• Childhood Kidney Diseases
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• v.9 no.2
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• pp.137-142
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• 2005

Purpose : Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A$\beta$-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. Methods : Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A $\beta$-hemolytic streptococcal infection were evaluated. Results : Six(12.5$\%$) patients showed elevation of serum creatinine level but there was no patient with Persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9$\%$). Hematuria disappeared within 6 months(79$\%$) and proteinuria within 6 months(100$\%$) from the disease onset. Conclusion : Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4$\%$) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequoia. (J Korean Soc Pediatr Nephrol 2005;9:137-142)

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.167-174
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• 2005

Purpose : Relapses are a major problem in children with steroid responsive nephrotic syndrome(SRNS). This study has been performed to determine the predictive factors for relapse in children with SRNS. Methods : The study group consisted of 7,3 children with SRNS who had been admitted to the Department of Pediatrics, Kyungpook National University Hospital, over 6 years from 1996 to 2001. The medical records were reviewed retrospectively and analyzed to determine significant relationships between selected variables[age at onset, sex, laboratory data, the rapidity of response(days to remission), interval to first relapse] and the frequency of relapse. Results : The age($mean{\pm}SD$) of patients was $4.53{\pm}2.53$ years old. The male to female ratio was 52:21. In 95$\%$, 39 out of the 41 children had a renal biopsy, and the final diagnosis was minimal change nephrotic syndrome. There was no significant correlation between the frequency of relapse and the following variables age at onset, sex, and presence of hematuria. However, the rapidity of response correlated well with the frequency of relapse, especially during the first year after the onset of the disease(P=0.005). Conclusion : The rapidity of response is expected to be one of the predictive (actors for relapse in children with SRNS. (J Korean Soc Pediatr Nephrol 2005;9:167-174)

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.953-959
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• 2005

Purpose : The administration of total parenteral nutrition(TPN) has become a standard procedure in the management of nutritionally deprived and critically low birth weight neonates. Sepsis remains the most frequent serious complication during TPN, resulting in increased morbidity, mortality and health care costs. This study was performed to evaluate the clinical efficacy and complications of percutaneous central venous catheterization(PCVC) in very low birth weight infants. Methods : A total of 56 very low birth weight infants below 1,500 g during the period from January 1998 to December 2003 were enrolled and their medical records reviewed. Study group(n=32) included the babies who had undergone PCVC and a control group(n=24) included babies who had not undergone PCVC. We compared the study group with the control group for factors such as subject characteristics and catheter-related complications. Results : There was no difference in subject characteristics, such as birth weight, gestational week, respiratory distress syndrome, duration of ventilator therapy, duration from tube to complete oral feeding, days at TPN and its total duration, body weight at discontinuation of TPN and the days taken to reach to 2,000 g. However, the morbidity rate due to patent ductus arterious, chronic lung disease, necrotizing enterocolitis, osteopenia, cholestasis, and sepsis showed no difference. The study group with infants below 1,000 g showed a higher incidence of sepsis compared to the control group of the same weight group. The study group with infants between 1,000 to 1,500 g showed significantly higher incidences of intraventricular hemorrhage and took longer reach the a body weight of 2,000 g. Conclusion : Considering the high incidence of sepsis in the PCVC group, every attempt should be made to minimize the length of TPN therapy and encourage early enteral feeding. We also recommend the use of PCVC carefully in patients requiring prolonged nutritional support.

• Clinical and Experimental Pediatrics
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• v.48 no.8
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• pp.871-876
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• 2005

Purpose : Recently, it was reported that tumor necrosis factor(TNF) and $lymphotoxin-{\alpha}$($LT-{\alpha}$) gene regions might be a susceptible loci to type 1 diabetes in Japanese. The purpose of this study was to investigate the association of TNF and $LT-{\alpha}$ gene polymorphisms with disease susceptibility in Korean children with type 1 diabetes. Methods : Forty-nine Korean children with type 1 diabetes(29 girls and 20 boys) and 94 healthy Koreans were investigated in this study. Genotyping for -857T/C polymorphism in the TNF promoter region and $LT-{\alpha}$ gene polymorphism were performed by PCR-RFLP(restriction fragment length polymorphism). TNF promoter -1031C/T polymorphism was detected by allele-specific PCR. Results : The distribution of the -857T/C and -1031C/T genotype in the TNF promoter region was not different between diabetic children and the controls. The frequency of TT genotype in the distribution of TNF -1031C/T polymorphism in diabetic children with diabetic ketoacidosis(DKA) at diagnosis was significantly lower than those without DKA(P<0.05). No significant difference in the distribution of $LT-{\alpha}$ gene polymorphism was observed between diabetic children and the controls. There was no association between clinical characteristics of type 1 diabetes and $LT-{\alpha}$ gene polymorphisms. Conclusion : These results suggest that TNF promoter -857T/C and $LT-{\alpha}$ gene polymorphisms are not associated with susceptibility to type 1 diabetes in Korean children. TNF promoter -1031C/T polymorphism might be related to clinical manifestations(DKA) of type 1 diabetes.

