• Title/Summary/Keyword: 3 Valve

Search Result 2,019, Processing Time 0.031 seconds

Estimation of Groundwater Table using Ground Penetration Radar (GPR) in a Sand Tank Model and at an Alluvial Field Site (실내 모형과 현장 충적층에서 지하투과레이더를 이용한 지하수면 추정)

  • Kim, Byung-Woo;Kim, Hyoung-Soo;Choi, Doo-Houng;Koh, Yong-Kwon
    • The Journal of Engineering Geology
    • /
    • v.23 no.3
    • /
    • pp.201-216
    • /
    • 2013
  • Ground penetrating radar (GPR) surveys were conducted in a sand tank model in a laboratory and at an alluvial field site to detect the groundwater table and to investigate the influence of saturation on GPR response in the unsaturated zone. In the sand tank model, the groundwater table and saturation in the sand layer were altered by injecting water, which was then drained by a valve inserted into the bottom of the tank. GPR vertical reflection profile (VRP) data were obtained in the sand tank model for rising and lowering of the groundwater table to estimate the groundwater table and saturation. Results of the lab-scale model provide information on the sensitivity of GPR signals to changes in the water content and in the groundwater table. GPR wave velocities in the vadose zone are controlled mainly by variations in water content (increased travel time is interpreted as an increase in saturation). At the field site, VRP data were collected to a depth of 220 m to estimate the groundwater table at an alluvial site near the Nakdong river at Iryong-ri, Haman-gun, South Korea. Results of the field survey indicate that under saturated conditions, the first reflector of the GPR is indicative of the capillary fringe and not the actual groundwater table. To measure the groundwater table more accurately, we performed a GPR survey using the common mid-point (CMP) method in the vicinity of well-3, and sunk a well to check the groundwater table. The resultant CMP data revealed reflective events from the capillary fringe and groundwater table showing hyperbolic patterns. The normal moveout correction was applied to evaluate the velocity of the GPR, which improved the accuracy of saturation and groundwater table information at depth. The GPR results show that the saturation information, including the groundwater table, is useful in assessing the hydrogeologic properties of the vadose zone in the field.

20 Years Surgical Experiences for Ebstein's Anomaly (엡스타인 기형의 20년 수술 치험)

  • Lee, Sak;Park, Han-Ki;Lee, Chang-Young;Chang, Byung-Chul;Park, Young-Hwan
    • Journal of Chest Surgery
    • /
    • v.40 no.4 s.273
    • /
    • pp.280-287
    • /
    • 2007
  • Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

Surgical Treatment of Anomalous Origin of Coronary Artery from the Pulmonary Artery: Postoperative Changes of Ventricular Dimensions and Mitral Regurgitation (관상동맥-폐동맥 이상기시증(Anomalous Origin of Coronary Artery from Pulmonary Artery)의 수술적 치료: 중기 성적과 좌심실 및 승모판 기능의 변화 양상에 대한 연구)

