaclor and sulfa drug may not be useful as first line empirical antibiotics to treat pediatric patients with community UTI in Korea. But, 3rd generation cephalosporin such as cefixime can be used as effective second line antibiotics after primary treatment failure, also may be useful as an empirical first line antibiotic. Finally, we conclude that a continuous surveillance study to monitor susceptibility patterns of E. coli in community UTI will be needed for the standard guide lines of empirical oral antibiotic treatment.