Jeong, Hyeon Kyoung;Ahn, Eun Young;Rim, Sung Soo;Kim, Eun Young;Kim, Kyoung Sim;Kim, Yong Wook;Kim, Ki Bok
Clinical and Experimental Pediatrics
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v.45
no.3
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pp.311-319
/
2002
Purpose : We have performed this study to obtain reference data for the distribution of chromosomal aberrations in Korea. Methods : We analyzed 1,180 chromosomal study cases from Kwang ju Christian Hospital during the past 25 years. 756 cases suspected of characteristic chromosomal aberration syndromes and 424 cases with hermaphroditism, mild sexual abnormalities, multiple anomalies, or mental & growth retardation were included. Results : The male to female ratio of autosomal aberration syndromes was 1.2 : 1. 78.6% of autosomal aberrations were diagnosed under 1 year of age, whereas 89.8% of sex chromosomal aberrations were diagnosed over 12 years of age. Among 1,180 cases, 612 ones had chromosomal aberrations(51.9%) : 590 of 756 cases suspected of chromosomal aberration syndromes had aberrations( 78.0%), whereas 22 of 424 showing the above other features had aberrations(5.2%). Autosomal aberrations appeared in 514 cases(83.8%) and sex chromosomal aberrations appeared in 98 cases(16.2%). The most frequently observed abberation in autosomal aberrations was Down syndrome, followed by E, D, B, A and C group aberrations. The most common abberation in sex chromosomal aberrations was Turner syndrome, followed by Klinefelter syndrome and Fragile X syndrome. Conclusion : It is of vital importance that patients suspected of chromosomal aberrations undergo chromosomal analysis. Further advanced chromosomal staining and molecular genetic methods will raise the detection rate of chromosomal aberrations.
Purpose : It is known that single umbilical artery is frequently associated with gastrointestinal or urogenital anomaly, however, routine renal sonography has been debated in healthy neonate with isolated single umbilical artery. This study is designed to determine the usefulness of routine renal sonography in apparently healthy infants with an isolated single umbilical artery. Methods : Thirty healthy neonates with a single umbilical artery without a major anomaly from January 1995 to July 2002 were enrolled. The authors investigated the clinical background of babies and their mothers after renal sonography after 72 hours of age. When the abnormalities were found at the first renal sonography, the severity of hydronephrosis and degree of obstruction and renal function were analyzed by follow up renal sonography, voiding cystourethrography(VCUG) and technetium-99m-dimercaptosuccinic acid(DMSA) scan or technetium-99m-mercaptoacetyl-triglycerine (MAG3) scan. Results : Among the 30 healthy patients with isolated single umbilical artery, five patients(16.7%) showed abnormalities on first renal sonography with one major(3.3%) and four(13.4%) minor renal anomaly(minimal or mild hydroneohrosis). One major renal anomaly(severe hydronephrosis) showed severe decreased renal function on MAG3 scan without reflux, and the other four minor regressed spontaneously on follow up study. Conclusion : The value of routine early renal sonograpy for detecting renal anomaly in healthy infants with an isolated single umbilical artery remained unclear because most of the anomalies would regress spontaneously in the follow up study.
Purpose : Fragile sites are points on chromosomes which tend to break non-randomly when exposed to specific chemical agents or conditions of tissue culture. The chromosomal break induced by the antineoplastic drug, 1-${\beta}$-D-arabinofuranosyl-cytosine(Ara-c), was investigated to study the laboratory conditions in which the incidence of chromosomal break could be enhanced. Besides, the fragile sites induced by Ara-C were investigated and compared to the already known locations of the specific chromosomal alterations observed in specific neoplasms. Methods : T-lymphocytes from theree normal males and three females were cultured for 48 hours. Cells from each individual were exposed to the Ara-C for an additional 24 hours. After the caffeine was added during the last six hours culture, the metaphase chromosomes were prepared following the conventional method. A site was considered fragile if it was found to break two or more per 100 chromosomal breaks in more than four of six individuals tested. Results : Ara-C induced 252.1 chromosomal breaks per 100 mitotic cells and this result was significantly higher than that of the control, which induced 25.2 breaks(P<0.05). The incidence of the chromosomal break by Ara-C was higher, if cultured in the MEM-FA, which has no folic acid, than in the RPMI 1640 which contains enough folic acid(P<0.05). The most common break site by Ara-C was 3p14.2(FRA3B). There were 20 fragile sites induced by Ara-C. Among these 20 fragile sites, seven coincided with the locations of the mapped oncogenes, JUN, SKI, REL, N-MYC, FHIT, MET, ETS-1, and FOS. Conclusion : S phase specific chemotherapeutic agent, Ara-C, induced the expression of the chromosomal fragile sites effectively using the T-lymphocyte in vitro. Some of the fragile sites by Ara-C highly coincided with the oncogenes and neoplasm specific chromosome breakpoints. In this regard, the fragile sites reported here could provide the unknown neoplasm related chromosomal alternation points.