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.991-997
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• 2005

Purpose : Cow's milk protein-induced enterocolitis(CMPIE) is a symptom complex of vomiting and/or diarrhea caused by delayed hypersensitivity and may result in serious complications. This study was undertaken to identify high risk factors to facilitate the early recognition of CMPIE. Methods : We reviewed the data of 101 patients, aged 15 to 45 days, admitted due to vomiting and/or diarrhea between 2003 and 2004. After excluding 13 patients absolutely breast-fed and 2 patients transferred from other hospitals with the impression of CMPIE, the 86 study subjects were divided into three groups based on the underlying etiologies; CMPIE, infectious and non-infectious group. Results : CMPIE was diagnosed in 11 patients(12.8%). On admission, failure to gain weight(P=0.003), hypoalbuminemia(P=0.003), peripheral leukocytosis(P=0.015), and metabolic acidosis(P=0.014) were more significant in the CMPIE group than in the others. Multiple logistic regression analysis showed that the independent predictors of high risks for CMPIE were failure to gain weight <10 g/day(OR, 10.25[95% CI, 1.62-65.06]) and serum hypoalbuminemia <3.5 g/dL(OR, 9.18[95% CI, 1.69-49.74]). Cow's milk challenges were performed in the 11 CMPIE patients; vomiting(81.8%), abnormal stool test(80.0%), peripheral leukocyte count and absolute neutrophil count(ANC) increase(100.0%) (P<0.05), and enteropathy(100.0%). Conclusion : CMPIE is not a rare clinical disease in early infancy. The high risk factors of CMPIE were identified as follow : failure to gain weight below 10 g/day, hypoalbuminemia on admission and a rapid decrease during admission. Cow's milk challenge test with endoscopic duodenal biopsy was helpful to confirm CMPIE.

• Clinical and Experimental Pediatrics
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• v.48 no.9
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• pp.969-975
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• 2005

Purpose : We performed a study of clinical findings of Mycoplasma Pneumonia in children, to know differences between recent clinical manifestations of Mycoplasma pneumonia and previous studies. Methods : The subjects of this study were 393 children who were diagnosed as Mycoplasma pneumonia with high titers of Mycoplasma antibody(${\geq}1$ : 160) or fourfold rises of Mycoplasma antibody at Chung Ang University Hospital from January 1998 to December 2003. We practiced a retrospective study on the clinical manifestations of Mycoplasma pneumonia based on their medical records. Results : Male to female ratio was 1.06 to 1 and mean age was $4.32{\pm}2.94years$. The highest incidence was in the age of 2 to 3 years(18.6 percent). Most frequent months were October, and November in 2000, April in 2002, and October and, December in 2003. Twenty six point seven percent showed allergic diseases. Second degree relatives of 10.7 percent patients had allergic diseases. Forty three point three percent were admitted before this admission for pneumonia. Allergic tests were positive in 65.7 percent. Cough, and sputum were the most common symptoms and abdominal pain, and vomiting were the most frequent extrapulmonary symptoms. Atelectasis and pleural effusion were seen in 2.5 percent and 1.8 percent. Infiltrations were more common on the right side. Titers of each simultaneous test for cold agglutinin and mycoplasma antibody were not in proportion to each other (P=0.163). Conclusion : The onset age of mycoplasma pneumonia was found to be lower than it used to be. More patients had a past medical history or a family history of allergic disease, and more wheezing was heard and associated with recurrent pneumonia.

