• Clinical and Experimental Pediatrics
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• v.45 no.3
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• pp.370-375
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• 2002

Purpose : In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. Methods : Ten children with MDS(CMMoL 5, RAEB 3, RAEBt 2) underwent HSCT(HLA-matched sibling transplantation 4, HLA-matched unrelated transplantation 4, cord blood transplantation 1, HLA-mismatched familial transplantation 1) between November 1995 and January 2001 at St. Mary's Hospital. Median follow-up duration was 11 months. Results : Engraftment was successful in all cases and 8 patients are alive without disease. Three cases of VOD were observed and improved without complication. Four cases of grade II and 1 case of grade III acute GVHD were observed and well controlled with treatment. Three patients relapsed after transplantation. One patient is alive without disease after cytoreduction with allogenic stem cell rescue and 2 patients died of relapse. Conclusion : HSCT is a curative strategy of MDS and the survival rate is relatively higher than that of adults. But there is an obvious need for more studies because of the small number of patients and the short duration of the follow-up.

• Clinical and Experimental Pediatrics
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• v.46 no.6
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• pp.566-571
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• 2003

Purpose : Leukoencephalopathy(LE) is one of the most serious complications in children with hematologic malignancies during the course of treatment. Early recognition is important to reduce the impact and sequelae from LE. We therefore investigated the clinical features of LE following central nervous system(CNS) prophylaxis in children with hematologic malignancies and evaluated the significance of regular check-ups of brain MRI. Methods : We retrospectively reviewed children with hematologic malignancies who had CNS prophylaxis including intrathecal(IT) methotrexate(MTX) and/or cranial irradiation at the Department of Pediatrics, Kyungpook National University Hospital from Oct. 1995 to May 2002. Fifteen cases of acute leukemia and one case of lymphoma who experienced LE following CNS prophylaxis were included in the study. Clinical data were analyzed from the medical records and brain MRIs were reviewed by neuroradiologists. Results : The ages ranged from 1 to 13 years(median age=5.2 years), and the male to female ratio was 3 : 1. The time interval from the beginning of chemotherapy to the time of diagnosis of LE ranged from 2 to 17 months. They all had IT MTX two to 15 times and ten underwent cranial irradiation(1,800 rads). At the time of diagnosis, ten of them had neuropsychiatric symptoms including seizures, personality changes, headache, etc. After the change of treatment modality, four cases showed significant improvement on follow-up MRIs, six cases had no significant changes and two had worsening of LE. Four patients died of infection and bone marrow relapse. Conclusion : CNS prophylaxis with IT therapy and cranial irradiation may cause leukoencephalopathy during the course of treatment. As a result, regular brain MRI check-up is recommended for the early detection and reducing the incidence of LE, along with changes in the treatment modality.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.370-375
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• 2003

Purpose : It has been suggested that the exposure to aeroallergens during early infancy after birth is important in the subsequent development of sensitization and allergic diseases. In Korea, the level of house dust mites as one of the important aeroallergens is known to be the highest in autumn. The aim of this study was to test whether the distribution of month of birth bears a relationship to the presence of mite sensitization in children with respiratory allergy. Methods : Skin prick tests and methacholine provocation tests were performed on 1,327 patients with chronic respiratory symptoms who visited Seoul National University Children's Hospital from January 1995 to May 2002. An analysis of patients' month of birth distribution according to the presence of mite sensitization was performed. Results : Atopic subjects who had at least one positive skin test numbered 864(65.1%); and non-atopic subjects numbered 463(34.9%). Among atopic subjects, 787(59.3%) had positive skin tests to mites and 77(5.8%) had positive skin test only to minor allergens. A significantly greater than expected number of mite atopic subjects were born in the months between August and November(P=0.03), however, the birth month of non-atopic subjects didn't show a consistent seasonal preference. Asthma patients numbered 543(40.9%). Among these, atopic asthmatics numbered 421(77.5%) and non-atopic asthmatics, 122(22.5%). Dust-mite atopic asthmatics numbered 387(91.9%) out of 421 atopic asthmatics. Dust-mite atopic asthmatics were born significantly higher in the season lasting from August to November in comparison to non-atopic asthmatics(P=0.002). Conclusion : Month of birth seems to be related with sensitization to allergens. Our results show that August to November is the risk period for the development of mite sensitization in Korea.