Background: We analyzed five hundred patients who underwent either isolated or concomitant coronary artery bypass grafting(CABG) between November 1981 and June 1997. Material and Method: There were 330 males and 170 females with a mean age of 57.4$\pm$8.9 years. To evaluate the preoperative status, we performed electrocardiograghy, echocardiography, MIBI scan, Duplex sonogram, common blood test including CK and LDH and coronary angiography. Result: Preoperative clinical diagnoses were unstable angina in 282 (56.4%), stable angina in 141 (28.2%), postinfarction angina in 58 (11.6%), acute myocardial infarction in 8 (1.6%), variant angina in 7 (1.4%) and failed percutaneous transluminal coronary angioplasty in 4 (0.8%) patients. Preoperative angiographic diagnoses were three-vessel disease in 263 (52.6%), two-vessel disease in 93 (18.6%), one-vessel disease in 71 (14.2%), left main disease in 68 (13.6%), and others in 5 (1.0%) patients. Patients had various risk factors for coronary disease, and the frequency of the risk factors such as hypertension, diabetes and smoking showed increasing tendency year by year. We used saphenous vein grafts in 1143, internal thoracic artery grafts in 442, radial artery graft in 17, and gastroepiploic artery graft in 1 anastomosis. The mean number of grafts was 3.2$\pm$1.2 per patient. Concomitant operations were prosthetic valve replacement or valvuloplasty in 31, coronary endarterectomy and angioplasty in 27, left main coronary angioplasty in 13, carotid endarterectomy in 5, and neurologic problems, bleeding, and perioperative myocardial infarction. The mean follow-up period was 25$\pm$23 months and there were 5 cases of reoperation. Conclusion: We hope that the surgical results would improve with the accumulation of experience, application of new myocardial protection technique, and timely intervention of mechanical assisted devices.
Excellent clinical results of the arterial switch operation and the limited availablity of the intraventricular rerouting has recently made an arterial switch operation to become the therapeutic method of choice for the repair of double-outlet right ventricle (DORV) with subpulmonary ventricular septal defect (VSD). The early and midterm outcomes of arterial switch operation for this anomaly were evaluated. Material and Method: Between August 1994 and July 2002, 13 patients underwent an arterial switch operation for the correction of double-outlet right ventricle with subpulmonary VSD at Dong-A university hospital.. The 50% rule was used to define DORV. Median age and mean body weight were 27 days (range, 3-120 days) and 3.8$\pm$0.7kg (range, 2.92-5.3kg) respectively. Aortic arch anomalies were associated in 6 cases (46.2%), which were all repaired through one-stage operation. The relationship of the great arteries were side-by-side in 8 cases (61.5%) and anteroposterior in 5 (38.5%). Coronary artery patterns were 1 LCx-2R in 6 cases, retropulmonary left coronary artery (LCA) in 6, and intramural LCA in 1 respectively. The enlargement of VSD was required in 1 patient and the patch enlargement of right ventricular outflow tract was performed in another one patient. The Lecompte maneuver was used in all but 3 patients with a side by side relationship of the great arteries. Result: Overall postoperative hospital mortality was 23.1 % (3/13). All operative deaths were occurred in the patients with aortic arch anomalies. There was one late death related to the postoperative complication of the central nerve system during the mean follow-up of 41.3$\pm$30.7 months. Pulmonary valvar stenosis (>30mmHg of pressure gradient) developed in 1 patient (10%) and left pulmonary artery stenosis in 2 (20%), among them, one required reoperation 52 months after repair. There was an asymptomatic patient with moderate aortic regurgitation. 5-year survival rate including operative deaths was 68.3%. Conclusion: Although the operative mortality is high in the patients with aortic arch anomaly, the arterial switch operation for DORV with supbpulmonary VSD can be performed with low operative mortality and low reoperation rate in the patients Without arch anomaly. The arterial switch operation can be considered a good option for this complex anomaly.
