• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.161-166
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• 2003

Purpose: The purpose of this study was to evaluate the efficiency of treatment of living-related liver transplantation (LRLT) with the parental heterozygote carrier graft in children with Wilson disease. Methods: We retrospectively evaluated 7 children with Wilson disease who had received liver transplantation from 1994 to 2002 at Asan Medical Center. All the donors were parental. Liver functions, Kayser-Fleischer ring, and other factors regarding to copper metabolism were analyzed. Results: Of the 7 children, 5 had fulminant hepatitis and 2 had decompensated liver cirrhosis irresponsive to medical therapy. All donors being parental, all grafts came to be heterozygote carrier grafts. Survival rate was 100% in those 7 children, 87% in all children with liver transplantation in the same period, and 84% in children with non-metabolic liver disease. After liver transplantation, all 7 children could stop low copper diet and penicillamine therapy and their AST, total bilirubin and prothrombin time were recovered to normal. After liver transplantation, ceruloplasmin and serum copper levels were also recovered to normal. A marked reduction in 24 hr-urinary copper excretion was observed in all recipients after transplantation. During follow-up, Kayser-Fleischer rings resolved completely after LRLT in 5 children and partially in 1 child. Conclusion: We concluded that living-related liver tranplantation in children with Wilson disease with parental heterozygote carrier graft is an effective treatment modality.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.2
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• pp.116-121
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• 2008

Purpose: Cricopharyngeal incoordination is a rare cause of swallowing difficulties in newborns and infants; it is characterized by delayed pharyngeal contractions related to cricopharyngeal relaxation. Dysphagia and repeated aspiration are common findings despite normal sucking. We conducted this study to assess the clinical features of cricopharyngeal incoordination in newborns and infants. Methods: An analysis of the clinical data from 17 patients with cricopharyngeal incoordination who were admitted to the Department of Pediatrics, Pusan National University Hospital, between 2000 and 2006 was conducted retrospectively. The diagnosis of cricopharyngeal incoordination was established by the clinical characteristics and the videofluoroscopic swallowing studies. Results: The male to female ratio was 1:1.1 (males 8, females 9) the age range 1 to 60 days. The body weight of 11 patients (64.7%) was less than the $10^{th}$ percentile at diagnosis. Six patients (35.3%) were born prematurely. The associated anomalies or diseases were chromosomal anomaly (2 cases), congenital heart disease (3 cases), and laryngomalacia, hypoxic brain damage or neonatal seizures (1 case each). The chief complaints of patients were recurrent aspiration pneumonia (10 cases), feeding difficulty (9 cases), dyspnea (4 cases), and chocking (4 cases). The severity of aspiration on the videoesophagogram or esophagogram was mild in 12 cases. The correlation between the severity of aspiration and the duration of tube feeding after the diagnosis was significant (p<0.05). Conclusion: Cricopharyngeal incoordination should be considered in the differential diagnosis of newborns and infants, without known risk factors associated with swallowing dysfunction, when they present with unexplained respiratory problems. Although the prognosis of cricopharyngeal incoordination is good, early diagnosis and tube feeding are recommended to prevent the complications associated with this disorder.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.2
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• pp.130-136
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• 2008

Purpose: Lamivudine is known to be effective for the treatment of chronic hepatitis B in adults. However, data on lamivudine therapy in pediatrics is limited. The aim of this study was to evaluate the efficacy and durability of lamivudine therapy for chronic hepatitis B in Korean children. Methods: A total of 44 children (27 males and 17 females, ages 6 months to 14.8 years, mean age 6.7 years) with chronic hepatitis B who received lamivudine (3 mg/kg/day, max 100 mg) for at least 12 months were enrolled. We evaluated the serum AST, ALT and serological HBV markers (HBsAg and anti-HBs, HBeAg and anti HBe, and HBV DNA) periodically. Predictive three year cumulative seroconversion rates were obtained using the Kaplan-Meier method. Results: Twenty one (48%) of 44 children achieved seroconversion of HBeAg by three years, while 23 (42%) children did not. HBV DNA was cleared in 34 (77%) children and the serum ALT levels were normalized in 41 children (93%). The three year cumulative seroconversion rates were 60% for HBeAg, and the clearance rates were 76% for HBV DNA. Eighteen children who discontinued lamivudine after HBeAg seroconversion maintained the therapeutic response for three years (treatment duration 13~58 months mean 24 months). Viral breakthrough developed in 12 children (27%) during the therapy and the YMDD mutation was documented in 11 children (25%). The mean duration for the development of a mutation was 22.7 months. Loss of HBsAg occurred in 6 children (14%). The pretreatment ALT levels were higher in responders; however, the differences were not statistically significant (p>0.05). Conclusion: The results of this study showed that lamivudine treatment had a favorable effect and durable therapeutic response in children with chronic hepatitis B. Long term follow-up and alternative therapy are warranted for those patients who do not respond to this treatment.

