2,113 results match your criteria Pediatrics Tachycardia


Neonatal ventricular tachycardia: Adverse event possibly due to maternal ritodrine.

Pediatr Int 2019 Feb 7. Epub 2019 Feb 7.

Department of Pediatrics, Kagoshima University, Kagoshima, Japan.

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http://dx.doi.org/10.1111/ped.13763DOI Listing
February 2019
1 Read

Evaluation using a four-dimensional imaging tool before and after pulmonary valve replacement in a patient with tetralogy of Fallot: a case report.

J Med Case Rep 2019 Feb 5;13(1):30. Epub 2019 Feb 5.

Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan.

Background: Pulmonary regurgitation is a common complication after tetralogy of Fallot repair, resulting in right ventricular dysfunction, arrhythmia, and sudden death. However, the indications and optimal timing for pulmonary valve replacement are not fully known. We describe a case in which a four-dimensional imaging tool was useful in the decision to re-operate, thus resulting in decreased energy loss and improved right ventricular function after the re-operation for tetralogy of Fallot. Read More

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https://jmedicalcasereports.biomedcentral.com/articles/10.11
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http://dx.doi.org/10.1186/s13256-018-1964-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362591PMC
February 2019
6 Reads

Ca-Binding Proteins of the EF-Hand Superfamily: Diagnostic and Prognostic Biomarkers and Novel Therapeutic Targets.

Authors:
Claus W Heizmann

Methods Mol Biol 2019 ;1929:157-186

Department of Pediatrics, Division of Clinical Chemistry and Biochemistry, University of Zürich, Zürich, Switzerland.

A multitude of Ca-sensor proteins containing the specific Ca-binding motif (helix-loop-helix, called EF-hand) are of major clinical relevance in a many human diseases. Measurements of troponin, the first intracellular Ca-sensor protein to be discovered, is nowadays the "gold standard" in the diagnosis of patients with acute coronary syndrome (ACS). Mutations have been identified in calmodulin and linked to inherited ventricular tachycardia and in patients affected by severe cardiac arrhythmias. Read More

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http://link.springer.com/10.1007/978-1-4939-9030-6_11
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http://dx.doi.org/10.1007/978-1-4939-9030-6_11DOI Listing
January 2019
4 Reads

Successful radiofrequency catheter ablation of life-threatening atrial tachycardia in an infant with asplenia syndrome.

J Cardiol Cases 2019 Jan 14;19(1):1-4. Epub 2018 Sep 14.

Department of Pediatric Cardiology, Saitama Medical University International Medical Center, Saitama, Japan.

A 1-year-old infant with asplenia syndrome and congenital heart disease consisting of common atrium, common inlet left ventricle with a common atrio-ventricular (AV) valve, pulmonary atresia, and total anomalous pulmonary venous connection was admitted to our hospital for radiofrequency catheter ablation (RFCA) of supraventricular tachycardia (SVT) before total cavo-pulmonary connection. After antiarrhythmic medications were discontinued for RFCA, she suffered from SVT that resulted in the rapid deterioration of hemodynamic status. Antiarrhythmic medications and cardioversion were not effective in terminating SVT. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18785409183007
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http://dx.doi.org/10.1016/j.jccase.2018.08.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6342611PMC
January 2019
4 Reads

Challenging diagnosis of thyroid hormone resistance initially as Hashimoto's thyroiditis.

J Pediatr Endocrinol Metab 2019 Feb;32(2):203-206

Department of Pediatrics, Division of Pediatric Endocrinology, SUNY Downstate Medical Center, Brooklyn, NY, USA.

Background Resistance to thyroid hormone (RTH) commonly presents with goiter, attention deficit hyperactivity disorder (ADHD), short stature and tachycardia. However, due to its variable presentation with subtle clinical features, a third of the cases are mistreated, typically as hyperthyroidism. Case presentation A 15-year-old female with ADHD and oligomenorrhea was initially diagnosed as Hashimoto's thyroiditis but found to have a rare heterozygous mutation in c803 C>G (p Ala 268 Gly) in the THRβ gene, confirming resistance to thyroid hormone. Read More

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http://dx.doi.org/10.1515/jpem-2018-0284DOI Listing
February 2019
6 Reads

Sudden cardiac death in football players: Towards a new pre-participation algorithm.

Exp Ther Med 2019 Feb 30;17(2):1143-1148. Epub 2018 Nov 30.

Exercise Physiology and Sports Medicine Clinic, Center for Adolescent Medicine and UNESCO Chair on Adolescent Health Care, First Department of Pediatrics, Medical School, National and Kapodistrian University of Athens, Aghia Sophia Children's Hospital, 11527 Athens, Greece.

Athletic pre-participation screening is essential for minimizing the risk for sudden cardiac death (SCD) in athletes participating in either competitive or leisure sporting activities. The primary causes of SCD in young athletes (<35 years of age) include hypertrophic cardiomyopathy, congenital anomalies of the coronary artery and arrhythmogenic right ventricular cardiomyopathy. Other abnormalities, such as malignant arrhythmia due to blunt trauma to the chest (commotio cordis), myocarditis, valvular disease, aortic rupture (in Marfan syndrome) and ion channelopathies (catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome, long or short QT syndrome), also contribute to a lesser degree to SCD. Read More

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http://www.spandidos-publications.com/10.3892/etm.2018.7041
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http://dx.doi.org/10.3892/etm.2018.7041DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6327574PMC
February 2019
11 Reads

Paediatric intensive care admissions during the 2015-2016 Queensland human parechovirus outbreak.

