• Journal of Chest Surgery
• /
• v.30 no.11
• /
• pp.1062-1068
• /
• 1997

From August 1989 to January 1996, a total of 105 cases of bidirectional Glean operations have been done as the interim stage for the patien s with some risk of univentricular correction at Sejong General Hospital. From December 1992, we started the conversion to Fontal operations for them, and 42 cases underwent Fontal-stage operation till February 1996. Their diagnoses were univentricular heart in 19(right ventricular type : 14), tricuspid atresia 11, double outlet of right ventricle 9, and others in 3 cases. The median age of bidirectional Glerln-stage operation was 12.5 months(range 2 months to 8 years) and Fontan-stage operation was at 59.6 months of median age(range 1 year 5 months to ,9 year 7 months). The mean waiting interval between the two operations was 33.88 $\pm$ 17.85 months with a range of 10 months to 6 years 3 months. During the waiting periods, 18 patients developed significant systemic-pulmonary collaterals andfor systemic verso-veno collateral channels. There were 5 hospital deaths after operations due to low cardiac output in 4 and sepsis in one. Most of the Fontal-stage operations were done by the late al tunneling with Core-Tex tube graft patch and fenestrated with the size of 2.5 ~6 mm. All the patients were followed-up(7 months to 4 years 2 months, mean 21.97$\pm$10.82 months) and there were 5 late deaths(postoperatively 6 months to 2 years) due to thromboembolism in 1, after heart transplantation 1, plastic bronchitis 1, protein loosing enteropathy 1, and pneumonia in 1. Dividing the patients by the waiting interval of 2 years, the early correction to Fontal group (N=16) showed the better results(hospital mortality 1116, late mortality 1116, significant collateral development 2/16) compared to the other group(N=26) (4/26, 4/26, 16/26). In conclusion, after the bidirectional Glean-stage operation successfully got rid of the previous risk factors, we recommand to do the Fontan-stage operation no later than 2 years of interval.

• Journal of Chest Surgery
• /
• v.29 no.11
• /
• pp.1218-1222
• /
• 1996

The reported incidence of postcardiotomy cardiogenic shock not responding to conventional therapy is still 0.1 to 0.8%. For this group of patients, more aggressive form of circulatory support must be employed. Centrifugal pumps are a ventricular assist device most commonly used on this purpose, due to low cost and easy availability. Currently, however, clinical experience of centrifugal pumps as a ventricular assist device is rarely reported in Korea. From January 1992 to January 1996, 2986 patients underwent cardiac operations on cardiopulmonary bypass at Seoul National University Hospital. Refractory postcardiotomy cardiac failure requring ventricular support with a Biomedicus centrifugal pump developed in ten of these patients. There were eight men and two women, ranged in age from nine years to 77 years with a mean of 50$\pm$20 years. The primary surgical procedures consisted of isolated coronary revascularization in four patients, combined coronary revascularization and aortic valve replacement in two, aortic dissection repair in two, pulmonary embolectomy in one, and heart transplantation in one. Of the ten patients, five had left ventricular assistance, one had right ventricular assistance, and four had biventricular assistance. Duration of ventricular assistance ranged from 24 to 175 hours, with a mean of 76$\pm$51 hours. Seven patients were weaned from ventricular assistance, and four of them discharged. The causes of death for nonsurvivors were progressive cardiac failure in two patients and multiorgan failure, intractable ventricular fibrillation, irreversible brain injury, and mechanical problem, respectively, in the other four. Survival was not predicted by time on cardiopulmonary bypass, aortic cross-clamp time, or duration of ventricular support. Major complications included bleeding(7), renal failure(6), infection(3) and neurologic complication(2). These results indicate that a centrifugal pump can provide reasonably satisfactory short-term circulatory support.

