keyword
https://read.qxmd.com/read/38473714/arrhythmogenic-right-ventricular-cardiomyopathy-post-mortem-assessment-a-systematic-review
#21
REVIEW
Vincenzo Cianci, Elena Forzese, Daniela Sapienza, Alessio Cianci, Antonio Ieni, Antonino Germanà, Maria Cristina Guerrera, Fausto Omero, Desirèe Speranza, Annalisa Cracò, Alessio Asmundo, Patrizia Gualniera, Cristina Mondello
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder characterized by the progressive fibro-fatty replacement of the right ventricular myocardium, leading to myocardial atrophy. Although the structural changes usually affect the right ventricle, the pathology may also manifest with either isolated left ventricular myocardium or biventricular involvement. As ARVC shows an autosomal dominant pattern of inheritance with variable penetrance, the clinical presentation of the disease is highly heterogeneous, with different degrees of severity and patterns of myocardial involvement even in patients of the same familiar group with the same gene mutation: the pathology spectrum ranges from the absence of symptoms to sudden cardiac death (SCD) sustained by ventricular arrhythmias, which may, in some cases, be the first manifestation of an otherwise silent pathology...
February 20, 2024: International Journal of Molecular Sciences
https://read.qxmd.com/read/38472792/study-of-the-interactions-between-muscle-fatty-acid-composition-meat-quality-related-genes-and-the-ileum-microbiota-in-tibetan-sheep-at-different-ages
#22
JOURNAL ARTICLE
Fanxiong Wang, Yuzhu Sha, Xiu Liu, Yanyu He, Jiang Hu, Jiqing Wang, Shaobin Li, Pengyang Shao, Xiaowei Chen, Wenxin Yang, Qianling Chen, Min Gao, Wei Huang
The intestinal microbiota of ruminants is an important factor affecting animal production and health. Research on the association mechanism between the intestinal microbiota and meat quality of ruminants will play a positive role in understanding the formation mechanism of meat quality in ruminants and improving production efficiency. In this study, the fatty acid composition and content, expression of related genes, and structural characteristics of the ileum microbiota of ewes of Tibetan sheep at different ages (4 months, 1...
February 23, 2024: Foods (Basel, Switzerland)
https://read.qxmd.com/read/38472635/the-oral-health-related-quality-of-life-of-children-with-fetal-alcohol-spectrum-disorder
#23
JOURNAL ARTICLE
Mohammad Saad Khawer, Keith Da Silva
INTRODUCTION: A child's oral health impacts their development and quality of life. Children who live with fetal alcohol spectrum disorder (FASD) face barriers to dental care combined with poorer oral health outcomes. However, how this affects their oral health-related quality of life (OHRQoL) is largely unknown. Thus, the aim of this study is to examine the OHRQoL of children living with FASD. METHODS: This cross-sectional survey used the Child Oral Health Impact Profile-Short Form-19 (COHIP-SF-19) to evaluate the OHRQoL quality of life of children (aged 8 to 15) living with FASD, compared to healthy controls...
March 12, 2024: Special Care in Dentistry
https://read.qxmd.com/read/38471666/left-ventricular-global-longitudinal-strain-and-acute-myocardial-injury-in-patients-with-sickle-cell-disease-admitted-to-the-intensive-care-unit-for-vaso-occlusive-crisis
#24
JOURNAL ARTICLE
Guillaume Roger, Pierre Denormandie, Thibaut Gobe, Danila Azzolina, Tài Pham, Christelle Chantalat, Daphnée Cuveillier, Amir Bouchachi, Patrick Jourdain, Christopher Lai, Arthur Pavot, Nicolas Fage, Paul Domnariu, Jean-Louis Teboul, Xavier Monnet
In patients with sickle cell disease (SCD), SCD-related cardiomyopathy may be partly due to repeated ischaemic events related to sickling during vaso-occlusive crises, but few clinical studies support this hypothesis. We evaluated the incidence of acute myocardial ischaemia during vaso-occlusive crises as assessed by the left ventricular global longitudinal strain (LVGLS) and high-sensitive cardiac troponin T (hs-cTnT). We included adult patients with SCD admitted to the intensive care unit (ICU) for vaso-occlusive crisis...