J Paediatr Child Health 2019 Jan 24. Epub 2019 Jan 24.

Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia.

Aim: The human parechovirus (HPeV) has emerged as a pathogen causing sepsis-like presentations in young infants, but there is a lack of data on HPeV presentations requiring intensive care support. We aimed to characterise the clinical presentation, disease severity, management and outcome of a population-based cohort of children with microbiologically confirmed HPeV infection requiring admission to paediatric intensive care units (PICUs) in Queensland, Australia during a recent outbreak.

Methods: This was a multicentre retrospective study of children admitted to PICU between 1 January 2015 and 31 December 2016 with confirmed HPeV infection. Read More

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http://doi.wiley.com/10.1111/jpc.14336
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http://dx.doi.org/10.1111/jpc.14336DOI Listing
January 2019
4 Reads

Bioavailability of endotracheal epinephrine in an ovine model of neonatal resuscitation.

Early Hum Dev 2019 Jan 15;130:27-32. Epub 2019 Jan 15.

Department of Pediatrics, UC Davis School of Medicine, Sacramento, CA, United States of America.

Background: Distressed infants in the delivery room and those that have completed postnatal transition are both resuscitated according to established neonatal resuscitation guidelines, often with endotracheal (ET) epinephrine at the same dose. We hypothesized that ET epinephrine would have higher bioavailability in a post-transitional compared to transitioning newborn model due to absence of fetal lung liquid and intra-cardiac shunts.

Methods: 15 term fetal (transitioning newborn) and 6 postnatal lambs were asphyxiated by umbilical cord and ET tube occlusion respectively. Read More

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http://dx.doi.org/10.1016/j.earlhumdev.2019.01.006DOI Listing
January 2019
2 Reads

Association Between Time to Defibrillation and Survival in Pediatric In-Hospital Cardiac Arrest With a First Documented Shockable Rhythm.

JAMA Netw Open 2018 Sep 7;1(5):e182643. Epub 2018 Sep 7.

Center for Resuscitation Science, Department of Emergency Medicine, Beth Israel Deaconess Medical Center, Boston, Massachusetts.

Importance: Delayed defibrillation (>2 minutes) in adult in-hospital cardiac arrest (IHCA) is associated with worse outcomes. Little is known about the timing and outcomes of defibrillation in pediatric IHCA.

Objective: To determine whether time to first defibrillation attempt in pediatric IHCA with a first documented shockable rhythm is associated with survival to hospital discharge. Read More

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http://jamanetworkopen.jamanetwork.com/article.aspx?doi=10.1
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http://dx.doi.org/10.1001/jamanetworkopen.2018.2643DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6324599PMC
September 2018
7 Reads

Catecholaminergic Polymorphic Ventricular Tachycardia: The Cardiac Arrest Where Epinephrine Is Contraindicated.

Pediatr Crit Care Med 2019 Jan 11. Epub 2019 Jan 11.

Green Lane Paediatric and Congenital Cardiac Services, Starship Children's Hospital, Auckland, New Zealand.

Objectives: To raise awareness among pediatric intensive care specialists of catecholaminergic polymorphic ventricular tachycardia; an uncommon cause of polymorphic ventricular tachycardia and ventricular fibrillation arrest in children and young adults where epinephrine (adrenaline), even when given according to international protocols, can be counter-productive and life-threatening. We review three cases of cardiac arrest in children, later proven to be catecholaminergic polymorphic ventricular tachycardia related, where delay in recognition of this condition resulted in significantly longer resuscitation efforts, more interventions, and a longer time to return of spontaneous circulation.

Design: Retrospective case series. Read More

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http://dx.doi.org/10.1097/PCC.0000000000001847DOI Listing
January 2019
9 Reads

Frequency Domain Indices of Heart Rate Variability are Useful for Differentiating Vasovagal Syncope and Postural Tachycardia Syndrome in Children.

J Pediatr 2019 Jan 7. Epub 2019 Jan 7.

Department of Pediatrics, Peking University First Hospital, Beijing, China; Key Laboratory of Molecular Cardiovascular Sciences, Ministry of Education, Beijing, China. Electronic address:

Objective: To explore the value of frequency domain indices of heart rate variability (HRV) in the differential diagnosis between pediatric vasovagal syncope and postural tachycardia syndrome (POTS).

Study Design: Eighty-five patients aged 7-16 years with either vasovagal syncope or POTS were enrolled in the experimental group; 18 healthy children served as controls. Holter electrocardiography was used to detect HRV frequency-domain indices in patients with vasovagal syncope, patients with POTS, and control subjects. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00223476183170
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http://dx.doi.org/10.1016/j.jpeds.2018.11.054DOI Listing
January 2019
7 Reads

Nonautoimmune congenital hyperthyroidism due to p.Asp633Glu mutation in the TSHR gene.

Ann Pediatr Endocrinol Metab 2018 Dec 31;23(4):235-239. Epub 2018 Dec 31.

Department of Pediatrics, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Korea.

