Full Text Journal Articles about
Pediatric Dilated Cardiomyopathy

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Uncovering sleep in young males with Duchenne muscular dystrophy.

Amy G Hartman, Lauren Terhorst, Natalie Little, Roxanna M Bendixen,

OBJECTIVES:Sleep health in rare disease is often overlooked due to the complex nature of the disease. For males with Duchenne muscular dystrophy, sleep assessment is typically focused on pulmonary function and identification of sleep disordered breathing. Unfortunately for young boys with Duchenne muscular dystrophy, sleep assessment is often neglected, resulting ... Read more >>

Eur. J. Paediatr. Neurol. (European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society)
[2020, :]

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Peak Cough Flow in Children with Neuromuscular Disorders.

Nidhi Kotwal, Prateek J Shukla, Geovanny F Perez,

PURPOSE:Patients with neuromuscular disease (NMD) experience weakened cough due to progressive respiratory muscle weakness. Peak cough flow (PCF) measurements derived from adult populations are used to recommend initiation of assisted cough therapies. The objective of this study was to characterize PCF values among pediatric patients with NMD. METHODS:Retrospective chart review ... Read more >>

Lung (Lung)
[2020, :]

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Implantable Cardioverter Defibrillator Use in Males with Duchenne Muscular Dystrophy and Severe Left Ventricular Dysfunction.

Michael A McCulloch, Ashwin K Lal, Kenneth Knecht, Ryan J Butts, Chet R Villa, Jonathan N Johnson, Jennifer Conway, Matthew J Bock, Kurt R Schumacher, Sabrina P Law, Joshua M Friedland-Little, Shriprasad R Deshpande, Shawn C West, Irene D Lytrivi, Katheryn E Gambetta, Carol A Wittlieb-Weber,

Duchenne muscular dystrophy (DMD) is characterized by myocardial fibrosis and left ventricular (LV) dysfunction. Implantable cardioverter defibrillator (ICD) use has not been characterized in this population but is considered for symptomatic patients with severe LV dysfunction (SLVD) receiving guideline-directed medical therapy (GDMT). We evaluated ICD utilization and efficacy in patients ... Read more >>

Pediatr Cardiol (Pediatric cardiology)
[2020, :]

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The association between trunk control and upper limb functions of children with Duchenne muscular dystrophy.

Numan Bulut, Ipek Alemdaroğlu-Gürbüz, Haluk Topaloğlu, Öznur Yılmaz, Ayşe Karaduman,

Introduction: The aim of this study was to investigate whether trunk control is associated with the upper limb function of children with Duchenne Muscular Dystrophy (DMD).Methods: The children included in the study were divided into two groups according to the Trunk Control Measurement Scale total scores. Twenty-five children whose trunk ... Read more >>

Physiother Theory Pract (Physiotherapy theory and practice)
[2020, :1-9]

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Ventricular Tachycardia in an Infant Without Congenital Anomaly: A Case Report.

Nouhad El Joueid, Marianne Touma Boulos, Simon Abou Jaoude, Linda Daou,

Ventricular tachycardia (VT) is a serious form of arrhythmia that can be life-threatening; that's why diagnosis and treatment are very important in order to avoid serious complications. We are reporting this case of VT which is a rare entity, especially, in healthy infants. This infant, without cardiac pathology known from ... Read more >>

Cardiol Res (Cardiology research)
[2020, 11(1):61-65]

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Recombinant human insulin-like growth factor-1 therapy for 6 months improves growth but not motor function in boys with Duchenne muscular dystrophy.

Meilan M Rutter, Brenda L Wong, James J Collins, Hemant Sawnani, Michael D Taylor, Paul S Horn, Philippe F Backeljauw,

INTRODUCTION:Recombinant human insulin-like growth factor-1 (rhIGF-1) is a growth factor and has anabolic effects on muscle. We investigated whether rhIGF-1 therapy: 1) improves or preserves muscle function; and 2) improves growth in boys with Duchenne muscular dystrophy (DMD). METHODS:In this study we compared prepubescent, ambulatory, glucocorticoid-treated boys with DMD (n ... Read more >>

Muscle Nerve (Muscle & nerve)
[2020, :]

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Shedding light on pediatric diseases: multispectral optoacoustic tomography at the doorway to clinical applications.