Kim, Hyung-Tae;Sung, Si-Chan;Kim, Si-Ho;Bae, Mi-Ju;Lee, Hyoung-Doo;Park, Ji-Ae;Chang, Yun-Hee
Journal of Chest Surgery
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v.43
no.4
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pp.364-374
/
2010
Background: We assessed the early and mid-term results of the modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome (HLHS) and its variants to identify the risk factors for hospital mortality. Material and Method: Between March, 2003, and December, 2009, 23 patients (18 males and 5 females) with HLHS or variants underwent the modified Norwood procedure. The age at operation ranged from 3 to 60 days (mean, $11.7{\pm}13.2days$) and weight at operation ranged from 2.2 to 4.8 kg (mean, $3.17{\pm}0.52kg$). We used a modified technique that spared the anterior wall of the main pulmonary artery in 20 patients. The sources of pulmonary blood flow were RV-PA conduit in 15 patients (group I) and RMBTS in 8 (group II). Follow-up was completed in 19 patients (19/20, 95%) in our hospital (mean $26.0{\pm}22.8months$). Result: Early death occurred in 3 patients (3/23, 13%), of whom 2 had TAPVC. Fourteen patients underwent subsequent bidirectional cavopulmonary connection (BCPC, stage 2) and seven underwent the Fontan operation (stage 3). Three patients died between stages, 2 before stage 2 and one before stage 3. The estimated 1-year and 5-year survival rates were 78% and 69%, respectively. On multivariate regression analysis, aberrant right subclavian artery (RSCA) and associated total anomalous pulmonary venous connection (TAPVC) were risk factors for hospital mortality after stage 1 Norwood procedure. Conclusion: HLHS and its variants can be palliated by the modified Norwood procedure with low operative mortality. Total anomalous pulmonary venous connection adversely affects the survival after a stage 1 Norwood procedure, and interstage mortality rates need to be improved.
Purpose : To Investigate the pathways of radiation induced apoptosls and the effect of cysteamine (${\beta}$-mercaptoethyiamine), as a radioprotector, on it. Materials and Methods : HL-50 ceils were assigned to control, irradiated, and cysteamlne (1 mM, 10mM) pretreated groups. Irradiation was given In a single fraction of 10 Gy (6 MV x-ray) and cysteamine was administered 1 hour before irradiation. The activities of caspase-8 were measured in control and irradiated group to evaluate its relation to the radiation Induced apoptosis. To evaluate the role of cysteamine In radiation Induced apoptosis, the number of viable cells, the expression and activity of caspase-3, and the expression of poly (ADP-ribose) polymerase (PARP) were measured and compared after irradiating the HL-60 celis with cysteamine pretreatment or not. Results : The intraceliular caspase-8 activity, known to be related to the death receptor induced apoptosis, was not affected by irradiation(p>0.05). The number of viable cells began to decrease from 6 hours after irradiation (p>0.05), but the number of viable cells In 1 mM cysteamine pretreated group was not decreased after irradiation and was similar to those in the control group. In caspase-3 analyses, known as apoptosis executioner, its expression was not different but its activity was Increased by irradiation(p>0.05). However, this Increase of activity was suppressed by the pretreatment of 1 mM cysteamine. The cleavage of PARP, thought to be resulted from caspase-3 activation, occurred after irradiation which was attenuated by the pretreatment of 1 mM cysteamine. Conclusion : These results show that radiation induced apoptotic process is somewhat different from death receptor induced one and the pretreatment of 1 mM cysteamine has a tendency to decrease the radiation-induced apoptosis in HL-60 cells.