March 12, 2024: British Journal of Haematology
https://read.qxmd.com/read/38469996/description-of-a-national-multi-center-registry-of-patients-with-sickle-cell-disease-and-sars-cov-2-infection-data-from-the-pediatric-covid-19-united-states-registry
#25
JOURNAL ARTICLE
Aleksandra S Dain, Caroline Diorio, Brian T Fisher, Jane S Hankins, Char M Witmer, Mickael Boustany, Madeline Burton, Jose Ferrolino, Salma Sadaf, Hailey S Ross, Gabriela Maron
Children with sickle cell disease (SCD) are at risk of complications from viral infections, including SARS-CoV-2. We present the clinical characteristics and outcomes of pediatric patients with SCD from the Pediatric COVID-19 United States Registry who developed acute COVID-19 due to SARS-CoV-2 infection (n = 259) or multisystem inflammatory syndrome in children (MIS-C; n = 4). Nearly half of hospitalized children with SCD and SARS-CoV-2 infection required supplemental oxygen, though children with SCD had fewer intensive care (ICU) admissions compared to the general pediatric and immunocompromised populations...
March 12, 2024: Pediatric Blood & Cancer
https://read.qxmd.com/read/38469592/predictors-of-non-primary-auditory-and-vestibular-symptom-persistence-following-surgical-repair-of-superior-canal-dehiscence-syndrome
#26
JOURNAL ARTICLE
Liliya Benchetrit, Samantha Shave, Alejandro Garcia, Janice J Chung, Krish Suresh, Daniel J Lee
OBJECTIVE: Patients with superior canal dehiscence syndrome (SCDS) can present with a plethora of auditory and/or vestibular symptoms associated with a bony defect of the superior semicircular canal. While surgical repair is a reasonable option for patients with significant localizing symptoms, the degree of clinical improvement will vary among patients and poses challenges in outcome prediction. This study aims to assess the relationship between preoperative and postoperative symptoms and identify predictors of symptom persistence following repair...
2024: Frontiers in Neurology
https://read.qxmd.com/read/38469144/key-genes-involved-in-nonalcoholic-steatohepatitis-improvement-after-bariatric-surgery
#27
JOURNAL ARTICLE
Xiyu Chen, Shi-Zhou Deng, Yuze Sun, Yunhu Bai, Yayun Wang, Yanling Yang
BACKGROUND: Nonalcoholic steatohepatitis (NASH) is the advanced stage of nonalcoholic fatty liver disease (NAFLD), one of the most prevalent chronic liver diseases. The effectiveness of bariatric surgery in treating NASH and preventing or even reversing liver fibrosis has been demonstrated in numerous clinical studies, but the underlying mechanisms and crucial variables remain unknown. METHODS: Using the GSE135251 dataset, we examined the gene expression levels of NASH and healthy livers...
2024: Frontiers in Endocrinology
https://read.qxmd.com/read/38468150/digital-nasoalveolar-molding-through-presurgical-orthopedics-in-newborns-infants-with-cleft-lip-and-palate-a-comprehensive-review-and-case-study
#28
Katia Villarreal-Martínez, Victor Fierro-Serna, Miguel A Rosales-Berber, Vanessa Alejandri-Gamboa, Gabriela Torre-Delgadillo, Socorro Ruiz-Rodríguez, Amaury Pozos-Guillén, Arturo Garrocho-Rangel
AIMS: This study aims to provide a comprehensive review and case study about the advantages and disadvantages of the application of digital technologies in presurgical orthopedics in newborns/infants with cleft lip and palate (CLCP). Positive changes in the nasal anatomy, maxillary arch, and cleft width could be achieved. METHODS: Three representative cases of newborns/infants with CLCP were managed using the presurgical newborn/infant orthopedics (PSIO) approach...
March 11, 2024: Special Care in Dentistry
https://read.qxmd.com/read/38467355/cardiac-conduction-disorders-in-young-adults-clinical-characteristics-and-genetic-background-of-an-underestimated-population
#29
JOURNAL ARTICLE
Cristina Balla, Alice Margutti, Beatrice De Carolis, Luca Canovi, Assunta Di Domenico, Ilaria Vivaldi, Francesco Vitali, Martina De Raffele, Michele Malagù, Biagio Sassone, Mauro Biffi, Rita Selvatici, Alessandra Ferlini, Francesca Gualandi, Matteo Bertini
BACKGROUND: Cardiac conduction disorders (CCD) in patients <50 years are a rare and mostly unknown condition. OBJECTIVE: We aimed to assess clinical characteristics and genetic background of patients < 50 years with CCD of unknown origin. METHODS: We retrospectively review a consecutive series of patients with a diagnosis of CCD before the age of 50 referred to our Centre between January 2019 and December 2021. Patients underwent complete clinical examination and genetic evaluation...
March 9, 2024: Heart Rhythm: the Official Journal of the Heart Rhythm Society
https://read.qxmd.com/read/38466269/opioid-prescribing-and-outcomes-in-patients-with-sickle-cell-disease-post-2016-cdc-guideline
#30
JOURNAL ARTICLE
Hyeun Ah Kang, Bofei Wang, Jamie C Barner, Kenneth I Ataga, Robert C Mignacca, Alicia Chang, Yahan Zhang
IMPORTANCE: Although the intention of the 2016 US Centers for Disease Control and Prevention (CDC) Guideline for Prescribing Opioids for Chronic Pain was not to limit pain treatment for patients with sickle cell disease (SCD), clinicians and patients have recognized the possibility that the guideline may have altered outcomes for this population. However, the outcomes of the 2016 guideline for this patient population are unknown. OBJECTIVE: To examine changes in opioid prescribing patterns and health outcomes among patients with SCD before and after the release of the 2016 CDC guideline...
March 11, 2024: JAMA Internal Medicine
https://read.qxmd.com/read/38466042/prognostic-implications-of-premature-ventricular-contractions-and-non-sustained-ventricular-tachycardia-in-light-chain-cardiac-amyloidosis
#31
JOURNAL ARTICLE
Zhongli Chen, Anteng Shi, Hongbin Dong, Natallia Laptseva, Feng Chen, Jiandu Yang, Xiaogang Guo, Firat Duru, Keping Chen, Liang Chen
BACKGROUND AND AIMS: Premature ventricular contractions (PVC) and non-sustained ventricular tachycardia (NSVT) are commonly observed in light chain cardiac amyloidosis (AL-CA), but their association with prognosis is still unclear. We aimed to evaluate the prognostic value of PVCs and NSVT in patients with moderate-to-advanced AL-CA. METHODS: We retrospectively included patients with AL-CA at modified 2004 Mayo stages II-IIIb between February 2014 and December 2020...
March 11, 2024: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://read.qxmd.com/read/38465910/systemic-medications-for-sickle-cell-disease-and-potential-applications-for-sickle-cell-retinopathy
#32
JOURNAL ARTICLE
Anupam K Garg, Adrienne W Scott
PURPOSE OF REVIEW: To review the literature evaluating systemic medications for treatment of sickle cell disease (SCD) and their applications for sickle cell retinopathy. RECENT FINDINGS: Prior studies have demonstrated the efficacy of traditional systemic therapies in reducing the risk of development of sickle cell retinopathy. Since 2017, several new and promising disease-modifying therapies for sickle cell disease have been approved for clinical use, including the first genetic therapies such as exagamglogene autotemcel (exa-cel) and lovotibeglogene autotemcel (lovo-cel)...
