• 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.

• Radiation Oncology Journal
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• v.28 no.3
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• pp.133-140
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• 2010

Purpose: We retrospectively investigated the effect of irradiation using helical tomotherapy in recurrent pelvic tumors that underwent prior irradiation. Materials and Methods: Fourteen patients with recurrent pelvic tumors consisting of rectal cancer (57.1%), cervical cancer (35.7%) and cancer with an unknown origin (7.1%) were treated with tomotherapy. At the time of irradiation, median tumor size was 3.5 cm and 7 patients complained of pain originating from a recurrent tumor. The median radiation dose delivered to the gross tumor volume, clinical target volume, and planning target volume was 50 Gy, 47.8 Gy and 45 Gy, respectively and delivered at 5 fractions per week over the course of 4 to 5 weeks. Treatment response and duration of local disease control were evaluated using the Response Evaluation Criteria in Solid Tumors (ver. 1.0) and the Kaplan-Meyer method. Treatment-related toxicities were assessed through Common Terminology Criteria for Adverse Events (ver. 3.0). Results: The median follow-up time was 17.3 months, while the response rate was 64.3%. Symptomatic improvement appeared in 6 patients (85.7%). The median duration time of local disease control was 25.8 months. The rates of local failure, distant failure, and synchronous local and distant failure were 57.1%, 21.4%, and 7.1%, respectively. Acute toxicities were limited in grade I or II toxicities, except for one patient. No treatment related death or late toxicity was observed. Conclusion: Helical tomotherapy could be suggested as a feasible palliative option in recurrent pelvic tumors with prior radiotherapy. However, to increase treatment effect and overcome the limitation of this outcome, a large clinical study should be performed.

• Radiation Oncology Journal
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• v.19 no.1
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• pp.40-44
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• 2001

Purpose : To evaluate effect and tolerance of external beam radiotherapy for carcinoma of the prostate and define the optimal radiotherapeutic regimen. Materials and methods : We retrospectively analyzed the records of 60 patients with prostate cancer who were treated with external beam radiotherapy with curative intent in our institution between September, 1987 and March, 2000. Histologic diagnosis was established by transurethral resection or ultrasonography guided biopsy. The major presenting symptoms were a nodule at routine prostatic examination and frequency and urgency of urination, along with dysuria. The median age was 63 years with range of 51 to 87 years. There were 6 patients in Stage A, 20 in Stage 8, 26 in Stage C, and 8 in Stage Dl. All patients were treated with megavoltage equipment producing 10 MV photons. The 4 field pelvic brick technique was used to a dose of 45 Gy or 50.4 Gy at 1.8 Gy per day in 5 to 6 weeks, after which a small boost field was delivered 2.0 Gy per day to a total dose of 66 to 70 Gy. The follow-up period ranged from 1 to 8 years. Results : Actuarial 5-year and 7-year survival rates for Stage A, B, C, and D1 were $100\%\;and\;84\%$, $83\%\;and\;72\%$, $67\%\;and\;54\%$, and $v$, respectively. The corresponding 5-year and 7-year relapse free survival rates were $84\%\;and\;84\%$, $77\%\;and\;67\%$, $48\%\;and\;40\%$, and $33\%\;and\;25\%$, respectively. Relapse free 5-year survival rates for Stage B were $80\%,\;80\%,\;and\;50\%$ for well, moderately, and poorly differentiated tumors, respectively. These were $64\%,\;44\%,\;and\;33\%$ for Stage C, respectively. The local control rates at 5 years were $84\%,\;85\%,\;78\%,\;and\;60\%$ for Stage A, B, C, and D1, respectively. Mild to moderate complications were observed in $22\%$ of patients. Severe complications requiring surgical procedures were documented in only $3\%$ of patients. Conclusion : This study confirms that external beam irradiation is an effective and safe treatment for prostatic cancer, providing long-term local control and good survival with acceptable complications.

