• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.129-139
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• 2003

Purpose: The aim of the study was to evaluate the long-term outcome of glycogen storage disease (GSD) type 1 with particular reference to hepatic adenoma and hepatocellular carcinoma, and to analyze risk factors affecting the development of hepatic adenoma in GSD type 1. Methods: Forty-three GSD type 1 patients (31 males and 12 females, mean age $13.9{\pm}6.4$ years) were analyzed retrospectively. Hepatic adenoma was detected on abdominal USG and diagnosed on histologic examination. Clinical profiles were compared between patients with hepatic adenoma (n=16) and age-matched controls without hepatic adenoma (n=16). Results: 1) Of 43 GSD type 1 patients, 16 (37.2%) had hepatic adeoma. Hepatic adenoma was detected at the age of mean $14.2{\pm}4.1$ years (range: 7.9~25.7 years). Fourteen (87.5%) adenomas were multiple at detection. 2) Comparison of the clinical profiles between adenoma group and non-adenoma group revealed that age at first introduction of uncooked cornstarch treatment was significantly late in adenoma group compared with non-adenoma group ($9.1{\pm}5.2$ years vs. $3.0{\pm}1.8$ years, p=0.003). Portocaval shunt surgery was performed in 11 (68.8%) patients in adenoma group and 3 (18.8%) in non-adenoma group (p=0.004). Hepatic adenoma developed mean $5.8{\pm}4.2$ years after shunt operation. 3) One patient was diagnosed as hepatocellular carcinoma at the age of 25.7 years. Conclusion: Hepatic adenoma is an important late complication of GSD type 1 with the risk of malignant transformation. Early introduction of cornstarch therapy with strict metabolic control is needed to prevent the development of hepatic adenoma in GSD type 1.

• 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.

• Childhood Kidney Diseases
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• v.9 no.1
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• pp.64-68
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• 2005

Purpose : Renal abscess is very rare in children and its diagnosis is difficult because symptoms are often nonspecific. In previous studies, on]y 15% to 25% of patients were reported to be diagnosed at the time of admission. Early diagnosis and treatment are important be cause mortality rate correlates positively with the time of diagnosis. The purpose of this study is to clarify the clinical features of children with renal abscess and to investigate the possible indicators of this disease for early diagnosis and Proper treatment. Methods : Twelve children diagnosed with renal abscess from Jan. 1996 to Jul. 2004 were included. The age of patients ranged from S months to 15 years. We retrospectively analyzed the demographics of patients, their symptoms, predisposing factors, diagnostic methods and causative organisms and the treatment modalities. Results : Fever was the most common manifestation, Five children(42%) had vesicoureteral reflux. Renal ultrasonography and computerized tornography were the most frequently used imaging tools to detect renal abscess. Gram negative bacteria were isolated in 7 patients and Staphylococcus aureus grew in 2 patients. All patients received intravenous antibiotics and 4 patients underwent aspiration or drainage of renal abscess. The average admission duration was 30 days. Conclusion : Renal abscess should be included in the differential diagnosis of prolonged fever in children, especially when flank pain is combined. For early diagnosis and a better prognosis, patients should be promptly investigated with ultrasonography or computerized tomography.

• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.650-654
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• 2008

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

• Childhood Kidney Diseases
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• v.15 no.1
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• pp.58-65
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• 2011