March 11, 2024: Current Opinion in Ophthalmology
https://read.qxmd.com/read/38462801/-whether-to-implant-a-defibrillator-or-not-the-possibility-of-using-the-madit-icd-benefit-score-calculator-in-real-practice
#33
JOURNAL ARTICLE
N N Ilov, S A Boytsov, A A Nechepurenko
AIM: To study the predictive capabilities of the MADIT-ICD Benefit Score calculator in assessing the benefit of implantable cardioverter defibrillator (ICD) placement for the primary prevention of sudden cardiac death (SCD). MATERIAL AND METHODS: This study included 388 patients with NYHA II-IV functional class chronic heart failure (CHF) with a left ventricular ejection fraction (LVEF) ≤35 % who underwent ICD placement for the primary prevention of SCD. Patients were followed up for two years to record the endpoints of first-time paroxysmal sustained ventricular tachyarrhythmia (VT) or non-arrhythmic death...
February 29, 2024: Kardiologiia
https://read.qxmd.com/read/38462769/prevalence-of-duffy-null-and-its-impact-on-hydroxyurea-in-young-children-with-sickle-cell-disease-in-the-united-states
#34
JOURNAL ARTICLE
Fathia Oladipupo, Joseph Stanek, Joseph Walden, Jennifer Young, Melissa J Rose, Kathleen Nicol, Anthony Villella, Susan Creary
Consistent with studies showing a high prevalence of the Duffy null phenotype among healthy Black Americans, this retrospective study found that Duffy null was present in >75% of a young and contemporary cohort of children with sickle cell disease (SCD) in the United States. Despite the potential for this phenotype to impact absolute neutrophil counts, hydroxyurea (HU) dosing, and outcomes, it was not associated with being prescribed a lower HU dose or having increased acute SCD visits early in the HU treatment course...
March 10, 2024: Pediatric Blood & Cancer
https://read.qxmd.com/read/38462562/barriers-and-mediators-for-routine-dental-care-visits-from-the-experiences-of-immigrant-parents-of-autistic-children-living-in-the-united-states
#35
JOURNAL ARTICLE
Sara Imanpour, Rukhsar Ullah
INTRODUCTION: Immigrants are reluctant to attend routine dental care visits. Moreover, children with autism have a higher chance of developing periodontal disease. In this study, we describe the experiences of immigrant parents with autistic children and the barriers and mediators to routine dental care visits for their children in the United States. METHOD: We conducted semistructured interviews with 19 immigrants who have children with autism. RESULTS: We found that lack of transportation, support system, and insurance; prioritization of basic needs; and a mistrust of healthcare providers are the main barriers to attending routine dental care visits for autistic children of immigrant parents...
March 10, 2024: Special Care in Dentistry
https://read.qxmd.com/read/38462030/study-of-the-antigenic-characteristics-of-red-blood-cells-units-and-their-sickle-cell-disease-recipients-and-the-g6pd-activity-of-transfused-red-blood-cells-units
#36
JOURNAL ARTICLE
Morgane Le Gallo, Stéphane Moutereau, Mélanie Gentil, France Pirenne
INTRODUCTION: Transfusion has a central place in the treatment of patients with sickle cell disease (SCD). Matching blood groups of red blood cell (RBC) units with the blood groups of the patient is essential to prevent alloimmunization and delayed hemolytic transfusion reaction. African ancestry donors have the best phenocompatibility with patients of the same origin, however their RBCs may present characteristic that can alter quality of the unit such as glucose-6-phosphate dehydrogenase (G6PD) deficiency...
March 8, 2024: Transfusion Clinique et Biologique: Journal de la Société Française de Transfusion Sanguine
https://read.qxmd.com/read/38461297/sudden-cardiac-death-among-workers-a-systematic-review-and-meta-analysis
#37
JOURNAL ARTICLE
Carlotta Amantea, Enrico Pilia, Maria Francesca Rossi, Valerio Flavio Corona, Giuseppe Amato, Corrado Ciccu, Filippo Gavi, Paolo Emilio Santoro, Ivan Borrelli, Walter Ricciardi, Maria Rosaria Gualano, Umberto Moscato
OBJECTIVE: Sudden cardiac death (SCD) is a rare and yet unexplained condition. The most frequent cause is myocardial infarction, while a small proportion is due to arrhythmogenic syndromes (e.g., channelopathies). This systematic review and meta-analysis aimed to provide a comprehensive overview of the prevalence and risk factors associated with SCD in workers. MATERIAL AND METHODS: A search for eligible studies was performed utilizing three databases (PubMed, ISI Web of Knowledge, and Scopus)...
