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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
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    • v.40 no.4 s.273
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    • pp.280-287
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    • 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.

Executive Dysfunction and It's Relation to K-WAIS Scores in Mild Traumatic Brain Injury Patients with Normal Intelligence Quotient (정상 지능의 경도 외상성 뇌손상 환자에서 실행기능 장애 및 K-WAIS 점수들과의 관련성)

  • Lee, Dae-Bo;Yoon, In-Seon;Kim, Seon-Kyung;Rho, Seung-Ho;Park, Min-Cheol;Lee, Sang-Yeol
    • Korean Journal of Psychosomatic Medicine
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    • v.20 no.1
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    • pp.50-58
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    • 2012
  • Objectives : This study examined the selective deficits of executive function in patients with mild traumatic brain injury that in normal range of general intelligence level and aimed to analysis of the correlation between K-WAIS result and executive function. Methods : 59 subjects were included in this study, who were diagnosed as mild traumatic brain injury(MTBI) and they have visited in neuropsychiatric department of Wonkwang University Hospital during from March, 2005 to September, 2010. For measurement of general intelligence quotient, the Korean-Wechsler Adults Intelligence Scale(K-WAIS) was administered and for measurement of executive intelligence quotient(EIQ), Executive Intelligence Test(EXIT) was administered. Results : Of patients, 50.8% included at abnormal EIQ group. The patients of abnormal EIQ showed poorer full scale IQ(FIQ), performance IQ(PIQ) and in subscale that picture arrangement, digit symbol, digit span, block design, object assembly and comprehension were significantly different. In terms of relationships between K-WAIS and EIQ, FIQ and PIQ have positive correlation with EIQ. And in subscale, picture arrangement, digit symbol, digit span, block design, object assembly and comprehension show positive correlation with EIQ. Conclusion : This study suggest that MTBI patients with have normal range of general intelligence level may have deficit of executive function is common. The decline of FIQ, PIQ and some subscales of K-WAIS may suggest executive dysfunction in MTBI patients.

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Surgical Treatment of Aneurysm of the Sinus of Valsalva Eight Year Experience (발살바동 동맥류의 외과적 치료)

  • 오상준;유완준
    • Journal of Chest Surgery
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    • v.29 no.9
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    • pp.983-988
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    • 1996
  • Aneurysm of the sinus of Valsalva is fairly rare. Between 1987 and 1994, we operated on a total of 12 cases of aneurysm of the sinus of Valsalva at the Inje University Seoul Paik Hospital. This represents 0.7% of all cardiac operation under cardiopulmonary bypass. There were eight male and four female patients aged from 8 to 38 years(mean, 19.2 years). 8 of 12 patients had ruptured aneurysms. The origin of aneurysm of the sinus of Valsalva was the right coronary sinus in 10(83. 3%), and the noncoronary sinus in 2(16.6%). In ruptured aneurysm, the origin was the right oronary sinus in 6, and the noncoronary sinus in 2. The aneurysms originating from the right coronary sinus ruptured into the right ventricle in 5, and into both the right atrium and right ventricle in 1. The aneurysms originating from the noncoronary sinus ruptured into the right ventricle in 1, and into the right atrium in 1. Associated congenital cardiac defects included ventricular septal defect in 10(83.3%) patients 39 cases of these were associated with the aneurysms of the right coronary sinus), aortic regurgi- tation in 3 (all of these had an additional ventricular septal defect), mitral regurgitation in 1, and double chambered right ventricle in 1. No hospital deaths occurred, although one late death occurred as a result of endocarditis 15 months after the first operation. The mean follow-up period was 29 months, range from 4 to 60 months. Eleven patients except one late death were in New York Heart Association class 1. Due to the low mortality risk o( an operation for aneur sm of the sinus of Valsalva, a ruptured aneurysm of the sinus of Valsalva should be corrected surgically when the diagnosis is mane, and unruptured aneurysm of the sinus of Valsava with complication should also be operated. In most cases the aorta was opened to examine the morphology of the aneurysm and the aortic cusps, and an associated aortic valve defect should be corrected simultaneously.