• Clinical and Experimental Pediatrics
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• v.46 no.8
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• pp.784-788
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• 2003

Purpose : Perinatal asphyxia occurring in newborn is one of the major causes of acute mortality and chronic neurological disability in survivors. We have studied the relationship between early electroencephalography(EEG) findings and clinical course and neurologic outcome in severe asphyxiated neonates. Methods : Between the period of July 1999 and June 2002, 25 neonates who were diagnosed with severe perinatal asphyxia(1-minute Apgar score of ${\leq}3$ and initial pH is less than 7.2) at NICU in Dongsan Medical Center were enrolled. An EEG was recorded and analyzed within three days of life and divided into two groups - group 1(normal or focal change on EEG) and group 2(generalized abnormal EEG). Between the two groups, clinical courses and neurologic outcomes were compared. Results : Fifteen infants(60%) were group 1 and ten infants(40%) were group 2(polyspikes, burst-suppression, generalized low voltage). Associated maternal disease, days of hospitalization, need for ventilator support, delay of oral feeding and convulsion duration are significantly higher and longer in group 2. Also, poor neurologic outcome(expire, developmental delay) was significantly higher in group 2(60%) than group 1(13.3%). Conclusion : Thus, the early neonatal EEG in asphyxiated newborn can be a predictable diagnostic tool in assessment of neurologic outcome.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.299-306
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• 2008

Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

• Clinical and Experimental Pediatrics
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• v.50 no.9
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• pp.891-895
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• 2007

Purpose : Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system and mostly develops after viral illness or vaccinations. We investigated the clinical differences and neurologic outcomes according to the distribution of the lesions on brain MRI. Methods : The study group was composed of 21 patients from January 1995 to August 2003 in Kyunghee University hospital. We grouped the patients according to the MRI findings as follows. Group I (14 cases): Multi- or unifocal lesions only in the cerebral white matter. Group II (7 cases): lesions in the gray matter with or without white matter involvement. Results : 1. Preceding events were as follows: no defined prodrome (38.1%), upper respiratory tract infection (28.6%), nonspecific febrile illness (19.0%), gastointestinal disturbance and vaccination. 2. Presenting symptoms were as follows: seizures (76.2%), headache/vomiting (47.6%), altered consciousness (38.1%), hemiparesis, cerebellar ataxia, visual disturbance and facial nerve palsy. 3. Laboratory findings were as follows: CSF pleocytosis (76.2%), leucocytosis (38.1%) and elevated CSF protein (28.6%). 4. Fifteen patients were recovered completely without neurological sequelae. Three patients in group I and 1 patient in group II had intractable seizures. Two patients in group I and 2 patients in group II had motor disturbance. Conclusion : There were no statistically significant differences in preceding events, presenting symptoms, and neurological outcomes according to the distribution of the lesions on brain MRI. However, the ADEM have quite diverse clinical manifestations and neuroimage findings. MRI plays an important role in making diagnosis of the patients who are suspected of ADEM.

• Clinical and Experimental Pediatrics
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• v.51 no.3
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• pp.315-322
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• 2008

Purpose : Prader-Willi syndrome (PWS) is a complex genetic disorder, caused by the deletion of the paternally derived 15q11-13 region or the maternal uniparental disomy of chromosome 15 (mUPD(15)). In this study, we compared phenotypic differences between those patients whose disease was caused by microdeletion and those caused by mUPD(15). In addition, a comparison of the efficacy of growth hormone (GH) therapy between these two PWS genotypes was analyzed. Methods : Fifty-three patients were diagnosed as having PWS based on molecular and cytogenetic analyses and clinical features. Data that included maternal age, birth weight, a feeding problem in the neonatal period, cryptorchidism, developmental delay or mental retardation, short stature, hypopigmentation, changes in height, weight, and body mass indexes (BMI) before and after GH treatment were obtained by a retrospective review of medical records. The data from the patients with microdeletion were compared with those from the patients with mUPD(15). Results : Of the 53 patients with genetically confirmed PWS, 39 cases had microdeletion and 14 mUPD(15). Maternal ages were significantly higher in the mUPD(15) group, and hypopigmentation and a feeding problem in the neonatal period were more frequent in the microdeletion group. Growth hormone was administered to 20 patients [14 with microdeletion, 6 with mUPD(15)]. There were no differences between the two groups in height velocity, weight and height SDS, and BMI after GH therapy. Conclusion : Phenotype and genotype correlations were observed in Korean PWS patients, such as more advanced maternal ages in the mUPD(15) group and more feeding problems and hypopigmentations in the microdeletion group. Further long-term prospective studies are needed to correlate other aspects of the phenotypes.