Shone's syndrome is a congenital cardiac malformation that consists of multiple levels of left heart obstruction including supravalvular mitral ring, congenital mitral stenosis(parachute mitral valve), subaortic stenosis, and coarctation of aorta. This syndrome is a very rare congenital anomaly and its prognosis is poor. We experienced 9 patients with Shone's syndrome between 1985 and 1994. There were 8 male and 1 female patients, and mean age was 33.0$\pm$31.0 months ranged from 2 months to 1 1 years. The congenital mitral, stenosis and coarctation of aorta existed in all patients and the supravalvular mitral ring and subaortic stenosis in 4 patients. Two patients had all four anatomic lesions. 3 patients underwent one stage total correction and the other 6 patients underwent two staged operation that was initial coarctoplasty with thoracotomy and later correction of intracardiac anomalies with median sternotomy. A third operation was performed in 2 patients. These procedures included reoperation for coarctation and replacement of mitral valve for persistent mitral stenosis. There was no operative death at the first operation but two operative deaths at the second operation. The cause of death in two cases was severe heart failure secondary to left ventricular hypoplasia. There was no operative death at the third operation. The seven survivors have beeli followed from 11 months to 12 years(mean follow-up 6.7 $\pm$ 3.6 years). There was no late death and the New York HeArt Association activity level was class I for all patients. We conclude that a food lone-term outcome can be expected by proper surgical treatment tailred to each individual's anatomy and pathophysiology although the operative mortality and morbidity of Shone's syndrome are high.
The advantages of mitral valve reconstruction have been well established and so mitral valve reconstruction is now considered as the procedure of choice to correct mitral valve disease. This is the report of intermediate-term results of 38 cases that performed mitral valve reconstruction for valve insufficiency(the total number of mitral valve reconstruction were 49 cases, but 11 cases that performed mitral valve replacement due to incomplete reconstruction were excluded). Material and Method : From March 1991 to March 2001, 38 patients underwent mitral valve repair due to mitral valve regurgitation with or without stenosis. Mean age was 47.6$\pm$14.7 years(range 15 to 70 years) : 11 were men and 27 were women. The causes of mitral valve regurgitation were degenerative in 14, rheumatic in 21, infective in 2 and the other was congenital. Result : According to the Carpentier's pathologic classification of mitral valve regurgitation, 3 were type 1 , 16 were type II and 19 were type III. Surgical procedures were annuloplasty 15, commissurotomy 19, leaflet resection and annular plication 9, chordae shortening 11, chordae transfer 5, new chordae formation 2, papillary muscle splitting 2 and vegetectomy 2. These procedures were combined in most patients. There were 2 early death and the causes of death were respiratory failure, renal failure and sepsis. There was no late death. Valve replacement was done in 6 patients after repair due to valve insufficiency or stenosis 3 weeks, 1, 3, 51, 69, 84months later respectively. These patients have been followed up from 1 to 116 months(mean 43.0 months). The mean functional class(NYHA) was 2.36 pre-operatively and improved to 1.70. Conclusion : In most cases of mitral valve regurgitation, mitral valve reconstruction when technically feasible is effective operation that can achieve stable functional results and low surgical and late mortality.