• The Korean Journal of Nuclear Medicine
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• v.30 no.1
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• pp.77-85
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• 1996

Radioiodine($^{131}I$) has been used for the treatment of Graves' hyperthyroidism since the late 1940's and is now generally regarded as the treatment of choice for Graves' hyperthyroidism who does not remit following a course of antithyroid drugs. But for the dose given, several different protocols have been described by different centers, each attempting to reduce the incidence of long-term hypothyroidism while maintaining an acceptable rate control of Graves' hyperthyroidism. Our goals were to evaluate effective half-life and predict absorbed dose in Graves' hyperthyroidism patients, therefore, to calculate and readminister radioiodine activity needed to achieve aimed radiation dose. Our data showed that the mean effective $^{131}I$ half-life for Graves' disease is 5.3 days(S.D=0.88) and mean biologic half-life is 21 days, range 9.5-67.2 days. The mean admininistered activity and the mean values of absorbed doses were 532 MBq(S.D.=254), 112 Gy (S.D.=50.9), respectively. The mean activity needed to achieve aimed radiation dose were 51MBq and marked differences of $^{131}I$ thyroidal uptake between tracer and therapy ocurred in our study. We are sure that the dose calculation method that uses 5 days thyroidal $^{131}I$ uptake measurements after tracer and therapy dose, provides sufficient data about the effective half-life and absorbed dose of $^{131}I$ in the thyroid and predict the effectiveness of $^{131}I$ treatment in Graves' hyperthyroidism.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.142-149
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• 2014

Purpose: There are 15 types of Glycogen storage disease (GSD) that have been identified, and GSD type Ia is the most common type. There are several studies of Korean GSD type Ia patients' long-term complications. The aim of this study to find out clinical symptoms and prognosis of GSD type Ia patients. Methods: We performed clinical, biochemical and genetic analysis retrospectively on five patients diagnosed with GSD type Ia in a Soonchunhyang University Hospital from July 2002 to July 2014. Results: All patients had hepatomegaly at diagnosis, and they were all confirmed to have fatty liver at abdomen USG. They had no developmental delay, but two of them had growth retardation. Elevated blood lactate, triglyceride, and uric acid levels can find out all patients, but only one patient had hypoglycemia. They are diagnosed with GSD through gene analysis, and by gene analysis, they have c.648G>T (homozygote, splicing mutation), c.122G>A/c.648G>T, c.248G>A/c.648G>T mutations. Treatment with three times meals, three times snacks and four to six times use of uncooked constarch for all patients. Following the progress, one of them resulted in hypothyroidism, other one had renal stones. A patient diagnosed at 16 years old had liver cirrhosis and started having hemodialysis for ESRD. Conclusion: GSD type Ia patients had hepatomegaly, hyperlipidemia, hyperuricemia, and lactacidemia. Therefore patients who have such these symptoms are recommended gene analysis. A patient diagnosed at 16-years-old had liver cirrhosis and ESRD in progress, early diagnosis and treatment are important for GSD type Ia patients.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.1
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• pp.31-40
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• 2005

Purpose: This study aimed to provide, as a basic material, the experiences of endoscopy in diagnosis and treatment of tumorous conditions in the upper gastrointestinal tract in children. Methods: The objects were 26 patients diagnosed as having tumorous conditions in the upper gastrointestinal tract among 1,283 patients who underwent upper gastrointestinal endoscopic examination at the Department of Pediatrics, Pusan National University Hospital, from January 1994 to July 2004 retrospectively. The characteristics of patients, the chief complaints for endoscopic examination, the sorts of tumors diagnosed, the endoscopic findings of tumors, and the treatment of tumors were analysed. Results: 1) Eleven male and fifteen female were included, whose mean age was $6.93{\pm}4.02years$. 2) The chief complaints for endoscopic examination were abdominal pain (80.7%), vomiting or nausea (30.8%), and gastrointestinal beeding (30.7%) in order. 3) Six cases of ectopic pancreas, five cases of sentinel polyp, three cases of papilloma and vallecular cyst, two cases of Brunner's gland hyperplasia and gastric submucosal tumor, one case of gastrointestinal stromal tumor, duodenal intramural hematoma, T cell lymphoma, lipoma, and Peutz-Jeghers syndrome were diagnosed by endoscopy with or without biopsy. 4) The location of tumors was in the pharynx (19.2%), esophagus (7.7%), gastro-esophageal junction (23.0%), stomach (30.7%) and duodeneum (26.9%). 5) The size of tumors was less than 10 mm in 53.8%, 10~20 mm in 26.9%, more than 20 mm 19.2%. 6) Treatments for tumors included resection by laser, surgical resection, endoscopic polypectomy with a forcep or snare, and observation 7) There was no significant complication. Conclusion: Various and not a few tumors were found in the upper gastrointestinal tract. The endoscopy was accurate, effective, and safe means for diagnosis and treatment of those lesions in children.

• Tuberculosis and Respiratory Diseases
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• v.48 no.3
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• pp.357-364
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• 2000

Background : Complicated exudative pleural fluid collections have traditionally been treated by either closed tube thoracostomy drainage or by open surgical drainage. Complete drainage is important in order to control pleural sepsis, restore pulmonary function, and entrapment. Recently intracavitary fibrinolytic therapy has been advocated as a method to facillitate drainage of complicated exudative pleural effusion and to allow enzymatic debridemant of the restrictive fibrinous sheets covering the pleural surface. The purpose of this study is to prospectively evaluate the effects of image-guided catheter drainage with high dose urokinase(UK) instillation in the treatment of complicated pleural effusions. Patients : Twenty complicated pleural effusion patients that poorly respond to image-guided drainage were allocated to receive UK. There were 8 pneumonia and 12 tuberculosis. Methods : Drugs were diluted in 250 mL normal saline and were infused intrapleurally through the chest tube or pig-tail catheter in a daily dose of 250,000 IU of UK. Response was assessed by clinical outcome, fluid drainage, chest radiography, pleural ultrasound and/or computed tomography. Results : The mean UK instillation time was $1.63{\pm}0.10$. The mean volume drained UK instillation was $381.3{\pm}314.4\;mL$, and post-UK was $321.6{\pm}489.5\;mL$. The follow up duration after UK therapy was mean $212.9{\pm}194.5$ days. We had successful results in 19 cases (95.0%). There were 12 pleural thickenings (60.0%), 2 markedly decreased effusions (10.0%) and 5 cases of no thickening or effusion. There was recurrence after treatment in only one patient(5%) with complicated pleural effusiondue to tuberculosis. Conclusions : Image-guided drainage with high dose UK instillation (250,000 U/day) in complicated pleural effusion is a safe and more effective method than closed thoracostomy drainage. And this management, in turn, can obviate surgery in most cases.

• The Korean Journal of Nuclear Medicine
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• v.34 no.2
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• pp.119-128
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• 2000

