• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.2
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• pp.218-225
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• 2006

Purpose: With a remarkable increase in the prevalence of childhood obesity, the prevalence of nonalcoholic fatty liver disease is assumed to be increasing. The aim of this study is to evaluate the prevalence of nonalcoholic fatty liver disease, hyperlipidemia, and glucose intolerance in normal and obese children. Methods: A total of 2,206 elementary students (boys: 1340, girls: 866) were grouped according to obesity index; normal group and obesity group (mild, moderate, severe). Aspartate aminotransferase (AST, SGOT) and alanine aminotransferase (ALT, SGPT) were measured with total cholesterol, triglyceride, and fasting blood glucose. Results: Compared with the 4.6% of elevated aminotransferases in normal group, obese groups showed significantly higher prevalence; 12.1% in mild obesity group, 19.4% in moderate group, and 21.6% in severe group (p<0.0001). The prevalence of hypertriglyceremia was 16.9% in normal weight group, which was significantly lower than obesity group (mild obesity group 30.3%, moderate and severe 37.6%, 38.2% each). In boys, the prevalences of elevated aminotransferases in normal weight and obese groups (mild, moderate, severe) were 6.8%, 18.0%, 23.0%, and 26.0%, respectively (p<0.0001). In girls, those were 2.1%, 5.1%, 12.0%, and 12.6%, respectively (p< 0.0001). The prevalence of hypertriglyceremia was relative to severity of obesity in boys and girls (p<0.0001). Conclusion: The prevalence of elevated serum liver enzymes increased with severity of obesity. For the prevention and treatment of fatty liver and hypertriglycemia, it is important to lower the obesity degree and enforce the education for a weight loss in the student and the parents.

• Clinical and Experimental Pediatrics
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• v.45 no.5
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• pp.622-628
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• 2002

Purpose : The purpose of this study is to ascertain the clinical manifestations and associated cardiac anomalies of dextrocardia. Methods : Twenty-four pediatric patients, who were admitted to, or visited, Dongsan Medical Center Keimyung University and were diagnosed with dextrocardia between January 1996 and July 2001, were enrolled in this study. All patients received echocardiogram and abdominal ultrasonogram to identify structural cardiac anomalies and visceral position. Results : Among 24 patients, 7 cases were situs solitus(29.2%), 10 cases(41.6%) were situs inversus and 7 cases(29.2%) were situs ambiguous. Most were diagnosed within a week of life(87.5%). They were most commonly cyanotic(45.8%), but eleven cases(45.8%) had no specific symptoms or signs. Nineteen cases(79.2%) had accompanied cardiac anomalies, and pulmonary stenosis or atresia were the most common among them. All the cases of situs solitus and ambiguous had associated cardiac anomalies, but half of the situs inversus had that. Eleven cases were dead by cardiac or extracardiac anomalies during follow up and the mortality rate was higher in situs solitus or ambiguous group than situs inversus group. Conclusion : Dextrocardia accompanies different cardiac and extracardiac anomalies. It's very important to diagnose dextrocardia and associated cardiac or extracardiac anomalies in the early stages of life to improve prognosis.

• The Korean Journal of Nuclear Medicine
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• v.36 no.6
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• pp.368-380
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• 2002

