• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.875-881
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• 2006

Purpose : The aims of this study were to analyze the clinical characteristics of children with Kikuchi's disease(KD) at a medical center and to investigate the etiologic role of human herpesvirus 8(HHV 8) or Epstein-Barr virus(EBV) in children with KD. Methods : Twenty six children who were diagnosed as KD between Jan. 1998 and Dec. 2005 were included. Medical records were reviewed on the clinical characteristics of children with KD. Follow up data were collected by chart review and telephone contact. Polymerase chain reaction(PCR) was performed in order to detect HHV 8 DNA, and in situ hybridization(ISH) was perfomed in order to detect EBV RNA from 20 lymph node tissues. Results : There were 15 girls and 11 boys with a mean age of 13 years. Posterior cervical lymph nodes were involved in 72 percent(18/25) of the patients. Extracervical lymphadenopathy was associated in one patient. Fever was an associated symptom in 31 percent(8/26) of the patients. Leukopenia was observed in six (46 percent) patients. The cervical lymphadenopathy usually resolved spontaneously within 6 months. Only one patient had a recurrence of lymphadenopathy with fever during follow-up. No children with KD in our series developed systemic lupus erythematosus. HHV 8 DNA was not amplified by nested PCR in any of the cases, and all cases were negative for EBV RNA by ISH. Conclusion : KD should be differentiated as a cause of cervical lymphadenopathy in children. HHV 8 and EBV may not play major causative roles in KD in children.

• Clinical and Experimental Pediatrics
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• v.49 no.3
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• pp.287-291
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• 2006

Purpose : Aneurysms of sinus valsalva are rare anormalies thought to be primarily congenital in origin, progressing into death by acute heart failure in cases of rupture. Surgical correction is the only method of treatment. With these clinical implications, we reviewed the clinical characteristics and surgical results of patients with ruptured sinus of valsalva aneurysm. Methods : Between January 1991 and February 2004, 17 patients with ruptured sinus of valsalva aneurysm were retrospectively reviewed for their clinical symptoms, physical findings, past history, coexistent cardiac anormalies, surgical results, and mid-term prognosis. Results : The 17 patients included 13 men and four women, with a mean age of 30 years(10-59 years). Preoperatively accompanying cardiac anormalies were ventricular septal defect(VSD, eight cases of doubly committed juxta-arterial VSD) and aortic insufficiency(11 cases). During operations, patterns of fistulous tracts were found to be right colonary sinus-right ventricle in 13 patients, right coronary sinus-right atrium in one, noncoronary sinus - right ventricle in two, noncoronary sinus - right atrium in one, and VSD was noticed in 14 patients(all were doubly committed juxta-arterial in type). The defects were closed with a patch in 13 patients, without a patch in four, with concommitant aortic valve replacement in four and with aortic valvuloplasty in two. There were no mortalities during operations or the mid-term follow-up periods($40{\pm}49$ months). Conclusion : Because, at least in Orientals, VSD(especially doubly committed juxta-arterial) was accompanied in large numbers of patients with aneurysms of sinus valsalva, preoperative evaluations of this congenital heart disease should be made very careful. And we may need to revise the algorithm of treatment policy in small sized doubly committed juxta-arterial VSD.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.889-894
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• 2006

Purpose : Asthma is characterized by the presence of airway hyperresponsiveness(AHR) and inflammation. The extensive eosinophil infiltration into the lung is the hallmark of asthma and contributes to the damage of respiratory epithelium during late phase airway responses. Eotaxin is the major eosinophil chemoattractant found in bronchoalveolar lavage(BAL) fluid of allergic inflammation. IL-13 has been known to induce the expression of exotaxin and eosinophilia. IL-13 also induces airway inflammation, mucus production and leads to marked fibrosis, airway remodeling and AHR. We investigated whether serum IL-13 levels can reflect the presence of airway hyperresponsiveness in children with asthma, and the relationship between serum IL-13 and eotaxin levels. Methods : Using sandwich enzyme-linked immunosorbent assay, the serum IL-13 and eotaxin levels were measured in 13 atopic asthmatics, 5 atopic non-asthmatics and 12 control subjects. Metacholine challenge tests were performed in all subjects. Airway hyperresponsiveness to metacholine was expressed as provocative concentration of metacholine causing a 20% fall in FEV1[$PC_{20}mg/mL$]. $PC_{20}$ value of 25 mg/mL was used as a cut-off for defining a AHR. Results : Serum IL-13 levels showed positive correlation with eotaxin levels. Serum IL-13 and eotaxin levels showed no differences among atopic asthmatics, atopic non-asthmatics and control subjects. And there were no differences serum IL-13 and eotaxin levels in children with and without AHR and atopy. Serum IL-13 and eotaxin levels did not correlate with $logPC_{20}$ levels. Conclusion : IL-13 is closely related to the eotaxin release. But serum IL-13 and eotaxin per se can't predict the severity of airway hyperresponsiveness. IL-13 and eotaxin may have local effect on respiratory epithelium or there can be some factors to induce airway hyperresponsiveness other than serum IL-13 in asthmatic airways.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.967-972
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• 2002

