• Clinical and Experimental Pediatrics
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• v.51 no.1
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• pp.78-83
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• 2008

Purpose : Osteosarcoma is the most common primary malignant bone tumor in children and adolescents. Although survival rate of osteosarcoma patients has markedly improved, about 20-30% of patients still have a relapse. This study was aimed to find factors that influence postrelapse survival of osteosarcoma in childhood and adolescents. Methods : Between 1985 and 2004, of 461 patients who were diagnosed and treated as osteosarcoma in Korean Cancer Center Hospital, 180 patients with recurrent osteosarcoma were retrospectively reviewed. We examined survival rates and analyzed prognostic factors, such as relapse site, post-relapse treatment methods, pathologic response to neoadjuvnat chemotherapy, metastasis at first diagnosis, and relapse free interval. Results : The overall recurrence rate of patients with osteosarcoma was 39%. The 5-year and 10-year postrelapse survival rates in the recurrent osteosarcoma were 13% and 4%, respectively. The 5-year post-relapse survival rate was influenced by site of relapse (lung, 39%; local, 0%; lung & bone, 25%; others, 12%; P<0.05), relapse-free interval (<12 months, 13%; ${\geq}12$ months, 44%, P<0.05), and post-relapse treatment methods (with surgery, 38%; without surgery, 11%; P<0.05). Conclusion : The survival rate of recurrent case is very low after 10 years, so new second-line chemotherapy and active treatment is needed to increase survival. Aggressive surgery with the removal of recurrence sites combined with multi-agent chemotherapy could either cure patients with recurrent osteosarcoma or significantly prolong their survival.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.77-81
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• 1998

The author exprienced a case of glycogen storage disease type Ia(GSD-I) in an 18-year-old male patient who was admitted to our hospital due to proteinuria and hypertension. he was suspected to have GSD when 12 years old because of his family history of short stature and hepatomegaly. On admission, physical examination revealed short stature, heparomegaly, and The diagnosis of GSD-I was confirmed by compatible liver biopsy finding and enzyme assay which erealeddeficiency of glcose-6-phosphatase if hepatocyte. Sympromatic treatment was done using antihypertensive drugs and allopurinol with diet control. The authors report a case of glycogen storage disease type Ia completely confirmed by typical clinical manifestation, pathologic findings of the liver and the kidney, and the result of enzyme assay which revealed deficiency of glucose-6-phosphatase in hepatocytes with brief review fo related literatures.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.301-307
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• 2005

Primary cardiac tumors are known to be rare. We studied the surgical results for primary cardiac tumors. Material and Method: Between August 1980 and December 2003, we classified 86 patients who had operation for primary cardiac tumors in our center into 3 groups; myxoma, nonmyxoma benign tumors, and malignant tumors. The mean age was $44.3\pm20.8$ years and 59 patients $(66.3\%)$ were female. In postoperative pathologic diagnosis, there were 81 cases $(94.2\%)$ of benign tumors in which myxoma was the most common tumor $(70\;cases,\;78.7\%);$ 5 fibroma $(5.6\%)$, 3 rhabdomyoma $(3.4\%)$, and 5 malignant tumors $(5.8\%)$. Result: $86.4\%$ of benign tumor was myxoma and the mean age was $50.4\pm15.4\;(range\;7\~80)$ years. Tumor was more common in females (49 cases) and most common preoperative symptom was dyspnea $(62.9\%)$. 57 cases were located at left atrial septum and only one case, which was located at right ventricular septum, was resected incompletely. There were no hospital deaths and one patient had mitral valve replacement on the first operative day due to newly developed postoperative mitral regurgitation. The mean follow up period was $109.3\pm71.8$ months and there was no evidence of recurrence in this period. 11 cases $(12.8\%)$ were non myxoma benign tumors; 5 fibromas, 3 rhabdo-myomas, etc. There were two hospital deaths and the causes of death were fungal endocarditis and hypoxia. There were no reoperations in nonmyxoma benign tumors. Malignant tumors were in 5 cases $(5.8\%);$ undifferentiated sarcoma in 2, rhabdomyosarcoma in 1, etc. Although there were no hospital mortalities, 3 patients who were followed up died from complications of tumors. Conclusion: Myxomas showed very excellent prognosis after complete resection and nonmyxoma benign tumors showed relatively good results for relief of symptoms. Surgery helped to relieve symptoms for malignant tumors, but the prognosis was poor.

