• Title/Summary/Keyword: Cardiac diseases

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The Role of Enzyme Replacement Therapy in Fabry Disease in Cardiology Perspective

  • Hongo, Kenichi
    • Journal of mucopolysaccharidosis and rare diseases
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    • v.4 no.1
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    • pp.21-25
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    • 2018
  • Fabry disease is a hereditary lysosomal storage disorder caused by the reduction or absence of lysosomal enzyme alpha-galactosidase A and the accumulation of glycosphingolipids, such as globotriaosylceramide (Gb3), in various organs, including the heart. The prevention of cardiac involvement in Fabry disease can only be achieved by enzyme replacement therapy (ERT), and the method of assessing the efficacy of ERT should be confirmed. Changes in the electrocardiogram, such as the shortening of PQ interval, prolongation of QTc and repolarization abnormalities as well as left ventricular hypertrophy in voltage criteria, can be used to identify Fabry disease patients; however, the usefulness of electrocardiograms for evaluating the efficacy of ERT is limited. The assessment of left ventricular hypertrophy using echocardiography has been established to evaluate the efficacy of ERT during long-term period. A new technique involving speckled tracking method might be useful for detecting early cardiac dysfunction and identifying the effect of ERT for a relatively short period. The estimation of left ventricular hypertrophy using cardiac magnetic resonance (CMR) is also useful for assessing the efficacy of ERT. Identifying late gadolinium enhancement in CMR may affect the effectiveness of ERT, and the new technique of T1 mapping might be useful for monitoring the accumulation of Gb3 during ERT. Histopathology in cardiac biopsy specimens is another potentially useful method for identifying the accumulation of GB3; however, the use of histopathology to evaluate of the efficacy of ERT is limited because of the invasive nature of an endomyocardial biopsy.

Cardiopulmonary Resuscitation: New Concept

  • Lee, Kwang-Ha
    • Tuberculosis and Respiratory Diseases
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    • v.72 no.5
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    • pp.401-408
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    • 2012
  • Cardiopulmonary resuscitation (CPR) is a series of life-saving actions that improve the chances of survival, following cardiac arrest. Successful resuscitation, following cardiac arrest, requires an integrated set of coordinated actions represented by the links in the Chain of Survival. The links include the following: immediate recognition of cardiac arrest and activation of the emergency response system, early CPR with an emphasis on chest compressions, rapid defibrillation, effective advanced life support, and integrated post-cardiac arrest care. The newest development in the CPR guideline is a change in the basic life support sequence of steps from "A-B-C" (Airway, Breathing, Chest compressions) to "C-A-B" (Chest compressions, Airway, Breathing) for adults. Also, "Hands-Only (compression only) CPR" is emphasized for the untrained lay rescuer. On the basis of the strength of the available evidence, there was unanimous support for continuous emphasis on high-quality CPR with compressions of adequate rate and depth, which allows for complete chest recoil, minimizing interruptions in chest compressions and avoiding excessive ventilation. High-quality CPR is the cornerstone of a system of care that can optimize outcomes beyond return of spontaneous circulation (ROSC). There is an increased emphasis on physiologic monitoring to optimize CPR quality, and to detect ROSC. A comprehensive, structured, integrated, multidisciplinary system of care should be implemented in a consistent manner for the treatment of post-cardiac arrest care patients. The return to a prior quality and functional state of health is the ultimate goal of a resuscitation system of care.

Variation in clinical usefulness of biomarkers of acute kidney injury in young children undergoing cardiac surgery