Most cases of congenital hyperthyroidism are autoimmune forms caused by maternal thyroid stimulating antibodies. Nonautoimmune forms of congenital hyperthyroidism caused by activating mutations of the thyrotropin receptor (TSHR) gene are rare. A woman gave birth to a boy during an emergency cesarean section at 33 weeks of gestation due to fetal tachycardia. Read More

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http://e-apem.org/journal/view.php?doi=10.6065/apem.2018.23.
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http://dx.doi.org/10.6065/apem.2018.23.4.235DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312916PMC
December 2018
6 Reads

Phase I/II Trial of Electrophysiology-Guided Noninvasive Cardiac Radioablation for Ventricular Tachycardia.

Circulation 2019 Jan;139(3):313-321

Department of Internal Medicine, Cardiovascular Division (K.M.S.M., D.H.C., M.F., A.N., T.W.S., P.S.C.), Washington University, St Louis, MO.

Background: Case studies have suggested the efficacy of catheter-free, electrophysiology-guided noninvasive cardiac radioablation for ventricular tachycardia (VT) using stereotactic body radiation therapy, although prospective data are lacking.

Methods: We conducted a prospective phase I/II trial of noninvasive cardiac radioablation in adults with treatment-refractory episodes of VT or cardiomyopathy related to premature ventricular contractions (PVCs). Arrhythmogenic scar regions were targeted by combining noninvasive anatomic and electric cardiac imaging with a standard stereotactic body radiation therapy workflow followed by delivery of a single fraction of 25 Gy to the target. Read More

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http://dx.doi.org/10.1161/CIRCULATIONAHA.118.038261DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6331281PMC
January 2019
3 Reads

2018 American Heart Association Focused Update on Pediatric Advanced Life Support: An Update to the American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.

Circulation 2018 Dec;138(23):e731-e739

This 2018 American Heart Association focused update on pediatric advanced life support guidelines for cardiopulmonary resuscitation and emergency cardiovascular care follows the 2018 evidence review performed by the Pediatric Task Force of the International Liaison Committee on Resuscitation. It aligns with the International Liaison Committee on Resuscitation's continuous evidence review process, and updates are published when the group completes a literature review based on new published evidence. This update provides the evidence review and treatment recommendation for antiarrhythmic drug therapy in pediatric shock-refractory ventricular fibrillation/pulseless ventricular tachycardia cardiac arrest. Read More

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http://dx.doi.org/10.1161/CIR.0000000000000612DOI Listing
December 2018
1 Read

Prognostic Significance of PR Interval Prolongation in Adult Patients With Total Correction of Tetralogy of Fallot.

Circ Arrhythm Electrophysiol 2018 Nov;11(11):e006234

Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan (Y.K., K. Fukuda, M.N., Y.H., K. Fukasawa, T.C., K.M., S.T., H.S.).

Background: Several studies have demonstrated the importance of mechanoelectrical interaction in patients with surgically corrected tetralogy of Fallot. However, the significance of atrioventricular conduction disturbance, that is PR interval prolongation, on adverse cardiac events in those patients remains to be elucidated.

Methods: We examined electrocardiograms at baseline and their temporal change in a total of 176 patients with repaired tetralogy of Fallot (49% men; median age, 17. Read More

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http://dx.doi.org/10.1161/CIRCEP.118.006234DOI Listing
November 2018
1 Read

Plasma Homocysteine Level in Children With Postural Tachycardia Syndrome.

Front Pediatr 2018 3;6:375. Epub 2018 Dec 3.

Department of Pediatrics, Peking University First Hospital, Beijing, China.

The study was designed to evaluate the changes of plasma homocysteine (Hcy) level in children with postural tachycardia syndrome (POTS) and explore its significance. A total of 65 subjects were recruited in our study, of whom 35 children were in the POTS group and 30 healthy children were in the control group. Plasma Hcy levels were determined in all subjects. Read More

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http://dx.doi.org/10.3389/fped.2018.00375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287046PMC
December 2018

Contemporary Outcomes of Pediatric Restrictive Cardiomyopathy: A Single-Center Experience.

Pediatr Cardiol 2018 Dec 12. Epub 2018 Dec 12.

Cincinnati Children's Hospital Medical Center, Heart Institute, Cincinnati, OH, USA.

Background: Pediatric restrictive cardiomyopathy (RCM) has high mortality in historical cohorts, and traditional management often involves early referral for heart transplantation (HTx). This study sought to determine outcomes of pediatric RCM at a center that has favored medical management over early listing for HTx.

Methods: All patients (N = 43) with pure RCM phenotype (RCM, N = 26) and hypertrophic cardiomyopathy with restrictive physiology (RCM/HCM, N = 17) managed at our center over a 15-year period were investigated. Read More

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http://dx.doi.org/10.1007/s00246-018-2043-0DOI Listing
December 2018

Incidence and effect of early postoperative ventricular arrhythmias after congenital heart surgery.

Heart Rhythm 2018 Dec 5. Epub 2018 Dec 5.

Department of Pediatrics, Thomas P. Graham Jr. Division of Cardiology, Vanderbilt University Medical Center, Nashville, Tennessee.

Background: Postoperative arrhythmias after pediatric congenital heart disease (CHD) surgery are a known cause of morbidity and are associated with mortality. A comprehensive evaluation of early postoperative ventricular arrhythmias (VAs) after CHD surgery has not been reported.

