• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1118-1123
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• 2003

Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous purpura, abdominal pain, arthralgia and renal involvement. The clinical features of HSP have been reasonably well documented but there are still many gaps in our understanding of HSP. The aim of this study was to present the clinical features of 125 children with HSP and compare them with previous reports, placing particular emphasis on clinical information. Methods : We collected the clinical data of 125 patients with acute HSP who visited Chungbuk National University Hospital from March 1992 to April 2002. Data were expressed as the mean or $mean{\pm}SD$ and statistical analysis was performed using Chi-square approximation. P<0.05 was considered as significant. Results : The patient population consisted of 87 boys and 38 girls ranging in age from one to 14 years. HSP occurs throughout the year, but this study shows seasonal skewing, with most patients presenting from fall through spring and a paucity of cases in summer. All patients had non-thrombocytopenic purpura concentrated on the buttocks and lower extremities. Purpuric lesions were also scattered on the arms and occaisionally on the face and ears, but the trunk was largely spared. A recurrence of purpura was defined as the reappearance of a rash or other symptoms following resolution of disease for at least two weeks. The mean number of recurrences was 0.51. Eighty eight patients(70.4%), 18 patients(14.4%) and 67 patients(53.6%) complained of abdomianl pain, gastrointestinal bleeding and arthralgia, respectively. Nephritis occurred in 48(38.4%) patients. Fifteen boys (17.2%) developed epididymitis. Neurologic features occurred in 13(10.4%) and two(15%) of these were seizures. Conclusion : HSP all showing purpura as defined is characterized by various clinical features, including abdominal pain, arthralgia, epididymitis and nephritis which could occur before the appearance of purpura. Therefore, we suggest that the possibility of HSP should be considered in children before invasive procedures, even if the above symptoms and signs present without purpura.

• 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.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.512-517
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• 2008

Purpose : Syncope is relatively common in children and adolescents. Among the etiologies of syncope, neurocardiogenical syncope is the most common, but it is often confounded with seizure. We investigated the clinical features of patients under the age of 15 with neurocardiogenical syncope, compared to patients with neurologic disorders including epilepsy. Methods : Among the children who visited the Department of Pediatrics at Cheongju St. Mary's Hospital and Chungbuk National University Hospital from March 2005 to February 2007, we retrospectively analyzed 69 patients whose chief complaint was syncope. We classified the patients by syncope etiology and made comparisons between the neurocardiogenical syncope (NCS) group and the neurological disorders (ND) group regarding to age; location; time and season in which syncope occurred; associated symptoms, including seizures, provocation factors; prodromes; duration of syncope; frequency of previous syncope; birth history; associated disease; past medical history; family history; neurological exam; physical exam; laboratory findings; electrocardiography; electroencephalography (EEG); head-up tilt test; brain CT; and MRI. Results : Among 69 syncope patients, 53 (76.8%) were in the NCS group and 11 (15.9%) were in the ND group. There were no statistically significant differences between the two study groups except for the presence of prodromes and EEG abnormalities. The presence of prodromes in the NCS group was more common than in the ND group [46.9% (23/49) vs. 9.1% (1/11), (P=0.038)]. The EEG abnormality in the ND group was more common than in the NCS group [90% (9/10) vs. 5.8% (3/52), (P<0.01)]. Conclusion : Our study suggests that detailed history-taking, including that concerning prodromes, is important for the accurate diagnosis of neurocardiogenical syncope, and EEG should be obtained if neurological disorders are suggested.

• 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.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.487-491
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• 2008

Purpose : Although Mycoplasma pneumoniae (M. pneumoniae) infection can cause wheezing in non-asthmatic children, the mechanisms of this symptom remain unclear. Vascular endothelial growth factor (VEGF) is a major mediator of angiogenesis and vascular permeability, and is also known to be elevated in cases of chronic pulmonary disease such as asthma. We hypothesized that VEGF may increase in children with acute M. pneumoniae pneumonia and wheezing. Methods : Nine patients with clinical and laboratory evidence of acute M. pneumoniae pneumonia were enlisted from children admitted to Korea University Hospital. They had had more than one episode of wheezing during the illness, which was confirmed by a physician; they comprised the wheezer group. The individuals with M. pneumoniae pneumonia without wheezing were 63 in number, and they comprised the non-wheezer group. Patients with a history of asthma or who had received asthma medications were excluded. Serum concentrations of VEGF, total IgE, eosinophil cationic protein (ECP), and peripheral blood eosinophil counts were measured. Results : The serum VEGF concentrations were higher in the wheezer group ($mean{\pm}SD$; $650.2{\pm}417.9pg/mL$) than in the non-wheezer group ($376.5{\pm}356.2pg/mL$, P=0.049). M. pneumoniae antibody (1:1,380 vs. 1:596, P=0.048) and serum total IgE (591.8 IU/mL vs. 162.2 IU/mL, P=0.032) were higher in the wheezer group than in the non-wheezer group. There were no differences between the two groups in terms of serum ECP concentration or blood eosinophil count. Conclusion : In the presence of wheezing, serum VEGF concentrations were higher in the children with M. pneumoniae pneumonia. This finding suggests that VEGF may associate with wheeze-related symptoms in children with acute M. pneumoniae pneumonia.

