• Investigative Magnetic Resonance Imaging
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• 제17권3호
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• pp.200-206
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• 2013

목적: 자기공명 심장영상을 이용한 좌심실 파라미터 측정에 있어, 자동화 방식을 적용하였을 경우에 나타나는 효과를 분석하고 이를 수동 및 반자동 방식을 적용했을 경우 나타나는 결과와 비교하였다. 대상과 방법: 1.5T 자기공명 심장영상 촬영을 시행한 62명의 환자를 대상으로, 심장 주기당 20상의 단축 항정상태 자유세차 동영상과 심첨2방 및 심첨4방 영상을 얻었다. 심내막 경계와 심외막 경계를 수동, 자동, 반자동 방식으로 각각 구하여 이를 바탕으로 이완말기와 수축말기 용적, 박출 계수, 일회 박출량, 좌심실 질량을 계산하고 각 방식간 평균값 차이를 일원분산분석법을 이용 통계적 분석하였다. 결과: 이완말기와 수축말기 용적의 경우에는 세 방식으로 측정한 결과는 통계적으로 유의하게 다르지 않았다. (P = .399 and .145). 그러나, 박출 계수, 일회 박출량, 좌심실 질량의 경우에는 통계적으로 유의하게 다르게 나타났으며 (P=.001, < 001, < 001) 자동화 방식으로 측정한 측정치가 다른 두 방식에 비해 일관되게 큰 결과치를 보였다. 결론: 자동화 방식을 적용하여 측정한 좌심실의 박출 계수, 일회 박출량, 좌심실 질량의 측정치는 수동, 반자동 방식에 비해 과장된 값을 나타낸다. 자동화 방식으로 많은 노력을 절감할 수 있으나, 임상적으로 민감한 케이스에 대해서는 이에 더하여 수동적 교정을 고려해야 할 것이다.

• Journal of Chest Surgery
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• 제44권2호
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• pp.131-136
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• 2011

Background: We evaluated the surgical results and predictors of long-term survival in patients who underwent coronary artery bypass grafting (CABG) at the time of an aortic valve replacement (AVR) due to aortic stenosis. Materials and Methods: Between January 1990 and December 2009, 183 consecutive patients underwent CABG and concomitant aortic valve replacement for aortic stenosis. The mean follow-up period was $59.8{\pm}3.3$ months and follow-up was possible in 98.3% of cases. Predictors of mortality were determined by Cox regression analysis. Results: There were 5 (2.7%) in-hospital deaths. Follow-up of the in-hospital survivors documented late survival rates of 91.5%, 74.8%, and 59.6% at 1, 5, and 10 postoperative years, respectively. Age (p<0.001), a glomerular filtration rate (GFR) less than 60 mL/min (p=0.006), and left ventricular (LV) mass (p<0.001) were significant predictors of mortality in the multivariate analysis. Conclusion: The surgical results and long-term survival of aortic valve replacement with concomitant CABG in patients with aortic stenosis and coronary artery disease were acceptable. Age, a GFR less than 60 mL/min, and LV mass were significant predictors of mortality.

• Journal of Chest Surgery
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• 제36권9호
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• pp.651-658
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• 2003