  • Kang, Chang-Hyun;Kim, Woong-Han;Seo, Hong-Joo;Kim, Jae-Hyun;Lee, Cheul;Chang, Yoon-Hee;Hwang, Seong-Wook;Back, Man-Jong;Oh, Sam-Se;Na, Chan-Young;Han, Jae-Jin;Lee, Young-Tak;Kim, Chong-Whan
    • Journal of Chest Surgery
    • /
    • v.37 no.1
    • /
    • pp.19-26
    • /
    • 2004
  • Background: The aims of this study are to verify the result of the surgical treatment of ALCAPA and to identify the postoperative changes of left ventricular dimensions and mitral regurgitation (MR), Material and Method: Fifteen patients operated on since 1985 were included in the study. The patients operated on before 1998 (n=9) showed heterogeneous properties with various surgical strategies and cardiopulmonary bypass techniques. However, six patients were operated on with the established surgical strategy since 1998; 1) Dual perfusion and dual cardioplegic solution delivery through ascending aorta and main pulmonary artery, 2) Coronary transfer by rolled-conduit made of pulmonary artery wall flap, and 3) Additional mitral valvular procedure was not peformed. Result: Median age of the study group was 6 months (1 month to 34 years). The operative methods were left subclavian artery to left coronary artery anastomosis in 1, simple ligation in 2, Takeuchi operation in 2, and coronary reimplantation in 10 patients. The mean follow up period was 5.5<5.8 years (2 months 14 years), There were one early death (6.7%) and one late death. Overall 5-year survival rate was 85.6$\pm$9.6%. The Z-value of left ventricular end-diastolic and end-systolic dimensions were 6.4$\pm$3.0 and 5.1 $\pm$3.6 preoperatively, and decreased to 1.7$\pm$ 1.9 and 0.8$\pm$ 1.6 in 3 months (p<0.05). Significant preoperative MR was identified in 6 patients (40%) and all the patients showed immediate improvement of MR within f month postoperatively. There were 3 cases of reoperation due to coronary anastomosis site stenosis and recurrence of MR. However, there was no mortality nor late reoperation in the patients operated on after 1998. Conclusion: The surgical treatment of ALCAPA showed favorable survival and early recovery of ventricular dimensions and mitral valvular function. Although long-term reintervention was required in some cases of earlier period, all the cases after 1998 showed excellent surgical outcome without long-term problem.

Effects of Percutaneous Balloon Mitral Valvuloplasty on Static Lung Function and Exercise Performance (승모판협착증 환자에서 경피적 풍선확장판막성형술의 폐기능 및 운동부하 검사에 대한 효과)

  • Kim, Yong-Tae;Kim, Woo-Sung;Lim, Chae-Man;Chin, Jae-Yong;Koh, Youn-Suck;Kim, Jae-Joong;Park, Seong-Wook;Park, Seung-Jung;Lee, Jong-Koo;Kim, Won-Dong
    • Tuberculosis and Respiratory Diseases
    • /
    • v.41 no.1
    • /
    • pp.1-10
    • /
    • 1994
  • Background: Patients with mitral stenosis(MS) have been demonstrated to have a variable degree of pulmonary dysfunction and exercise impairment. The hemodynamic changes of MS can be reversed after percutaneous mitral balloon valvuloplasty(PMV), but the extent and time course of the imporvement in pulmonary function and exercise capacity are not defined. Methods: In order to investigate the early(3 weeks or less)and late(3 months or more) effects of PMV on pulmonary function and determine if the pulmonary dysfunction is reversible even in patients with moderate to severe pulmonary hypertension, we performed the spirometry, measurements of diffusing capacity and lung volumes, and incremental exercise tests in patients with MS before and after PMV. Results: In 46 patients with MS(age: $40{\pm}12$years, male to female ratio: 1:2, mitral valve area: $0.8{\pm}0.2cm^2$) there was a significant increase in FVC(P<0.0025), $FEV_1$(P<0.001), $FEF_{25-75%}$(P<0.001, $FEF_{50%}$(P<0.001), PEF(P<0.0005), MVV(P<0.005), $\dot{V}O_2$max (P<0.0001), and AT(P<0.0001) after average 10 days of PMV. Also there was a significant decrease in DLco(P<0.0001) and DL/VA(P<0.0001). At later($5{\pm}2$months) follow-up in 11 patients, there was no further improvement in any parameters of pulmonary function and exercise test. Twenty nine patients with sinus rhythm were divided into 16 patients with pulmonary arterial pressure(PAP) more than 35mmHg and/or tricuspid regurgitation grade n or more(group A) and 13 patients with PAP less than 35mmHg(group B). Group A Patients had significantly lower FVC(P<0.001), $FEV_1$(P<0.001), DLco(P<0.05), $\dot{V}O_2$ max(P<0.025) and mitral valve area(P<0.025) than group B patients. Group A patients after PMV, showed significant increase in FVC(P<0.001), maximum $O_2$ pulse(P<0.00001) and $\dot{V}O_2$ max(P<0.00025). Both group showed an increase in AT(P<0.0001, P<0.005), but group A showed greater decrease in $\dot{V}E/\dot{V}O_2$ and $\dot{V}E/\dot{V}CO_2$ both at AT(P<0.001, P<0.001) and $\dot{V}O_2$ max(P<0.0001, P<0.0001) after PMV compared with group B. Conclusion: These data suggest that patients with MS can show increased pulmonary function and exercise performance within 1 month after PMV. Patients with moderate to severe pulmonary hypertension had a significant increase in exercise performance compared with those with mild to no pulmonary hypertension and it is thought to be related to a significat decrease of ventilation for a given oxygen consumption at maximum exercise.