• Pediatric Infection and Vaccine
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• v.10 no.2
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• pp.186-192
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• 2003

Purposes : Recently, acute gastroenteritis broke out among the neonates transferred from postpartum care centers located in Seoul, and Gyeounggi area. Thus, we researched the cause, progress and characters of the disease by analyzing the cases from 9 hospitals in Seoul and Gyeounggi area. Methods : We conducted retrospective study of 33 neonates transferred from postpartum care centers from October 2001 to July 2002 : we divided the neonates into 2 groups. The severe group had any of following 5 conditions : blood pH <7.20, respiratory difficulty, mechanical ventilation, shock, and disseminated intravascular coagulation. And the rest were classified into the moderate group. Results : The severe group was significantly more aged than the moderate group(P= 0.005). Weight loss was significantly severe in the severe group(P=0.0512). In blood gas analysis, bicarbonate was less in the severe group than the moderate group(P=0.032). In the virus examination, rotavirus was detected in 7 cases, and astrovirus was detected in 1 case. In the severe group, 4 neonates were dead. Conclusion : In acute gastroenteritis of neonates, early diagnosis and treatment are important. Thus, the legislation of postpartum care center is needed, and the medical specialists should be stationed in postpartum care center. We assume that the severe group had severe conditions because they were treated comparatively late. However, to find out the cause of the disease and to cope with it, the nationwide epidemiologic study on acute gastrenteritis of neonates is needed.

• Pediatric Infection and Vaccine
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• v.17 no.1
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• pp.30-35
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• 2010

Purpose : We investigated the causative organism and its antibiotic susceptibility of community acquired urinary tract infection (UTI) in children at a secondary hospital to test the adequacy of the current guidelines. Methods : Children diagnosed with UTI at the Department of Pediatrics, Kwandong University MyMyongji Hospital by pyuria and bacterial growth of greater than $1.0{\times}10^5CFU/mL$ on clean catch midstream urine from January 2005 to December 2008 were studied retrospectively. The epidemiologic data, causative organism, and the antibiotic susceptibility were analyzed. Results : Sixty two children were diagnosed with sixty four cases of UTI's. Two bacteria were isolated in one case and thus data on 65 urine cultures were analyzed. The male:female ratio was 1.6:1 and 78.1% were less than 12 months of age. Escherichia coli was the predominant cause consisting of 53 cases (82.8%) of the cases. K. pneumoniae (5), Enterobacter (4), Enterococcus (1), $\beta$-streptococcus (1), Diphtheroides (1) were isolated. The antibiotic resistance of E. coli were as follows; ampicillin 69.8%, cefotaxime 1.9%, gentamicin 15.1%, amikacin 0.0%, levofloxacin 1.9%, and trimethoprim/sulfamethoxazole 26.4 %. Only one case of the E. coli was extended spectrum $\beta$-lactamase (ESBL) positive. Conclusion : Compared to prior reports from other tertiary hospitals in Korea, E. coli was the predominant cause in childhood UTI and the rate of ESBL positivity was low. The antibiotic resistance was also different compared to prior reports. We conclude that a difference in the cause and antibiotic resistance of childhood UTI exists between centers and this should be taken into consideration when prescribing antibiotics for childhood UTIs.

• Pediatric Infection and Vaccine
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• v.17 no.1
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• pp.36-48
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• 2010