Purpose: Some hormonal and electrolyte abnormalities have been reported in pediatric patients with urinary tract infection (UTI). This study aimed to investigate the relationships between the imbalance of electrolytes and the severity of infection and associated urologic anomalies in children with febrile UTI. Methods: We retrospectively reviewed 267 patients with febrile UTI who were admitted to Korea University Guro Hospital during the period from January, 2007 until February, 2010. According to the presence of hyponatremia or hyperkalemia, clinical parameters and associated renal anomalies, such as hydronephrosis, cortical defects and vesicoureteral reflux, were compared. Results: 42.7% of all patients had decreased concentration of serum sodium. In patients with decreased concentration of serum sodium, cortical defects were significantly increased compared to normal patients (40.4% vs. 14.4%, P <0.05). White blood cell (WBC) counts ($15,721{\pm}6,553/uL$ vs. $12,885{\pm}5,367/uL$, P <0.05), C-reactive protein (CRP) ($61.8{\pm}56.1$ mg/L, vs. $29.9{\pm}39.8$ mg/L, P <0.05), and erythrocyte sedimentation rate (ESR) ($43.9{\pm}34.3$ mm/hr vs. $27.4{\pm}26.8$ mm/hr, P <0.05) in peripheral blood showed significant increases in the group with decreased concentration of serum sodium. Duration of fever, presence of gastrointestinal symptom, the incidence of hydronephrosis and vesicoureteral reflux did not differ between the two groups. None of the patients had significant hyperkalemia. Conclusion : We suggest that decreased concentration of serum sodium in febrile UTI might be a helpful marker for leukocytosis and increased CRP and ESR in peripheral blood, and acute pyelonephritis.

• Journal of Hospice and Palliative Care
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• v.8 no.2
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• pp.152-155
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• 2005

Purpose: The lower extremity edema (LEE) is a common distressful symptom in advanced cancer patients and is hard to manage. We analyze the characteristics of LEE in patients with advanced cancer to provide the basic information of causes and adequate management. Methods: Physical examination, assessment of the location and severity of edema, blood chemistry (albumin, creatinine), Doppler Sono for patients with suspecting deep vein thrombosis (DVT), and abdomen CT scan for patient with suspecting lymph edema were performed. Severity of edema was classified according to NCI lymph edema scaling and improvement was defined as lowering at least 1 grade of edema after management. Results: Among 154 patient who had been admitted to Hospice Ward from Mar 2003 to Jan 2004, 33 had LEE, and 6 had both upper extremity edema and LEE except generalized edema. Their underlying cancers were stomach (7), lung (6), biliary tract (5), liver (5), colorectal (5), pancreas (2), and others (9). There were 12 patient with grade I, 20 patients with grade II, and 7 patients with grade III edema. The causes were hypoalbuminemia (11), lymph edema (10), DVT (7), obstruction of inferior vena cava (IVC) or portal vein (6), and dependent edema (5). The common managements were including leg elevation and diuretics. Elastic stocking was applied for patients with DVT and leg massage and pneumatic compression was used for lymph edema. The 2/3 patients were improved after management. Conclusion: The incidence of LEE in terminal cancer pts was high (25.3%) and their causes were variable including lymph edema, DVT, hypoalbuminemia and dependent edema. Active noninvasive management according to causes could result in good palliation.

• Childhood Kidney Diseases
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• v.3 no.1
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• pp.72-79
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• 1999

Renal scarring associated with vesico-ureteral reflux(VUR) is one of the major causes of end stage renal failure and renal hypertension in children. Urinary ${\beta}_2$-microglobulin(MG) has been suggested as a potential marker for presence of renal tubular damage. This study was designed to evaluate the significance of random urine ${\beta}_2$-MG as a predictor of presence of vesico-ureteral reflux in children with urinary tract infection(UTI). 57 children with urinary tract infection were studied. Patients were devided into two groups; 35($78.9\%$) children have UTI without VUR and 12($21.1\%$) children have UTI and VUR. Beta2-MG and creatinine in random urine sample was measured to decide the excretion ratio(${\beta}_2$-MG/creatinine). Among the 57 children with UTI, 44 children were confirmed by urine culture study and 13 children suspected by compatible clinical feature. Random urine ${\beta}_2$-MG of VUR group ($2.2{\pm}5.91$ mg/L) were significantly higher than that of simple UTI group($0.19{\pm}0.16mg/L$)(P=0.03). The ${\beta}_2$-MG/creatinine ratio of VUR group($32.41{\pm}25.7$) were significantly higher than that of simple UTI group($3.93{\pm}3.44$)(P=0.007). In conclusion, random urine ${\beta}_2$-MG and excretion ratio deserved early predictor of presence of VUR in children with UTI. And this method was more simple and inexpensive than the method of measuring ${\beta}_2$-MG with 24 hour urine collection, so might be a useful screening test for VUR in children with UTI.