  • PDF

The study of Intercostal Nerve Block and Patient-Controlled Analgesia for Post-Thoracotomy Pain (개흉술 후 동통억제에 대한 신경차단법 및 환자자가 치료법의 비교연구)

  • 김우종;이길노
    • Journal of Chest Surgery
    • /
    • v.30 no.9
    • /
    • pp.920-926
    • /
    • 1997
  • Remarkable effect of pain relief and prevention of the postoperative Complications after thoracotomy has been achieved by continuous intravenous analgesia. This study was carried out with thirty patients who underwent posterolateral thoraco tony. The patients were divided into three groups: Group I(n= 10), the patients with intermittent intramuscular analgesia(piroxicam 20 mg), Group II(n=10), the patients with continuous epidural analgesia(0.5% bupivacaine 30m1 + normal saline 30 ml + morphine 10 mg), and Group III(n= 10) the patients with controlled intravenous infusion of analgesics(fentanyl 2500 mfg +normal saline 10 ml). The results w re as follows; 1) There were no significant changes of vital signs, between groups. 2) Tidal volume and FVC were significantly improved in the group II and III compared with the group I during the first postoperative day. 3) A significant reduction of immediate post-thoracotomy pain was achieved in the group II and III compared with the group I. 4) The limitation of motion in the operative side was less in the group II and III compared with the group I. 5) A signi(icant reduction of the postoperative analgegics consumption was noticed in group II and III. 6) Significant complications were not occured during follow-up period in all groups.

  • PDF

Coronary Artery Bypass Surgery with Radial Artery -Early Results (요골동맥을 이용한 관상동맥우회술 -조기성적)

  • 나찬영;이영탁;박국양;이해영;김욱성;박?현;홍민수;심재천;권오춘
    • Journal of Chest Surgery
    • /
    • v.30 no.3
    • /
    • pp.275-281
    • /
    • 1997
  • The radial artery as a graft for myocardial revascularization was introduced by Carpentier in the early 1970s. Mid-term results were unfortunately discoura ing, and the clinical experience with this graft was interrupted. At the end of the 1980s, these authors reproposed the same arterial conduit with more satisfying results, because of improved technique and pharmacological management of the graft. Between October 1994 and July 1995, 36 patients underwent myocardial revascularization with a radial artery graft in Seiong General Hospital. Left internal mammary artery was concomitantly used as a pedicled Vift in 34 patients. Fifteen patients (42%) had a complete arterial waft revascularization. A total of 12) distal anastomoses were performed (average 3.4 per patient), including 36 left internal mammary artery wafts (two sequential in 2 patients), and 23 saphenous vein grafts. The remaining 64 distal anastomoses were perFormed with radial artery grafts (mean 1.8 per patient). The radial arteries were anastomosed to the circumflex (n=38), diagonal (n= 18), right coronary(n=G), and left anterior descending coronary artery(n=2). The percent ge of radial artery graft anastomoses (64) to the total anastomoses(123) was 52%. The radial artery was used as a single graft in 10 patients, as a sequential graft in 25 patients, and two grafts in 1 patient. Twenty patients underwent associated procedures coronary endarterectomy (14), coronary artery patch angioplasty (4), mitral valve repair (1), and repair of ventricular septal rupture (1). One patient died of low cardiac output syndrome and the others had no perioperative myocardial infarction. There are no ischemic and functional complications in the arm or hand aftcr removal of the radial artery. Only 1 patient required reexploration of the am, for the hematoma evacuation, and 2 patients complained transient thumb dysesthesia of the side of the havested arm. This dysesthesia improved within one month. Postoperative angiovaphic controls were obtained in 11 patients(31%) postoperative 79 to 210 days (mean 126 days). The patency rate were as follows : left internal mammary artery (100%), saphcnous vein (100%), and radial artery(95%). We concluded that the radial artery is useful alternative graft, but long term clinical and angiographic studies are required to derterminc whether wider application is warranted.