  • Baek, Hee Sun;Lee, Youngok;Jang, Hea Min;Cho, Joonyong;Hyun, Myung Chul;Kim, Yeo Hyang;Hwang, Su-Kyeong;Cho, Min Hyun
    • Clinical and Experimental Pediatrics
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    • v.63 no.4
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    • pp.151-156
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    • 2020
  • Background: Acute kidney injury (AKI) is one of the most significant postoperative complications of pediatric cardiac surgery. Because serum creatinine has limitations as a diagnostic marker of AKI, new biomarkers including neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), and interleukin-18 (IL-18) are being evaluated to overcome these limitations and detect AKI at an early stage after cardiac surgery. Purpose: This study aimed to investigate the clinical usefulness of these biomarkers in young children. Methods: Thirty patients with congenital heart diseases who underwent cardiac surgery using cardiopulmonary bypass (CPB) were selected, and their urine and blood samples were collected at baseline and 6, 24, and 48 hours after surgery. Serum creatinine and blood urea nitrogen levels as well as NGAL, KIM-1, and IL-18 levels in urine samples were measured, and clinical parameters were evaluated. Results: Of the 30 patients, 12 developed AKI within 48 hours after cardiac surgery. In the AKI group, 8 of 12 (66.6%) met AKI criteria after 24 hours, and urine KIM-1/creatinine (Cr) level (with adjustment of urine creatinine) peaked at 24 hours with significant difference from baseline level. Additionally, urine KIM-1/Cr level in the AKI group was significantly higher than in the non-AKI group at 6 hours. However, urine NGAL/Cr and IL-18/Cr levels showed no specific trend with time for 48 hours after cardiac surgery. Conclusion: It is suggested that urine KIM-1/Cr concentration could be considered a good biomarker for early AKI prediction after open cardiac surgery using CPB in young children with congenital heart diseases.

Analysis of clinical characteristics and causes of chest pain in children and adolescents

  • Chun, Ji Hye;Kim, Tae Hyeong;Han, Mi Young;Kim, Na Yeon;Yoon, Kyung Lim
    • Clinical and Experimental Pediatrics
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    • v.58 no.11
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    • pp.440-445
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    • 2015
  • Purpose: Chest pain is common in children and adolescents and is a reason for referral to pediatric cardiologists. Although most cases of chest pain in these age groups are benign and do not require treatment, timely diagnosis is important not to miss life-threatening diseases requiring prompt treatment. We investigated certain clinical characteristics that may be useful in the diagnosis of such critical diseases. Methods: Patient medical records between July 2006 and September 2013 were retrospectively examined. We included 517 patients who presented with chest pain to the Department of Pediatrics at Kyung Hee University Hospital in Gangdong. Results: Most cases of chest pain were idiopathic in origin (73.6%), followed by cases with respiratory (9.3%), musculoskeletal (8.8%), cardiac (3.8%), gastrointestinal (2.9%), and psychiatric (1.4%) causes. In 6 patients (1.2%) with air-leak syndrome including pneumothorax or pneumomediastinum, the pain was abrupt, continuous, and lasted for a short period of 1-2 days after onset in the older adolescents. Of the patients with cardiac pain, 13 had cardiac arrhythmias (65.0%), 6 had congenital heart diseases (30%), and 1 had coronary aneurysms caused by Kawasaki disease (5.0%). One patient with atrial flutter had only symptoms of syncope and chest pain. Conclusion: The abrupt, continuous chest pain of a short duration in the older children was characteristic of air-leak syndrome. In patients with pneumomediastinum, radiological diagnosis was difficult without careful examination. Combined syncope should not be neglected and further cardiac workup is essential in such patients.

Cardiac Differentiation of Chicken Spermatogonial Stem Cells-A Directional Approach

  • Sodhi, Simrinder Singh;Jeong, Dong Kee
    • Reproductive and Developmental Biology
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    • v.38 no.4
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    • pp.137-142
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    • 2014
  • A tremendous increase in the human population has put poultry industry under an increased pressure to meet steep increase in the demand. Poultry is contributing 25% of the total world's meat production and lesser cost of investment per bird makes it more suitable for the further breeding programmes. Major poultry diseases frequently lead to cardiac damage and cause huge economic losses to poultry industry due to mortality. The in vitro embryonic stem cell (ESC) technology has a futuristic approach for homogeneous populace of differentiated cells, for their further transplantations. During in vitro conditions the differentiated cell populace can be used in grafting and transplantation processes to regenerate damaged tissues. Therefore, the current study targeted the use of spermatogonial stem cells (SSCs) in the poultry production system through cardiac regeneration. The current study will also open new boulevard for the similar kind of research in other livestock species for the management of heart diseases.

