• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.640-645
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• 2008

Purpose : It is widely known that allergic diseases progress through an allergic march. However, there have not been any recent reports in Korea on how the diseases progress. Methods : Parents who visited one of the Pediatric Allergy Clinics of four university hospitals in Seoul with a child under the age of 6 years during the period from May 1-30, 2006 were asked to complete a questionnaire. Results : A total of 229 parents answered the questionnaire about their children, of which 122 were male and 107 were female. The most common allergic disease before 2 years of age was atopic dermatitis (79%). Meanwhile, in 2- to 4-year age group, allergic rhinitis (38%) and asthma (37%) were predominant. The most common allergic disease in the 4- to 6-year age group was asthma (72%), followed by allergic rhinitis (64%). Seventy-three percent of the children had a family history of allergic disease, most often in the fathers (39%). Among patients with allergic rhinitis, 50% had experienced asthma in their earlier days and 30% had experienced atopic dermatitis. In addition, 57% of the children with asthma had suffered from atopic dermatitis. Parents believed that asthma was the most serious allergic disease. Conclusion : In the present study, allergic disease showed a tendency to march from atopic dermatitis to asthma and then to allergic rhinitis. Early diagnosis and treatment of atopic dermatitis is, therefore, considered important for prevention of the allergic march.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.546-550
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• 2008

In this untreated classic phenylketonuria (PKU) case, mental retardation is severe; however, there have been individuals- like the mother of this case- who have escaped mental retardation and all the other potential sequelae of phenylketonuria, despite having high blood phenylalanine levels, and very poor dietary control. It appears that they have nearly normal brain phenylalanine levels despite high blood phenylalanine (Phe) levels. A number of studies have now demonstrated considerable variability in blood vs. brain phenylalanine levels in phenylketonuria patients. Outcome of phenylketonuria appears to be related to brain phenylalanine levels. We report a case of "undiagnosed" maternal phenylketonuria syndrome. A female infant had low birth weight (2,400 g) with microcephaly. We examined her family and discovered that her mother was an undiagnosed phenylketonuria patient with a borderline intelligence quotient (IQ). The infant's sister, six years old, was diagnosed with phenylketonuria at the age of four years was mentally retarded and had received an operation for cleft lip and palate. the sister had also had a low birth weight (2,300 g). Her sister and mother were compound heterozygotes (mother: R243Q/Y325X; sister: Y325X/P407S). The infant and father were heterozygous carriers (baby: R243Q/ -; father: P407S/ - ).

• Childhood Kidney Diseases
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• v.1 no.1
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• pp.60-66
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• 1997

Even though the urinary stones are rare in children, careful observation and monitoring are necessary because the incidence has been increasing. This study is aimed to document the characteristics of urinary stones in children including the symptoms, diagnosis, with or without urinary tract infection, other urinary tract anomaly and treatment. 45 patients under the age of 15 years with urinary stones hospitalized during Jan. 1986 to Jun. 1996 at Severance Hospital were reviewed retrospectively. Patients' mean age was 6.5 years and sex ratio (male : female) was 5.4:1. The most common symptom was gross hematuria. Stones accompanied with urinary tract infection was 46.7%, and stones associated with urinary tract anomaly was 35.6%. Extracorporeal shock wave lithotripsy, percutaneous nephrolithotorny, hydration and diuretics were the treatment modality used. Urinary stone were found to be a significant cause of urinary tract obstruction in children, requiring prompt diagnosis and treatment. Although KUB, IVP, and ultrasonography were commonly used to make the diagnosis, many cases were detected only by ultrasonographic study.

• Child Health Nursing Research
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• v.22 no.4
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• pp.309-316
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• 2016

Purpose: This study of secondary analysis aims to compare family management style according to severity in children with atopic dermatitis. Methods: A convenience sample of 109 Korean mothers caring for a child with atopic dermatitis, were recruited from the pediatric departments of two general hospitals in Seoul. Data were collected from November 1, 2015 to February 28, 2016. A structured self-report questionnaire was used. Data were analyzed using descriptive statistics, correlation analysis, and one-way ANOVA with IBM SPSS Version 22.0. Results: For family management style, condition management effort, family life difficulty, and view of condition impact were significantly different according to the severity of the atopic dermatitis in these children. Conclusion: The findings indicate that family management strategies to enhance care of children with atopic dermatitis as well as members of the family should include these significant variables in a family-centered approach.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.232-239
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• 2002

