• Clinical and Experimental Reproductive Medicine
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• v.35 no.2
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• pp.99-110
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• 2008

Objectives: Preimplantation genetic diagnosis (PGD) has become an assisted reproductive technique for couples carrying genetic conditions that may affect their offspring. Osteogenesis imperfecta (OI) is an autosomal dominant disorder of connective tissue characterized by bone fragility and low bone mass. At least 95% of cases are caused by dominant mutations in the COL1A1 or COL1A2. In this study, we report on our experience clinical outcomes with 5 PGD cycles for OI in two couples. Methods: Before clinical PGD, we assessed the amplification rate and allele drop-out (ADO) rate of alkaline lysis and nested PCR protocol using heterozygous patient's single lymphocytes in the pre-clinical diagnostic tests for OI. We performed 5 cycles of PGD for OI by nested PCR for the causative mutation loci, COL1A1 c.2452G>A and c.3226G>A, in case 1 and case 2, respectively. The PCR products were analyzed by agarose gel electrophoresis, restriction fragment length polymorphism (RFLP) analysis with HaeIII restriction enzyme in the case 1 and direct DNA sequencing. Results: We confirmed the causative mutation loci, COL1A1 c.2452G>A in case 1 and c.3226G>A in case 2. In the pre-clinical tests, the amplification rate was 94.2% and ADO rate was 22.5% in case 1, while 98.1% and 1.9% in case 2, respectively. In case 1, a total of 34 embryos were analyzed and 31 embryos (91.2%) were successfully diagnosed in 3 PGD cycles. Eight out of 19 embryos diagnosed as unaffected embryos were transferred in all 3 cycles, and in the third cycle, pregnancy was achieved and a healthy baby was delivered without any complications in July, 2005. In case 2, all 19 embryos (100.0%) were successfully diagnosed and 4 out of 11 unaffected embryos were transferred in 2 cycles. Pregnancy was achieved in the second cycle and the healthy baby was delivered in March, 2008. The causative locus was confirmed as a normal by amniocentesis and postnatal diagnosis. Conclusions: To our knowledge, these two cases are the first successful PGD for OI in Korea. Our experience provides a further demonstration that PGD is a reliable and effective clinical techniques and a useful option for many couples with a high risk of transmitting a genetic disease.

• Journal of Chest Surgery
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• v.31 no.5
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• pp.472-480
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• 1998

We reviewed the surgical results of intracardiac lateral tunnel Fontan procedure for the repair of functional single ventricles. Between 1990 and 1996, 104 patients underwent total cavopulmonary anastomosis. Patients' age and body weight averaged 35.9(range 10 to 173) months and 12.8(range 6.5 to 37.8) kg. Preoperative diagnoses included 18 tricuspid atresias and 53 double inlet ventricles with univentricular atrioventricular connection and 33 other complex lesions. Previous palliative operations were performed in 50 of these patients, including 37 systemic to pulmonary artery shunts, 13 pulmonary artery bandings, 15 surgical atrial septectomies, 2 arterial switch procedures, 2 resections of subaortic conus, 2 repairs of total anomalous pulmonary venous connection and 1 Damus-Stansel-Kaye procedure. In 19 patients bidirectional cavopulmonary shunt operation was performed before the Fontan procedure and in 1 patient a Kawashima procedure was required. Preoperative hemodynamics revealed a mean pulmonary artery pressure of 14.6(range 5 to 28) mmHg, a mean pulmonary vascular resistance of 2.2(range 0.4 to 6.9) wood-unit, a mean pulmonary to systemic flow ratio of 0.9(range 0.3 to 3.0), a mean ventricular end-diastolic pressure of 9.0 (range 3.0 to 21.0) mmHg, and a mean arterial oxygen saturation of 76.0(range 45.6 to 88.0)%. The operative procedure consisted of a longitudinal right atriotomy 2cm lateral to the terminal crest up to the right atrial auricle, followed by the creation of a lateral tunnel connecting the orifices of either the superior caval vein or the right atrial auricle to the inferior caval vein, using a Gore-Tex vascular graft with or without a fenestration. Concomitant procedures at the time of Fontan procedure included 22 pulmonary artery angioplasties, 21 atrial septectomies, 4 atrioventricular valve replacements or repairs, 4 corrections of anomalous pulmonary venous connection, and 3 permanent pacemaker implantations. In 31, a fenestration was created, and in 1 an adjustable communication was made in the lateral tunnel pathway. One lateral tunnel conversion was performed in a patient with recurrent intractable tachyarrhythmia 4 years after the initial atriopulmonary connection. Post-extubation hemodynamic data revealed a mean pulmonary artery pressure of 12.7(range 8 to 21) mmHg, a mean ventricular end-diastolic pressure of 7.6(range 4 to 12) mmHg, and a mean room-air arterial oxygen saturation of 89.9(range 68 to 100) %. The follow-up duration was, on average, 27(range 1 to 85) months. Post-Fontan complications included 11 prolonged pleural effusions, 8 arrhythmias, 9 chylothoraces, 5 of damage to the central nervous system, 5 infectious complications, and 4 of acute renal failure. Seven early(6.7%) and 5 late(4.8%) deaths occured. These results proved that the lateral tunnel Fontan procedure provided excellent hemodynamic improvements with acceptable mortality and morbidity for hearts with various types of functional single ventricle.