• Journal of radiological science and technology
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• v.36 no.4
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• pp.313-318
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• 2013

Positron emission tomography/computed tomography (PET/CT) imaging with fluorodeoxyglucose (FDG) have been used as a powerful fusion modality in nuclear medicine not only for detecting cancer but also for staging and therapy monitoring. Nevertheless, there are various causes of FDG uptake in normal and/or benign tissues. The purpose of present study was to investigate whether additional delayed imaging can improve the diagnosis to differentiate the rates of FDG uptake at axillary lymph nodes (ALN) between malignant and benign in breast cancer patients. 180 PET/CT images were obtained for 27 patients with ALN uptake. The patients who had radiotherapy and chemotherapy were excluded from the study. $^{18}F$-FDG PET/CT scan at 50 min (early phase) and 90 min (delayed phase) after $^{18}F$-FDG injection were included in this retrospective study. The staging of cancers was confirmed by final clinical according to radiologic follow-up and pathologic findings. The standardized uptake value (SUV) of ALN was measured at the Syngo Acquisition Workplace by Siemens. The 27 patients included 18 malignant and 9 ALN benign groups and the 18 malignant groups were classified into the 3 groups according to number of metastatic ALN in each patient. ALNs were categorized less than or equal 3 as N1, between 4 to 9 as N2 and more than 10 as N3 group. Results are expressed as the mean${\pm}$standard deviation (S.D.) and statistically analyzed by SPSS. As a result, Retention index (RI-SUV max) in metastasis was significantly higher than that in non-metastasis about 5 fold increased. On the other hand, RI-SUV max in N group tended to decrease gradually from N1 to N3. However, we could not prove significance statistically in malignant group with ANOVA. As a consequence, RI-SUV max was good indicator for differentiating ALN positive group from node negative group in breast cancer patients. These results show that dual-time-point scan appears to be useful in distinguishing malignant from benign.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.13 no.2
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• pp.154-163
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• 2010

Purpose: Colonic polyposis is less common in children than in adults. The clinical data pertaining to colonic polyposis in children are limited. Children with colonic polyposis have complications associated with numerous polyps, malignant transformation of the polyps, and extraintestinal neoplasms. We studied the clinical spectrum, endoscopic characteristics, and histologic findings of colonic polyposis in Korean children. Methods: We reviewed the clinical data of 37 children with multiple colonic polyps between 1987 and 2009. The mean age at the time of diagnosis of colonic polyposis was 8.0${\pm}$3.2 years. Results: Peutz-Jeghers syndrome, juvenile polyposis syndrome, familial adenomatous polyposis (FAP), and lymphoid polyposis was diagnosed in 22, 7, 6, and 2 children, respectively. The most common clinical presentation in children with colonic polyposis was hematochezia. A family history of colonic polyposis was noted in 7 children. The colonoscopic findings of colonic polyposis varied with the size and number of polyps. The majority of polyps were multi-lobulatd and pedunculated in children with Peutz-Jeghers syndrome. The polyps in children with juvenile polyposis syndrome were primarily round and pedunculated. For the children with FAP, the colon was carpeted with small, sessile polyps. There were multiple sessile polyps in the patients with lymphoid polyposis. Surgical polypectomy was performed in 14 children (38%). Intestinal segmental resection was performed in 13 children (35%). Four patients with FAP underwent total colectomy. Four children with Peutz-Jeghers syndrome had extraintestinal neoplasms. No malignant transformation of polyp was identified. Conclusion: Children with colonic polyposis should undergo a careful initial evaluation and require periodic re-evaluation.