Purpose : A retrospective study was undertaken to evaluate the usefulness of low risk criteria for identifying febrile infants younger than three months unlikely to have serious bacterial infection. Methods : We conducted a retrospective study of 527 infants younger than three month with a axillary temperature ${\geq}37.4^{\circ}C$. If they met the following all four criteria, appear well, WBC $5,000-20,000/mm^3$, urine stick WBC(-) and nitrite(-), CSF WBC < $10/mm^3$, they were considered at low risk for serious bacterial infection(SBI). SBI was defined as a positive culture of urine, blood, or cerebrospinal fluid. The sensitivity, specificity, negative predictive value and positive predictive value of the low risk criteria were calculated. Results : Of 527 febrile infants, 110(21.0%) had serious bacterial infections. The 2.7% who met the low risk criteria had SBI and negative predictive value was 97.3%. SBI was diagnosed in 103 infants(38.6%) who didn't meet the low risk criteria including urinary tract infection(78.6%), most commonly, bacteremia(16.5%), bacterial meningitis(8.7%), Salmonella gastroenteritis(1%), osteomyelitis( 1%), septic arthritis of hip joint(1%). There were no differences in the sensitivity and negative predictive value according to the monthly-age-group. Conclusion : This low risk criteria to identify infants unlikely to have SBI early is available, however low risk infants must be carefully observed.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1075-1082
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• 2002

Purpose : Obesity is known to be associated with hypertension, dyslipidemia, and fatty liver and is thought to be associated with increased levels of free fatty acids. One of the strategies for decreasing free fatty acid levels is stimulation of hepatic lipid oxidation with L-carnitine. Carnitine is an essential cofactor for transport of long-chain fatty acid into mitochondria for oxidation. This study was designed to evaluate the changes of serum fatty acids and carnitine levels after exogenous injection of L-carnitine. Methods : Sprague Dawley rats were divided into two groups. Group A was control. Group B was given intraperitoneal injection with L-carnitine(200 mg/kg) daily for two weeks. Serum lipid (total cholesterol, triglyceride, HDL-cholesterol, LDL-cholesterol) and fatty acid levels were analyzed on the first day of the first and second weeks after injection of L-carnitine. Total, free, and acyl carnitine levels also were performed by a enzymatic cycling techniques at the same day intervals. Results : There was no significant difference between the two groups in total cholesterol, HDL-cholesterol, LDL-cholesterol levels before and after the administration of L-carnitine. But triglyceride levels were significantly decreased at the first week in group B compared with group A. Among free fatty acids, linoleic acid showed significant decrement(A group : $131.3{\pm}31.3mg/dL$ vs B group : $90.0{\pm}7.0mg/dL$) at the first week. Total, free, and acyl carnitine levels showed significant increments at all days intervals, but only free carnitine showed significant increments according to cumulative doses of carnitine. Conclusion : Plasma linoleic acid, a long-chain fatty acid, showed significant decrement after administration of L-carnitine in the first week. This may suggest that L-carnitine can be used as an antilipidemic agent for obese patients. A prospective study will investigate obese children in the future.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1065-1074
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• 2002

Purpose : The purpose of this study is to analyze the epidemiologic characteristics of sudden unexpected death in infancy and to evaluate the importance of postmortem autopsy. Methods : We reviewed, retrospectively, medical records of 34 infants admitted to Kangnam General Hospital from January 1987 to December 2001 because of sudden unexpected death. We investigated the cause of death through medical history, death scene examination, autopsy findings, acylcarnitine and organic acid analysis. Results : Among the total 34 infants, 18 were male(52.9%) and 16 were female(47.1%). Thirty infants(88%) were below the six months of age. Winter was the most affected season(38.2%). Eighteen infants(52.9%) died between 6 and 12AM. The prone sleeping position was observed more frequently than the supine position at death; nine cases in the prone position, six cases in the supine position. The cause of death of 23 cases could not be found by only history and death scene examination. Autopsy was done in 13 cases. Seven cases of them were thought to be SIDS. In six cases, we explained the cause of death with autosy findings. They were an endocardial fibroelastosis, a nesidioblastosis, a subdural hematoma, a bronchopneumonia and two fatty changes of liver. Metabolic screening tests performed in three cases to rule out metabolic disorder since 2000 were all normal. Conclusion : We concluded that autopsy and metabolic screening test should be performed to find out the cause of death in sudden unexpected death in infancy.