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Analysis of domain required for aggregates formation of ALS (Amyotrophic lateral sclerosis)/FTD (Frontotemporal dementia)-linked FUS in mammalian cells (루게릭병 및 전측두엽성 치매 연관 단백질 Fused in Sarcoma (FUS)의 스트레스 응집체 형성에 관여하는 도메인 분석)

  • Jun, Mi-Hee;Lee, Jin-A
    • Analytical Science and Technology
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    • v.28 no.5
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    • pp.331-340
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    • 2015
  • Mutations in Fused in Sarcoma (FUS) have been identified in patients with amyotrophic lateral sclerosis (ALS) or Frontotemporal Dementia (FTD). Pathological FUS is mis-localized to cytosol and forms aggregates associated with stress granules (SG), while FUS is normally localized to nucleus. However, it is largely unknown how pathological FUS forms SG-aggregates and which domains are responsible for this process. In this study, we examined cellular localization and aggregation of ALS-linked FUS missense mutants (P525L, R521C, R521H, R521G), analyzed the domains responsible for cytosolic FUS aggregation in HEK293T cells, and confirmed this in cultured mouse neurons. To do this, we firstly generated missense mutants of FUS and then examined their cellular localization. We found that P525L was mostly mis-localized to cytosol and formed FUS-positive SG aggregates while R521C, R521H, or R521G was localized to both nucleus and cytosol. To further characterize the domains required for aggregate formation of cytosolic FUS, we generated different domain-deletion mutants using FUS-∆17 which has a deletion of nuclear localization signal. Interestingly, cytosolic FUS without SYGQ and RGG1 domain or cytosolic FUS without RGG2-ZnF-RGG3 domain did not form FUS-positive SG aggregates, while cytosolic FUS without RRM domain generated more aggregates compared to FUS-∆17. Taken together, these data suggest that SYGQ-RGG1 or RGG2-ZnF-RGG3 domain contributes to formation of cytosolic aggregate, while RRM domain might interfere with FUS aggregation. Therefore, our studies will provide important insight for understanding cellular pathogenesis of neurodegeneration associated with FUS aggregate as well as finding therapeutic targets for ALS or FTD.

Mid-term Results of Neonatal Surgical Management of Pulmonary Atresia with Intact Ventricular Septum (온전한 심실 중격을 가진 폐동맥 폐쇄증의 신생아기 수술 치료의 중기 성적)

  • Kwak Jae Gun;Kim Woong-Han;Kim Dong Jin;Lee Chang Ha;Lee Jeong Ryul;Kim Yong Jin;Rho Joon Ryang
    • Journal of Chest Surgery
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    • v.38 no.12 s.257
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    • pp.815-820
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    • 2005
  • Background: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. Material and Method: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. Result: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8$\pm$1.50 (-3.2$\∼$1.2) and -2.5$\∼$0.5 (-3$\∼$ -2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states. Conclusion: Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.

Surgical Results and Risk Facor Analysis of the Patients with Single Ventricle Associated with Total Anomalous Pulmonary Venous Connection (총폐정맥연결이상증을 동반한 단심증 환아의 수술결과 및 위험인자 분석)