On-X 기계판막은 1997년 처음으로 사용된 이후 전 세계적으로 사용빈도가 증가하고 있다. 본 연구는 이 새로운 기계판막에 대한 유용성과 안전성, 그리고 술 후 혈역학적 변화에 대해 알아보고자 시행되었다. 대상 및 방법: 1999년 4월부터 2002년 8월까지 전남대학교병원 흉부외과에서 On-X 판막을 사용하여 대동맥판막 치환술을 시행했던 52명의 환자를 대상으로 분석하였다. 환자 중 52%가 남자였으며 평균연령은 50$\pm$13세였다. 연구는 판막수술 후 결과보고에 대한 AATS/STS 지침에 따라 분석하였다. 술 전 뉴욕 심장병학회의 기능분류상 class III 이상의 환자가 32명(61.5%)이였으며, 2명의 환자는 이전에 대동맥판막 수술을 받았던 환자였다. 71.1%의 환자에서 동반된 질환에 의해 대동맥판막 치환술과 함께 동반 수술을 시행하였고, 이식된 판막의 크기는 19 mm 13명, 21 mm 26명, 23 mm 10명과 25 mm 3명이었다 평균 외래 추적관찰 기간은 16.6$\pm$10.5 (1∼39)개월이었다. 수술 전과 수술 직후 그리고 3, 6, 12개월에 심초음파를 반복 시행하여 술 후 혈역학적 변화양상을 분석하였다. 결과: 평균 체외순환시간과 대동맥 차단시간은 각각 191$\pm$94.7분과 142$\pm$51.7분이었다. 술 후 조기 및 만기 사망은 없었다. 술 후 12개월간 event-free rate는 혈전색전증 95.6$\pm$6%; 출혈성 합병증 90.2$\pm$4%: 판막 주위누출 92.3$\pm$4%이었으며 전체적인 event-free rate는 76.6$\pm$3%이었다. 수술 후 판막혈전이나 심내막염, 구조적 판막부전은 발생하지 않았다. 술 후 12개월의 평균심박출량은 62.7$\pm$9.8%로 수술 전(55.8$\pm$15.9%)에 비해 의미 있는 증가를 보였다(p=0.006). 좌심실 체적지수와 최대 판막압력차는 수술 전에 247.3$\pm$122.3 g/$\textrm{cm}^2$, 62.5$\pm$38.0 mmHg을 보였으나 술 후 12개월에 각각 155.5$\pm$58.2 g/$\textrm{cm}^2$(p=0.002), 18.0$\pm$10.8 mmHg (p<0.0001)로 감소하는 소견을 보였다. 결론: On-X 기계판막을 이용한 대동맥판막 치환술은 임상적 및 혈역학적으로 만족스러운 조기 결과를 보여주었다. 그러나 대상 환자들에 대한 장기간의 외래 추적관찰과 함께 기존 판막과의 비교가 이루어짐으로써 이 새로운 판막에 대한 유용성과 안정성이 확립될 수 있을 것으로 생각한다.

• 대한한의학회지
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• 제23권3호
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• pp.1-10
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• 2002

Objective: There exist gender differences in pulsatile contour waveform. Women have a greater age-related increase in left ventricular mass than do men and more likely to experience symptomatic heart failure after infarction. SDPTG (the second derivative of photoplethysmogram waveform) is a noninvasive method for evaluating the pulse wave and is correlated with age and other risk factors for atherosclerosis. We studied the effect of gender on SDPTG and made clear why the gender differences appear. Methods: To study the effects of effect factors, including height and blood pressure, on SDPTG in the fourth decade, data on height, weight, PTD (pulse transit distance), blood pressure, serum lipid levels, and SDPTG were collected in 115 laboratory healthy men and women. SDPTG is derived from double-differential processing of fingertip photoplethysmography and consists of a, b, c, and d waves in systole and an e wave in diastole; SDPTG aging index (AI) was calculated as (b-c-d-e)/a. Results: There were significant gender-related differences of SDPTG AI, height, and blood pressure. Age, height, and mean blood pressure were respectively and significantly correlated with SDPTG AI. SDPTG is dependent upon age, height, and blood pressure. Restricting analysis to SDPTG AI, age, height, and mean blood pressure, yielded that there were gender-related differences in SDPTG AI (P<0.05) which were derived from those of height (F<0.001, df=l, P=0.994). Conclusions: These new data may help to explain previous findings about age-related differences in pulsatile contour waveforms and why gender differences of SDPTG appear. The results of this study suggest that SDPTG AI, used for evaluation of biological vascular aging, should be calibrated by height as well as age and blood pressure.

• 대한유전성대사질환학회지
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• 제14권1호
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• pp.37-41
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• 2014

Fabry disease is an X-linked disease caused by deficiency of the lysosomal enzyme alpha-galactosidase A. Affected males present anhydrosis, acroparesthesia and angiokeratoma, and subsequently cardiac, cerebral and renal complications are followed. Females and atypical variants show heterogeneous clinical symptoms. In 2001, two recombinant enzymes were approved for Fabry disease: agalsidase alpha and agalsidase beta. Since the introduction of enzyme replacement therapy (ERT), the number of long-term follow-up studies has been reported. Long-term ERT showed effectiveness on renal function in patients with chronic kidney disease, decrease or stabilization of left ventricular mass, and improvement of pain and quality of life. However, there were limited effects on cerebrovascular events and their mortality. Current literatures on the clinical effect of ERT have reported limited datain adult patients who have already advanced disease. Therefore, further study for pre-symptomatic patients and atypical variants is needed to verify the impact of ERT. This review summarized recent progresses in ERT and limitations of long-term effect of ERT in patients with Fabry disease.