Background: Hypoplastic left heart syndrome is uniformly fatal if this condition is not properly treated. We reviewed the surgical results of treating hypoplastic left heart syndrome, and we evaluated the hemodynamics and functional status of these patients after they underwent a Fontan operation. Material and Method: To assess the surgical results, we retrospectively reviewed the medical records of 6 (M/F=4/2) patients who underwent a staged operation, including a Norwood procedure, a bidirectional Glenn procedure and a Fontan procedure between October 1997 to May 2005. The mean age of the patients was $17.3{\pm}10.8$ days (range: 9~36 days) at the $1^{st}$ staged operation, $8.9{\pm}7.1$ months (4.6~23.3 months) at the $2^{nd}$ staged operation (the Bidirectional Glenn procedure) and $32.4{\pm}9.8$ months at the final staged operation (the Fontan procedure). During the $2^{nd}$ staged operation, one of the patients received tricuspid valve repair due to regurgitation. All the patients underwent an extracardiac Fontan procedure using Gore-Tex conduit (20 mm: 2 patients, 18 mm: 4 patients) and one of them required fenestration. Result: 21 patients underwented a Norwood procedure. There were 7 early deaths and 4 interstage deaths. Bidirectional cavopulmonary shunt was performed in 10 patients and the Fontan procedure was done in 6 (mortality: 1 patient, Flow up loss: 1 patient, Awaiting a Fontan procedure: 2 patients). After the Fontan procedure, there was no complication except for one case of post operative bleedings. All the patients had good ventricular function and 2 had grade I tricuspid regurgitation, as noted on their echocardiography. The average follow up period after the Fontan procedure was $19.6{\pm}14.9$ months (range: 1.5~39.1 month). All the patients had normal sinus rhythm and they were put on aspirin and cardiac medication. During follow up period, all the patients had a good functional status (NYHA functional class I). Conclusion: All the patients who suffered with hypoplastic left heart syndrome and who underwent a Fontan procedure achieved a good hemodynamic and functional status, even though there was a relatively high operative mortality rate after stage I Norwood palliation. Therefore, thise staged operation should be strongly recommended as an important surgical strategy for treating hypoplastic left heart syndrome.
Purpose : The purpose of this study is to analyze the epidemiologic characteristics of sudden unexpected death in infancy and to evaluate the importance of postmortem autopsy. Methods : We reviewed, retrospectively, medical records of 34 infants admitted to Kangnam General Hospital from January 1987 to December 2001 because of sudden unexpected death. We investigated the cause of death through medical history, death scene examination, autopsy findings, acylcarnitine and organic acid analysis. Results : Among the total 34 infants, 18 were male(52.9%) and 16 were female(47.1%). Thirty infants(88%) were below the six months of age. Winter was the most affected season(38.2%). Eighteen infants(52.9%) died between 6 and 12AM. The prone sleeping position was observed more frequently than the supine position at death; nine cases in the prone position, six cases in the supine position. The cause of death of 23 cases could not be found by only history and death scene examination. Autopsy was done in 13 cases. Seven cases of them were thought to be SIDS. In six cases, we explained the cause of death with autosy findings. They were an endocardial fibroelastosis, a nesidioblastosis, a subdural hematoma, a bronchopneumonia and two fatty changes of liver. Metabolic screening tests performed in three cases to rule out metabolic disorder since 2000 were all normal. Conclusion : We concluded that autopsy and metabolic screening test should be performed to find out the cause of death in sudden unexpected death in infancy.
Shin, Mee Yong;Hwang, Jong Hee;Chung, Eun Hee;Moon, Jeong Hee;Lee, Ju Suk;Park, Yong Min;Ahn, Kang Mo;Lee, Sang Il
Clinical and Experimental Pediatrics
/
v.45
no.9
/
pp.1090-1096
/
2002
Purpose : We evaluated the clinical manifestations, bronchoscopic findings and therapeutic effects of flexible fiberoptic bronchoscopy in atelectasis of children. Methods : Sixty six children who received bronchoscopy due to persistent atelectasis, acute severe atelectasis and incidental atelectasis on plain chest radiography were studied retrospectively. Results : The most common causative underlying disease was pneumonia(60.4%). Other underlying conditions were pulmonary tuberculosis, chronic lung disease, postoperative state, bronchial asthma and chest trauma. The most common abnormal findings were inflammatory changes such as bronchial stenosis(n=15), mucosal edema and large amount of secretion(n=14), granulation tissue( n=3) and mucus plug(n=3) although 39.4% showed normal airways. Other findings were congenital airway anomalies, endobronchial tuberculosis, extrinsic compression and obstruction by blood clot. In 32 children with pneumonia-associated atelectasis, 43.7% revealed normal airways, and the most common abnormal findings were also inflammatory changes. Eighteen out of 39 patients who received therapeutic intervention such as suctioning of secretion, bronchial washing and intrabronchial administration of N-acetylcysteine($Mucomyst^{(R)}$) had complete or partial resolution of their atelectasis. In 32 patients with pneumonia-associated atelectasis, 56.5% showed improvement by therapeutic intervention. Conclusion : In this study, atelectasis was mainly associated with inflammatory airway diseases such as pneumonia. The most common abnormal bronchoscopic findings were inflammatory changes such as mucosal edema and large amounts of secretion and bronchial stenosis, although about 40% revealed normal airway. Flexible bronchoscopy is helpful for either diagnosis or treatment, especially in pneumonia-associated atelectasis.