  • PDF

Effect of Simple VSD Repair on Doppler-Derived Right Ventricular Systolic Time Interval (심실중격결손 봉합이 우심실 수축기 시간 간격에 미치는 영향)

  • 정태은;이영환
    • Journal of Chest Surgery
    • /
    • v.32 no.2
    • /
    • pp.124-129
    • /
    • 1999
  • Background: Ventricular septal defect(VSD) that causes pulmonary hypertension increase right ventricular workload. Echocardiographic assessment of right ventricular systolic time interval (RVSTI) has been used to predict pulmonary artery pressure in various cardiopulmonary diseases. This study was undertaken in infants with simple VSD to observe the alteration of the right ventricular workload through the changes of RVSTI after repair of VSD. Material and Method: We evaluated heart rate, the ratio of the left atrium/aortic root diameter (LA/Ao), right ventricular pre-ejection period(RVPEP), right ventricular ejection time(RVET), and its ratio(RVPEP/RVET) as a predictor of right ventricular workload in 12 children with simple VSD. These were measured three times at the preoperative period, at the 3 month and between 6 month and 1 year(average 9.5${\pm}$1.8month) after repair of VSD by M-mode & Doppler echocardiograph from the pulmonic valve echogram. Result: Heart rate was decreased significantly after repair(137.1${\pm}$13.7 vs 114.4${\pm}$21.1 and 104.1${\pm}$10.2, p<0.01). LA/Ao ratio was decreased significantly after repair(1.71${\pm}$0.32 vs 1.47${\pm}$0.33 and 1.39${\pm}$0.23, p<0.05). RVPEP/RVET were decreased after repair (0.38${\pm}$0.09 vs 0.32${\pm}$0.08 and 0.29${\pm}$0.09, p<0.01). Heart rate corrected RVPEP/RVET were significantly decreased only after 6 months(0.32${\pm}$0.03 vs 0.30${\pm}$0.05 and 0.28${\pm}$0.06, p<0.05). Conclusion: We found elevated right ventricular workload was progressively decreased until more than 6 months after repair and the RVSTI may serve a useful guide in postoperative care for children with VSD.

  • PDF

Clinical Experience of the Surgical Treatment of Cardiac Tumor (심장 종양의 수술적인 치료의 임상적 고찰)