Objectives: We sought to determine the incidence of in-hospital VAs after CHD surgery and assess the clinical relevance of this arrhythmia during the postoperative hospital course. Read More

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http://dx.doi.org/10.1016/j.hrthm.2018.11.032DOI Listing
December 2018
2 Reads

Safety of Sports for Young Patients With Implantable Cardioverter-Defibrillators.

Circ Arrhythm Electrophysiol 2018 Nov;11(11):e006305

Yale University School of Medicine, New Haven, CT (J.D., C.B., L.S., F.L., R.J.L.).

Background: Despite safety concerns, many young patients with implantable cardioverter-defibrillators (ICDs) participate in sports. We undertook a prospective, multinational registry to determine the incidence of serious adverse events because of sports participation. The primary end points were death or resuscitated arrest during sports or injury during sports because of arrhythmia or shock. Read More

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http://dx.doi.org/10.1161/CIRCEP.118.006305DOI Listing
November 2018
12 Reads

Postural orthostatic tachycardia syndrome (POTS) in teens: A guide for behavior change to manage symptoms.

Pacing Clin Electrophysiol 2019 Feb 18;42(2):283-286. Epub 2018 Dec 18.

Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

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http://dx.doi.org/10.1111/pace.13571DOI Listing
February 2019
26 Reads

Low Sensitivity of Abbreviated Tilt Table Testing for Diagnosing Postural Tachycardia Syndrome in Adults With ME/CFS.

Front Pediatr 2018 16;6:349. Epub 2018 Nov 16.

Stichting CardioZorg, Hoofddorp, Netherlands.

Orthostatic intolerance is common among individuals with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). In some ME/CFS case definitions, orthostatic intolerance is considered a core feature of the disorder. Some studies have employed tilt table tests lasting 2-5 min to diagnose one common form of orthostatic intolerance, postural tachycardia syndrome (POTS). Read More

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http://dx.doi.org/10.3389/fped.2018.00349DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6250822PMC
November 2018
1 Read

Lassa fever outbreak in adolescents in North Central Nigeria: report of cases.

J Virus Erad 2018 Oct 1;4(4):225-227. Epub 2018 Oct 1.

Department of Paediatrics, Jos university Teaching Hospital, Jos, Nigeria.

Background: Lassa fever (LF) is a viral haemorrhagic fever endemic to West Africa. The clinical presentation and course is variable, making diagnosis difficult.

Aim: To report the outbreak and identify the common clinical presentations of LF in paediatric patients in Jos, Plateau State, North Central, Nigeria. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6248838PMC
October 2018
1 Read

Arrhythmia in thiamine responsive megaloblastic anemia syndrome.

Turk J Pediatr 2018 ;60(3):348-351

Department of Pediatric Endocrinology, Erciyes University Medical Faculty, Kayseri, Turkey.

Argun M, Baykan A, Hatipoğlu N, Akın L, Şahin Y, Narin N, Kurtoğlu S. Arrhythmia in thiamine responsive megaloblastic anemia syndrome. Turk J Pediatr 2018; 60: 348-351. Read More

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http://dx.doi.org/10.24953/turkjped.2018.03.021DOI Listing
January 2018
4 Reads

Value of Immediate Heart Rate Alteration From Supine to Upright in Differential Diagnosis Between Vasovagal Syncope and Postural Tachycardia Syndrome in Children.

Front Pediatr 2018 19;6:343. Epub 2018 Nov 19.

Department of Pediatrics, Peking University First Hospital, Beijing, China.

To explore the predictive value of immediate heart rate alteration from supine to upright in the differential diagnosis between vasovagal syncope (VVS) and postural tachycardia syndrome (POTS) in children. A total of 76 pediatric outpatients or inpatients who visited the Peking University First Hospital from July 2016 to November 2017 were recruited in the study. Among them, 52 patients were diagnosed with VVS and 24 patients were diagnosed with POTS. Read More

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https://www.frontiersin.org/article/10.3389/fped.2018.00343/
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http://dx.doi.org/10.3389/fped.2018.00343DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6252323PMC
November 2018
9 Reads

Pre-excitation induced ventricular dysfunction and successful berlin heart explantation after accessory pathway ablation.

J Electrocardiol 2018 Nov - Dec;51(6):1067-1070. Epub 2018 Sep 17.

Department of Pediatrics, Pediatric Electrophysiology, University of Arkansas for Medical Sciences, Little Rock, AR, USA. Electronic address:

A 13 kg, 20 month-old, Caucasian girl, presented with cardiomyopathy, biventricular dysfunction and pre-excitation on electrocardiogram. She had normal intracardiac anatomy with severely dilated left ventricle and severely diminished biventricular function (Fig. 1). Read More

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http://dx.doi.org/10.1016/j.jelectrocard.2018.09.008DOI Listing
September 2018
1 Read

Pediatric dysautonomia: Much-maligned, often overmedicated, but not as complex as you think.

Congenit Heart Dis 2018 Nov 28. Epub 2018 Nov 28.

Division of Pediatric Cardiology, Department of Pediatrics, East Carolina University Brody School of Medicine, Greenville, North Carolina.