Kim Young-Boong;Jeon Ki-Hong;Rho Jung-Hae;Kang Suk-Nam
Food Science of Animal Resources
/
v.25
no.4
/
pp.365-372
/
2005
This study was carried out to investigate the Physiochemical Properties of loin and rump in the native horse meat from Jeju. In the analysis of chemical composition of loin and rump, the result showed $72.2\%\;and\;73.8\%$ in moisture content $20.1\%\;and\;21.2\%$ in crude protein, $2.42\%\;and\;3.08\%$ in crude Int and $0.13\%\;and\;0.14\%$ in crude ash respectively. Glutamic acid was 3,275mg/100g and 3,577mg/100g in loin and rump each and it had highest result in amino acid analysis. K content was 388.0mg/100g which showed highest result in mineral analysis and next contents were P>Na>Mg>Ca. Oleic acid had highest result in fatty acid composition which were $62.64\%\;and\;63.77\%$ in loin and rump respectively. Cholesterol content of loin and rump were 43.25 and 43.57 mg/100g but showed no significant differences to the part. pH of loin and rump were 5.60 and 5.75 which had no significant differences. Loin had Higher result than that of rump with no significant differences in WHC and springiness of texture analysis. Redness of rump was higher than that of loin. In the sensory evaluation, there were significant differences in the color and odor. Loin had higher result than that of rump in the overall palatability but showed no significant differences. With the result of this experiment native horse meat from Jeju could be understood as good meat resources.
The purpose of this study is to analyze the human rights found in the North Korean Constitution and their core problem by focusing on elements of human rights suggested by Daesoon Jinrihoe's doctrine of Haewon-sangsaeng (解冤相生 the Resolution of Grievances for Mutual Beneficence). Haewon-sangsaeng is seemingly the only natural law that could resolve human resentment lingering from the Mutual Contention of the Former World while leading humans work for the betterment of one another. Haewon-sangsaeng, as a natural law, includes the right to life, the right to autonomous decision-making, and duty to act according to human dignity (physical freedom, the freedom of conscience, freedom of religion, freedom of speech, freedom of press, etc.), the right to equal treatment in one's social environment, and the right to ensure the highest level of health through treatment. The North Korean Constitution does not have a character as an institutional device to guarantee natural human rights, the fundamental principle of the Constitution, and stipulates the right of revolutionary warriors to defend dictators and dictatorships. The right to life is specified so that an individual's life belongs to the life of the group according to their socio-political theory of life. Rights to freedom are stipulated to prioritize group interests over individual interests in accordance with the principle of collectivism. The right to equality and the right to health justify discrimination through class discrimination. The right to life provided to North Koreans is not guaranteed due to the death penalty system found within the North Korean Criminal Code and the Criminal Code Supplementary Provisions. The North Korean regime deprives North Koreans of their right to die with dignity through public executions. The North Korean regime places due process under the direction of the Korea Worker's Party, recognizes religion as superstition or opium, and the Korea Worker's Party acknowledge the freedoms of bodily autonomy, religion, media, or press. North Koreans are classified according to their status, and their rights to equality are not guaranteed because they are forced to live a pre-modern lifestyle according to the patriarchal order. In addition, health rights are not guaranteed due biased availability selection and accessibility in the medical field as well as the frequent shortages of free treatments.
