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.
Kim Myung Se;Kim Kyung Ae;Kim Sung Kyu;Shin Sei One;Lee Sung Ho;Chang Jae Chun
Radiation Oncology Journal
/
v.7
no.1
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pp.59-70
/
1989
Cervix cancer is the most common female cancer in Korea. In spite of their relatively local invasive tendency, still $44\%$ of patient will develop recurrent cancer. This result suggests that more aggressive local treatment may increase the cure rate but increased complication risk also cannot be avoidable. Various institutions proposed different treatment regimen, but recommended dose were about 4500 cGy for whole pelvis and 8000 cGy at point A, even though they agreed that those doses may not be satisfactory for control of bulky disease. 96 cases of invasive cervical cancer, treated with postoperative or primary radiation therapy were analyzed to determine the complication rate and prognostic factor in our treatment regimen Which is $5500\~1000 CGy$ higher than Other institution. Mean follow up duration was 21 months. Symptomatic patients including mild but persistent abdominal discomfort was $46\%$, but only 1 patient $(1\%)$ had operative treatment because of incomplete obstruction of small bowel. Most symptoms appeared within 12 months and most common complaints were frequent bowel movement. Barium enema and sigmoidoscopy were performed for persistent symptomatic patients. Only one patient had abnormal finding in barium enema which showed inefficiency of this method for detecting bowel complication. Patient's age, total tumor dose, total TDF, rectal dose were not significant risk factors for complication, but boost dose, previous history of operation had some relationship with complication risk. Even though dose of point A and rectum is $500\~1,000cGy$ higher than other institution, such a low rate of severe complications may suggest that fear of complications should not be overestimated than cure rate and the possibility of more aggressive treatment for better local control should not be underestimated.
Background: Postinfarction ventricular septal rupture is associated with mortality as high as $85\sim90%$, if it is treated medically. This report documents our experience with postinfarction ventricular septal rupture that was treated surgically, Material and Method: We retrospectively reviewed the medical records of 11 patients who were operated on due to postinfarction ventricular septal rupture between August 1996 and August 2006. There were 4 men and 7 women, with a mean age of $70{\pm}11$ years (age range: $50\sim84$ years). The location of the rupture was anterior in 7 cases and posterior in 4 cases. The interval between the onset of acute myocardial infarction and the occurrence of the ventricular septal rupture was $2.0{\pm}1.3$ days (range: $1\sim5$ days). Operation was performed at an average of $2.4{\pm}2.7$ days (range: $0\sim8$ days) after the diagnosis of septal rupture. Preoperative intraaortic balloon pump therapy was performed in 10 patients. Result: The infarct exclusion technique was used in all cases. Coronary artery bypass grafting was done in 8 cases, with the mean number of distal anastomosis being $1.0{\pm}0.8$. There was one operative death. In 2 patients, reoperation was performed due to a residual septal defect. The postoperative morbidities were transient atrial fibrillation (n=7), paroxysmal supraventricular tachycardia (n=1), low cardiac output syndrome (n=3), bleeding reoperation (n=2), delayed sternal closure (n=2), acute renal failure (n=2), pneumonia (n=1), intraaortic balloon pump-related thromboembolism (n=1), and transient delirium (n=2). Nine patients have been followed up for a mean of $38{\pm}40$ months except for one follow-up loss. There have been 3 late deaths. At the latest follow-up, all 6 survivors were in a good functional class. Conclusion: We demonstrated satisfactory operative and midterm results with our strategy of preoperative intraaortic balloon pump therapy, early repair of septal rupture by infarct exclusion and combined coronary revascularization.
Kim, Kyung-Hwan;Ahn, Hyuk;Hwang, Ho-Young;Choi, Jin-Ho;Kim, Ki-Bong
Journal of Chest Surgery
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v.43
no.5
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pp.475-481
/
2010
Background: We evaluated mid-term results of mitral annuloplasty using a flexible band and a completely rigid ring for mitral valve repair in patients with degenerative mitral regurgitation (MR). Material and Method: From January 2004 to September 2008, 71 patients (M:F=36:35, $55{\pm}13$ years) underwent mitral valve repair with mitral annuloplasty for degenerative MR. Ring annuloplasty was done using a Cosgrove-Edwards flexible band (Group I, n=43) or a Carpentier-Edwards classic ring (Group II, n=28). There were no differences in preoperative characteristics of the participants. Average duration of follow-up was 36 months (range: 2~69 mos). Result: There was no in-hospital mortality. Postoperative morbidity, which included atrial fibrillation (n=7) and low cardiac output syndrome (n=5) in groups I and II were similar. There was one late death in group II. The proportion exhibiting freedom from recurrent mitral regurgitation ($\geq$moderate) at 4 years in Groups I and II were, respectively, 94.5 and 91.8%, (p=0.695). Left ventricular ejection fraction decreased in the early postoperative period ($7{\pm}2$ days) and recovered by last follow-up ($25{\pm}16$ mos; p=0.002). The pattern was similar in groups I and II (p=0.905). Re-operation was performed in 3 patients (1 in Group I and 2 in Group II, p=0.316). Four-year event-free survival (free of adverse valve-related events) was 95.2% for Group I and 92.6% for Group II; this difference was not significant, p=0.646). Conclusion: The type of technique used in mitral annuloplasty to repair the mitral valve repair after degenerative MR did not affect mid-term clinical and functional results.