• The Korean Journal of Nuclear Medicine
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• v.35 no.6
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• pp.378-388
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• 2001

Purpose: In this study, we developed a new method for the determination of renal depth with anterior and posterior renal scintigrams in a dual-head gamma camera, considering the attenuation factor $e^{-{\mu}x}$ of the conjugate-view method. Material and Method: We developed abdomen and kidney phantoms to perform experiments using Technetium-99m dimercaptosuccinic acid ($^{99m}Tc$-DMSA). The phantom images were obtained by dual-head gamma camera equipped with low-energy, high-resolution, parallel-hole collimators (ICONf, Siemens). The equation was derived from the linear integration of omission ${\gamma}$-ray considering attenuation from the posterior abdomen to the anterior abdomen phantom surface. The program for measurement was developed by Microsoft Visual C++ 6.0. Results : Renal depths of the phantoms were derived from the derived equations and compared with the exact geometrical values. Differences between the measured and the calculated values were the range of 0.1 to 0.7 cm ($0.029{\pm}0.15cm,\;mean{\pm}S.D.$). Conclusion: The present study showed that the use of the derived equations for renal depth measurements, combined with quantitative planar imaging using dual-head gamma camera, could provide more accurate results for individual variation than the conventional method.

• 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.

• Radiation Oncology Journal
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• v.26 no.2
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• pp.83-90
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• 2008

Purpose: For the first time, a nationwide survey of the Patterns of Care Study(PCS) for the various radiotherapy treatments of esophageal cancer was carried out in South Korea. In order to observe the different parameters, as well as offer a solid cooperative system, we compared the Korean results with those observed in the United States(US) and Japan. Materials and Methods: Two hundreds forty-six esophageal cancer patients from 21 institutions were enrolled in the South Korean study. The patients received radiation theraphy(RT) from 1998 to 1999. In order to compare these results with those from the United States, a published study by Suntharalingam, which included 414 patients[treated by Radiotherapy(RT)] from 59 institutions between 1996 and 1999 was chosen. In order to compare the South Korean with the Japanese data, we choose two different studies. The results published by Gomi were selected as the surgery group, in which 220 esophageal cancer patients were analyzed from 76 facilities. The patients underwent surgery and received RT with or without chemotherapy between 1998 and 2001. The non-surgery group originated from a study by Murakami, in which 385 patients were treated either by RT alone or RT with chemotherapy, but no surgery, between 1999 and 2001. Results: The median age of enrolled patients was highest in the Japanese non-surgery group(71 years old). The gender ratio was approximately 9:1(male:female) in both the Korean and Japanese studies, whereas females made up 23.1% of the study population in the US study. Adenocarcinoma outnumbered squamous cell carcinoma in the US study, whereas squamous cell carcinoma was more prevalent both the Korean and Japanese studies(Korea 96.3%, Japan 98%). An esophagogram, endoscopy, and chest CT scan were the main modalities of diagnostic evaluation used in all three countries. The US and Japan used the abdominal CT scan more frequently than the abdominal ultrasonography. Radiotherapy alone treatment was most rarely used in the US study(9.5%), compared to the Korean(23.2%) and Japanese(39%) studies. The combination of the three modalities(Surgery+RT+Chemotherapy) was performed least often in Korea(11.8%) compared to the Japanese(49.5%) and US(32.8%) studies. Chemotherapy(89%) and chemotherapy with concurrent chemoradiotherapy(97%) was most frequently used in the US study. Fluorouracil(5-FU) and Cisplatin were the most preferred drug treatments used in all three countries. The median radiation dose was 50.4 Gy in the US study, as compared to 55.8 Gy in the Korean study regardless of whether an operation was performed. However, in Japan, different median doses were delivered for the surgery(48 Gy) and non-surgery groups(60 Gy). Conclusion: Although some aspects of the evaluation of esophageal cancer and its various treatment modalities were heterogeneous among the three countries surveyed, we found no remarkable differences in the RT dose or technique, which includes the number of portals and energy beams.