Dysautonomia is an increasingly recognized yet still poorly understood disease within the field of pediatrics. Symptoms, including dizziness, headaches, fatigue, joint pain, anxiety, and intolerance of heat or cold, are often significant and difficult to sort, especially in terms of their relation to each other. This often leads to referral to multiple subspecialists, who then proceed to treat seemingly familiar symptoms in kind. Read More

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http://doi.wiley.com/10.1111/chd.12720
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http://dx.doi.org/10.1111/chd.12720DOI Listing
November 2018
8 Reads

Polymorphic ventricular tachycardia associated with an episode of reflex syncope: Is this the needle in the haystack?

HeartRhythm Case Rep 2018 Nov 4;4(11):510-513. Epub 2018 Aug 4.

Division of Cardiology, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada.

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http://dx.doi.org/10.1016/j.hrcr.2018.07.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6241039PMC
November 2018
1 Read

Autonomic dysfunction and HPV immunization: an overview.

Immunol Res 2018 Nov 27. Epub 2018 Nov 27.

Rheumatology Department, National Institute of Cardiology, Mexico City, Mexico.

This article reviews the case series reported from several countries describing patients with suspected severe side effects to the HPV vaccines. The described symptom clusters are remarkably similar and include disabling fatigue, headache, widespread pain, fainting, gastrointestinal dysmotility, limb weakness, memory impairment episodes of altered awareness, and abnormal movements. This constellation of symptoms and signs has been labeled with different diagnoses such as complex regional pain syndrome (CRPS), postural orthostatic tachycardia syndrome (POTS), small fiber neuropathy (SFN), myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), or fibromyalgia. Read More

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http://dx.doi.org/10.1007/s12026-018-9036-1DOI Listing
November 2018
2 Reads

Postmortem Findings in a Young Man With Congenital Generalized Lipodystrophy, Type 4 Due to CAVIN1 Mutations.

J Clin Endocrinol Metab 2019 Mar;104(3):957-960

Division of Nutrition and Metabolic Diseases, Department of Internal Medicine and the Center for Human Nutrition; UT Southwestern Medical Center, Dallas, Texas.

Context: Congenital generalized lipodystrophy, type 4 (CGL4) is a rare autosomal recessive disorder caused by mutations in caveolae-associated protein 1. Patients with CGL4 also have myopathy and cardiomyopathy with a predisposition for sudden death due to ventricular arrhythmias. However, the underlying pathology for these morbidities remains unknown. Read More

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https://academic.oup.com/jcem/advance-article/doi/10.1210/jc
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http://dx.doi.org/10.1210/jc.2018-01331DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6364506PMC
March 2019
10 Reads

A novel pacing maneuver to verify the postpacing interval minus the tachycardia cycle length while adjusting for decremental conduction: Using "dual-chamber entrainment" for improved supraventricular tachycardia discrimination.

Heart Rhythm 2018 Nov 20. Epub 2018 Nov 20.

Department of Pediatrics, Stanford University School of Medicine, Stanford, California.

Background: The postpacing interval (PPI) minus the tachycardia cycle length (TCL) is frequently used to investigate tachycardias. However, a variety of issues (eg, failure to entrain, decremental conduction, and oscillating TCLs) can make interpretation of the PPI-TCL challenging.

Objective: The purpose of this study was to investigate a novel maneuver to confirm the PPI-TCL value without using either the ventricular PPI or the TCL interval and to assess the ability of this maneuver to identify decremental conduction and differentiate supraventricular tachycardias. Read More

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http://dx.doi.org/10.1016/j.hrthm.2018.11.021DOI Listing
November 2018
7 Reads

Small-conductance calcium-activated potassium current modulates the ventricular escape rhythm in normal rabbit hearts.

Heart Rhythm 2018 Nov 13. Epub 2018 Nov 13.

Krannert Institute of Cardiology and Division of Cardiology, Department of Medicine, Indiana University School of Medicine, Indianapolis, Indiana. Electronic address:

Background: The apamin-sensitive small-conductance calcium-activated K (SK) current I modulates automaticity of the sinus node. I blockade by apamin causes sinus bradycardia.

Objective: The purpose of this study was to test the hypothesis that I modulates ventricular automaticity. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15475271183111
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http://dx.doi.org/10.1016/j.hrthm.2018.10.033DOI Listing
November 2018
9 Reads

Long-term control of abdominal pain related to POTS: two case reports in pediatrics.

Pain Manag 2018 Nov 9;8(6):437-440. Epub 2018 Nov 9.

Carolinas Pain Institute, 145 Kimel Park Drive, Winston-Salem, NC 27103, USA.

Aim: To describe two consecutive cases of Postural Orthostatic Tachycardia Syndrome (POTS)-related chronic abdominal pain control in children after bilateral splanchnic block.

Patients & Methods: Two pediatric patients with chronic abdominal pain related to POTS received celiac and T11 splanchnic plexus block for pain control.

Results: While celiac plexus block did not provide any substantial relief of their pain, splanchnic block provided a long-term improvement in pain scores, nausea and vomiting cessation. Read More

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http://dx.doi.org/10.2217/pmt-2018-0013DOI Listing
November 2018
4 Reads

Catecholaminergic polymorphic ventricular tachycardia patients with multiple genetic variants in the PACES CPVT Registry.

PLoS One 2018 7;13(11):e0205925. Epub 2018 Nov 7.

Departments of Medicine, Pediatrics, and Biochemistry & Molecular Biology, University of British Columbia, Vancouver, BC, Canada.

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is often a life-threatening arrhythmia disorder with variable penetrance and expressivity. Little is known about the incidence or outcomes of CPVT patients with ≥2 variants.