• Clinical and Experimental Pediatrics
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• v.52 no.10
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• pp.1161-1166
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• 2009

Purpose : Kawasaki disease—the most common cause of acquired heart disease in children—incidence is increasing yearly. Therefore, we evaluated the predictive indicators of coronary complications of Kawasaki disease based on clinical and laboratory data. Methods : Between January 2005 and March 2008, of the 201 children with Kawasaki disease treated at the Gil Hospital of Gachon University of Medicine and Science, 51 had coronary artery lesions (Group II) and 150 had no lesions (Group I). The reasons for coronary artery lesions were deduced from the clinical and laboratory data. Results : Analysis of the 2 groups revealed that fever duration and days of fever after and before initial intravenous gammaglobulin (IVIG) treatment were significantly longer in Group 2 than in Group I. IVIG infusions were statistically higher in Group II than in Group I. As per the laboratory data, C-reactive protein (CRP) value was significantly higher in Group II. Collectively, >10 days of fever duration, >48 h of fever duration after, and >10 days of fever before IVIG treatment increased the risk of coronary artery lesions 6-, 5-, and 3.5-fold, respectively. Furthermore, additional IVIG courses and higher CRP level increased the risk of coronary artery lesions 4-fold and 2-3-fold, respectively. Conclusion : The following 3 factors were responsible for increased risk of coronary artery lesions in children with Kawasaki disease: fever duration and days of fever after and before IVIG treatment. To identifythe predictive indicators of coronary complications, it is necessary to further elucidate the relationship between well-known forecasting factors.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.11 no.1
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• pp.3-15
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• 2000

Objectives：There has been a rapid expansion of studies aimed at elucidating the genetic basis of autistic disorder, especially it’ relationship to fragile-X syndrome. The detection of fragile X chromosome(Xq27.3) by cytogenetic analysis has revealed many difficulties in testing. Therefore, to explore the relationship between autistic disorder and fragile X syndrome, this study administered molecular biologic methods which examined an unstable CGG repeat within the fragile X mental retardation-1(FMR-1) gene. Methods：Ninety nine autistic children and eight normal control children were tested. The number of CGG repeats within FMR-1 gene was measured after amplification by PCR, and cytogenetic analysis was also carried out to detect fragile site Xq27.3. Southern blot hybridization, using StB12.3 and/or Pfxa3 probe, was done for the patients showing expansion of more than 50 CGG repeats (premutation). Results：All but two autistic patients had no expansion in CGG repeats by PCR and there was no significant statistical difference in number of CGG repeat in comparison with normal control. Two autistic patients, considered as premutation by PCR analysis, had no full mutation or premutation by Southern blot hybridization. All autistic children tested did not have any abnormal karyotype or fragile site Xq27.3. Conclusions：These results suggest that autistic patients may not have abnormality in FMR-1 gene or abnormal expansion in CGG repeat. In conclusion, fragile X syndrome may not be antecedent of autistic disorder.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.17 no.1
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• pp.19-26
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• 2006

Objectives : The object of this study was to examine computer game playing patterns and psychopathologies related to computer game addiction in school-age children. Methods : The subjects were 533 elementary school students (4th to 6th grade) in Kangdonggu, Seoul. We evaluated computer playing patterns of all subjects using computer game playing pattern questionnaire, and determined the risk group of computer game addiction by internet game addiction scale score. We evaluated subscale score of K-CBCL from parents of all subjects, and conducted correlation analysis and logistic regression analysis between computer game addiction and subscale score of K-CBCL. Results : In 488 responders, 10.2% of started playing computer game in preschool age, and 67.2% started at low grade of elementary school. The mean frequency of computer game play per week was 3.66 days. Mean time spent playing computer games per day was 1.89 hours. 'Simply for fun' was the most common reason far playing computer games (40.8%). Male subjects showed statistically significant differences in age of starting computer game, frequency of computer game play per week, reasons for playing computer game and computer game addiction scale scores. There were significant correlations between computer game addiction scale scores and academic performance, somatic complaints, attention problems, and internalizing problems in K-CBCL. But In logistic regression analysis, only attention problems among K-CBCL subscales showed significant predictability to computer game addiction. Conclusion : Upper grade elementary school students experienced computer game playing at the very early age, and spend much time in playing computer games. There were significant correlation and predictability between computer game addiction and attention problems.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1016-1023
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• 2002

Purpose : Brain tumors are the most common solid tumor in children. We retrospectively investigated the clinical characteristics of pediatric brain tumors, such as age, sex, tumor site and survival, as seen in a single institution over the last 15 years. We tried to evaluate the role of chemotherapy on the survival of some brain tumors. Methods : Three hundred fifty four children with primary brain tumor who were treated at Severance Hospital from Jan. 1985 to Sep. 2001 were enrolled. Results : Pediatric brain tumors were found most frequently in 10-15 years of age group(35.3%) and the ratio of male to female was 1.3 : 1. Supratentorial tumors(52%) were more frequent than infratentorial tumors(48%). Medulloblastoma/primitive neuroectodermal tumor(PNET) was the most common type(24.6%), followed by cerebellar astrocytoma(14.1%). Ten year survival rate of medulloblastoma, cerebellar astrocytoma and cerebral astrocytoma were 59.4%, 79.3% and 71%, respectively. The prognosis for brain stem glioma and glioblastoma multiforme were still grim with a 10 year survival rate of 12.7% and 13.3%, respectively. The addition of chemotherapy for high grade medulloblastoma led to an improved 10 year survival rate of 54.5%, compared with 40% without chemotherapy. Conclusion : The combined use of chemotherapy and radiation and surgery improved survival rate of pediatric brain tumors in our study. Chemotherapy for high grade medulloblastoma improved the 10 year survival rate. Further data analysis of the treatment modalities will lead to better comparisons.