Purpose : In children on anticancer chemotherapy, bloodstream infections (BSIs) are a major cause of morbidity and mortality. We investigated febrile episodes and bloodstream infections in pediatric cancer patients to guide proper selection of empiric antibiotics for febrile pediatric hemato-oncologic patients. Methods : All febrile episodes treated in the division of hematology-oncology, the department of pediatrics, Hanyang University Hospital, between July 2005 and June 2008 were reviewed. Episodes with and without bloodstream infections were compared. Results : Forty cases (18.9%, 25 patients) of BSI occurred in 212 febrile episodes (63 patients). Thirty-seven cases (23.6%, 22 patients) of BSI occurred in 157 febrile episodes with neutropenia (54 patients). Microorganisms identified in BSI corresponded to 23 gram-positive bacteria (51.2%), 20 gram-negative bacteria (44.5%), and 2 fungi (4.4%). Rates of BSI between those who had received umbilical cord blood transplantation and those who had received transplantation from other source were significantly different (55.0% vs. 7.7%, P =0.001). No differences in mortality rate were observed among organisms in BSI patients. For febrile episodes the rate of BSI was higher among those with Chemoport than those with Hickman catheter (P =0.029) and gram-positive pathogens were more likely to be associated with Chemoport (P =0.001). Conclusion : The study showed the rate of BSI, distribution of pathogens with regard to neutropenia, transplantation, central venous catheters, and antimicrobial susceptibility of pathogens in order to help guide in the choice of optimal empiric antibiotics in pediatric febrile neutropenic hemato-oncologic patients.

• Pediatric Infection and Vaccine
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• v.21 no.2
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• pp.121-128
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• 2014

Purpose: To compare the clinical characteristics and laboratory finding between adenoviral and group A streptococcal (GAS) pharyngitis. Methods: A retrospective review of medical records was performed in the patients with adenovirus infection among those who were admitted for febrile respiratory disease from January 2011 to July 2013 and GAS pharyngitis among those who visited for symptoms of scarlet fever from August 2006 to July 2013. Results: 179 patients (AV1 group) were diagnosed with adenoviral pharyngitis and 37 (AV2 group) of these patients had adenovirus single infection. 26 patients (GAS group) were diagnosed with scarlet fever. Adenoviral infection (AV2 group) developed in younger patients compared to GAS group ($2.8{\pm}2.1$ years vs. $5.4{\pm}1.8$ years, P =0.000). Total durations of fever and admission were longer in AV2 ($6.3{\pm}2.6$ days vs. $3.3{\pm}1.9$ days, P =0.000; $4.1{\pm}1.2$ days vs. $1.9{\pm}1.8$ days, P =0.000, respectively). WBC counts were higher in AV2 ($11,449{\pm}5,680$ cells/$mm^2$ vs. $6,722{\pm}6,941$ cells/$mm^2$, P =0.000). CRP was not significantly different between AV2 and GAS group ($3.8{\pm}3.2$ mg/dL vs. $5.2{\pm}5.1$ mg/dL, P =0.368). No difference was found between two groups in the percentage of antibiotics use (91.9% vs. 100%, P =0.261). Conclusion: Clinical characteristics and measures of inflammation in the laboratory findings were similar between adenoviral and GAS pharyngitis group. It is necessary to conduct the test for respiratory virus and bacteria in early stage to differentiate in the pharyngitis patients with leukocytosis and elevation of CRP level.

• Radiation Oncology Journal
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• v.20 no.2
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• pp.100-107
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• 2002

Purpose : The aim of this retrospective study was to assess the treatment results of 30 patients with pineal region tumors who were underwent radiation therapy under the diagnosis by either CT or MRI. There was no histological verification. We analyzed the prognostic factors that have a significant effect on the overall survival (OS) and disease free survival (DFS) rates. Materials and Methods : A total 30 patients with pineal region tumors were treated between March 1983 and August 1995. After a trial radiation therapy of $20\~30\;Gy/2\~3$ weeks, the patients were evaluated for their clinical response and radiological response by either CT or MRI and the final treatment direction was then decided. According to their response to the trial radiation therapy and the involved site, radiation treatment was given in various fields i.e., local, ventricle, whole brain and craniospinal field. The radiation dose ranged from 40.8 to 59.4 Gy (Median 50.4 Gy). The median follow up was 36.5 months $(4\~172\;months)$. Results : An improvement or stability in the clinical symptoms was observed in 28 patients $(93.3\%)$ after the trial RT. Nineteen patients $(63.3\%)$ showed a partial or complete response by CT or MRI. The two-year and five-year survival rates of the patients were $66.7\%$ and $55.1\%$, respectively. No significant difference in the survival rates according to the degree of the radiological response was abserved after the trial RT. The results of univariate analysis showed that age, the primary site, the performance status $(KPS\geq70)$, the degree of response after completing RT and the RT field were significant prognostic factors affecting the survival and disease free survival rates (p<0.05). Conclusion : The clinical and histological characteristics of pineal region tumors are quite complex and diverse. Therefore, it is difficult to predict the histological diagnosis and the possibility of radiocurability only with the initial response to RT. We think that the development of less invasive histological diagnostic techniques and tailored treatment to the histological type of each tumor are needed.