Background: Thoracic empyema remains a serious problem despite the availability of modern diagnostic methods and appropriate antibiotics. The condition presents in many different forms and stages that require different therapeutic options. Video-assisted thoracic surgery (VATS) has become increasingly popular for use in the treatment of empyema. Material and Method: From January 2005 to May 2009, VATS was performed in 36 patients with pleural empyema and for whom chest-tube drainage and antibiotic therapy had failed or the CT scan showed multiseptate disease. The perioperative clinical factors were analyzed for all the study patients. Result: All the patients underwent VATS, but it was necessary to convert to thoracotomy in one patient. The mean operation time was $90{\pm}38.5\;min$. For the operative evaluation, 11 patients were compatible with ATS stage III. The duration of chesttube insertion was $11.9{\pm}5.8$ (3~24) days. One patient did not improve and therefore this patient underwent additional open drainage. At discharge, costophrenic angle blunting was observed in 22 patients, pleural thickening was noted in 20 patients, both were noted in 17 patients and neither was noted in 11 patients. However, at follow-up, each of these changes was observed in 9, 7, 4 and 24 patients, respectively. All except one patient showed radiographic improvement. Conclusion: VATS is suitable for the treatment of early and fibrinopurulent thoracic empyema, and even in selected patients with stage III disease.
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.
Journal of the Korea Organic Resources Recycling Association
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v.21
no.4
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pp.72-81
/
2013
The objective of this study was to verify whether SCB(Slurry Composting & Bio-filtration) system can be applied for the treatment of anaerobic digestion(AD) wastewater and also, to identify the most effective set among three filtration compost beds tested. Results can be summarized as these; (a) When AD wastewater was sprayed on the top of beds which were mainly composed of sawdust and/or other media and, subsequently, filtrates collected and analyzed, there were large drop in the values of Electric Conductivity(EC), Total Suspended Solid(TSS), Biochemical Oxygen Demand(BOD), and Chemical Oxygen Demand(COD). In contrast, Total Nitrgen(T-N) and Total Phosphorus(T-P) were progressively elevated. We consider these changes as positive if the filtrate are to be utilized as liquid fertilizer. (b) When three sets of filtration beds (T1, T2, T3) were compared for their effectiveness, no significant difference was found among them. These indicate that expensive sawdust can be replaced in part with cheaper media such as woodchip, rice husks, or others. (c) At early stage of operation (within 20 days), BOD in filtrates were maintained at high level probably due to the lack of microbial activity. During the same stage, T-N, T-P was at low level but, were elevated to higher levels thereafter. These data, when combined, indicate that the filtration system needs at least a couple of weeks for the optimized microbial functioning. (d) The temperatures of the experimental beds were progressively dropped as the experiment continued through the fall season, although filtration effectiveness was not noticeably influenced.