Purpose: Liver transplantation (LT), one of the therapeutic options of primary liver cancer has been suffering from recurrence caused by metastasis in 8-54% of patients. This study was performed to investigate whether FDG-PET is useful for detecting hidden metastasis in LT candidates. Materials and Methods: Twenty-six patients (male:female=23:3, mean age 55.7 years) underwent FDG-PET. Their previous conventional diagnostic studies (CDS) like abdomen US and CT, chest x-ray and CT, and bone scan were negative (n=22) or equivocal (n=4) for metastasis. Positive FDG-PET findings were confirmed by biopsy or clinical follow-up. Results: Among 4 patients with equivocal metastatic lesions on CDS, 3 had 6 hypermetabolic lesions on FDG-PET, which were confirmed as metastasis and subsequently LTs were cancelled. Of these, 5 lesions were initially negative on CDS. Remained 1 patient underwent LT with a negative FDG-PET result. Among 22 patients without metastasis on CDS, 5 had 7 hypermetabolic lesions on FDG-PET. One of these patients proved to have 2 metastatic lesions, and LT was cancelled. The other 4 patients had S hypermetabolic lesions on FDG-PET, which were confirmed as benign lesions, and 3 patients of them underwent LT. In summary, FDG-PET was useful in avoiding 4 unwarranted LT by detecting unsuspected metastatic lesions on CDS. A total of 17 patients underwent LT. In comparison with pathology, the sensitivity and specificity of FDG-PET for detecting viable primary liver cancer were 55.6% (5/9) and 87.5% (7/8), respectively. Conclusion: FDG-PET can detect additional hidden metastasis and contribute to reducing unwarranted LT in the patients with primary liver cancer.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.2
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• pp.55-62
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• 2020

A 22-month-old girl who had taken iron supplements due to iron deficiency anemia, presented bloody mucoid stool for one month. She had a bruise at the right periorbital area due to minor trauma and hepatosplenomegaly. Laboratory studies showed anemia, thrombocytopenia, elevated alkaline phosphatase (ALP), hypophosphatemia, decreased haptoglobin, hypocomplementemia, negative direct/indirect Coomb's test, normal vitamin D3 level and high PTHi. Wrist x-ray showed no signs of rickets. The abdominal ultrasound showed only accessory spleen. Tandem mass spectrometry was normal. During follow up, bloody stool regressed after seven days of withdrawal of iron supplement and cow milk, and the total CO2 level had been within 15-20 mEq/L with normal anion gap. NGS (next generation sequencing) panel test for evaluation of renal tubular acidosis showed negative results. After low dose steroid and vitamin D supplements under the impression of hypocomplementemic vasculitis, thrombocytopenia, C3/C4, decreased haptoglobin, and elevated ALP level became normal. At 57 months of age, laboratory findings showed elevated liver enzyme, ALP and gamma-glutamyl transferase again. And liver cirrhosis with splenomegaly and diffuse renal disease were reported with abdomen CT scan. Liver biopsy reported macro- and micronodular cirrhosis. Urine organic acid profile showed elevated succinylacetone level. Whole exome sequencing revealed novel compound heterozygous mutations (NM_00137.2:c.107T>C, NM_00137, 2:c.614T>C) in FAH gene and confirmed by Sanger sequencing. Consequently, the patient was diagnosed as chronic hereditary tyrosinemia type I. She started low phenylalanine/tyrosine diet and nitisinone treatment. Our case had presented symptoms very slowly, which is the first case of chronic tyrosinemia type I in South Korea.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.143-155
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• 2002

A survey on biliary atresia was made among 26 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and a case registration form for each patient during the twentyone-year period of 1980-2000. Three hundred and eighty patients were registered from 18 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:1.3. The age of patients on diagnosis with biliary atresia was on average $65.4{\pm} 36.2$ days old. The national distribution was 32.8% in Seoul, 25.3% in Gyoungki-Do, 21.6% in Gyoungsang-Do, 9.27% in Choongchung-Do, etc. in order. The most common clinical presentation was jaundice (98.4%) and change of stool color (86.2%) was second. Two hundred eighty (74.7%) of 375 patients were operated by 80 days of age. Three hundred thirty six (9 1.9%) of 366 patients were operated on by the original Kasai procedure, and 305 (84.3%) of 362 patients were observed by bile-drainage postoperatively. The overall postoperative complication rate was 18.5% and the overall postoperative mortality rate was 6.8%. The associated anomalies were observed in 72 cases (22.5%). One hundred ninty five (64.7%) of 302 patients have been alive in follow-up and 49 (25.1%) have survived over 5 years without problem after operation. Ascending cholangitis, varices and ascites affected survival significantly, and the important long-term prognostic factor was the occurrence of complications.