Adrian P Regensburger, Alexandra L Wagner, Jing Claussen, Maximilian J Waldner, Ferdinand Knieling,

Optoacoustic imaging (OAI), or photoacoustic imaging (PAI), has fundamentally influenced basic science by providing high-resolution visualization of biological mechanisms. With the introduction of multispectral optoacoustic tomography (MSOT), these technologies have now moved closer to clinical applications. MSOT utilizes short-pulsed near-infrared laser light to induce thermoelastic expansion in targeted tissues. This ... Read more >>

Mol Cell Pediatr (Molecular and cellular pediatrics)
[2020, 7(1):3]

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Early experience with the HeartMate 3 continuous-flow ventricular assist device in pediatric patients and patients with congenital heart disease: A multicenter registry analysis.

Matthew J O'Connor, Angela Lorts, Ryan R Davies, Francis Fynn-Thompson, Anna Joong, Katsuhide Maeda, Christopher E Mascio, Patrick I McConnell, Michael C Mongé, Deipanjan Nandi, David M Peng, David N Rosenthal, Ming-Sing Si, David L Sutcliffe, Christina J VanderPluym, Melita Viegas, Farhan Zafar, Matthew Zinn, David L S Morales,

BACKGROUND:The HeartMate 3 ventricular assist device (VAD) is a newer centrifugal continuous-flow VAD used for bridge-to-transplant and destination therapy in adults. However, there is limited experience regarding its use in children and adults with complex congenital heart disease (CHD). METHODS:The Advanced Cardiac Therapies Improving Outcomes Network (ACTION) is a multicenter ... Read more >>

J. Heart Lung Transplant. (The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation)
[2020, :]

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The relationship between electrocardiographic data and mortality in children diagnosed with dilated cardiomyopathy.

Mehmet Türe, Hasan Balık, Alper Akın, Meki Bilici, Ahmet Nergiz,

The mortality causes of patients followed up due to dilated cardiomyopathy (DCM) include complications related to heart failure, ventricular arrhythmia, and transplantation. This study aims to evaluate the electrocardiographic findings of patients diagnosed with dilated cardiomyopathy and determine its relationship with mortality. The electrocardiographic, clinical, and laboratory findings of patients ... Read more >>

Eur. J. Pediatr. (European journal of pediatrics)
[2020, :]

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A significance of school screening electrocardiogram in the patients with ventricular noncompaction.

Keiichi Hirono, Nariaki Miyao, Masao Yoshinaga, Eiki Nishihara, Kazushi Yasuda, Shigeru Tateno, Mamoru Ayusawa, Naokata Sumitomo, Hitoshi Horigome, Mari Iwamoto, Hideto Takahashi, Seiichi Sato, Shigetoyo Kogaki, Seiko Ohno, Tadayoshi Hata, Daisuke Hazeki, Naomi Izumida, Masami Nagashima, Kunio Ohta, Nobuo Tauchi, Hiroya Ushinohama, Shozaburo Doi, Fukiko Ichida, ,

Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy and is associated with high morbidity and mortality. However, the role and significance of school screening for LVNC have not been fully elucidated. In this multicenter, retrospective cohort study, a total of 105 children with LVNC were included from 2000 to 2017. ... Read more >>

Heart Vessels (Heart and vessels)
[2020, :]

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Latent process model of the 6-minute walk test in Duchenne muscular dystrophy : A Bayesian approach to quantifying rare disease progression.

Janelle L Lennie, John T Mondick, Marc R Gastonguay,

Duchenne muscular dystrophy (DMD) is a rare X-linked genetic pediatric disease characterized by a lack of functional dystrophin production in the body, resulting in muscle deterioration. Lower body muscle weakness progresses to non-ambulation typically by early teenage years, followed by upper body muscle deterioration and ultimately death by the late ... Read more >>

J Pharmacokinet Pharmacodyn (Journal of pharmacokinetics and pharmacodynamics)
[2020, 47(1):91-104]

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Bridge to recovery with Berlin Heart EXCOR in children <10 kg with dilated cardiomyopathy: a histological analysis.

Yuji Tominaga, Takayoshi Ueno, Takashi Kido, Tomomitsu Kanaya, Jun Narita, Hidekazu Ishida, Koichi Toda, Toru Kuratani, Yoshiki Sawa,

OBJECTIVES:This study aimed to identify the histological characteristics associated with bridge to recovery using Berlin Heart EXCOR® (BHE) in paediatric patients <10 kg with dilated cardiomyopathy. METHODS:Of the 10 consecutive patients <10 kg with dilated cardiomyopathy who underwent BHE implantation between 2013 and 2018, 4 patients showed improvement in left ventricular (LV) ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, :]

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Cardiac phenotype in propionic acidemia - Results of an observational monocentric study.

A Kovacevic, S F Garbade, G F Hoffmann, M Gorenflo, S Kölker, C Staufner,

BACKGROUND:Propionic acidemia (PA) is an organic aciduria caused by inherited deficiency of propionyl-CoA carboxylase. