• 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.6 no.1
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• pp.63-73
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• 1988

One hundred fifty-four patients with the carcinoma of the uterine cervix were studied retrospectively to assess the result and impact of treatment at Department of Radiation Oncology, Korea University, Hae-Wha Hospital from Feb 1981 through Dec. 1986. Prior to radiotherapy, the patients were evaluated and staged by recommendation of FIGO including physical examination, pelvic examination, cystoscopy, rectosigmoidoscopy, chest X-ray, IVP. Ba enema. Also, an additional pelvic CT scan was obtained for some of the patients. The patients were treated by radiotherapy alone or adjuvant postoperative irradiation; in case of radiation therapy only, whole pelvic irradiation was given with Co-60 teletherapy unit via AP and PA parallel opposing fields or 4-oblique fields, 180 cGy per day, 5 days per week and intracavitary insertion was performed. In satges Ia, Ib, and IIa with small primary lesion, external irradiation was initially given to pelvis up to $2,000\~3,000\;cGy/2frac{1}{2}\;-3frac{1}{2}$ weeks and then intracavitary insertion was performed using Fletcher-Mini-Declos Applicator with cesium-137 cources and followed by external irradiation of $1,000\~2,000\;cGy/1frac{1}{2}\;-2frac{1}{2}$weeks via AP and PA parallel opposing fields with midline shield to spare of bladder and rectum. However, if the primary lesion is large, external irradiation was given without midline shield. More than stages IIb, the patients were treated by external beam irradiation up to 5,400cGy/30f for 6 weeks via 4-oblique portals and at the dose of 5,040cGy/28f the field was cut 5cm from the top margin for spare of small bowel, and followed by intracavitary irradiation, If there was residual tumor an additional dose of $900\~l,200cGy/5\~7f$ was given to parametrium and/or residual tumor area. Total dose of radiation to A and B-point were as follows; A-point; In early stages, Ia, Ib, IIa; $8,000\~9,000$ B-point $5,000\~6,000 cGy$ A-point; In advanced stages IIb, IIIa, IIIb; $9,000\~10,000$ B-point $60,000\~7,000cGy$ The results were obtained and as fellows; 1 The patients distribution according to FIGO staging system were stage Ia 6, Ib 27, IIa 28, IIb 54, IIIa 12, IIIb 18, and stage IVa 9. 2. Value of CT scan were demonstration of cervix tumor mass, parametrial and pelvic side wall tumor spread, pelvic and inguinal lymph nodes metastases, and hydronephrosis. Three dimensional quantitative demonstration of tumor volume is also important in planning radiation therapy. Another advantage of CT scan was detection of recurrent tumor after radiation or surgery. 3. Local control rate of tumor according to the size was $91.3\%$ for less than 5cm in size and $44.6\%$ in tumor over 5cm (p<0.0068). 4. Thirty out of 50 recurrent sites has locoregional failures and 17 cases has distant metastases. And the para-aortic lymph nodes were the most common site for distant metastases. 5. The most common complication was temporal rectal bleeding which was controlled most by conservative management. However, 4 patients required for endoscopic cauterization. 6. The 5-year survival rates showed; stage la and Ib $95\%,\;stage\;IIa\;81\%\;stage\;lIb\;67\%,\;stage\;IIIa\;37.7\%,\;stage\;IIIb\;23\%$ and 3-year survival rate of stage IVa showed $11.6%$, retrospectively.

• Tuberculosis and Respiratory Diseases
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• v.40 no.5
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• pp.565-574
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• 1993

Background: Pulmonary hamartomas are benign tumors that occur in the parenchyma or in the bronchi. They present as a solitary pulmonary nodule(SPN) or as a cause of bronchial obstruction. The incidence, once minimal, is increasing in Korea. To get clinical spectrum about the tumor, we analyzed all the reported cases in Korea since 1964. Methods: We reviewed the clinical, radiological and pathological findings of 13 patients of intrapulmonary or endobronchial hamartomas in Severance Hospital and of 38 reported cases in Korea published in literatures from 1964 to 1992 retrospectively. Results: Including 17 endobronchial hamartomas, 54 cases were studied. There were 25 men and 29 women, with a mean age of 47.2 years; 45.3 years in endobronchial type and 51.3 years in parenchymal type. Pulmonary symptoms were present in 8 patients (22%) of intrapulmonary type and in all patients of endobronchial type: cough (65%), dyspnea (53%), sputum (35%), fever (29%) in order. On chest X-rays, atelectasis was seen in 10 patients (59%) in endobronchial type; but SPN was noted in 36 patients (97%) of intrapulmonary type. Calcification was present in 7 intapulmonary hamartomas (23%); but is in 2 endobronchial hamartomas (12%). The diagnostic yield was 6 out of 14(43%) in endobronchial ones; 4 out of 7(57%) in intrapulmonary ones. Fifty patients underwent operations as follows: lobectomy (28), enucleation (8), resection (8), bilobectomy (4), pneumonectomy (2). The hamartomas were 1.2 times more common in the right lung; mean transverse diameter at the time of operation was 2.3 cm in endobronchial type, 3.8 cm in intrapulmonary ones. Chondroid components were present in 11(65%) of 17 endobronehial ones but in 30(91%) of 33 intrapulmonary hamartomas. No malignant changes were seen perioperative period and up to early 1993. Conclusion: The younger age in endobronchial hamartomas, the preponderance of the female sex and the more incidence in the right lung, and the diagnostic choice of lobectomies were different from the studies of the Western countries.

• Clinical and Experimental Pediatrics
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• v.50 no.7
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• pp.649-654
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• 2007

Purpose : The objective of this study was to clarify the presentation, associated preceding illness, pathologic organisms, treatment and outcome of deep neck abscess in children according to age and location. Methods : We retrospectively reviewed the in-patient charts of children treated at our hospital for deep neck abscess. Thirty-five such patients were identified as having been treated from March 1990 to December 2005. Results : A total of 35 were enrolled in our study: 25 boys and 10 girls. Their ages ranged from 11 months to 15 years. Presenting symptoms included mass, fever, irritability, trismus and dysphagia. The most commonly known associated preceding illness was viral upper respiratory infection (53%). The most common site of infection was the submandibular space (37%). Bacteria was identified in 16 patients. The most common pathogen was Staphylococcus aureus. Thirteen (37%) children recovered from the infection with conservative treatment and twenty-four (68%) children received surgical drainage. The duration of hospitalization was longer in the group who underwent surgery than in the group who were managed with conservative treatment. No complication occurred. Conclusion : Unexplained torticollis, trismus or irritability in children were suggestive of deep neck abscess. Our results demonstrate that deep neck abscesses in children is respond well to conservative treatment if diagnosed early.

• Radiation Oncology Journal
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• v.15 no.2
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• pp.105-111
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• 1997

group($22\%\;vs.\;38\%$, p=0.24). The 5YSRs of 21 patients of primary tumor extension to adjacent sites and the other 13 patients of tonsillar proper site were $28\%\;and\;38\%$, respectively but the difference was not significant statistically(p=0.52) There was a statistically significant difference in 5YSRs between the groups of the Patients who received radiotherapy in less than 61days vs more than 60days($60\%\;vs.\;18\%$, p=0.027). All living Patients without any tumor progression(n=11) had suffered from serious late sequelae such as xerostomia, edentia, dental caries and one patient had the osteoradionecrosis of mandible. On univariate analysis. the duration of radiotherapy and T-stage were the significant prognostic factors affecting 5YSR. On multivariate analysis, also the duration of radiotherapy was the only significant Prognostic factor(p=0.01). Conclusion : There was no survival difference between the radiotherapy alone and with neoadiuvant chemothe groups. Although it was a retrospective study, the role of conventional radiotherapy alone could be effective as the local treatment modality only for the early stage of tonsillar carcinomas. But for the purpose of more improved survivals and better quality of lives of living patients, other altered fractionation such as hyperfractionated radiotherapy with shorter treatment time and smaller fraction size rather than conventional radiotherapy might be beneficial and these prospective studies are needed.

• Radiation Oncology Journal
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• v.25 no.4
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• pp.261-267
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• 2007

Purpose: To evaluate the association between radiation pneumonitis and dose-volume histogram parameters and to provide practical guidelines to prevent radiation pneumonitis following radiotherapy administered for breast cancer including internal mammary lymph nodes. Materials and Methods: Twenty patients with early breast cancer who underwent a partial mastectomy were involved in this study. The entire breast, supraclavicular lymph nodes, and internal mammary lymph nodes were irradiated with a dose of 50.4 Gy in 28 fractions. Radiation pneumonitis was assessed by both radiological pulmonary change (RPC) and by evaluation of symptomatic radiation pneumonitis. Dose-volume histogram parameters were compared between patients with grade <2 RPC and those with grade ${\geq}$2 RPC. The parameters were the mean lung dose, V10 (percent lung volume receiving equal to and more than 10 Gy), V20, V30, V40, and normal tissue complication probability (NTCP). Results: Of the 20 patients, 9 (45%) developed grade 2 RPC and 11 (55%) did not develop RPC (grade 0). Only one patient developed grade 1 symptomatic radiation pneumonitis. Univariate analysis showed that among the dose-volume histogram parameters, NTCP was significantly different between the two RPC grade groups (p<0.05). Fisher's exact test indicated that an NTCP value of 45% was appropriate as an RPC threshold level. Conclusion: This study shows that NTCP can be used as a predictor of RPC after radiotherapy of the internal mammary lymph nodes in breast cancer. Clinically, it indicates that an RPC is likely to develop when the NTCP is greater than 45%.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.10 no.1
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• pp.36-43
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• 2007

Purpose: The ultrasonography (USG) and computed tomography (CT) are popular diagnostic tools for the diagnosis of acute appendicitis in children, but there are many debates about their clinical significance. The purpose of this study is to clarify the clinical significance of USG, CT and follow-up CT performed subsequently to USG, especially in perforated acute appendicitis in children. Methods: We have reviewed 419 cases of surgically confirmed acute appendicitis in children under the age of sixteen, who had been treated in Inje University Ilsan Paik Hospital from March 2002 to February 2006. All the clinical data including the results of USG and CT were collected and analyzed. Results: Sensitivity, specificity, positive and negative predictive values of USG were 98.7%, 96.8%, 98.1%, 97.8% in non-perforation group and 90.8%, 100%, 100%, 81.9% in perforation group. Those of CT were 96.4%, 100%, 100%, 96.5% and 86.6%, 100%, 100%, 87.5% respectively. Those of follow-up CT after USG were 100%, 100%, 100%, 100% and 87.5%, 100%, 100%, 92.0% respectively. The duration of using antibiotics in seven patients showed positive correlation with the interval between two imaging studies (r=0.0472, p=0.019). There was no statistical significance of correlation when these imaging studies performed within 30 hours together. Conclusion: In most of the cases, single choice between USG and CT would be enough to diagnose the acute appendicitis in children. But, it may be helpful to perform CT as early as possible subsequently to USG when there is discrepancy between initial USG and clinical impression.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.60-66
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• 2003

Purpose : Neonatal hydronephrosis has been detected with increasing frequency with the widespread use of prenatal ultrasonography, but the consensus about its postnatal management has not yet been reached, especially about surgical intervention. We attempted to determine the guideline of follow-up study and surgical intervention of hydronephrosis by analyzing clinical outcomes of neonates with hydronephrosis. Materials and Methods : Between 1994 and 2000, 128 hydronephrotic kidneys were postnatally confirmed. Cases associated with other urologic anomalies were excluded and 90 unilateral hydronephrotic kidneys with a minimum follow-up of 12 months were enrolled in this study. We classified the patients into 6 groups according to the anterior posterior pelvic diameter(APPD) at initial ultrasonography(USG) within 1 month after birth. Renal USG and $Tc^{99m}-mercaptoacetyl$ triglycerine(MAG3) scan were done according to a set protocol, and pyeloplasty was performed when indicated according to our protocol. Results : Most cases whose APPD were below 10 mm improved or resolved. Only few cases with APPD above 20 mm showed spontaneous improvement and most(88%) had undergone operation. Those with initial APPD within 10-19 mm showed variable outcomes. When the risk factors for irreversible renal functional deterioration were analyzed, the age at pyeloplasty and pre-operative functional deficit were significant. Conclusion : We concluded that in infants with initial APPD below 10 mm, consideration of surgery is not needed, and in those with initial APPD above 20 mm, early operation is recommended. Our set protocol based on initial USG is useful, but the cut-off value of relative renal function(RRF) for operation might be increased to 40% to improve post operative RRF.

• Nuclear Medicine and Molecular Imaging
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• v.41 no.4
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• pp.309-316
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• 2007

Purpose: Sentinel lymph node biopsy became the standard procedure in early breast cancer surgery. Faculty members might be exposed to a trace amount of radiation. The aim of this study is to quantify the radiation exposure and verify the safety of the procedure and the facilities, especially during pathologic process. Materials and Methods: Sentinel lymph node biopsies with Tc-99m human serum albumin were performed as routine clinical work. Exposed radiation doses were measured in pathologic technologist, nuclear medicine technologist, and nuclear medicine physician using a thermoluminescence dosimeter (TLD) during one month. We also measured the residual radioactivities or absorbed dose rates, the exposure distance and time during procedure, the radiation dose of the waste and the ambient equivalent dose of the pathology laboratory. Results: Actual exposed doses were 0.21 and 0.85 (uSv/study) for the whole body and hand of pathology technologist after 47 sentinel node pathologic preparations were performed. Whole body exposed doses of nuclear medicine physician and technologist were 0.2 and 2.3 (uSv/study). According to this data and the exposure threshold of the general population (1 mSv), at least 1100 studies were allowed in pathology technologist. The calculated exposed dose rates (${\mu}$ Sv/study) from residual radioactivities data were 2.47/ 22.4 ${\mu}$ Sv (whole body/hand) for the surgeon; 0.22/ 0 ${\mu}$ Sv for operation nurse. The ambient equivalent dose of the pathology laboratory was 0.02-0.03 mR/hr. The radiation dose of the waste was less than 100 Bq/g and nearly was not detected. Conclusion: Pathologic procedure relating sentinel lymph node biopsy using radioactive colloid is safe in terms of the radiation safety.(Nucl Med Mol Imaging 2007;41(4);309-316)

• Sleep Medicine and Psychophysiology
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• v.18 no.2
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• pp.87-94
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• 2011

Objective: OSAS (obstructive sleep apnea syndrome) is a common disorder and its consequences are often serious. It is important to detect the disorder early in the course for proper treatment. This study is to grasp the snoring clinic visitors' knowledge level of OSAS. Method: One hundred and seventy-nine visitors at the of snoring clinic of Seoul National University Hospital were surveyed by questionnaire about reasons of visit and knowledge of treatment methods of snoring and OSAS, diagnostic method, OSAS-related symptoms, and complications. Results: Most of the respondents (89.4%) "have already heard about OSAS" and the major sources of information was the mass media (58.1%) such as television and radio. More than half (60.3%) were aware that snoring is closely related to OSAS. More than half (59.8%) recognized that a nocturnal polysomnograpy was necessary for proper diagnosis. Two thirds (67%) of the respondents noted surgery as a treatment for snoring. More than half (55.9%) answered that they would follow the doctor's advice on the treatment choice. Only 12.3% of respondents "have heard about nCPAP". No one chose nCPAP (nasal continuous positive airway pressure) as a treatment for either snoring or OSAS. About one third (34.6%) of the respondents were aware that OSAS is related to hypertension. Only 12.8% noted that OSAS is related to diabetes mellitus. Conclusion: Visitors at the snoring clinic were found to have substantially limited knowledge of health risks and proper treatments of OSAS. We suggest that it is crucially important to educate patients and offer easy-to-understand information on snoring and OSAS. We predict that provision of educaiton and information to patients and general public will faciliate the diagnosis and treatment of snoring and OSAS and reduce the related disorders such as hypertension, stroke, and diabetes mellitus.