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The Long QT syndrome (LQTS) is an inherited arrhythmogenic disease occurring in the structurally normal heart that may cause sudden death and that usually manifests in children and teen-agers (1). The prevalence of this disorder is still undefined, however it is estimated to be between 1:10000–1:5000.
Priori, SG, Bloise, R, Crotti, L
openaire +4 more sources
Abstract Background As previously reported, an increased repolarization temporal imbalance induces a higher risk of total/cardiovascular mortality. Hypothesis The aim of this study was to assess if the electrocardiographic short period markers of repolarization temporal dispersion could be predictive of the hospital stay length and mortality in ...
Gianfranco Piccirillo+11 more
wiley +1 more source
Regulatory authorities put major emphasis on QT (interval)–prolonging properties of new molecular entities. Product information/Summaries of Product Characteristics (SmPCs) of multiple drugs contain warnings or contraindications regarding QT prolongation, e.g., on coadministration of QT‐prolonging drugs (QT drugs).
Melanie I. Then+5 more
wiley +1 more source
Medium‐chain Acyl‐COA dehydrogenase deficiency: Pathogenesis, diagnosis, and treatment
MCADD is a commonly inherited metabolic disease with serious implications for health outcomes, particularly in children, that may be successfully managed with proper intervention. In this review, we discuss the genetics, pathogenesis, clinical presentation, diagnosis, and treatments for Medium‐Chain Acyl‐CoA Dehydrogenase Deficiency (MCADD ...
Emily Mason+2 more
wiley +1 more source
A 34-year-old female who is 4 months postpartum presents after a nocturnal seizure. She was awakened at night by an alarm clock to feed her baby, spoke briefly with her husband, and suddenly lost consciousness, appearing to have epileptic-type movements before spontaneously recovering.
Dominic Abrams, Calum A. MacRae
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Phase 1b study of anlotinib combined with TQB2450 in pretreated advanced biliary tract cancer and biomarker analysis. Abstract Background and Aims We evaluated the efficacy and safety of the antiangiogenic tyrosine kinase inhibitor anlotinib plus TQB2450, a programmed death‐ligand 1 inhibitor in pretreated advanced biliary tract cancers (BTCs ...
Jun Zhou+13 more
wiley +1 more source
Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study. Abstract Background and Aims Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real‐world data are lacking.
Shannon M. Vandriel+93 more
wiley +1 more source
Evaluating a general pediatric/adult genetic counseling clinic in a Midwest medical center
Abstract In 2018, the Munroe‐Meyer Institute for Genetics & Rehabilitation (MMI) at the University of Nebraska Medical Center (UNMC) in Omaha, NE created a genetic counseling clinic (GCC) to increase access to genetics services and decrease the time spent between a referral and being seen in a general genetics outpatient clinic.
Charlie King+4 more
wiley +1 more source
The familial long-QT syndrome (LQTS) is an infrequently occurring disorder in which affected family members have QT prolongation on the ECG, often associated with recurrent syncope and fatal ventricular arrhythmias. Autosomal recessive and autosomal dominant modes of inheritance were suggested by the pattern of occurrence of this disorder in the first ...
Arthur J. Moss, Jennifer L. Robinson
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ПРОГНОЗУВАННЯ РИЗИКУ ВИНИКНЕННЯ СЕРЦЕВО-СУДИННИХ УСКЛАДНЕНЬ НА ОСНОВІ КЛАСТЕРНОГО АНАЛІЗУ ТРИВАЛОСТІ ІНТЕРВАЛУ QT ПРИ ЦУКРОВОМУ ДІАБЕТІ 1 ТИПУ У ДІТЕЙ [PDF]
Clinical and scientific experience of curation and analysis of children with type 1 diabetes and with acquired or congenital long QT syndrome (LQTS) health is not enough discovered for full control, treatment and risks prediction.
Gnyloskurenko, Ganna+3 more
core +2 more sources