• Tuberculosis and Respiratory Diseases
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• v.47 no.5
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• pp.642-649
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• 1999

Background: It is very important to determine an accurate staging of the non-small cell lung cancer(NSCLC) for an assessment of operability and it's prognosis. However, it is difficult to evaluate tumor involvement of mediastinal lymph nodes accurately utilizing noninvasive imaging modalities. PET is one of the sensitive and specific imaging modality. Unfortunately PET is limited use because of prohibitive cost involved with it's operation. Recently hybrid SPECT/PET(single photon emission computed tomography/positron emission tomography) camera based PET imaging was introduced with relatively low cost. We evaluated the usefulness of coincidence detection(CoDe) PET in the detection of metastasis to the mediastinal lymph nodes in patients with NSCLC. Methods: Twenty one patients with NSCLC were evaluated by CT or MRI and they were considered operable. CoDe PET was performed in all 21 patients prior to surgery. Tomographic slices of axial, coronal and sagittal planes were visually analysed. At surgery, mediastinal lymph nodes were removed and histological diagnosis was performed. CoDe PET findings were correlated with histological findings. Results: Twenty of 21 primary tumor masses were detected by the CoDe PET. Thirteen of 21 patients was correctly diagnosed mediastinal lymph node metastasis by the CoDe PET. Pathological N0 was 14 cases and the specificity of N0 of CoDe PET was 64.3%. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of N1 node was 83.3%, 73.3%, 55.6%, 91.7%, and 76.2% respectively. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of N2 node was 60.0%, 87.5%, 60.0%,87.5%, and 90.0% respectively. There were 3 false negative cases but the size of the 3 nodes were less than 1cm. The size of true positive nodes were 1.1cm, 1.0cm, 0.5cm respectively. There were 1 false positive among the 12 lymph nodes which were larger than 1cm. False positive cases consisted of 1 tuberculosis case, 1 pneumoconiosis case and 1 anthracosis case. Conclusion: CoDe PET has relatively high negative predictive value in the enlarged lymph node in staging of mediastinal nodes in patients with NSCLC. Therefore CoDe PET is useful in ruling out metastasis of enlarged N3 nodes. However, further study is needed including more number of patients in the future.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.2 no.2
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• pp.153-163
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• 1999

Purpose: The purpose of our study is to provide useful information for diagnostic methods of fatty liver by childhood simple obesity and to provide correlation between serum alanine aminotransferase (ALT) for screening test and abdominal computerized tomography (CT) and liver biopsy for confirmative diagnostic methods of fatty liver. Methods: Among 78 obese childrens who visited our hospital, CT was carried out in 26 childrens. Of these, liver biopsy was carried out in 15 childrens who had high obesity index or severe elevated ALT. Based on the level of serum ALT, 26 cases were classified into 3 groups, and compared with physical measurements and degree of fatty infiltration on CT and liver biopsy. Results: 1) Correlation between ALT and physical measurements: Of 26 obese children, ALT was abnormally elevated (>30 IU/L) in 17 cases (67.4%) but there was no significant correlation between ALT and physical measurements (p>0.05). 2) Correlation between degree of fatty infiltration on CT and ALT: Of 26 cases, 13 cases (50%) revealed fatty liver on CT. The degree of fatty liver on CT had significant correlation with elevation of ALT (p<0.05). 3) Correlation between the degree of fatty infiltration on liver biopsy and ALT: Liver biopsy was performed in 15 cases of which 14 cases revealed fatty liver. But one case had normal hepatic histology with severe obesity and normal ALT. Fourteen fatty liver cases on liver biopsy were classified into 3 groups by the degree of fatty infiltration and analysed with obesity index and ALT. The histologic hepatic steatosis had no significant correlation with obesity index (p>0.05), but significant correlation with ALT (p<0.05). 4) Correlation between CT and liver biopsy finding: Both CT and liver biopsy were performed in 15 cases of which 6 cases revealed normal finding on CT and 9 cases manifested fatty liver. There was significant correlation between CT and liver biopsy findings (r=0.6094). Conclusion: The results of our study suggest that abdominal CT and liver biopsy are useful and accurate methods of estimating fatty liver in the childhood obesity. But biochemical abnormalities of routine liver function tests dot not correlate well with severity of the fatty liver and liver injury.