• Clinical and Experimental Pediatrics
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• v.50 no.5
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• pp.457-461
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• 2007

Purpose : Urinary tract infection (UTI) is a common bacterial infectious disease in childhood. Especially UTI in infant and young children is associated with urinary tract anomalies such as hydronephrosis, vesicoureteral reflux. The aim of this study was to compare the clinical and laboratory characteristics, and uroradiologic findings of UTI caused by pathogens other than E. coli with UTI caused by E. coli in infant and young children. Methods : We retrospectively reviewed medical records of 170 infants and children, who had been admitted for UTI to Il Sin Christian Hospital from January 2003 to December 2005. All patients were divided into two groups; E. coli and non-E. coli UTI, and they were compared for demographic data, clinical data (degree and duration of fever, time to defervescence, and length of hospital stay), underlying urinary tract anomalies (by history and ultrasonography), recurrent infection (by history and past medical records), and laboratory data [urinalysis, white blood cells (WBC) count in peripheral blood, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and serum creatinine level]. Results : Of the 170 UTI patients, the number of non-E. coli UTI was 114 (67.1%) and E. coli UTI was 56 (32.9%). As compared to E. coli group, non-E. coli group was younger in age ($0.52{\pm}0.59years$ vs $0.84{\pm}1.39years$, P<0.05), had higher rates of urinary tract anomalies [n=46 (82.1%) vs n=53 (46.5%), P<0.001], higher recurrence rate, shorter time to defervescence, less peripheral blood WBC count, lower level of CRP, lower level of ESR. Conclusion : The characteristics of non-E. coli UTI compared to E. coli UTI was younger age, milder clinical symptoms and signs, higher rates of urinary tract anomalies and higher recurrence rate.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.88-94
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• 1999

Purpose : Malformation of urinary tract is among the most common of all congenital anomalies and can progress to irreversible renal damage before diagnosis due to difficulty of early diagnosis. Present study was undertaken to determine the clinical characteristics of urinary tract anomaly and to find out the most appropriate diagnostic and therapeutic measures for children with these anomalies. Methods : During the past 10 years from 1987 to 1998, review of medical records revealed 65 children with congenital anomaly of urinary tract and the following results were obtained. Results : The most common anomalies were ureteropelvic junction obstruction occuring in 26 cases ($36\%$), followed by ureteral duplication in 11 cases, renal agenesis in 10 cases and ureterovesical function obstruction in 7 cases. Complex anomaly of urinary tract was found in 8 cases and anomaly of other systems such as congenital heart disease was detected in 11 cases. The most frequent age group at the time of diagnosis was below 1 year of age constituting 39 cases ($60\%$) and male preponderance was noted as male to female ratio being 2.25:1. Presenting symptoms were urinary tract infection in 25 cases, followed by hematuria, abdominal mass, abdominal pain and voiding difficulty, etc, and in 11 cases, the anomaly was picked up by routine prenatal ultrasonography. Azotemia was noted in 9 cases and the underlying anomaly was obstructive uropathy in 4 out of these 9 cases. Surgical correction was undertaken in 38 cases (most frequently in cases of obstructive uropathy) and in 2 out off cases with obstructive uropathy in whom surgical correction was done, azotemia disappeared during follow up period of 1-5years. No new cases of deteriorating renal function appeared during follow-up period. Conclusion : In spite of high incidence of congenital malformation of urinary tract, early diagnosis is still hampered by nonspecific symptoms and signs. Therefore, in patients with symptoms such as urinary tract infection, abdominal pain and voiding problems, etc, it Is advisable to take various diagnostic tests promptly to pick up any urinary tract anomaly and to apply proper therapy in order to avoid progression to irreversible renal damage. In this regard, prenatal ultrasonography should be utilized more widely as a routine procedure to detect any urinary tract anomalies before birth.

• Journal of Chest Surgery
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• v.42 no.5
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• pp.597-603
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• 2009