• Clinical and Experimental Pediatrics
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• v.45 no.8
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• pp.1007-1015
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• 2002

Purpose : The aim of this study is to find a method to diagnose and treat children who showed benign hemophagocytic histiocytes in bone marrow examination. Methods : We analyzed the clinical data of thirty patients retrospectively who showed benign hemophagocytic histiocytes in bone marrow examination from January 1995 to November 2001 at Keimyung University Dong-san Hospital. Bone marrow histiocytes were classified into a few, some, and many according to the number of histiocytes comparing with the white cells. Results : The age of thirty patients ranged from two months to 15 years. The median age was 5.6 years with male predominance(2.3 : 1). The most frequent clinical manifestation was fever; others were respiratory symptom, hepatosplenomegaly, lymphadenopathy and skin rash(in order of frequency). Common laboratory findings were leukopenia, thrombocytopenia, anemia and abnormal liver function test. Infection was present in 30 patients; causative organisms were documented in 15 patients, and bacterial infection was more common. Epstein Barr virus was the cause of infection in four patients. Bone marrow examination showed a few(20.0%), some(75.0%) and many(30.0%) hemophagocytic histiocytes. Combination immunochemotherapy including immunoglobulin, steroids and cyclosporine were helpful in 22 out of 30 patients. The mortality rate was high in young patients who showed some to many hemophagocytic histiocytes. Conclusion : Bone marrow examinations and early detection of histiocytes will be helpful in children who have fever, hepatosplenomegaly and abnormal liver function test. Immunochemotherapy were helpful and further investigation will be needed for the detection of the relationship between the bone marrow findings and prognosis.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1067-1072
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• 2003

Purpose : Non-accidental intracranial hemorrhage in children is not low in incidence and results in high mortality and serious sequelae. So, the authors have researched the distribution of sex and age, causes, symptoms and signs, hemorrhagic types, mortality rate and sequelae of the patients hospitalized with non-accidental intracranial hemorrhage at Hallym University Sacred Heart Hospital. Methods : The medical records of twenty patients, aged 15 or younger, and excluding neonatal patients, were analyzed retrospectively. The patients in this study were admitted with non-accidental intracranial hemorrhage from January 1999 to June 2002. Results : Of the twenty cases, the ratio of male to female was 1 : 0.8. The patients aged one or less and between 11 and 15 were discovered to be the most frequent cases. Shaken baby syndrome and arteriovenous malformation were found to be the most frequent causes. Seizure was most frequently found to be a symptom and a sign. Hemorrhagic type was classified into subdural hemorrhage eight, intracerebral hemorrhage five. There were three mortal cases. Twelve surviving patients, excluding five not-followed ones, were reclassified into six cases of complete recovery and six of sequalae. Conclusion : Non-accidental intracranial hemorrhage in children is not low in incidence, with a high mortality rate and a high incidence of serious sequelae after survival. Consequently, early diagnosis and appropriate treatment are required. In addition, appropriate rehabilitation after treatment is needed because the high survival rate due to advanced medical treatment results in an increasing number of neurologic sequelae.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.2
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• pp.138-146
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• 2007

Purpose: Ischemic enterocolitis (IEC) is the total or partial infarction of the intestine in the absence of occlusion of a major mesenteric blood vessel. The purpose of this study was to evaluate the clinical features of IEC in children. Methods: A clinical analysis of 6 patients with IEC who were admitted to the Department of Pediatrics at Pusan National University Hospital, between 1996 and 2005 was conducted retrospectively. Patients were diagnosed with IEC based on clinical characteristics, including radiologic, endoscopic, histopathologic, and intraoperative findings. Results: Four boys and 2 girls between the age of 6 weeks and 6 years were included in this study. Most of the patients were born at term and had a birth weight that was appropriate for their gestational age. The major symptoms of IEC observed included hematochezia or hematemesis (5 cases), vomiting, diarrhea, abdominal pain or irritability (4 cases), as well as abdominal distension and fever (3 cases). IEC occurred in thecolon in 5 cases (2 descending colon, 1 descending and sigmoid colon, 1 sigmoid colon, 1 whole colon) and the duodenal bulb and gastric antrum in 1 case each. The type of the lesions observed includedulcera, which were found in 3 cases, perforation, which was pbserved in 2 cases, necrotic patches, which were observed in 2 cases, stricture, which was observedin 1 cases, and massive membranous desquamation of the epithelium, which was observed in 1 case. Two of the patients received surgical treatment and the remaining four were treated conservatively. None of the patients died. Conclusion: The presentation of IEC varies, and the findings of this study will be helpful in managing patients with IEC.