Methods: The phenotypes, genotypes and outcomes of patients in the Pediatric and Congenital Electrophysiology Society CPVT Registry with ≥2 variants in genes linked to CPVT were ascertained. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0205925PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6221297PMC
November 2018
12 Reads

S100 proteins: Diagnostic and prognostic biomarkers in laboratory medicine.

Authors:
Claus W Heizmann

Biochim Biophys Acta Mol Cell Res 2018 Oct 28. Epub 2018 Oct 28.

Department of Pediatrics, Division of Clinical Chemistry and Biochemistry, University of Zürich, CH-8032 Zürich, Switzerland. Electronic address:

S100 proteins are members of the superfamily of Ca-binding proteins characterized by the specific Ca-binding motif, the EF-hand. Proteins of this superfamily are of clinical use as important diagnostic and prognostic biomarkers in adult and pediatric Laboratory Medicine. For example, measurements of troponin are nowadays the 'gold standard' in the diagnosis of patients with acute coronary syndrome. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01674889183047
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http://dx.doi.org/10.1016/j.bbamcr.2018.10.015DOI Listing
October 2018
17 Reads

A case of early onset life-threatening epilepsy associated with a novel ATP1A3 gene variant.

Brain Dev 2018 Nov 1. Epub 2018 Nov 1.

Division of Molecular Genetics, Institute for Comprehensive Medical Science, Fujita Health University, Toyoake, Japan. Electronic address:

Introduction: Mutations of the ATP1A3 gene are associated with a wide spectrum of neurological disorders including rapid onset dystonia-parkinsonism and alternating hemiplegia of childhood (AHC). The genotype-phenotype correlations in these cases remain unclear however. We here report a pediatric case of catastrophic early life epilepsy, respiratory failure, postnatal microcephaly, and severe developmental disability associated with a novel heterozygous ATP1A3 mutation. Read More

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http://dx.doi.org/10.1016/j.braindev.2018.10.008DOI Listing
November 2018
9 Reads

Channelopathies That Lead to Sudden Cardiac Death: Clinical and Genetic Aspects.

Heart Lung Circ 2019 Jan 4;28(1):22-30. Epub 2018 Oct 4.

Heart Centre AMC, Department of Clinical and Experimental Cardiology, Academic Medical Center, University of Amsterdam, Amsterdam, The Netherlands; Department of Medicine, Columbia University Irving Medical Center, New York, NY, USA.

Forty per cent (40%) of sudden unexpected natural deaths in people under 35 years of age are associated with a negative autopsy, and the cardiac ion channelopathies are the prime suspects in such cases. Long QT syndrome (LQTS), Brugada syndrome (BrS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are the most commonly identified with genetic testing. The cellular action potential driving the heart cycle is shaped by a specific series of depolarising and repolarising ion currents mediated by ion channels. Read More

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http://dx.doi.org/10.1016/j.hlc.2018.09.007DOI Listing
January 2019
2 Reads

Safety, Tolerability, and Feasibility of Young Plasma Infusion in the Plasma for Alzheimer Symptom Amelioration Study: A Randomized Clinical Trial.

JAMA Neurol 2018 Oct 29. Epub 2018 Oct 29.

Department of Neurology and Neurological Sciences, Stanford University, Stanford, California.

Importance: Young mouse plasma restores memory in aged mice, but, to our knowledge, the effects are unknown in patients with Alzheimer disease (AD).

Objective: To assess the safety, tolerability, and feasibility of infusions of young fresh frozen plasma (yFFP) from donors age 18 to 30 years in patients with AD.

Design, Setting, And Participants: The Plasma for Alzheimer Symptom Amelioration (PLASMA) study randomized 9 patients under a double-blind crossover protocol to receive 4 once-weekly infusions of either 1 unit (approximately 250 mL) of yFFP from male donors or 250 mL of saline, followed by a 6-week washout and crossover to 4 once-weekly infusions of an alternate treatment. Read More

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http://archneur.jamanetwork.com/article.aspx?doi=10.1001/jam
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http://dx.doi.org/10.1001/jamaneurol.2018.3288DOI Listing
October 2018
9 Reads

Clinical and molecular characterization of Korean children with infantile and late-onset Pompe disease: 10 years of experience with enzyme replacement therapy at a single center.

Korean J Pediatr 2018 Oct 23. Epub 2018 Oct 23.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

Purpose: Pompe disease (PD) is an autosomal recessive disorder caused by a deficiency of acid alpha-glucosidase resulting from pathogenic GAA variants. The present study described clinical features, genotypes, changes before and after enzyme replacement therapy (ERT), and long-term outcomes in patients with infantile-onset PD (IOPD) and late-onset PD (LOPD) in one tertiary medical center.

Methods: Five Korean patients (two males and three females) diagnosed with PD between 2002 and 2013 at Samsung Medical Center in Seoul, Republic of Korea. Read More

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http://kjp.or.kr/journal/view.php?doi=10.3345/kjp.2018.06968
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http://dx.doi.org/10.3345/kjp.2018.06968DOI Listing
October 2018
11 Reads

The change of QRS duration after pulmonary valve replacement in patients with repaired tetralogy of Fallot and pulmonary regurgitation.

Korean J Pediatr 2018 Nov 24;61(11):362-365. Epub 2018 Oct 24.