Purpose : Renal cystic diseases comprise a mixed group of heritable, developmental and acquired disorders. Recently the use of imaging modalities such as ultrasonography and radionuclide scanning has increased the detection rate of renal cystic diseases. We studied to review the clinical features and treatment of renal cystic diseases in children. Methods : This study was performed in 95 children with renal cystic diseases in the Department of Pediatrics, Asan Medical Center from October 1989 to June 2001. Results : In 95 patients, there were 55 cases(58.0%) with multicystic dysplastic kidney(MCDK), 19 cases(20.0%) with simple renal cysts, 13 cases(13.7%) with hereditary polycystic kidney diseases( 7 with autosomal recessive type, 5 with autosomal dominant type, 1 with undetermined), 6 cases(6.3%) with renal cysts in tuberous sclerosis and 1 case(1.0%) with medullary cystic disease. All MCDK patients had no renal dysfunction and hypertension during the follow-up period. Three out of 13 with polycystic kidney diseases had progressed to end-stage renal disease during the follow-up period. One case with a simple cyst underwent laparoscopic malsupialization for decompression. Conclusion : Renal cystic diseases have diverse clinicopathologic features and variable prognosis. We emphasize that routine follow-up should be performed to prevent and to detect early treatable complication in renal cystic diseases. Therefore, their natural history and treatment need further investigation and long term follow-up is required.

• Pediatric Infection and Vaccine
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• v.18 no.2
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• pp.182-192
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• 2011

Purpose : Atopic findings may be associated with severity of pneumonia in 2009 pandemic influenza A (H1N1) infection, which could suggest a possible association between atopic findings and the severity of viral infections. Thus, we studied association between atopic findings and severity of disease in children with H1N1 influenza infection. Methods : A retrospective study was performed in 74 children admitted in a single tertiary institute and confirmed as H1N1 patients by reverse transcriptase (RT) - polymerase chain reaction (PCR). They were divided into 2 groups according to the severity of pneumonia. We evaluated whether the atopic finding is risk factor between the two groups. Results : Children with severe pneumonia had higher percentages of serum eosinophilia (88% vs 40%, P <0.001), asthma (65% vs 35%, P =0.011), allergic rhinitis (71% vs 40%, P =0.009), and IgE level (P =0.007). We found positive correlations between aeroallergen sensitizations and severity of pneumonia (82% vs 53%, P =0.007). Conclusion : Among patients with H1N1 pneumonia, asthma and atopic findings are risk factors for severity of pneumonia.

• Childhood Kidney Diseases
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• v.5 no.1
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• pp.1-8
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• 2001

Purpose : Clinical manifestations and pathologic findings of thin glumerular basement membrane disease, recognized as a common underlying disease of benign, familaiar and asymptomatic hematuria has not been reported systemically in Korera. We analyzed clinical and pathologic findings of patients who were diagnosed as thin glomerular basement membrane disease Methods : We analyzed clinical and pathologic findings of twenty-six patients who were diagnosed as thin glomerular basement membrane disease by renal biopsy among who complained asymptomatic hematuria from 1990 to 2000. Results : The subjects were aged 9.4${\pm}$3.2 (3.0-15.8) years-old at onset of hematuria, and 11.1${\pm}$2.2 (4.7-16.3) years-old at renal biopsy. Sexual discrepancy was more common in girls (eight boys and eighteen girls). A family history of hematuria was found in 8 patients(30.7$\%$). Major clinical manifestation on admission was microscopic hematuria according to the findings of 3case(11.5$\%$) of gross hematuria, 23cases(88.5$\%$9) of microscopic hematuria, and 1 case(3.8$\%$) of proteinuria. Microscopic hematuria persisted in all cases. Kidney biopsy showed few changes by light microscopy, but IgM, C3 and fibrinogen deposit in mesangium was found by immunofluorescent microscopy in a few cases. Electron microscopic findings have revealed thinning of the glomerular basement membrane varied from 180.9${\pm}$35.8nm. Conclusion : Thin glomerular basement membrane disease might be a common cause of microscopic hematuria of children and family history was revealed in about 30$\%$. Clinical progression was good in majorities.(J. Korean Soc Pediatr Nephrol 5 : 1-8, 2001)

