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  • Open Access

    REVIEW

    Functions of ULK1 in autophagy and non-autophagy pathways and its implications in human physiology and disease

    LINNA TAN1,2, YUYONG TAN1,2, DELIANG LIU1,2,*

    BIOCELL, Vol.44, No.4, pp. 535-543, 2020, DOI:10.32604/biocell.2020.09171

    Abstract ULK1 (unc-51 like autophagy activating kinase 1), a mammalian serine/threonine kinase, is a key component of autophagy initiation complex and helps to induce all types of autophagy. Canonical autophagy is a process in which, through the interactions of a series of autophagy-related proteins, damaged organelles or misfolded proteins are engulfed by autophagosomes and then merged with lysosomes to be degraded. Thus, canonical autophagy is an important constituent part of the cellular “quality control.” Besides, accumulating evidence indicates that ULK1 exerts autophagy-independent effects in a cell-specific manner. For example, ULK1 facilitates neurite elongation through the regulation of endoplasmic reticulum (ER)–Golgi trafficking… More >

  • Open Access

    ARTICLE

    Numerical Investigation of an Idealized Total Cavopulmonary Connection Physiology Assisted by the Axial Blood Pump With and Without Diffuser

    Zhenxin Zhao1,#, Tong Chen2,#, Xudong Liu3, Shengzhang Wang2,4,*, Haiyan Lu5,*

    CMES-Computer Modeling in Engineering & Sciences, Vol.125, No.3, pp. 1173-1184, 2020, DOI:10.32604/cmes.2020.013702

    Abstract In order to improve the surgical treatment of the congenital heart disease patient with single ventricle defect, two axial flow blood pumps, one with diffuser and the other without diffuser, were designed and virtually implanted into an idealized total cavopulmonary connection (TCPC) model to form two types of Pump-TCPC physiological structure. Computational fluid dynamics (CFD) simulations were performed to analyze the variations of the hemodynamic characteristics, such as flow field, wall shear stress (WSS), oscillatory shear index (OSI), relative residence time (RRT), between the two Pump-TCPC models. Numerical results indicate that the Pump-TCPC with diffuser has better flow field stability,… More >

  • Open Access

    ARTICLE

    Detecting Outlier Behavior of Game Player Players Using Multimodal Physiology Data

    Shinjin Kang1, Taiwoo Park2,*

    Intelligent Automation & Soft Computing, Vol.26, No.1, pp. 205-214, 2020, DOI:10.31209/2019.100000141

    Abstract This paper describes an outlier detection system based on a multimodal physiology data clustering algorithm in a PC gaming environment. The goal of this system is to provide information on a game player’s abnormal behavior with a bio-signal analysis. Using this information, the game platform can easily identify players with abnormal behavior in specific events. To do this, we propose a mouse device that measures the wearer's skin conductivity, temperature, and motion. We also suggest a Dynamic Time Warping (DTW) based clustering algorithm. The developed system examines the biometric information of 50 players in a bullet dodge game. This paper… More >

  • Open Access

    ARTICLE

    Cognitive dysfunction is associated with abnormal responses in cerebral blood flow in patients with single ventricular physiology: Novel insights from transcranial Doppler ultrasound

    Rachel Wong1, Mohammed Al‐Omary2, David Baker2, Neil Spratt1,2, Andrew Boyle1,2, Natasha Baker Cert1, Peter Howe1, Nicholas Collins2

    Congenital Heart Disease, Vol.14, No.4, pp. 638-644, 2019, DOI:10.1111/chd.12763

    Abstract Objectives: Improvements in the management of complex congenital heart disease, including those with single ventricle physiology, have resulted in increased survival. As this population ages, the recognition of cognitive impairment is increasingly im‐ portant. At present, little is known about the potential mechanisms of cognitive dys‐ function. In this cross‐sectional study, we aimed to characterize the nature of abnormalities in cerebral blood flow and the relationship to cognitive deficits in adults with single ventricular physiology.
    Patients: Ten adults with single ventricular physiology (age 18‐40 years) and 12 age‐ and gender‐matched controls underwent transcranial Doppler ultrasound and ac‐ companying cognitive assessment.
    More >

  • Open Access

    REVIEW

    Cardiovascular adaptation to the Fontan circulation

    Gruschen R. Veldtman1, Alexander R. Opotowsky2, Samuel G. Wittekind1, Jack Rychik3, Daniel J. Penny4, Mark Fogel3, Bradley S. Marino5, Marc Gewillig6

    Congenital Heart Disease, Vol.12, No.6, pp. 699-710, 2017, DOI:10.1111/chd.12526

    Abstract Although medium-term survival following Fontan operations in the modern era has improved dramatically, late cardiovascular and extracardiac morbidity are common and are associated with impaired quality of life and premature late mortality. This serves as a reminder of the extraordinary adaptations required of the cardiovascular system when the systemic arterial, systemic venous and pulmonary circulations are placed in series coupled to a single ventricular pump. This article reviews the key features and principles that govern interactions between the ventricle, systemic arterial circulation, the systemic venous and pulmonary circulatory compartments, the microcirculation, and lymphatic circulations. The overarching aim is to provide… More >

  • Open Access

    ARTICLE

    Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies

    Tarek Alsaied1, Jouke P. Bokma2, Mark E. Engel3, Joey M. Kuijpers2, Samuel P. Hanke1, Liesl Zuhlke4, Bin Zhang5, Gruschen R. Veldtman6