• Journal of Preventive Medicine and Public Health
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• v.22 no.4 s.28
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• pp.555-577
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• 1989

This study was conducted to compare the delivery management including laboratory tests, medication and surgical procedures for the delivery in various medical facilities. Two university hospitals, two general hospitals, three hospitals, two private obstetric clinics, and two midwifery clinics in a large city were selected as they permitted the investigators to abstract the required data from the medical and accounting records. The total number of deliveries occurred at these 11 facilities between 15 January and 15 February, 1989 was 789 among which 606(76.8%) were vaginal deliveries and 183 (23.3%) were C-sections. For the normal vaginal deliveries, CBC, Hb/Hct level, blood typing, VDRL, hepatitis B antigen and antibody, and urinalysis were routinely done except the private clinics and midwifery clinics which did not test for hepatitis B and Hb/Hct level at all. In one university hospital ultrasonography was performed in 71.4% of the mothers and in one general hospital liver function test was done in 76.7% of the mothers. For the C-section, chest X-ray, bleeding/clotting time and liver function test were routinely done in addition to the routine tests for the normal vaginal deliveries. Episiotomy was performed in 97.2% of the vaginal deliveries. The type and duration of fluid infused and antibiotics administered showed a wide variation among the medical facilities. In one university hospital antibiotics was not administered after C-section at all while in the general hospitals and hospitals one or two antibiotics were administered for one week on the average. In one private clinic one pint of whole blood was transfused routinely. A wide variation was observed among the medical facilities in the use of vitamin, hemostatics, oxytocics, antipyreptics, analgesics, anti-inflammatory agents. sedatives. digestives. stool softeners. antihistamines. and diuretics. Mean hospital day for the normal vaginal deliveries of primipara was 2.6 days with little variation except one hospital with 3.5 days. Mean hospital day for the C-section of primipara was 7.5 days and that of multipara was 7.6 days and it ranged between 6.5 days and 9.4 days. Average hospital fee for a normal vaginal delivery without the medical insurance coverage was 182,100 Won for the primipara and 167,300 Won for the multipara. In case of the primipara covered by the medical insurance a mother paid 82,400 Won and a multiparous mother paid 75,600 Won. Average hospital fee for a C-section without the medical insurance was 946,500 Won for the primipara and 753,800 Won for the multipara. In case of the primipara covered by the medical insurance a mother paid 256,200 Won and a multiparous mother paid 253,700 Won. Average hospital fee for a normal vaginal delivery in the university hospitals showed a remarkable difference, 268,000 Won vs 350,000 Won, as well as for the C-section. A wide variation in the laboratory tests performed for a normal vaginal delivery and a C-section as well as in the medication and hospital days brought about a big difference in the hospital fee and some hospitals were practicing the case payment system. Thus, standardization of the medical care to a certain level is warranted for the provision of adequate medical care for delivery.