Division of Pediatric Cardiology, Department of Pediatrics, Kyungpook National University Children's Hospital, School of Medicine, Kyungpook National University, Daegu, Korea.

Purpose: This study aimed to analyze changes in QRS duration and cardiothoracic ratio (CTR) following pulmonary valve replacement (PVR) in patients with tetralogy of Fallot (TOF).

Methods: Children and adolescents who had previously undergone total repair for TOF (n=67; median age, 16 years) who required elective PVR for pulmonary regurgitation and/or right ventricular out tract obstruction were included in this study. The QRS duration and CTR were measured pre- and postoperatively and postoperative changes were evaluated. Read More

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http://kjp.or.kr/journal/view.php?doi=10.3345/kjp.2018.06765
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http://dx.doi.org/10.3345/kjp.2018.06765DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6258967PMC
November 2018
5 Reads

In Vivo Ryr2 Editing Corrects Catecholaminergic Polymorphic Ventricular Tachycardia.

Circ Res 2018 Sep;123(8):953-963

From the Cardiovascular Research Institute (X.P., L.P., S.K.L., T.A.W., N.L., J.O.R., J.L., X.H.T.W.), Baylor College of Medicine, Houston, TX.

Rationale: Autosomal-dominant mutations in ryanodine receptor type 2 ( RYR2) are responsible for ≈60% of all catecholaminergic polymorphic ventricular tachycardia. Dysfunctional RyR2 subunits trigger inappropriate calcium leak from the tetrameric channel resulting in potentially lethal ventricular tachycardia. In vivo CRISPR/Cas9-mediated gene editing is a promising strategy that could be used to eliminate the disease-causing Ryr2 allele and hence rescue catecholaminergic polymorphic ventricular tachycardia. Read More

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https://www.ahajournals.org/doi/10.1161/CIRCRESAHA.118.31336
Publisher Site
http://dx.doi.org/10.1161/CIRCRESAHA.118.313369DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6206886PMC
September 2018
3 Reads

The Incremental Benefit of Color Tissue Doppler in Fetal Arrhythmia Assessment.

J Am Soc Echocardiogr 2019 Jan 16;32(1):145-156. Epub 2018 Oct 16.

Fetal and Neonatal Cardiology Program, Department of Pediatrics, Division of Cardiology, Women's & Children's Health Research Institute, University of Alberta, Stollery Children's Hospital, Edmonton, Alberta, Canada. Electronic address:

Background: Accurate fetal arrhythmia (FA) diagnosis is key for effective management. Currently, FA assessment relies on standard echocardiography-based techniques (M mode and spectral Doppler), which require adequate fetal position and cursor alignment to define temporal relationships of mechanical events. Few data exist on the application of color Doppler tissue imaging (c-DTI) in FA assessment. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08947317183044
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http://dx.doi.org/10.1016/j.echo.2018.08.009DOI Listing
January 2019
14 Reads

Ablation of Atrial Arrhythmias After the Atriopulmonary Fontan Procedure: Mechanisms of Arrhythmia and Outcomes.

JACC Clin Electrophysiol 2018 Oct;4(10):1338-1346

Department of Cardiology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia; Sydney Medical School, The University of Sydney, Camperdown, New South Wales, Australia. Electronic address:

Objectives: This study sought to describe atrial arrhythmia mechanisms, acute outcomes, and long-term arrhythmia burdens following catheter ablation in adult atriopulmonary (AP) Fontan patients.

Background: Atrial arrhythmias are a significant cause of morbidity and mortality in the AP Fontan population.

Methods: Sixty consecutive atrial arrhythmia ablations were reviewed in 42 AP Fontan patients (31 ± 8 years of age), performed between 1998 and 2017. Read More

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http://dx.doi.org/10.1016/j.jacep.2018.08.012DOI Listing
October 2018
2 Reads

Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients.

JACC Clin Electrophysiol 2018 Oct 27;4(10):1300-1307. Epub 2018 Jun 27.

Arrhythmia Center, Department of Cardiology, University of California, Los Angeles, Los Angeles, California.

Objectives: The purpose of this study was to determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA).

Background: EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population. Read More

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http://dx.doi.org/10.1016/j.jacep.2018.05.009DOI Listing
October 2018
2 Reads

Predictive power of a single body temperature at different cutoff values for neonates in the nursery transferring to special care nursery.

Medicine (Baltimore) 2018 Oct;97(42):e12619

Department of Pediatric Emergency Medicine, Children's Hospital, China Medical University.

The aim of this study was to identify the clinical parameters indicative of serious etiology of neonatal hyperthermia and to determine the appropriate cutoff value of body temperature (BT) for predicting the need to transfer the newborn to the special care (SC) nursery.The nursery records of newborns diagnosed with hyperthermia between 2007 and 2013 were retrospectively reviewed. The clinical characteristics of newborns with hyperthermia remained in the nursery were compared with those transferred to the SC nursery. Read More

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http://dx.doi.org/10.1097/MD.0000000000012619DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6211842PMC
October 2018
2 Reads

Assessment of Efficacy of Oral Rehydration Salts in Children With Neurally Mediated Syncope of Different Hemodynamic Patterns.

J Child Neurol 2018 Oct 16:883073818803035. Epub 2018 Oct 16.