• Radiation Oncology Journal
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• v.15 no.3
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• pp.233-241
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• 1997

Purpose : This study was done to evaluate the survival rate and Prognostic factors of patients with inoperable non-small cell lung cancer(NSCLC) treated with radiation therapy. Materials and Methods A retrospective analysis was undertaken of 62 Patients who had inoperable NSCLC treated with radiation therapy from January 1991 through December 1993. According to AJCC slaging, stage IIIA was 14 patients and stage IIIB was 48 patients. Forty Gy to 70.2Gy to the primary tumor site was delivered with daily fractions of 1.8Gy or 2Gy, 5days per week. Thirty-seven patients received neoadjuvant chemotherapy. Results : Complete, partial and no response to radiation therapy were 3 patients, 34 Patients and 25 patients, respectively The median survival period of all patients was 11 month. One rear survival rate, 2 year survival rateand 5 rear survival rate for all patients were 45.0%, 14.3%, and 6.0% respectively The median survival period was 6.5 months in stage IIIA and 13 months in stage IIIB. One year survival rates were 28.6% in stage IIIA and 50.3% in stage IIIB In univariaite analysis, prognostic factors affecting survival were T-s1aging, AJCC staging, and response after radiation therapy (P<0.05) . Pretreatment peformance status affected survival but was not statistically significant (0.050.1). In multivariate analysis, Pathology and response to radiation therapy are independently significant prognostic factor. T stage was marginally significant (P=0.0809). During follow-up duration, distant metastasis developed in 20 patients-bone metastasis in 10 patients, brain metastasis in 3 patients, liver mentastasis in 3 patients, contralateral lung metastasis in 1 patients and multiple metastases in 3 patients. Conclusion :　Conventional radiotherapy alone or combined chemoradiotherapy are unlikely to achieve long term survival in patients with NSCLC.　Surgery after concurrent chemoradiotherapy is Ivied to improve the local　control in our hospital

• Radiation Oncology Journal
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• v.15 no.3
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• pp.207-213
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• 1997

Purpose : Intracranial germinoma is the most radiocurable tumor of theprimary intracranial neoplasm. But, the optimum radiation dose and target volume remain controversial In this retrospective study, we analysed the spreading pattern at presentation and the pattern of the failure and survival of intracranial germinoma, Materials and Methods : From 1989 to 1996, 23 Patients were treated for intracranial germinoma at Department of Radiation Oncology, Twenty-one Patients were treated at their initial Presentation and 2 Patients were treated for recurrent disease. Six patients had multiple tumor masses on MRI and 7patients had ventricular seeding on MRI. The examination of cerebrospinal fluid cytology was done in 15 patients and 3 out of 15 patients had positive cerebrospinal cytology. In tumor marker study of $\alpha-FP\;and\;\beta-hCG$, 6 patients had mildly elevated $\beta-hCG$ in serum or cerebrospinal fluid. Twentyone Patients were treated with whole craniospinal axis irradiation and 2 Patients were given whole ventricular radiation therapy. The total dose was ranged between 4500cGy and 5600cGy to primary tumor site (median 5580cGy) Dose to the entire ventricular system ranged from 1980cGy to 3960 cGy (median 2700cGy) and dose to the spinal axis ranged from 2160cGy to 3900cGy (median 2700cGy) Results : Of 23 patients, 21 Patients are alive without evidence of diseasefor median 4 years follow-up. One Patient who had markedly elevated $\alpha-FP\;and\;\beta-hCG$, suffered from Persistent disease after radiation therapy and received 2 cycles of chemotherapy. She died 9 months after chemotherapy One patient who developed ventricular seeding after gamma-knife was treated with whole craniospinal irradiation, he died after 1 year due to probably brain necrosis. The hematologic toxicity of 3 or 4 grade were seen in 7 patients and patient's endocrinologic dysfunction was not deteriorated after radiation therapy. One patient had been treated with growth hormone replacement due to short stature. Conclusions : This retrospective study has confirmed the excellent result of radiation therapy in intracranial germinoma. The complication rate during or after radiation therapy is considered within acceptable range. ft is necessary to further investigate the optimal dose and treatment volume of radiation therapy. The role of chemotherapy in the treatment of intracranial germinoma should be further investigated.