• Journal of the Korean Institute of Traditional Landscape Architecture
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• v.29 no.3
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• pp.106-115
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• 2011

This study, based on (edited in 18th century), analysed the landscape system and cultural landscape elements of Yuhoidang(Hageowon 何去園) Garden in Musu-dong, Daejeon, and the findings are as in the following. YuHoidang(Gwon Yijin 權以鎭) managed Hageowon Garden in Musu-dong, located on the southern branch of Mt. Bomun, to realize his utopia. The completion of Hageowon Garden was only possible due to his installation of a variety of facilities in family gravesite on the hill behind his house: Shimyoso(Samgeunjeongsa 三近精舍, in 1707), Naboji(納汚池, in 1713), Banhwanwon(in 1714) and expended exterior space(in 1727). With regard to the landscape system of the village, the main range of mountains consists of Mt. Daedun, Mt. Odae and Mt. Bomun. The main high mountain of the three is Mt. Bomun, where 'Blue Dragon' hill branches off on the east side(Eungbong), 'White Tiger' in the west(Cheongeun and Sajeong) and Ansan(inner mountain) in the south. The landscape system is featured by 'mountains in back and rivers in front'. The river in the south-west, with its source in Mt. Juryun is called as the 'Stream of outer perfect spot', while the 'Stream of inner perfect spot' rises from Eungbong, passing through the east part of the village into the south-western direction. Banhwanwon Garden(盤桓園) was created with the stream in the east and natural bedrocks, and its landscape elements includes Naboji, Hwalsudam, Gosudae, Sumi Waterfall, Dogyeong(path of peach trees), Odeeokdae(platform with persimmon trees), Maeryong(Japanese apricot tree), springs and observatories. An expanded version of Banhwanwon was Hageowon garden, where a series of 'water-trees-stone' including streams, four ponds, five observation platforms, three bamboo forests and Chukgyeongwon(縮景園) of an artificial hill gives the origin forest a scenic atmosphere. When it comes to semantics landscape elements, there are (1) Yuhoidang to cherish the memory of a deceased parents, (2) Naboji for family unification, (3) Gosudae to keep fidelity, (4) Odeokdae to collect virtue and wisdom, (5) Sumi Waterfall to aspire to be a man of noble character, (6) Yocheondae for auspicious life, (7) Sumanheon and Gigungjae to be in pursuit of hermitic life, (8) Hwalsudam for development of family and study, (9) Mongjeong to repay favor of ancestors, (10) Seokgasan, a symbol of secluded life, (11) Hageowon to enjoy guarding graves in retired life. The spatial composition of Hageowon was realized through (1) Yuhoidang's inside gardens(Naboji, Jucheondang, Odeokdae, Dogyeong, Back yard garden and others) (2) Sumanheon(收漫軒) Byeolup or Yuhoidang's back yard gardens (Seokyeonji, Yocheondae, Sumanheon, Baegyeongdae, Amseokwon and others) (3) Chukgyeongwon of the artificial hill(which is also the east garden of Sumanheon, being composed of Hwalsudam, Sumi Waterfall and Gasan or 12 mountaintops) (4) the scenic spots for unifying Confucianism, Buddhism and Taoism are Cemetry garden in the back hill of the village, the temple of Yeogyeongam, Sansinkak(ancestral ritual place of folk religion) and Geoeopjae(family school). On top of that, Chagyeongwon Garden(借景園) commands a panoramic distant view of nature's changing beauty through the seasons.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.143-155
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• 2002

A survey on biliary atresia was made among 26 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and a case registration form for each patient during the twentyone-year period of 1980-2000. Three hundred and eighty patients were registered from 18 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:1.3. The age of patients on diagnosis with biliary atresia was on average $65.4{\pm} 36.2$ days old. The national distribution was 32.8% in Seoul, 25.3% in Gyoungki-Do, 21.6% in Gyoungsang-Do, 9.27% in Choongchung-Do, etc. in order. The most common clinical presentation was jaundice (98.4%) and change of stool color (86.2%) was second. Two hundred eighty (74.7%) of 375 patients were operated by 80 days of age. Three hundred thirty six (9 1.9%) of 366 patients were operated on by the original Kasai procedure, and 305 (84.3%) of 362 patients were observed by bile-drainage postoperatively. The overall postoperative complication rate was 18.5% and the overall postoperative mortality rate was 6.8%. The associated anomalies were observed in 72 cases (22.5%). One hundred ninty five (64.7%) of 302 patients have been alive in follow-up and 49 (25.1%) have survived over 5 years without problem after operation. Ascending cholangitis, varices and ascites affected survival significantly, and the important long-term prognostic factor was the occurrence of complications.