1 Department of Pediatric Cardiovasology, Children's Medical Center, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.

Objective: To assess the efficacy of oral rehydration salts in children with neurally mediated syncope of different hemodynamic types.

Methods: Children with unexplained syncope or pre-syncope who visited or were hospitalized between March 2012 and February 2015 were enrolled in the study. Checked by the head-up tilt test, 105 children (aged 4-18 years, with a mean age of 11. Read More

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http://dx.doi.org/10.1177/0883073818803035DOI Listing
October 2018
3 Reads

Fetal hyperthyroidism associated with maternal thyroid autoantibodies: A case report.

Case Rep Womens Health 2018 Oct 26;20:e00081. Epub 2018 Sep 26.

Department of Endocrinology and Diabetes, "Alexandra" Hospital, Athens, Greece.

A 33-year-old Caucasian woman was referred at 24 + 3 weeks of gestation due to fetal tachycardia and hydrops. She had an uncomplicated pregnancy 16 years previously and was on levothyroxine after total thyroidectomy for Graves' disease 6 years previously, when she developed moderate exophthalmos. Laboratory evaluation revealed appropriate thyroid function for this time of gestation: thyroid stimulating hormone (TSH) 1. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22149112183011
Publisher Site
http://dx.doi.org/10.1016/j.crwh.2018.e00081DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171484PMC
October 2018
17 Reads

The gastrointestinal symptoms present in patients with postural tachycardia syndrome: A review of the literature and overview of treatment.

Auton Neurosci 2018 Dec 8;215:70-77. Epub 2018 Sep 8.

Department of Neurology; Medical College of Wisconsin, United States of America.

Orthostatic intolerance, including postural tachycardia syndrome, is often associated with gastrointestinal symptoms. In the vast majority of the cases, the gastrointestinal symptoms are not secondary to the orthostatic disorder, but rather just a comorbid condition. This concept is critical, since treatment aimed at the orthostatic condition will not improve the gastrointestinal symptoms. Read More

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http://dx.doi.org/10.1016/j.autneu.2018.09.003DOI Listing
December 2018
2 Reads

External Validation of the "Quick" Pediatric Logistic Organ Dysfunction-2 Score Using a Large North American Cohort of Critically Ill Children With Suspected Infection.

Pediatr Crit Care Med 2018 Dec;19(12):1114-1119

Research Institute, BC Children's Hospital (BCCH), Vancouver, BC, Canada.

Objectives: A quick Pediatric Logistic Organ Dysfunction 2 score on day 1, consisting of tachycardia, hypotension, and altered mentation, was shown to predict mortality with an area under the receiver operating characteristic curve of 82% (95% CI, 76-87%) in children admitted to a PICU with suspected infection. We performed an external validation of the quick Pediatric Logistic Organ Dysfunction 2, including its performance in predicting mortality in specific age groups.

Design: Analysis of retrospective data obtained from the Virtual Pediatric Systems PICU registry. Read More

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http://Insights.ovid.com/crossref?an=00130478-900000000-9837
Publisher Site
http://dx.doi.org/10.1097/PCC.0000000000001729DOI Listing
December 2018
10 Reads

Trial of Fingolimod versus Interferon Beta-1a in Pediatric Multiple Sclerosis.

N Engl J Med 2018 09;379(11):1017-1027

From the Partners Pediatric Multiple Sclerosis Center, Massachusetts General Hospital, Boston (T.C.); Montreal Neurological Institute, McGill University, and NeuroRx Research - both in Montreal (D.L.A.); Children's Hospital of Philadelphia (B.B.) and the Center for Neuroinflammation and Experimental Neurotherapeutics and the Department of Neurology (A.B.-O.), Perelman School of Medicine, University of Pennsylvania - all in Philadelphia; the Department of Neuropathology (W.B.) and the Department of Pediatrics and Adolescent Medicine, German Center for Multiple Sclerosis in Childhood and Adolescence (J.G.), University Medical Center Göttingen, Göttingen, and the Division of Pediatric Neurology, Children's Hospital Datteln, Witten/Herdecke University, Datteln (K.R.) - all in Germany; Gallarate Hospital, Gallarate, Italy (A.G.); Blizard Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London (G.G.); the University of Texas Southwestern Medical Center, Children's Health, Dallas (B.G.), and McGovern Medical School, University of Texas Health Science Center at Houston, Houston (J.S.W.) - both in Texas; Pediatric Multiple Sclerosis Center at NYU Langone, New York (L.K.); Hôpitaux Universitaires Paris-Sud, Assistance Publique-Hôpitaux de Paris, Paris (M.T.); the Department of Neurology, University of California at San Francisco, San Francisco (E.W.); Novartis Pharmaceuticals, East Hanover, NJ (T.S., Y.C., N.P.); and Novartis Pharma, Basel, Switzerland (M.M.).

Background: Treatment of patients younger than 18 years of age with multiple sclerosis has not been adequately examined in randomized trials. We compared fingolimod with interferon beta-1a in this population.

Methods: In this phase 3 trial, we randomly assigned patients 10 to 17 years of age with relapsing multiple sclerosis in a 1:1 ratio to receive oral fingolimod at a dose of 0. Read More

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http://www.nejm.org/doi/10.1056/NEJMoa1800149
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http://dx.doi.org/10.1056/NEJMoa1800149DOI Listing
September 2018
30 Reads