  • Bang, Jung-Hee;Woo, Jong-Soo;Choi, Pill-Jo;Cho, Gwang-Jo;Kim, Si-Ho;Park, Kwon-Jae
    • Journal of Chest Surgery
    • /
    • v.43 no.4
    • /
    • pp.375-380
    • /
    • 2010
  • Background: Primary cardiac tumors are extremely rare. The most common type are benign myxomas, and these are almost completely curable with early surgery. Malignant tumors, however, such as sarcomas, are difficult to remove surgically, and their prognosis is known to be poor. In this study, data on patients who had undergone surgical treatment of cardiac tumor in the authors' hospital were collected and analyzed. Material and Method: The subjects included 28 patients who had undergone surgical treatment of cardiac tumor from August 1993 to December 2008. Their medical records were reviewed and retrospectively analyzed. Result: The patients were aged from 20 to 76 years (mean age: $54.2{\pm}15.6$), and 11 were male (39%) and 17 female (61%). Fifteen of them (54%) underwent emergency surgery to improve heart failure symptoms. The most common preoperative symptom was dyspnea (15 cases, 54%). Preoperative echocardiography was performed on all the patients. The average size of the tumor as measured during the operation was $7.0{\pm}6.9cm$ (the average length of the long axis was 2∼40 cm), and the sites of tumor attachment were the interatrial septum (18 cases, 64%), the left atrium (9 cases, 32%), the mitral valve annulus (2 cases, 7%), and the left ventricle (2 cases, 7%). The operation was performed with an incision through both atria in all the patients, and a complete excision was made in 25 cases (89%). According to the biopsy results, there were 4 cases of sarcoma (14%), 1 case of lipoma (4%), and 23 cases of myxoma (82%). The three cases in which the tumors were not completely excised were sarcomas. No operative deaths occurred after the operations. Outpatient follow-up was possible for 24 cases (86%), with a mean follow-up period of $46.8{\pm}42.7$ months. Late death occurred in 3 of the 24 patients; each of these patients had sarcomas. Of these patients, the first had undergone two repeat surgeries, the second had metastatic sites removed, and the last had only chemotherapy. The average recurrence time was $12.7{\pm}10.8$ months, and the average metastasis time was $20.5{\pm}16.8$ months. Conclusion: Most cardiac tumors are benign myxomas. In principle, they should be surgically treated because they can create risks such as embolism, and can be radically treated when surgically removed. In most cases, however, malignant sarcomas are already considerably advanced with severe infiltration into the neighboring tissues at the time of diagnosis. The surgical removal of malignant sarcomas is known to be difficult because of the advanced stage and degree of infiltration. We suggest that excision of the removable portion of the tumor sites to alleviate symptoms such as heart failure can improve quality of life.

Study on Folk Caring in Korea for Cultural Nursing (문화간호를 위한 한국인의 민간 돌봄에 대한 연구 : 출생을 중심으로)

  • 고성희;조명옥;최영희;강신표
    • Journal of Korean Academy of Nursing
    • /
    • v.20 no.3
    • /
    • pp.430-458
    • /
    • 1990
  • Care is a central concept of nursing. Nursing would not exist without caring. Care and quality of life are closely related. Human behavior is a manifestation of culture. We can say that caring and nursing care are expression of culture. The nurse must understand the relationship of culture with care for ensure quality nursing care. But knowledge of cultural factors in nursing is not well developed. Time and in - depth study are needed to find meaningful relationships between culture and care. Nurses recognized the importance of culturally appropriate nursing There are two care systems in culturally based nursing. The folk care system and the professional nursing care system. The folk care system existed long before the professional nursing care system was introduced into this culture. If the discrepancy between these two care systems is great, the client may receive inappropriate nursing care. Culture and subcaltures are diverse and dynamic in nature. Nurses need to know the caring behaviors, patterns, and their meaning in their own culture. In Korea we have taken some first step to study cultural nursing phenomena. It is not our intent necessarily to return to the past and develop a nationalistic of nursing, but to identify the core of traditional caring and relate that to professional nursing care. Our Assumptions are as follows : 1) Care is essential for human growth, well being and survial. 2) 7here are diverse and universal forma, expressions, patterns, and processes of human care that exist transcul - turally. 3) The behaviors and functions of caring differ according to the social structure of each culture. 4) Cultures have folk and professional care values, beliefs, and practices. To promote the quality of nursing care we must understand the folk care value, beliefs, and practices. We undertook this study to understand caring in our traditional culture. The Goals of this study were as follows : 1) To identify patterns in caring behavior, 2) To identify the structural components of caring, and 3) To understand the meaning and some principles of caring. We faised several questions in this study. Who is the care-giver? Who is the care-receipient? Was the woman the major care -giver at any time? What are the patterns in caring behavior? What art the priciples underlying the caring process? We used an interdisciplinary team approach, composed of representatives from nursing and anthropology, to contribute in -depth understanding of caring through a socicaltural perspeetive. A Field study was conducted in Ro-Bong, a small agricultural kinship village. The subjects were nine women and one man aged be or more years of age. Data were collected from january 15 to 21, 1990 through opem-ended in-depth interviews and observations. The interview focused on caring behaviors sorrounding birth, aging, death and child rearing. We analysed these data for meaning, pattern and priciples of caring. In this report we describe caring behaviors surrounding childbirth. The care-givers were primarily mothers- in -low, other women in the family older than the mother - to- be, older neighbor woman, husbands, and mothers of the mother-to- be. The care receivers were the mother-to-be the baby, and the immediate family as a component of kinship. Emerging caring behavior included praying, helping proscribing, giving moral advice(Deug - Dam), showing concern, instructing, protecting, making preparations, showing consideration, touching, trusting, encouraging, giving emotional comfort, being with, worrying about, being patient, preventing problems, showing by an example, looking after bringing up, taking care of postnatal health, streng thening the health condition, entering into another's feelings(empathizing), and sharing food, joy and sorrow The emerging caring component were affection, touching, nurtuing, teaching, praying, comforting, encouraging, sharing. empathizing, self - discipline, protecting, preparing, helping and compassion. Emerging principles of. caring were solidarity, heir- archzeal relationships, sex - role distinction. Caring during birth expresses the valve of life and reflects the valued traditional beliefs that human birth is given by god and a unique unifying family event reaching back to include the ancestors and foreward to later generations. In addition, We found positive and rational foundations for traditionl caring behaviors surrounding birth, these should not be stigmatized as inational or superstitious. The nurse appropriately adopts the rational and positive nature of traditional caring behaviors to promote the quality of nursing care.