Journal of the Korea Organic Resources Recycling Association
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v.14
no.1
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pp.112-130
/
2006
In order to improve the waste management system for the unit of small villages, we visited 14 counties nationwide and investigated the present conditions of the farming and fishing villages on waste management. First, we selected one concentrated residential type village and one separated residential type village. Then we investigated the quantities and the characteristics of the domestic wastes generated from the 2 sample villages that we chose early on. The concentration of 7 heavy metals in open burned residuals was analyzed. The results are as follows: (1) We distributed the 799 questionnaires to public servants (83 Gun, 716 Myon) and 337 questionnaires to residents. In accordance with a result of questionnaire on the Volume-Based Waste Fee System, the rate of participated questionnaire was 70.3% from public servants and 31.8% from residents. (2) From the survey on two chosen villages, the generation of municipal waste was 48.6g/person/day in A Village, 54.3g/person/day in B Village. This amount was smaller than that of national average of 1.05kg because of excluding recyclable wastes and some of incinerated combustible waste. (3) The 14 counties entered the aged society. There were 2 aging societies, 8 aged societies and 4 super-aged societies. And the average ratios of Public financial independence and waste budget of financial independence were 16.1% and 17.9% respectively. These are lower than that of national average of 57.2% and 31.4%. This indicates that these factors seemed to be operated with the hindrance insettling Volume-Based Waste Fee System in the small villages.
The purpose of this study was to evaluate the changes of cancellous and cortical bone and the effect of estrogen in ovariectomized rats. Fifty female rats, 250gm in body weight, were divided into three groups : ovariectomized group(OVE), ovariectomized and estrogen-injected group(OVE-EST), and sham operated and estrogen-injected group(EST). Bilateral ovariectomy was performed at the onset of the experiment. In OVE-EST group and EST group, estrogen was injected $50{\mu}g/kg$ B.W. every other days from 3 weeks after surgery to sacrifice. Each five rats were sacrificed after 5, 6, 7 weeks. One side of mandibular body was radiographed with a soft x-ray apparatus(Hitex Co., Ltd., Japan). Thereafter the obtained microradiographs were used for the morphometric analysis using a Image analyzer. The morphometric analysis was perforrmed for parameters such as total bone area, cortex bone area and medullary bone area. The other side of the mandibular bone was decalcified and embedded in paraffin as using a general method. The specimens were sectioned and stained with Mallory's anilline blue and observed light microscopically. The results were as follows. 1 In all groups, the proportion of cortex to total bone area was not significantly different. 2. In ovariectomized(OVE) group, the proportion of marrow cavity to medullary bone area increased significantly from 5 to 7 weeks(p<0.05). In ovariectomized and estrogen-injected(OVE-EST) group, it decreased significantly at 7 weeks, and in estrogen-injected(EST) group, it decreased significantly from 6 weeks(p<0.05). 3. Microradiogram and histopathologic findings revealed that marrow cavity was enlarged and osteoclasts were observed around irregular bone surface in OVE group. In OVE-EST group, the size of marrow cavity at 7 weeks was similar to that of control group. In EST group, as dense trabecular bone increased from 5 to 7 weeks, marrow cavity decreased.
Background: Total anomalous pulmonary venous connection (TAPVC) is still one of the more challenging congenital heart defects in newborns and young infants. The purpose of the study is to evaluate the early and midterm results of the surgical corrections for patients in early infancy with isolated TAPVC. Material and Method: Hospital records of 15 consecutive patients in early infancy (January 1993 to August 1998) were retrospectively reviewed. There were 8 boys and 7 girls whose ages ranged from 4 days to 3.5 months (median age 22 days). Their body weight ranged from 1.75 kg to 4.9 kg (mean 3.54 kg). The abnormal anatomical connections were supracardiac in 11, cardiac in 3, and infracardiac in 1. In 6 patients (40%), the pulmonary venous drainage was obstructive. Total circulatory arrest was used in 13 patients. Anastomosis between the common pulmonary vein and the left atrium was performed with a continuous suture technique using a fine nonabsorbable polypropylene suture through a lateral approach behind the right atrium. Result: There was one hospital death (6.5%) caused by a sepsis 17 days after the operation in a neonate who had supracardiac drainage and was dependent on a ventilator preoperatively. There were 2 late deaths. One died sudde`nly of an unknown cause at home 2.5 years after the operation and the other died of a recurrent pulmonary hypertension 3 months after the reoperation due to pulmonary venous obstruction (PVO). Two patients required reoperations because of PVO 5 months and 10 months respectively after the initial operation. Of these patients, one patient is alive at the present time with persistent pulmonary hypertension. All survivors without postoperative PVO (78.6%) were in NYHA functional class I at mean follow-up of 25.8 months (0.5∼67 months). Conclusion: Surgical correction of TAPVC in early infancy can be performed at low risk. However, there were 2 postoperative PVOs (14.3%) which had bad results. The survivors without postoperative PVO had excellent functional status.
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