  • 이정렬;김창영;김홍관;이정상;김용진;노준량
    • Journal of Chest Surgery
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    • v.35 no.12
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    • pp.862-870
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    • 2002
  • The surgical results of the patients with single ventricle(SV) associated with total anomalous pulmonary venous connection(TAPVC) has been reported with high mortality and morbidity due to their morphologic and hemodynamic complexity. A retrospective review was undertaken to report the outcome of the first-stage palliative surgery in our institution and to determine the factors influencing early death. Material and Method: Between January 1987 and June 2002, 39 patients with SV and TAPVC underwent surgical intervention with or without TAPVC repair. Age at operation ranged from 1day to 10.7months (median age, 2.4month), and 29 patients were male. Preoperative diagnosis included 20 right-dominant SV, 15 SV with endocardial cushion defect, 3 left-dominant SV, and 1 tricuspid atresia. The pulmonary venous connection was supracardiac in 22, cardiac in 5, infracardiac in 11, and mixed in 1, Obstructed TAPVC was present in 11. First-stage palliative surgery was performed in 37. Repair of TAPVC, either alone or in association with other procedures, was performed during the initial operation in 31. Univariate and multivariate analyses were performed to analyze the risk factors influencing the operative death. Result: A mean follow-up period of survivors was 34.3 $\pm$ 43.0(0.53 ~ 146.2)months. Overall early operative mortality was 43.6%(17/39). The causes were low cardiac output in 8, failure of weaning from cardiopulmonary bypass in 3, sepsis in 2, pulmonary hypertensive crisis in 1, pulmonary edema in 1, pneumonia in 1, and postoperative arrhythmia in 1. Risk factors influencing early death in univariate analysis were body weight, surgical intervention in neonate, obstructive TAPVC, preoperative conditions including metabolic acidosis, and need for inotropic support, TAPVC repair in initial operation, operative time, and cardiopulmonary bypass(CPB) time. In multivariable analysis, body weight, age at initial operation, surgical intervention in neonate, preoperative conditions including metabolic acidosis, need for inotropic support and CPB time were the risk factors. Conclusion: In this study, we demonstrated that the patients with SV and TAPVC had high perioperative mortality. Preoperative poor condition, young age, the length of operative and CPB time, the presence of obstructive TAPVC had been proven to be the risk factors. This fact suggests that the avoidance of unnecessarily additional procedures may improve the surgical outcomes of the first-stage palliative surgery. However further observation and collection of the data is mandatory to determine the ideal surgical strategy.

Application of Bicuspidalized Cryopreserved Allograft Valves for the RVOT Reconstruction in Complex Cardiac Anomalies (복잡 심장기형 환자에서 우심실 유출로 재건술시 이첨판화 냉동보전 동종이식편의 적용에 관한 연구)

  • 오삼세;지현근
    • Journal of Chest Surgery
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    • v.30 no.3
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    • pp.270-274
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    • 1997
  • In recent years, the use of allograft conduits in repair of congenital cardiac disease is widely accepted. However, the supply of homograft. is currently limiting their increased clinical application, especially small cryopreserved homografts for use in neonates and inf'ants. We used a technique to surgically reduce the size of the more readily available large-diameter allografts, making them suitable for right ventricular outflow tract reconstruction in small infants and children. From December 1994 to March 1996, a total of 11 patients ranging in age from 10 months to 6 years (mean age, 27.3 months) and ranging in weight from 5.6 to 18.5 kg (mean 11.5 kg) underwent reconstruction of the right ventricular outflow tract using this surgical technique (pulmo ary atresia with ventricular septal defect, 9 cases ; tetralogy of Falloff, 2 cases). The diameter after downsizing ranged from 14 to 19 mm with a mean of 16.8 mm. There was one operative death due to rupture of the infected homograft. Evaluation of these patients between 2 and 15 months (mean 6.9 months) after homograft implantation reveals excellent clinical and echocardiographic results. There were no significant homograft insufficiency and RVOT obstructions. Although a longer follow-up is certainly required to evaluate the long term fate of the surgically modified bicuspid homografts, we believe that this technique may represent a valuable therapeutic alternative, at least in the short term, to the use of synthetic grafts when an appropriately sized homograft is not available.