Purpose: The aim of this study was to investigate the diagnostic role of $^{99m}Tc$-Tetrofosmin in detection of breast cancer and compared with that of $^{99m}Tc$-MIBI. Material and Methods: Forty-eight patients with a clinically palpable mass or abnormal mammographic or ultrasonographic findings had $^{99m}Tc-MIBI\;and\;^{99m}Tc$-Tetrofosmin scintimammographies after intravenous injection of 925 MBq of radiopharmaceuticals. The scintimammographs were correlated with histopathologic findings. Results: Thirty-three patients were diagnosed with breast cancer and 15 patients with benign breast diseases. The numbers of true positive, true negative, false positive, and false negative cases of $^{99m}Tc$-MIBI scintimammography were 29, 10, 5, and 4 respectively. The sensitivity, specificity, positive predictive value, and negative predictive value of $^{99m}Tc$-MIBI scintimammographies were 87.8%, 66.7%, 85.3%, and 71.4% respectively. The numbers of true positive, true negative, false positive, and false negative cases of $^{99m}Tc$-Tetrofosmin were 31,10, 5, and 2 respectively. The sensitivity, specificity, positive predictive value, negative predictive value of $^{99m}Tc$-Tetrofosmin were 93.9%, 66.7%, 86.1%, and 73.3% respectively. One patient was false negative in both $^{99m}Tc-MIBI\;and\;^{99m}Tc$-Tetrofosmin acintimammographies and its size was 0.5 cm. Conclusion: $^{99m}Tc-Tetrofosmin\;and\;^{99m}Tc-MIBI$ were non-invasive and useful in detection of breast cancer and $^{99m}Tc$-Tetrofosmin was comparable to the $^{99m}Tc$-MIBI in detection of primary breast cancer.

• Journal of Chest Surgery
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• v.38 no.2 s.247
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• pp.132-138
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• 2005

Background: Mitral valve repair (MVP) is the optimal procedure for mitral regurgitation (MR), however, failure and subsequent reoperations are the limitations. The current study assessed the procedure in relation to the primary valve related causes of recurrent MR. Material and Method: MR was treated in 493 patients undergoing MVP from January of 1994 to January of 2002. The causes of MR were degenerative $(n=252,\;51.5\%),$ rheumatic $(n=156,\; 31.6\%),$ and others $(n=85,\; 16.9\%).$ Surgery comprised 446 ring annuloplasties $(90.5\%),$ 227 new chordae formations $(46\%),$ 125 quadriangular resections $(25.3\%),$ 28 chordae transfers $(5.7\%),$ and 8 Alfieri's stitches $(1.6\%).$ The mean follow up was $29.04\pm22.81$ months. Result: There were 5 early $(1.01\%)$, and 5 late deaths $(1.01\%).$ The reoperation rate was $1.42\%$. There were 45 $(9.1\%)$ recurrent MR (grade III or IV). Of these, 24 were procedure related including incomplete repair (n=14), discordant new chordae length (n=8) and others (n=2). In 21 patients, the cause was valve related including rheumatic disease progression (n=10), recurrent chordae elongation or prolapse (n=5) and others (n=6). Severe MR was higher after incomplete repair (p < 0.001), and valve related failure strongly correlated with rheumatic progression (p < 0.05). Conclusion: Since completeness of operation is the prime risk factor that determine the repair durability, intra-operative assessment of the initial repair with trans-esophageal echocardiography is essential.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.158-165
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• 2002

Purpose: We designed this retrospective study to establish the incidence of diagnosic delay in children diagnosed with acute appendicitis and to identify associated factors with delayed diagnosis and its impact on the clinical course. Methods: All cases of children under 15 years of age who underwent appendectomy from 1996 to 2001 at Gangneung Asan Hospital were reviewed. We reviewed signs and symptoms, type of health professional first contacted, the advice given by the health professional and a history of appendicitis in first degree relatives. Diagnostic period is the time elapsed between first complaints and definitive diagnosis. Delay was defined as diagnostic period exceeded the 48 hours. Postoperative course and complications were also reviewed. Results: Incidence of diagnostic delay differed by whether diarrhea and fecalith on X-ray were present. Also children whose parents were advised to observe them at home were more likely to have a diagnostic delay. In almost half of the cases in delayed group, initial diagnosis was not acute appendicitis but gastroenteritis. The perforation rate in non-delayed group was 22%, whereas 87% in delayed group. The delayed group showed a higher number of postoperative complication and a longer hospitalization period. Conclusions: Diarrhea with abdominal pain and fever in children should not be dismissed as gastroenteritis, respiratory infections or other common disorders. Our study suggests that physicians have a responsibility to prevent diagnostic delay and resultant perforation of acute appendicitis in children by having a high index of suspicion about acute appendicitis.