March 9, 2024: Systematic Reviews
https://read.qxmd.com/read/38460164/blocking-ubiquitin-specific-protease-7-induces-ferroptosis-in-gastric-cancer-via-targeting-stearoyl-coa-desaturase
#38
JOURNAL ARTICLE
Xiaoqing Guan, Yichao Wang, Wenkai Yu, Yong Wei, Yang Lu, Enyu Dai, Xiaowu Dong, Bing Zhao, Can Hu, Li Yuan, Xin Luan, Kai Miao, Bonan Chen, Xiang-Dong Cheng, Weidong Zhang, Jiang-Jiang Qin
Gastric cancer (GC) presents a formidable global health challenge, and conventional therapies face efficacy limitations. Ubiquitin-specific protease 7 (USP7) plays pivotal roles in GC development, immune response, and chemo-resistance, making it a promising target. Various USP7 inhibitors have shown selectivity and efficacy in preclinical studies. However, the mechanistic role of USP7 has not been fully elucidated, and currently, no USP7 inhibitors have been approved for clinical use. In this study, DHPO is identified as a potent USP7 inhibitor for GC treatment through in silico screening...
March 9, 2024: Advanced Science (Weinheim, Baden-Wurttemberg, Germany)
https://read.qxmd.com/read/38459948/apoe-genotype-disclosure-influences-decisions-about-future-planning-but-not-adoption-of-healthy-lifestyle-changes-in-cognitively-unimpaired-individuals
#39
JOURNAL ARTICLE
Dominique L Popescu, Athene K Lee, Edmund Arthur, Louisa I Thompson, Jessica Alber
BACKGROUND: Studies have shown apolipoprotein E (APOE) genotype disclosure to be safe and well-tolerated in cognitively unimpaired (CU) older adults. This study aimed to examine the effect of the disclosure process on decisions about future directives and health behaviors in community-dwelling CU older adults from the Butler Alzheimer's Prevention Registry (BAPR). METHODS: CU APOE E4 non-carriers (n = 106) and carriers (n = 80) aged 58-78 completed in-person psychological readiness screening to undergo APOE disclosure...
March 9, 2024: Journal of Geriatric Psychiatry and Neurology
https://read.qxmd.com/read/38459131/can-we-screen-for-heart-disease-in-children-at-public-health-centres-a-multicentre-observational-study-of-screening-for-heart-disease-with-a-risk-of-sudden-death-in-children
#40
JOURNAL ARTICLE
Paula Greciano Calero, Silvia Escribá Bori, Juan Antonio Costa Orvay, Nina González Pons, María Del Carmen Martín Pérez, Dolores Cardona Alfonseca, Cristina Nogales Velázquez, Sergi Verd Vallespir, Alicia Esther Tur Salom, Antonella Chiandetti, Marcos Navarro Noguera, Anna Grau Blanch, María Magdalena Rotger Genestar, Marianna Mambié Meléndez, Mercedes Fernández Hidalgo, Juana María Seguí Llinas, Laura Martorell Bon, Patricia Arestuche Aguilar, Beatriz Garrido Conde, María Del Valle Sánchez Grao, Katia Sarraff Trujillo, Antoni Muntaner Alonso, Catalina Grimalt Ferragut, Andrea Soriano Marco, Viviana Gómez Rojas, Juan Pol Serra
Sudden cardiac death in children is a rare event, but of great social significance. Generally, it is related to heart disease with a risk of sudden cardiac death (SCD), which may occur with cardiovascular symptoms and/or electrocardiographic markers; thus, a primary care paediatrician (PCP) could detect them. Therefore, we proposed a study that assesses how to put into practice and conduct a cardiovascular assessment within the routine healthy-child check-ups at six and twelve years of age; that reflects cardiovascular signs and symptoms, as well as the electrocardiographic alterations that children with a risk of SCD in the selected population present; and that assesses the PCP's skill at electrocardiogram (ECG) interpretation...
March 8, 2024: European Journal of Pediatrics
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