Objective : Toll like receptor(TLR) is known to be involved in innate immunity. Many microbial antigens stimulate TLR, and as a result of intracellular signal transduction, they activate nuclear factor-kB which produces diverse inflammtory cytokines. Until now, many research topics in Kawasaki disease focused on cytokine increasement. In this study, we aim to reveal TLR increasement which might be associated with initiation of inflammatory response. Methods : We obtained the peripheral blood of ten patients who were diagnosed with Kawasaki disease in Yonsei University College of Medicine from March 2003 to August 2003, as well as those of a febrile control group and the same number of a normal control group. Flow cytometry was done in all samples for quantification of TLR-2 expression in CD14 positive monocyte. And we also extracted total RNA of periphral monocyte and quantificated expression of TLR-2 mRNA by RT-PCR. Results : The expression of TLR-2 in Kawasaki disease increased significantly compared with the normal control group but not when compared with the febrile control group. And the expression decreased slightly in the subacute phase of Kawasaki disease compared with the acute phase, but this was statistically insignificant. mRNA expression of TLR-2 in peripheral blood monocyte also increased in the acute phase of Kawasaki disease. Conclusion : Expression of TLR-2 in Kawasaki disease increased when compared with the normal control group, which means that innate immunity is associated with the pathogenesis of Kawasaki disease.
This study was practiced to provide basic data which is necessary for ethical education of dental hygiene students by comparing the bioethical perception between dental hygiene students and non-health students. Questionnaire surveys were conducted for 778 students in two universities in Gyeonggi-Do province and analyzed the collected data with SAS 9.2. The findings of the study were as follows : 1. there was no stastistical difference in the degree of bioethical perception between dental hygiene students and non-health students. 2. Students who have family member in medical field were more conscious of bioethics than students who doesn't. According to religion, the protestants were most conscious and no-religions scored the lowest. 3. In case of dental hygiene students, female was more conscious. According to religion, there were statistical differences between the Catholic and Buddhism, the Catholic and No-religion. 4. Dental hygiene students were more strongly opposed to let infant with fatal congenital disease die than non-health students. 5. Dental hygiene students were more strongly opposed to rejecting organ transplant, trade in human organs and using alternative organ of animal. Also, they estimated higher the possibility of medical irrationalities due to trade in human organs. From these results, a curriculum for bioethical education in department of dental hygiene should be developed to form and improve the more desirable bioethical perception of dental hygiene students.
A ketone body (acetoacetic acid, β-hydroxybutyric acid, and acetone) increases from blood or urine when bio-energy dependence pays more fatty acid than glucose. However, in case oxidation of fat is greater than the capacity of the citric acid cycle the fatty acid oxidation is made from acetoacetyl CoA to acetoacetate then, again form β-hydroxyburytic acid to acetone, the diffusion take place into the blood. Enzymes that oxidize ketone body in the brain and nerve tissue blood ketone dody is increased during prolonged fasting, brain used it as energy. In this study, we developed the rapid two step derivatization method for sensitive detection of the ketone body by GC-MS/SIM. The plasma was deproteinized and then the hydroxy and carboxyl groups of ketone body are subjected to extraction and drying then, keto-group were derivatized with hydoxylamine at 60℃ for 30 min for oximation. Then it was trimetyl-silylated with BSTFA at 80℃ for 30 min and analyzed using a GC-MS. The linear ranges were in between 0.001 μg/mL and 250 μg/mL for β-hydroxy butyrate, and acetoacetate. The method detection limits were below 0.1 pg over each target compound determined. The mean recoveries (%) of target compounds were ranged from 88.2 % to 92.3 % at 1 µg/mL, from 89.5 % to 94.8 % at 10 μg/mL, with RSD of 6.3-9.4 %. This method could be applied to quantification of ketone bodies which are seen in the keto-acidosis in children and adults from a variety of diseases that cause ketones in the blood and urine.
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