• Clinical and Experimental Pediatrics
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• v.45 no.9
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• pp.1126-1133
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• 2002

Purpose : Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical features and genetic basis of PWS patients for early detection and treatment. Methods : We retrospectively studied 24 patients with PWS in Department of Pediatrics, Samsung Medical Center, from September 1997 to September 2001. We performed cytogenetic and molecular genetic techniques using high resolution GTG banding techniques, fluorescent in situ hybridization and methylation-specific PCR for CpG island of SNRPN gene region. Results : The average birth weight of PWS patients was $2.67{\pm}0.47kg$ and median age at diagnosis was 1.3 years. The average height and weight of PWS patients under one year at diagnostic time were located in a 3-10 percentile relatively, and a rapid weight gain was seen between two and six years. Feeding problems in infancy and neonatal hypotonia were the two most consistently positive major criteria in over 95% of the patients. In 18 of the 24 cases(75%), deletion of chromosome 15q11-q13 was demonstrated and one case among 18 had an unbalanced 14;15 translocation. In four cases without any cytogenetic abnormality, it may be considered as maternal uniparental disomy and the rest showed another findings. Conclusion : We suggest diagnostic testing for PWS in all infants/neonates with unexplained feeding problems and hypotonia. It is necessary for clinically suspicious patients to undergo an early genetic test. As the genetic basis of PWS was heterogenous and complex, further study is required.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1101-1106
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• 2003

Purpose : In vivo, minocycline appears to be neuroprotective. Thus, the neuroprotective effects of minocycline were studied in a rat brain cortical cell culture induced by hypoxia. Methods : Cultured cells from the brains of Sprague-Dawley rats were divided into two sets of groups : normoxia groups treated with 5% $CO_2$ and hypoxia groups treated with 1% $CO_2$. After several days of incubation, the control groups were not treated with minocycline, while the sample groups were treated with either 1 or $10{\mu}g/mL$ of minocycline. The damaged cells were observed under a microscope, while apoptosis was detected using a TUNEL assay control-stained with DAPI. Results : Among the normoxia groups, the control and sample groups treated with 1 and $10{\mu}g/mL$ of minocycline were all statistically significantly different from each other. Meanwhile, among the hypoxia groups, although the control was significantly different from the sample groups, there was no statistically significant difference between the sample groups. When comparing the normoxia and hypoxia groups, there was a statistically significant difference between the control groups and sample groups treated with $1{\mu}g/mL$ of minocycline, yet no significant difference between the sample groups treated with $10{\mu}g/mL$ of minocycline. Conclusion : Minocycline was found to be neuroprotective in normoxia and hypoxia induced rat brain cortical cell cultures.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1095-1100
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• 2003

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 the right colon. Fourteen patients were managed by conservative treatment including antibiotics. A follow-up study was performed in 15 patients. There were symptomatic recurrences in two patients, but no significant complication was noted. The frequency of ACD was 11.7 per 1000 acute appendicitis. Conclusion : ACD in children can mostly be cured by conservative treatment. It is prudent to choose the management through the diagnostic work up, including abdominal sonography and computed tomography, because there was no significant difference of clinical findings between ACD and acute appendicitis.

• Clinical and Experimental Pediatrics
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• v.53 no.1
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• pp.28-32
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• 2010

Purpose : Although eosinophilia is a common laboratory finding in many neonatal intensive care units (ICUs), its causative mechanisms remain obscure. We aimed to determine the causes of eosinophilia in the neonatal ICU environment. Methods : Serial eosinophil counts were determined weekly for 288 hospitalized, appropriately grown neonates. Infants were divided into four groups according to gestational age, and the incidence and etiologic factors of eosinophilia were retrospectively studied. Results : Absolute eosinophilia (>$700/mm^3$) was documented in 18% (52/288) of neonates. Twenty-two infants (42.3%) exhibited mild eosinophilia ($700-999cells/mm^3$), 27 (51.9%) exhibited moderate eosinophilia ($1,000-2,999cells/mm^3$), and 3 (5.8%) exhibited severe eosinophilia (>$3,000cells/mm^3$). Of the 288 infants studied, 54 suffered sepsis. Thirty of these 54 infants (55.6%) showed eosinophilia, and 22 out of the remaining 234 infants (9%) without sepsis showed eosinophilia, indicating that eosinophilia was more prevalent in the sepsis group (P <0.05). All 5 infants suffering from bronchopulmonary dysplasia showed eosinophilia, and 47 out of the remaining 283 infants (16.7%) without bronchopulmonary dysplasia showed eosinophilia. Thus, eosinophilia was more prevalent in the bronchopulmonary dysplasia group (P<0.05). Furthermore, increased prevalence of eosinophilia was associated with respiratory distress syndrome, ventilator use, blood transfusion, and total parenteral nutrition (P<0.05). Conclusion : Our results suggest that eosinophilia is influenced by sepsis and bronchopulmonary dysplasia, although it can also occur idiopathically at birth. Moreover, the potential role of eosinophils in conditions such as wound healing and fibrosis in sepsis or chronic lung disease may be a cause of eosinophilia.