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A Study on Oral Health Status of Industrial Workers Gumi City (구미시 일부 근로자의 구강건강실태 조사연구)

  • Kim, Hye-Jin;Bae, Soo-Myung;Choi, Ok-Sun
    • Journal of dental hygiene science
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    • v.5 no.4
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    • pp.205-209
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    • 2005
  • The number of workers in the study were 831 in their academic backgrounds are 53.6% workers who graduated from high schools 80.7% of the subjects replied that their economic state belonged to the middle class. 55.7% brushed their teeth at least three times a day and 58.7% of them brushed their teeth after they had dinner. In questions about living habits, 68.4% were found to drink alcohol, among whom 55.7% had more than one bottle of So-ju. As well, 57.7% of the subjects smoked, among whom 34.8% smoked half the pack or one whole pack of cigarettes a day and 30.0% had smoked more than 10 years. The result of oral examination showed that 25.9% had more than 1 decayed tooth, 14.8% had their teeth missing, 42.2% suffered periodontal diseases, and 11.7% had problems with wisdom teeth.

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Analysis of Gliotransmitters in ADHD Mice (ADHD (주의력결핍 과잉행동장애) 생쥐 모델에서의 별아교세포 유래 신경전달물질 분석)

  • Kim, Ga-Yeon;Park, Jaewon;Yoon, Bo-Eun
    • Journal of Life Science
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    • v.28 no.5
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    • pp.597-604
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    • 2018
  • Although the core mechanisms of Attention Deficit/Hyperactivity Disorder (ADHD) are unknown, several ADHD-associated proteins have been studied. G-protein - coupled receptor kinase interacting protein-1 (GIT1) is a multifunctional adapter protein that affects neuron growth and dendrite formation. GIT1-deficient mice have shown ADHD-like behavior and also recovered through amphetamine treatment. In this study, gliotransmitters were investigated in both intracellular and extracellular space from GIT1-deficient mice. To measure the amount of gliotransmitters, primary astrocyte cultures were taken from the cerebral and cerebellar cortices of wild (WT), hetero (HE), and knock-out (KO) mice. Major gliotransmitters were analyzed using high-performance liquid chromatography. It was observed that the amount of excitatory and inhibitory gliotransmitters were dependent on genotype and showed a change in excitation/inhibition ratios. Interestingly, the major excitatory gliotransmitter, glutamate, existed at the lowest level in WT mice, but the amount of inhibitory gliotransmitters, gamma-aminobutyric acid (GABA) and glycine, varied depending on brain region. Remarkably, an increased amount of GABA was measured at the intracellular cerebrum in WT mice compared with KO mice. It was presumed that KO mice would secrete more inhibitory gliotransmitters to compensate for GIT1 depletion or else acquire a defect to reuptake-secreted GABA. This may be a possible mechanism for ADHD pathology.

A Potent Tissue Destructive Activity of Secreted Proteins of Aeromonas hydrophila (조직 괴사 활성을 지닌 Aeromonas hydrophila 의 분비 단백질에 관한 연구)

  • Kim, Kyu Lee;Choe, Yunjeong;Kang, Ho Young
    • Journal of Life Science
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    • v.25 no.2
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    • pp.214-222
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    • 2015
  • Aeromonas hydrophila is the most common water fish pathogen and cause diseases such as hemorrhagic septicemia, dropsy, ulceration and asymptomatic septicemia. A. hydrophila secretes many extracellular products (ECPs) which contribute to effective infection, wide distribution and great adaptability to environmental changes. Crude ECPs of A. hydrophila CK257, a strain used in this study, exhibits a toxic activity to the animals including mouse, rabbit and fish. Toxic symptoms were indicated by tissue damage and skin injuries in animal. When ECPs were subcutaneously injected to animals, skin damages were observed, appearing like necrosis. Preliminary research demonstrated that the active factors are protein component. The crude ECPs were collected after ammonium sulfate precipitation of cell-free culture supernatant. ECPs were fractionated with the use gel filtration chromatography. Five ECP fractions were obtained, of which one fraction was found to be toxic to goldfish. MALDI-TOF analyses provided two interesting proteases called M35 and M28. Both M35 and M28 are known as metalloprotease. Accordingly, proteins in an active fraction exhibited caseinolytic activity. These proteins were difference of caseinolytic activity under different metallic ions. Also active fraction has elastolytic activity. These results suggested that peptidase M28 and M35 may be a candidate factor for tissue necrosis activity about infection with A. hydrophila.