This study was undertaken to analyze the outcome of composite valve graftreplacement(CVGR) for the treatment of aneurysms of the ascending aorta involving the aortic root. Material and Method: Between April 1995 and June 2001, 56 patients had replacement of the ascending aorta and aortic root with a composite graft valve and were reviewed retrospectively. Aortic regurgitation was present in 50 patients(89%), Marfan's syndrome in 18 patients(32%), and bicuspid aortic valve in 7(12.5%). The indications for operation were annuloaortic ectasia(AAE) in 30 patients(53.6%), aortic dissection in 13(23.2%), aneurysms of the ascending aorta involving aortic root in 11(19.6%), and aortitis in 2(3.6%). Cardiogenic shock due to the aortic rupture was present in 2 patients. Nine patients(16%) had previous operations on the ascending aorta or open heart surgery. The operative techniques used for CVGR were the aortic button technique in 51 patients(91%), the modified Cabrol technique in 4, and the classic Bentall technique in 1. The concomitant procedures were aortic arch replacement in 24 patients(43%), coronary artery bypass graft in 8(14.3%), mitral valve repair in 2, redo mitral valve replacement in 1, and the others in 7 The mean time of circulatory arrest, total bypass, and aortic crossclamp were 21$\pm$14 minutes, 186$\pm$68 minutes, and 132$\pm$42 minutes, respectively. Result: Early mortality was 1.8%(1/56). The postoperative complications were left ventricular dysfunction in 16 patients(28.6%), reoperation for bleeding in 7(12.5%), pericardial effusion in 2, and the others in 7. Fifty-three patients out of 55 hospital survivors were followed up for a mean of 23.2 $\pm$ 18.7 months(1-75 months). There were two late deaths(3.8%) including one death due to the traumatic cerebral hemorrhage, and CVGR-related late mortality was 1.9%. The 1- and 6-year actuarial survival was 98.1$\pm$1.9% and 93.2$\pm$5.1%, respectively. Two patients required reoperation for complication of CYGR(3.8%) and two other patients required subsequent operations for dissection of the remaining thoracoabdominal aorta. The 1- and 6-year actuarial freedom from reoperation was 97.8$\pm$2.0% and 65.3$\pm$26.7%, respectively.
From March, 1992 to March, 1996, a total of 279 patients underwent coronary bypass surgery at the Sejong General Hospital, Puchon. We selected 22 patients with severe left ventricular(LV) dysfunction from them. The criteria were the presence of global or segmental abnormalities of left ventricular contraction and LV ejection fraction(EF) less than 35% based on biplane LV angiography by planimetry method. The mean age of 17 male and 5 female patients was 60$\pm$5.6years(range:47~73 years). All had the anginas, which were Canadian class II in 6, class 111 in 12 and class IV in 4. All patients except one had the history of previous myocardial infarction more than once. Seven of them had the symptoms and signs of congestive heart failure, such as dyspnea on excertion and increased pulmonary vascular markings. Their mean LVEF was 29.4$\pm$4 5%(range : 18~35%) and mean LV end-diastolic pressure was 18.7 $\pm$8. 2mmHg(range:10~42mmHg). 21 patients had 3 vessel-disease and 1 had 2 vessel-disease. Complete revascularization was tried with the use of 16 internal mammary arteries and 60 sapheuous veins and 3 radial arteries grafts. The mean number of distal anastomosis was 3.5$\pm$ 1.1. Concomitantly, one mitral valvuloplasty and annuloplasty was performed in the patient with moderate mitral regurtigation. The hospital mortality was 4.5%. During the follow-up, there were 3 late deaths. Of 18 survivors, 2 patients were lost in follow-up 24 and 27 month respectively after operation and the remaining 16 patients have bcen followed up with an average of 30.4 $\pm$ 13.4 months.15 patients had improvement with respect to angina but 8 patients still have the continuing or progressing heart failure. The 1-year, 2-year and 3-year actuarial survival rate was 85.2, 69.1, 46.1%, respectively. This study indicates that coronary artery bypass sur ery can be performed in the patients with severe LV dysfunction at acceptable risk but does not greatly contribute to the improvement of congestive heart failure.
Background: The use of video-assisted thoracic surgery (VATS) to perform major pulmonary resection with systematic node dissection (SND) for lung cancer by is commonly used in clinics. However, the feasibility of SND by VATS remains controversial. Video-assisted mediastinal lymphadenectomy (VAMLA) increases the quality of mediastinal lymph node staging in lung cancer. The video-mediastinoscope allows systematic lymphadenectomy by bimanual preparation. This study was conducted to assess safety and usefulness and clinical feasibility of VAMLA expanding Linder-Dahan mediastinoscope with VATS lobectomy for left sided lung cancer. Material and Method: Between February 2004 to April 2008, a total 50 patients who underwent VATS lobectomy for left sided lung cancer were analyzed retrospectively. Thirty patients (group A) underwent VAMLA followed by VATS lymphadenectomy and 20 patients (group B) underwent VATS lymphadenectomy for SND. Result: There were no statistical differences in operation times, chest tube indwelling times, or hospital days between the 2 groups. The number of dissected total nodes (p=0.001) and N2 nodes (p=0.013) were higher in group A than in group B, but there was no difference in N1 nodes. Postoperative complications included 2 prolonged air leakages (${\geq}$10 days) in each group, one pneumonia in group A, and one vocal cord palsy in group B. There were no early operative mortalities. Conclusion: Mediastinal staging of resectable lung cancer is performed by VAMLA. This new technique is the basis for VATS lobectomy particularly for left-sided lung cancer, because a higher percentage of mediastinal lymph nodes undergo complete resection using VAMLA.
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