• Childhood Kidney Diseases
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• 제24권2호
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• pp.107-114
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• 2020

Purpose: The purpose of this study was to evaluate the prevalence, clinical characteristics, and long-term clinical effects of hypertension in Korean childhood-onset systemic lupus erythematous (SLE) patients. Methods: The medical records of SLE patients, diagnosed by 2019 SLE European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria, who visited Samsung Medical Center from January 2009 to May 2019 were reviewed. Disease activity and long-term damage were evaluated using the Modified Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and the Pediatric Systemic Lupus International Collaborating Clinics/ACR Damage Index (Ped-SDI), respectively. The sex-, age- and height-blood pressure standards recommended by the American Academy of Pediatrics 2017 guideline was used to define hypertension. Results: A total of 32 patients were enrolled in this study. The median follow-up duration was 7.3 years and females were predominant. The median ages at SLE and hypertension diagnoses were 14.2 and 14.3 years, respectively. The biopsy-proven lupus nephritis was detected in 90.6% and 37.5% were class IV. During the follow-up, 12 patients (37.5%) had hypertension. Among them, 2 patients had 3 episodes of posterior reversible encephalopathy syndrome and 5 patients had left ventricular hypertrophy (LVH). Univariate analysis showed baseline hypertension was significantly correlated with a lower estimated glomerular filtration rate, higher body mass index and SLEDAI at baseline. The development of hypertension during the follow-up was significantly correlated with obesity, LVH, and higher Ped-SDI. Conclusion: Our study revealed that hypertension in pediatric SLE is associated with obesity and renal function at SLE diagnosis and could affect long-term damage.

• 한국임상수의학회지
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• 제36권3호
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• pp.159-165
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• 2019

Pimobendan is an inodilator used to treat canine heart failure, and pentoxifylline is reported to be beneficial for microcirculation and heart disease. The purpose of this study was to evaluate the pharmacokinetic and pharmacodynamic profiles of a novel pimobendan-pentoxifylline liquid mixture after oral administration to dogs. Eight healthy Beagle dogs were included in the study. The dogs were divided into the control group (orally administered water; n = 4) and experimental group (orally administered pimobendan-pentoxifylline liquid mixture [pimobendan 0.25 mg/kg, pentoxifylline 15 mg/kg]; n = 4). Plasma samples were obtained and echocardiographic indices were measured for 24 hours after administration. The concentrations of pimobendan and pentoxifylline were quantified by using a liquid chromatography-mass spectrometer (LC-MS). The elimination half-life ($T_{1/2}$) was $32.96{\pm}9.80mins$ for pimobendan and $29.49{\pm}6.67mins$ for pentoxifylline. The time to reach maximum concentration ($T_{max}$) were $52.50{\pm}31.22mins$ for pimobendan and $41.25{\pm}18.87mins$ for pentoxifylline. The maximum blood concentration ($C_{max}$) was $96.92{\pm}75.64ng/mL$ for pimobendan and $7074.07{\pm}3261.1ng/mL$ for pentoxifylline. Of the echocardiographic indices, fractional shortening (FS) and left ventricular internal diameter at end systole (LVIDs) were significantly altered at 1-3 hours after the administration of pimobendan-pentoxifylline liquid mixture. The pimobendan-pentoxifylline liquid mixture was well tolerated by the dogs, with no adverse effects observed during the study.

• Journal of Genetic Medicine
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• 제19권1호
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• pp.32-37
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• 2022