Reoperation after Open Heart Surgery -Clinical analysis of 27 cases- (개심술후 재수술에 대한 임상적 고찰)

  • 서의수
    • Journal of Chest Surgery
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    • v.22 no.1
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    • pp.50-58
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    • 1989
  • The emergence and expansion of cardiac surgery over the past decade has resulted in an increasing number of patients undergoing cardiac operations but many kinds of heart surgery was realized only palliative, resulting in increasing numbers of secondary cardiac procedures. From 1978 to 1988, 10 cases of various congenital heart diseases and 17 cases of acquired heart diseases were reoperated at Hanyang University Hospital. The leading indication of second operation was residual shunt or valvular malfunction due to technical failure in congenital heart disease and primary valve failure, endocarditis, paravalvular leakage were for acquired heart disease. The mortality of reoperation was 0% for congenital heart disease and 11.7%[2 death among the 17 patients] for acquired heart disease. The leading causes of death were myocardial failure, sepsis with endocarditis, acute renal failure and congestive heart failure.

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Recent Update of Advanced Imaging for Diagnosis of Cardiac Sarcoidosis: Based on the Findings of Cardiac Magnetic Resonance Imaging and Positron Emission Tomography

  • Chang, Suyon;Lee, Won Woo;Chun, Eun Ju
    • Investigative Magnetic Resonance Imaging
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    • v.23 no.2
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    • pp.100-113
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    • 2019
  • Sarcoidosis is a multisystem disease characterized by noncaseating granulomas. Cardiac involvement is known to have poor prognosis because it can manifest as a serious condition such as the conduction abnormality, heart failure, ventricular arrhythmia, or sudden cardiac death. Although early diagnosis and early treatment is critical to improve patient prognosis, the diagnosis of CS is challenging in most cases. Diagnosis usually relies on endomyocardial biopsy (EMB), but its diagnostic yield is low due to the incidence of patchy myocardial involvement. Guidelines for the diagnosis of CS recommend a combination of clinical, electrocardiographic, and imaging findings from various modalities, if EMB cannot confirm the diagnosis. Especially, the role of advanced imaging such as cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET), has shown to be important not only for the diagnosis, but also for monitoring treatment response and prognostication. CMR can evaluate cardiac function and fibrotic scar with good specificity. Late gadolinium enhancement (LGE) in CMR shows a distinctive enhancement pattern for each disease, which may be useful for differential diagnosis of CS from other similar diseases. Effectively, T1 or T2 mapping techniques can be also used for early recognition of CS. In the meantime, PET can detect and quantify metabolic activity and can be used to monitor treatment response. Recently, the use of a hybrid CMR-PET has introduced to allow identify patients with active CS with excellent co-localization and better diagnostic accuracy than CMR or PET alone. However, CS may show various findings with a wide spectrum, therefore, radiologists should consider the possible differential diagnosis of CS including myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, amyloidosis, and arrhythmogenic right ventricular cardiomyopathy. Radiologists should recognize the differences in various diseases that show the characteristics of mimicking CS, and try to get an accurate diagnosis of CS.

Functional roles and mechanisms of ginsenosides from Panax ginseng in atherosclerosis

  • Xue, Qianqian;He, Ningning;Wang, Zhibin;Fu, Xiuxiu;Aung, Lynn Htet Htet;Liu, Yan;Li, Min;Cho, Jae Youl;Yang, Yanyan;Yu, Tao
    • Journal of Ginseng Research
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    • v.45 no.1
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    • pp.22-31
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    • 2021
  • Atherosclerosis (AS) is a leading cause of cardiovascular diseases (CVDs) and it results in a high rate of death worldwide, with an increased prevalence with age despite advances in lifestyle management and drug therapy. Atherosclerosis is a chronic progressive inflammatory process, and it mainly presents with lipid accumulation, foam cell proliferation, inflammatory response, atherosclerotic plaque formation and rupture, thrombosis, and vascular calcification. Therefore, there is a great need for reliable therapeutic drugs or remedies to cure or alleviate atherosclerosis and reduce the societal burden. Ginsenosides are natural steroid glycosides and triterpene saponins obtained mainly from the plant ginseng. Several recent studies have reported that ginsenosides have a variety of pharmacological activities against several diseases including inflammation, cancer and cardiovascular diseases. This review focuses on describing the different pharmacological functions and underlying mechanisms of various active ginsenosides (Rb1,-Rd, -F, -Rg1, -Rg2, and -Rg3, and compound K) for atherosclerosis, which could provide useful insights for developing novel and effective anti-cardiovascular drugs.