    Congenital Heart Disease, Vol.12, No.4, pp. 393-398, 2017, DOI:10.1111/chd.12468

    Abstract Background: Reported long-term outcome measures vary greatly between studies in Fontan patients making comprehensive appraisal of mortality hazard challenging. We sought to create a clinical risk score to assist monitoring of Fontan patients in the outpatient setting.
    Methods: A systematic review was conducted to evaluate risk factors for long-term (beyond the first postoperative year) mortality in Fontan patients. Studies were eligible for inclusion if ≥90 patients were included or ≥20 long-term mortalities we reported. Risk factors for long-term mortality were determined. The pooled hazard ratios were used to create components of a clinical score for long-term mortality using meta-analysis techniques.
    More >

  • Open Access

    ARTICLE

    Pulsatile Glenn as long‐term palliation for single ventricle physiology patients

    Martin A. Chacon‐Portillo1,2, Rodrigo Zea‐Vera1,2, Huirong Zhu3, Heather A. Dickerson4,5, Iki Adachi1,2, Jeffrey S. Heinle1,2, Charles D. Fraser1,2, Carlos M. Mery1,2

    Congenital Heart Disease, Vol.13, No.6, pp. 927-934, 2018, DOI:10.1111/chd.12664

    Abstract Objective: There are limited studies analyzing pulsatile Glenn as a long‐term pallia‐ tion strategy for single ventricle patients. This study sought to determine their out‐ comes at a single institution.
    Design: A retrospective review was performed.
    Setting: Study performed at a single pediatric hospital.
    Patients: All single ventricle patients who underwent pulsatile Glenn from 1995 to 2016 were included.
    Outcome measures: Pulsatile Glenn failure was defined as takedown, transplant, or death. Further palliation was defined as Fontan, 1.5, or biventricular repair. Risk fac‐ tors were assessed by Cox multivariable competing risk analyses.
    Results: Seventy‐eight patients underwent pulsatile Glenn at age… More >

  • Open Access

    ARTICLE

    Exercise testing and spirometry as predictors of mortality in congenital heart disease: Contrasting Fontan physiology with repaired tetralogy of Fallot

    Keri M. Shafer1,2, Alexander R. Opotowsky1,2, Jonathan Rhodes1

    Congenital Heart Disease, Vol.13, No.6, pp. 903-910, 2018, DOI:10.1111/chd.12661

    Abstract Objective: Risk prediction using cardiopulmonary exercise testing (CPET) in complex congenital heart disease tends to either focus on single diagnoses or complete cohorts. We aimed to evaluate patients with two distinct anatomies cared for at a single institution over the same time period to determine CPET variables associated with mortality.
    Design: All Fontan and tetralogy of Fallot (TOF) subjects with CPET between November 1, 2002 and December 31, 2014 and subsequently died were identified (cases). Cases were matched 1:3 to controls with similar age, underlying anatomy and timing of exercise test.
    Results: Of the 42 cases, 27 had a Fontan… More >

  • Open Access

    ARTICLE

    Incidence of aspiration in infants with single‐ventricle physiology following hybrid procedure

    Jennifer P. Lundine1,2, Robert Dempster3,4, Kirby Carpenito5, Holly Miller‐Tate5, Wendelin Burdo‐Hartman4,6, Elizabeth Halpin2, Omar Khalid4,5

    Congenital Heart Disease, Vol.13, No.5, pp. 706-712, 2018, DOI:10.1111/chd.12636

    Abstract Background: Swallowing dysfunction is a known complication for infants with complex congenital heart disease (CHD), but few studies have examined swallowing outcomes following the hybrid procedure for stage 1 palliation in children with single ventricle physiology.
    Objectives: (1) Identify the incidence of aspiration in all infants with single ventricle physiology who underwent the hybrid procedure and (2) Compare results of clinical bedside and instrumental swallowing evaluations to examine the predictive value of a less invasive swallowing assessment for this population of high‐risk infants.
    Methods: This was a retrospective cohort chart review study. All patients with single‐ventricle physiology who underwent the… More >

  • Open Access

    ARTICLE

    Quality of life and sexual well-being in patients with a Fontan circulation: An explorative pilot study with a mixed method design

    Djoeke Wolff1, Henricus B. M. van de Wiel2, Mirthe E. de Muinck Keizer1, Joost P. van Melle3, Petronella G. Pieper3, Rolf M. F. Berger1, Tjark Ebels4, Willebrord C. M. Weijmar Schultz5

    Congenital Heart Disease, Vol.13, No.2, pp. 319-326, 2018, DOI:10.1111/chd.12576

    Abstract Objective: To get an impression of the quality of life (QOL) and sexual well-being in the Fontan population, and to generate hypotheses for future research.
    Methods: For this cross-sectional pilot study, questionnaires regarding health-related QOL, sexual function and fertility/pregnancy were completed by 21 patients with a Fontan circulation >16 years old, followed at the University Medical Center Groningen, the Netherlands. Semi-structured qualitative interviews were conducted in 8 patients.
    Results: Fontan patients scored significantly lower on general health than their healthy peers (t (19)=-3.0, P = .008), whereas their scores on other QOL domains and sexual well-being were comparable to normal… More >

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