• Journal of Chest Surgery
• /
• v.41 no.1
• /
• pp.12-24
• /
• 2008

Background: It is currently thought that tissue valve degeneration is related to an animal's immune response, which is mainly due to cell surface ${\alpha}$-Gal epitopes. Cell surface ${\alpha}$-Gal epitopes are known to be degraded by the enzyme called green coffee bean ${\alpha}$-Galactosidase. It is also well known that ${\alpha}$-Gal epitopes are immunologically stained by Griffonia Simplicifolia isolectin type B4. We know that many commercially available tissue valves are made of aortic valves and pericardial tissue of pig. So, we investigated whether ${\alpha}$-Gal epitopes of the aortic valve and pericardial tissue of a pig can be removed by green coffee bean ${\alpha}$-Galactosidase, and we did so by comparing immunologic staining of the tissues before and after the enzyme treatment. Material and method: After treating fresh porcine aortic valve and pericardial tissue with green coffee bean ${\alpha}$-Galactosidase at concentrations of 0.5 unit/mL, 1.0 unit/mL, 2.0 unit/mL, respectively, under the condition of pH 6.5, temperature. $4^{\circ}C$ and 24 hours of incubation, each sample was stained with Griffonia Simplicifolia isolectin type B4 immunpfluorescent labeling. We then examined whether the ${\alpha}$-Gal epitopes were reduced or abolished in each consecutive. concentration of green coffee bean ${\alpha}$-Galactosidase by comparing the degree of the Griffonia Simplicifolia isolectin B4 staining in each sample. Result: In the pig aortic valve tissue, a 1.0 unit/mL concentration of green coffee bean ${\alpha}$-Galactosidase at pH 6.5, $4^{\circ}C$ and reaction for 24 hours was enough for complete removal of ${\alpha}$-Gal epitopes from the cell sur face on the immunostaining with Griffonia Simplicifolia isolectin B4. On the other hand, more ${\alpha}$-Gal epitopes were present in the pig pericardial tissue on Griffonia Simplicifolia isolectin B4 staining before the enzyme treatment, and 1.0 unit/mL of galactosidase was not sufficient for complete removal of ${\alpha}$-Gal from the tissue. 2.0 units/mL of green coffee bean ${\alpha}$-Galactosidase was needed to completely remove the ${\alpha}$-Gal epitopes from the pericardial tissue on immunostaining. Conclusion: The ${\alpha}$-Gal epitopes of the pig's aortic valve and pericardial tissue were successfully stained with Griffonia Simplicifolia isolectin B4. We could remove nearly all the ${\alpha}$-Gal epitopes using green coffee bean ${\alpha}$-Galactosidase at the concentration of 1.0 unit/mL in the aortic valve. Of pig, and 2.0 unit/mL was need to nearly completely remove all the ${\alpha}$-Gal epitopes in the pericardial tissue of pig under the condition of pH 6.5, $4^{\circ}C$ and 24 hours of reaction time. In the near future, removal of ${\alpha}$-Gal epitapes in the pig's aortic valve and pericardial tissue will become a powerful tool for the improvement of the tissue valve durability. It needs to be determined if ${\alpha}$-galactosidase treated pig tissue is immune to human anti-Gal antibody or anit-Gal mooclonal antibodies.

• Journal of The Korean Society of Inherited Metabolic disease
• /
• v.17 no.3
• /
• pp.92-95
• /
• 2017

Fabry disease (FD) is an X-linked lysosomal storage disorder caused by an ${\alpha}$-galactosidase A (GLA, MIM 300644) enzyme deficiency due to pathogenic variants in the ${\alpha}$-galactosidase A gene (GLA). The disease leads to accumulation of globotriaosylceramide (Gb3) and related glycophospholipids affecting nearly all major organ systems, with the primary sites damaged by Gb3 including renal glomeruli, myocardium, neurons of the dorsal ganglion and autonomic nervous system, and vascular endothelial and smooth muscle. Progressive deposition in these organ systems present with various clinical manifestations including acroparesthesia, renal failure and heart failure. Here, we report a Chinese male diagnosed with Fabry disease in his late $4^{th}$ decades showing improvement of acroparesthesia during enzyme replacement therapy (ERT). A 48-year-old Chinese man who presented with chronic recurrent severe burning pain in his fingers and toes since the age of 10, with worse involvement of the former visited to our clinic for further evaluation. His medical history included a transient ischemic attack aged 40 and diagnosed with stage 4-5 chronic kidney disease aged 47. In the family history, the patient's brother was found to be have Fabry disease 1 month before his visit. Except for his brother, all other members of the family are healthy. Based on his medical history and family history, he was strongly suspicious for Fabry disease. He was found to have a galactose-alpha-1,3-galactose level 4.96 (Reference range, 42.5-67.9) suggestive of Fabry disease. The followed sequencing of GLA coding region in our patient revealed hemizyosity for the mutation c.988C>T (Q330X) in Exon 7. Since ERT start, he showed significant improvement in his symptoms of burning sensation of fingers and toes. On the contrary, due to deteriorating kidney function even with ERT, he is considered for kidney transplantation. Despite of diagnostic delay until late 4th decades, ERT showed a potential improvement of acroparesthesia in our patient. However, late start of ERT can lead to poor outcome in multiorgan function. Therefore, early diagnosis with high index of suspicion followed by continuous ERT with regular monitoring have an impact on quality of life in Fabry disease.