Mucopolysaccharidosis type VI (MPS VI) is an autosomal recessive lysosomal disorder caused by the deficiency of arylsulfatase B due to mutations in the ARSB gene. Here, we report the case of a Korean female with a novel variant of MPS VI. A Korean female aged 5 years and 8 months, who is the only child of a healthy non-consanguineous Korean couple, presented at our hospital for severe short stature. She had a medical history of umbilical hernia and recurrent otitis media. Her symptoms included snoring and mouth breathing. Subtle dysmorphic features, including mild coarse face, joint contracture, hepatomegaly, and limited range of joint motion, were identified. Radiography revealed deformities, suggesting skeletal dysplasia. Growth hormone (GH) provocation tests revealed complete GH deficiency. Targeted exome sequencing revealed compound heterozygous mutations in the ARSB genes c.512G>A (p.Gly171Asp; a pathogenic variant inherited from her father) and c.1157C>T (p.Ser386Phe; a novel variant inherited from her mother in familial genetic testing). Quantitative tests revealed increased urine glycosaminoglycan (GAG) levels and decreased enzyme activity of arylsulfatase B. While on enzyme replacement therapy and GH therapy, her height increased drastically; her coarse face, joint contracture, snoring, and obstructive sleep apnea improved; urine GAG decreased; and left ventricular mass index was remarkably decreased. We report a novel variant-c.1157C>T (p.Ser386Phe)-of the ARSB gene in a patient with MPS VI; these findings will expand our knowledge of its clinical spectrum and molecular mechanisms.

• Journal of Chest Surgery
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• 제57권3호
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• pp.255-262
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• 2024

Background: Sutureless aortic valves may enable shorter procedure times, which benefits patients with elevated surgical risk. We describe the outcomes of patients with aortic stenosis who underwent aortic valve replacement (AVR) using the sutureless Perceval aortic bioprosthesis. Methods: Data from a retrospective cohort were obtained from a clinical database. The study enrolled patients with symptomatic severe aortic stenosis who underwent surgical AVR with a sutureless bioprosthesis between August 2015 and December 2020. In total, 113 patients were included (mean age, 75.3±8.4 years; 57.5% women; median Society of Thoracic Surgeons score, 9.7%; mean follow-up period, 51.19±20.6 months). Of these patients, 41 were octogenarians (36.2%) and 3 were nonagenarians (2.6%). Transthoracic echocardiography was employed to assess changes in ejection fraction (EF), left ventricular mass index (LVMI), and mean pressure gradient (MPG). Results: The in-hospital mortality rate was 2.6%, and 13 patients developed new-onset atrial fibrillation. A permanent pacemaker was implanted in 3 patients (2.6%). The median intensive care unit stay was 1 day (interquartile range [IQR], 1-2 days), and the median hospital stay was 12 days (IQR, 9.5-15 days). The overall survival rate at 5 years was 95.9%. LVMI and MPG were reduced postoperatively, while EF increased over the follow-up period. No structural valve deterioration was observed, and no meaningful paravalvular leakage developed during follow-up. Conclusion: The use of a sutureless valve in the aortic position is safe and feasible, even for high-risk elderly patients requiring surgical AVR. LVMI and MPG decreased postoperatively, while EF increased over the follow-up period.

• Journal of Chest Surgery
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• 제41권4호
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• pp.439-446
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• 2008

배경: 본 연구의 목적은 좌심실 수축기능, 용적, 질량과 QT dispersion간의 관계를 알아보고, 관상동맥 협착이 있는 환자와 없는 환자에서 비교하는 것이다. 대상 및 방법: 174명의 ST분절 및 심근효소 이상소견이 없는 전형적인 안정성 협심증을 호소하는 환자들을 대상으로 하였다. Group I은 심혈관 촬영상 관상동맥 협착이 50%이상(n=101), Group II는 심혈관 촬영상 관상동맥 협착이 50%이하(n=73)인 환자로 나누었다. 심혈관 촬영 전에 좌심실 구출율, 용적, 질량을 심초음파로 측정하였고 QT dispersion측정을 위해 12-lead 심전도를 측정하였다. 결과: QT dispersion은 Group I에서 Group II보다 유의하게 길었다(39.8 ms vs. 33.3 ms; p<0.05). 모든 환자에서 좌심실용적, 질량은 QT dispersion과 통계학적으로 유의한 상관관계가 있었으며, 좌심실 질량은 유일한 독립적 관계요소였다.(p<0.05). 그런데 Group I에서만 보면 초음파상에 나타난 어떤 결과도 QT dispersion과 유의한 상관 관계가 없었으며, Group II에서는 좌심실 용적, 질량은 QT dispersion과 유의한 상관관계가 있었으며, 좌심실 질량은 여전히 독립적 관계 요소였다(p<0.05). 결론: 우리의 연구는 좌심실용적, 질량 등은 안정성 협심증환자에서 QT dispersion과 유의한 상관관계를 나타냈다. 이런 소견들은 저명한 관상동맥 협착이 없는 환자들에서만 나타난다.