• Radiation Oncology Journal
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• v.8 no.2
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• pp.219-223
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• 1990

This is a retrospective review of 33 patients with large cell lung carcinoma treated at Yonsei University Cancer Center between Jan.1985 and Dec.1989. Of the thirty-three patients, twenty eight were men and five women. Median age was 59 years. Large cell undifferentiated carcinoma was the most common pathologic type, $78.8\%$. Twenty one of thirty three patients had far advanced diseases, stage IIIB-IV at the time of initial diagnosis. Pleural effusion was initially presented in 12 patients, and SVC syndrome appeared in 5 patients. As to location of the primary tumor,19($57.6\%$) appeared in the right lung and 14 ($42.4\%$) in the left. Patients with a centrally located primary tumor mass were nearly the same as those peripherally located (17 vs.16). Fifteen of thirty three patients developed metastasis involving not only bone, brain, the opposite lung, adrenal gland but also soft tissue, skin, pancreas and appendix. Treatment was individualized with 19 treated radically and 14 palliatively. After treatment, only two patients showed a complete response. Long term survival was observed in 4 patients: 1 (24 mo.),2 (41 mo.) and 1 (54 mo.). The overall 2 year survival rate was $14.3\%$ while the median survival time was 6.0 months. Through the analysis of the various factors affecting survival, we observed that pleural effusion-absent group and complete response group had a statistical significant better survival rate (p<0.01).

• Journal of Yeungnam Medical Science
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• v.16 no.1
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• pp.94-100
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• 1999

The aim of this retrospective study was to determine whether a nephron sparing surgery might be feasible in patients with a small solid renal tumor. Materials and methods: Between 1988 and 1999, 21 patients with a radiologically detectable small solid renal tumor underwent enucleoresection, wedge resection and polar segmental nephrectomy. The mean age of the 11 men and 10 women in this study was 43 years (range 14 to 68). According to the preoperative radiological diagnosis, 15 among the 21 patients were considered to have renal cell carcinoma, 4 were considered to have angiomyolipoma, and in the remaining 2 patients, radiological differentiation of renal tumors was difficult. Among 15 patients considered to have renal cell carcinoma, 14 were found to have renal cell carcinoma and the remaining one patient was diagnosed as having oncocytoma on pathologic examination. Radiological determination of angiomyolipoma in four patients was confirmed to be correct on pathological examination. The 2 patients whose radiological diagnose was difficult were found to have cavernous hemangioma and angiomyolipoma. One patient with renal cell carcinoma developed arteriocaliceal fistula, the only immediate complication in this series and underwent nephrectomy on postoperative 10th day. The mean follow up duration for the 14 patients with renal cell carcinoma was 18.6 months (range:1-103). There was no other tumor involvement in the resection margins following the nephron sparing surgery. These results suggest that nephron sparing surgery provides an effective treatment for patients with a single, small, unilateral, localized renal tumor. Longer follow-up is suggested for more definite verification of the role of nephron sparing surgery.

• Clinical and Experimental Pediatrics
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• v.49 no.4
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• pp.410-416
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• 2006