• Journal of Chest Surgery
• /
• v.39 no.6 s.263
• /
• pp.427-433
• /
• 2006

Background: Current vascular prostheses are considered still inadequate for reconstruction of small-diameter vessels. To evaluate the potential use of xenograft vessels as small diameter arterial grafts, we implanted porcine vessels in goats. The grafts were treated with two different processes, freezing and acellularization, before implantation, and gross inspection as well as microscopic examination followed after a predetermined period. Material and Method: Bilateral porcine carotid arteries were harvested and immediately stored at $-70^{\circ}C$ within tissue preservation solution. One of them was designated as frozen xenograft vessel. The other one was put on acellularization process using NaCl-SDS solution and stored frozen until further use. Grafts were implanted in the place of carotid arteries of the same goat. The grafts have remained implanted for 1, 3, and 6 months in three animals, respectively. Periodic ultrasonographic examinations were performed during the observation period. After explantation, the grafts were analyzed grossly and histologically under light microscope. Result: All animals survived the experimental procedure without problems. Ultrasonographic examinations showed excellent patency of all the grafts during the observation period. Gross examination revealed nonthrombotic, patent lumens with smooth surfaces. Microscopic examinations of the explanted grafts showed cellular reconstruction at the 6-month stage in both grafts. Although more inflammatory responses were observed in the early phase of frozen xenografts, there was no evidence of significant rejection. Conclusion: These findings suggest that porcine xenograft vessels, regardless of pre-implantation processes of acelluarization or freezing, can be acceptably implanted in goats, although short duration of observation in a small number of animals may limit this study.

• Journal of Life Science
• /
• v.16 no.6
• /
• pp.1060-1065
• /
• 2006

Non-invasive evaluation of liver function in animal models remains a challenge. Hepatoscintigraphy provides information about changes in liver size and shape, and enables to understand general liver function. Futhermore it is readily used to diagnosis complications of liver transplantation like hepatitis, rejections and biliary complications. In this study, we investigated the usefulness of evaluating the liver function in miniature pigs with $^{99m}Tc-Tin$ colloid and $^{99m}Tc-DISIDA$ which are the most commonly used radiopharmaceuticals in human medicine. In result, $^{99m}Tc-Tin$ colloid was uptaked in lung, liver, gastric wall and kidney in miniature pigs. And $^{99m}Tc-DISIDA$ showed continuous uptake images of heart, lung, liver, gallbladder and duodenum, and it was similar to human's. Therefore we could conclude $^{99m}Tc-Tin$ colloid would not be suitable for evaluating hepatic function because of it's nonspecific affinity, however $^{99m}Tc-DISIDA$ scintigraphy would be an effective method for detecting hepatobiliary function in miniature pigs.