Background: Atrioventricular valve regurgitation in pediatric patients with a functional single ventricles (FSV) - has been known as one of the important risk factors for death and unfavorable long-term results after a Fontan operation. We evaluated early and mid-term results of bivalvation valvuloplasty in FSV patients. Material and Method: We retrospectively evaluated 11 patients with a functional single ventricle who underwent bivalvationvalvuloplasty between 1999 and 2007. The degree of common atrioventricular valve regurgitation (CAVVR) was determined by color Doppler echocardiography (regurgitation grade scoring, trivial; 1, mild; 2, moderate; 3, severe; 4). Mean age at valve surgery was $6.9{\pm}7.0$ months (median 4 months, 24 days$\sim$21 months)and mean body weight was $6.2{\pm}2.8\;kg$ ($3.1{\sim}11.3\;kg$). Nine patients had isomerism heart and two of them had TAPVC. The concomitant procedures were performed in all but one patient. Additional commissural closure was performed in 3 patients and commissural annuloplasty in another 3 patients. Result: There was one hospital death after. surgery. A 32-day old patient who had been preoperatively dependent on a ventilator died of air way and lung problems 4.3 months after pulmonary artery banding and bivalvation valvuloplasty. Mean follow-up duration was 40 months ($4.3{\sim}114$ months). Mean preoperative CAVVR score was $3.3{\pm}0.6$, which decreased to $1.9{\pm}0.7$ postoperatively (p<0.0001). This residual regurgitation slightly increased to $2.2{\pm}0.4$ (no statistical significance) after a mean follow-up of 1.4.3 months. Six patients (60%) required re-operations for residual regurgitation at a subsequent bidirectionalcavopulmonary shunt or Fontan operation. One patient with Ebsteinoid malformation of the right sided atrioventricular valve required valve replacement due to stenoinsufficiency. Another patient required edge-to-edge repair at the right sided AV valve (between the right mural leaflet and the bridging leaflets). The remaining 4 patients required additional suture placements between bridging leaflets with or without commissural annuloplasty. All survivor had trivial or mild CAVVR at the latest follow-up. Conclusion: Bivalvation valvuloplasty for CAVVR in FSV patients is. an effective and safe procedure. However, significant numbers of the patients have small residual regurgitation and require additional valve procedures at subsequent operations. Long-term observations to monitor progression of the CAVVR is mandatory.

• Tuberculosis and Respiratory Diseases
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• v.65 no.5
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• pp.390-395
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• 2008

Background: Since the early 1980s, interferon-alpha ($IFN-{\alpha}$) has been used as adjuvant therapy in pediatric patients with recurrent respiratory papillomatosis (RRP). However, its efficacy in adults needs to be validated. Since 2002, Samsung Medical Center's guidelines have mandated regular injection of $IFN-{\alpha}$ in patients with RRP to prevent recurrence. To evaluate these guidelines, patient data were investigated. Methods: Five patients diagnosed as having RRP by bronchoscopy and histopathology were included. After initial bronchoscopic intervention, including laser cauterization, all patients received subcutaneous injection of 6 million units of $IFN-{\alpha}$ every 2 months. Further bronchoscopic intervention was carried out as needed. Patients were regularly evaluated using bronchoscopy or computed tomography. Results: The median age of the patients was 44 years (range 13~68), and the median duration of papillomatosis was 31 years (range 1~45). Three and two patients had juvenile-onset and adult-onset disease, respectively. Two patients had a history of tracheostomy at the time of diagnosis. The median duration of $IFN-{\alpha}$ therapy was 56 months (range 12~66). Two patients showed complete remission at 12 and 36 months after $IFN-{\alpha}$ injection, respectively. The other three patients showed partial remission, and the number of laser therapy sessions was significantly reduced.Conclusion: Intermittent $IFN-{\alpha}$ injection is effective in patients with long-standing RRP and can reduce the number of laser therapy sessions required in their treatment.

• Clinical and Experimental Pediatrics
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• v.49 no.7
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• pp.745-750
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• 2006

Purpose : Enteroviruses are the most common cause of aseptic meningitis in patients of all ages. A definite diagnosis of enteroviral meningitis can be established by detection of virus directly in CSF specimens. But this is time-consuming and lacks sensitivity, so polymerase chain reaction(PCR) detecting of viral RNA in patient specimens such as CSF, stool has been demonstrated. But little is known about the influence of sampling time on the results of CSF PCR and stool PCR. We investigated diagnostic utility of PCR of CSF and stool according to sampling time after the onset of symptoms. Methods : PCR results were analyzed according to sampling time for 42 patients diagnosed aseptic meningits in our hospital from $11^{th}$ January to $30^{th}$ August, 2005. Results : The diagnostic yield of the test was higher of CSF specimens obtained ${\leq_-}2$ days after clinical onset(positive PCR results 9/18, 50 percent), compared with CSF collected >2 days after onset(positive PCR results 1/24, 4.2 percent)(P=0.001). Instead, positive PCR results of fecal specimens maintained highly(average 90.5 percent), 10 cases had also positive PCR results even 5-6 days after onset. 10 cases of CSF specimens had positive enterovirus PCR results containing coxsackievirus B5 (n=6), coxsackievirus B3(n=3). 38 cases of stool specimens had positive enterovirus PCR results containing echovirus 18(n=7), echovirus 9(n=3), coxsackievirus B5(n=8), coxsackievirus B3(n=3). 6 cases(coxackie B5) had positive CSF PCR and stool PCR, both. Conclusion : Stool PCR was clinically sensitive for detecting enterovirus during enteroviral meningits and could give a presumptive diagnosis throughout the disease course. A definite diagnosis was obtained by CSF PCR, but its utility was clearly lower for samples obtained >2 days after clinical onset. Therefore, it is recommended that, in addition to performance of CSF PCR, fecal samples obtained from patients with suspected enteroviral meningitis should be tested by PCR, especially when the duration of symptoms is >2 days.