Purpose : Ciliary abnormalities of the respiratory system usually accompany recurrent or persistent respiratory diseases such as paranasal sinusitis, bronchiectasis, rhinitis, and/or otitis media, since they cause certain derangements in ciliary cleaning activities. This disease is usually inherited by autosomal recessive trait, but may also be found to be acquired or transient in rare cases after heavy exposure to pollutants, cigarette smoking or severe infection. We performed this study in children with frequently recurrent or persistent respiratory diseases to clarify if the ciliary abnormalities are preceding factors. Methods : We enrolled 17 children with suspected respiratory ciliary abnormalities. The indications for evaluation of ciliary ultrastructure were recurrent or persistent respiratory infections. Children with immunologic abnormalities were excluded. From August 2000 to July 2003, we performed a biopsy on nasal mucosa and examined the structure of ciliary status by using an electron microscope. Results : Of the subjects, there were seven males and 10 females, aged 2 to 10 years. Out of the 17 subjects, 12 cases of chronic paranasal sinusitis, nine chronic coughs, nine frequent upper respiratory infections, seven cases of recurrent otitis media, four cases of recurrent pneumonia, and four cases of bronchial asthma were found. Out of the 17 cases on which histologic examinations were conducted, four cases showed pathologic findings, including one case of inner dynein arm defect, one of microtubular transposition, one of supernumerous tubules, and one singlet, respectively. Conclusion : It is essential for differential diagnosis and effective treatment to identify the abnormalities of ultrastructure of nasal cilia in children with symptoms of frequently recurrent or persistent respiratory diseases, if immunodeficiency or respiratory allergy could be excluded.

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.466-472
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• 2002

Purpose : The diagnosis of Meckel's diverticulum is difficult and delayed because it presents with various clinical symptoms. We evaluated clinical, imaging and pathologic findings of Meckel's diverticulum to facilitate detection of Meckel's diverticulum in children. Methods : Review of clinical, imaging, surgical and pathological findings in 10 children aged 7 days to 14 years with Meckel's diverticulum during an 8-year period, 1993-2001, at Ewha Womans University Hospital was undertaken. Results : The male to female ratio was 2.3 : 1. The chief complaint was painless lower gastrointestinal( GI) bleeding; others were abdominal pain, abdominal distention and vomiting, in order of frequency. The diagonsis before surgery were Meckel's diverticulum in 5 patients, non-reducible intussusception in 3 patients and intestinal obstruction in 2 patients. The diverticulum was located between 35 cm to 70 cm proximal to the ileocecal valve. The length of the diverticulum ranged from 4 cm to 12 cm and 80% of it was within 5 cm. A Meckel scan($^{99m}Tc-pertechnetate$ scintigraphy) after cimetidine administration was done in 6 cases. All 5 cases that presented with lower GI bleeding had ectopic gastric mucosa confirmed on pathology. Out of 5 cases of ectopic gastric mucosa, only 4 cases were positive on the Meckel's scan. Conclusion : In cases of unexplained GI bleeding, obstruction, or inflammation diagnostic workup should be carried out to rule out Meckel's diverticulum. Laparoscopy, high resolution ultrasonography and computed tomography of the abdomen may be indicated in the assessment of pediatric patient with lower GI bleeding, especially in patients with suspected bleeding from Meckel's diverticulum showing negative Meckel's scan.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1242-1247
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• 2003

Purpose : Gastroesophageal reflux disease(GERD) is known as one of the most common causes of chronic cough, especially in children. The purpose of this study is to evaluate the efficacy of parameters from proximal esophageal 24-hr pH monitoring through its comparison with those of distal esophageal 24-hr pH monitoring that we generally use for diagnosis of GERD. Methods : We performed chest CT scans to find out the cause of chronic cough in infants with no clinical manifestation suggesting GERD. Then, in case that they had air space consolidation in posterior segment of both upper lobes and superior segment of both lower lobes(dependent position), we performed proximal and distal esophageal 24-hr pH monitoring. Results : The proximal and distal pH monitoring were performed in 17 infants(male 12; female five). The patients with positive pathologic reflux in proximal esophagus were 15 of 17(88.2%) and in distal esophagus were four of 17(23.5%). Reflux index and the total number of reflux episodes were statistically significantly lower in the proximal than in the distal esophagus(P<0.05). There was no correlation between each parameters of proximal and distal esophageal 24-hr pH monitoring. Conclusion : This study suggests that proximal esophageal 24-hr pH monitoring can be used as a very useful diagnostic tool in infants with chronic cough in which there are suspicions that it resulted from aspiration due to GERD.