• KDI Journal of Economic Policy
• /
• v.12 no.1
• /
• pp.31-68
• /
• 1990

The concentration of economic power takes the form of one or a few firms controlling a substantial portion of the economic resources and means in a certain economic area. At the same time, to the extent that these firms are owned by a few individuals, resource allocation can be manipulated by them rather than by the impersonal market mechanism. This will impair allocative efficiency, run counter to a decentralized market system and hamper the equitable distribution of wealth. Viewed from the historical evolution of Western capitalism in general, the concentration of economic power is a paradox in that it is a product of the free market system itself. The economic principle of natural discrimination works so that a few big firms preempt scarce resources and market opportunities. Prominent historical examples include trusts in America, Konzern in Germany and Zaibatsu in Japan in the early twentieth century. In other words, the concentration of economic power is the outcome as well as the antithesis of free competition. As long as judgment of the economic system at large depends upon the value systems of individuals, therefore, the issue of how to evaluate the concentration of economic power will inevitably be tinged with ideology. We have witnessed several different approaches to this problem such as communism, fascism and revised capitalism, and the last one seems to be the only surviving alternative. The concentration of economic power in Korea can be summarily represented by the "jaebol," namely, the conglomerate business group, the majority of whose member firms are monopolistic or oligopolistic in their respective markets and are owned by particular individuals. The jaebol has many dimensions in its size, but to sketch its magnitude, the share of the jaebol in the manufacturing sector reached 37.3% in shipment and 17.6% in employment as of 1989. The concentration of economic power can be ascribed to a number of causes. In the early stages of economic development, when the market system is immature, entrepreneurship must fill the gap inherent in the market in addition to performing its customary managerial function. Entrepreneurship of this sort is a scarce resource and becomes even more valuable as the target rate of economic growth gets higher. Entrepreneurship can neither be readily obtained in the market nor exhausted despite repeated use. Because of these peculiarities, economic power is bound to be concentrated in the hands of a few entrepreneurs and their business groups. It goes without saying, however, that the issue of whether the full exercise of money-making entrepreneurship is compatible with social mores is a different matter entirely. The rapidity of the concentration of economic power can also be traced to the diversification of business groups. The transplantation of advanced technology oriented toward mass production tends to saturate the small domestic market quite early and allows a firm to expand into new markets by making use of excess capacity and of monopoly profits. One of the reasons why the jaebol issue has become so acute in Korea lies in the nature of the government-business relationship. The Korean government has set economic development as its foremost national goal and, since then, has intervened profoundly in the private sector. Since most strategic industries promoted by the government required a huge capacity in technology, capital and manpower, big firms were favored over smaller firms, and the benefits of industrial policy naturally accrued to large business groups. The concentration of economic power which occured along the way was, therefore, not necessarily a product of the market system. At the same time, the concentration of ownership in business groups has been left largely intact as they have customarily met capital requirements by means of debt. The real advantage enjoyed by large business groups lies in synergy due to multiplant and multiproduct production. Even these effects, however, cannot always be considered socially optimal, as they offer disadvantages to other independent firms-for example, by foreclosing their markets. Moreover their fictitious or artificial advantages only aggravate the popular perception that most business groups have accumulated their wealth at the expense of the general public and under the behest of the government. Since Korea stands now at the threshold of establishing a full-fledged market economy along with political democracy, the phenomenon called the concentration of economic power must be correctly understood and the roles of business groups must be accordingly redefined. In doing so, we would do better to take a closer look at Japan which has experienced a demise of family-controlled Zaibatsu and a success with business groups(Kigyoshudan) whose ownership is dispersed among many firms and ultimately among the general public. The Japanese case cannot be an ideal model, but at least it gives us a good point of departure in that the issue of ownership is at the heart of the matter. In setting the basic direction of public policy aimed at controlling the concentration of economic power, one must harmonize efficiency and equity. Firm size in itself is not a problem, if it is dictated by efficiency considerations and if the firm behaves competitively in the market. As long as entrepreneurship is required for continuous economic growth and there is a discrepancy in entrepreneurial capacity among individuals, a concentration of economic power is bound to take place to some degree. Hence, the most effective way of reducing the inefficiency of business groups may be to impose competitive pressure on their activities. Concurrently, unless the concentration of ownership in business groups is scaled down, the seed of social discontent will still remain. Nevertheless, the dispersion of ownership requires a number of preconditions and, consequently, we must make consistent, long-term efforts on many fronts. We can suggest a long list of policy measures specifically designed to control the concentration of economic power. Whatever the policy may be, however, its intended effects will not be fully realized unless business groups abide by the moral code expected of socially responsible entrepreneurs. This is especially true, since the root of the problem of the excessive concentration of economic power lies outside the issue of efficiency, in problems concerning distribution, equity, and social justice.