• Childhood Kidney Diseases
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• v.6 no.1
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• pp.48-55
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• 2002

Purpose: Thin glomerular basement membrane nephropathy (TGBMN) is recognized as the leading cause of microscopic hematuria in both children and adults. However thinning of glomerular basement membrane (TCBM) has been found in healthy adult and also is known to be associated with various renal diseases such as Alport syndronh, IgA nephropathy and mesangial proliferative glomerulonephritis. The association of TGBM with minimal change nephrotic syndrome (MCNS) has been very rare so that the present study was undertaken to determine the relationship between TGBM and MCNS. Methods: The study population consisted of 49 children with biopsy- proven MCNS who have been admitted to the pediatric department of Kyungpook University Hospital during the past 5 years from 1997 to 2001. Group I consisted of 8 children associated with TGBM and Group II 41 children without TCBM. Various parameters such as age of illness, duration from discovery of illness to the time of biopsy, family history of hematuria and other laboratory tests were compared between these two groups and the following results were obtained. Results: Age distribution showed slightly older age in Group I ($7.1{\pm}3.5$ years) compared to Group II ($4.8{\pm}2.9$ years). However this was not statistically different (P=0.056). Family history of hematuria was noted in 2 cases in Group II. Though statistically not significant, hematuria was seen in 2 out of 8 cases ($25\%$) in MCNS children with TGBM, compared to 7 out of 41 cases ($17\%$) with MCNS children without TGBM. Other parameters such as BUN, creatinine, 24 hours urine protein excretion, serum protein, albumin, cholesterol, and T4/T8 ratio, showed no difference. Also renal biopsy finding showed no significant difference and the thickness of glomerular basement membrane in Croup I was $188{\pm}30nm$. Conclusion: TGBM was found in 8 out of 49 children with MCNS ($16.3\%$). And this high frequency of occurrence indicates that these association is not an incidental findings. Typical clinical findings of TCBMN was not noted in all of the 8 children with MCNS associated with TGBM, suggesting that thinning of glomerular basement membrane (TCBN) is secondary to rather than the cause of MCNS. (J Korean Soc Pediatr Nephrol 2002;6: 48-55)

• Clinical and Experimental Pediatrics
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• v.50 no.5
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• pp.489-492
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• 2007

Idiopathic renal hypouricemia is a disorder characterized by impaired urate handling in the renal tubules. This disease usually produces no symptoms, but hematuria, uric acid nephrolithiasis or acute renal failure may develop. A defect in the SLC22A12 gene, which encodes the human urate transporter, is the known major cause of this disorder. We describe a 10-month-old boy with idiopathic renal hypouricemia. He was diagnosed with transient pseudohypoaldosteronism at admission, but hypouricemia was accidentally found through follow-up study. By gene analysis, his diagnosis was confirmed to idiopathic renal hypouricemia. In addition, we report a mutation in the human urate transporter 1 (hURAT1) gene identified in his family.

• Clinical and Experimental Pediatrics
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• v.45 no.11
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• pp.1403-1410
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• 2002

Purpose : In previous studies, various risk factors for recurrent febrile seizures have been identified. But none of these risk factors alone could sufficiently discriminate children at high or low risk for recurrent seizures. Therefore, we tried to identify patients at high risk of recurrent febrile seizures by combining risk factors. Methods : Two hundred and four children who had been admitted to our hospital from March, 1997 to July, 1999 with their first febrile seizures were enrolled in our study, and followed up over 2 years. We investigated the recurrence rate according to variables such as sex, age at first febrile seizure, family history of febrile seizures or epilepsy, type of the first seizure, neurologic abnormality and EEG abnormality. Results : Family history of febrile seizures and age at first febrile seizure(<12 months) were significant independent risk factors for recurrent febrile seizures. With these two combined factors, four groups were allocated and the recurrence rate by each group was designated as follows: group with no family history of febrile seizures and age at first febrile seizure ${\geq}12$ months (no risk factor), 43.8%; the group with no family history and age <12 months(one risk factor), 61.7%; group with family history and age ${\geq}12$ months(one risk factor), 64.5%; group with family history and age <12 months(two risk factors), 90.4%. Conclusion : A correlation between numbers of risk factors and recurrence rate was present and the children with a family history of febrile seizures and a young age at onset(<12 months) were regarded as a high risk group of recurrence.