  • PDF

Clinical Features, Molecular Analysis, and Outcome of ERT in Korean Patients with Mucopolysaccharidosis Type VI (국내 6형 뮤코다당증의 임상 양상, 분자유전학적 특징 및 효소치료의 효과에 대한 고찰)

  • Wichajarn, Khunton;Kim, Jinsup;Yang, Aram;Sohn, Young Bae;Lee, Beom Hee;Yoo, Han-Wook;Cho, Sung Yoon;Jin, Dong-Kyu
    • Journal of The Korean Society of Inherited Metabolic disease
    • /
    • v.16 no.1
    • /
    • pp.24-33
    • /
    • 2016
  • Mucopolysaccharidosis type VI (MPS VI) is a rare disease caused by the mutation of ARSB with prevalence range from 1/5,000 in northeast Brazil to 1/2,057,529 births in Czech Republic. In Asia, there is only one published figure in Taiwan of about 1/833,000 births. The exact prevalence in the Korean population is unknown, but we estimated the incidence of MPS VI is about 0.03/100,000 live births. Enzyme replacement therapy (ERT) with recombinant human Arylsulfatase B (rhASB) is a modality for the treatment of MPS VI that reduces the excretion of urine glycosaminoglycan (GAG) and improves joint motion, pulmonary function, and endurance. We presented the clinical features, molecular analysis and outcome of ERT in three Korean MPS VI patients. All patients had the typical characteristic clinical features of MPS IV. Short stature, dysostosis multiplex, corneal opacity and valvular heart disease were found at first presentation, while restrictive lung disease and carpal tunnel syndrome developed later in all patients. Molecular analysis demonstrated novel missense and nonsense mutation in the patients, including p.Ile 67Ser, p.Gly328Arg, $p.Arg191^*$, p.Asp352Asn, and p.Gly17Asp. After ERT, urine GAG was decreased in all patients. Skeletal involvement, corneal opacity, heart valve abnormalities and pulmonary function were not improved with ERT, but it had a better outcome on regarding joint motion and endurance. One patient underwent allogeneic bone marrow transplantation (BMT) prior to ERT, but their clinical response was not improved much after BMT. This study demonstrates clinical phenotypes and molecular analysis of the severe form of MPS VI in Korean patients.

  • PDF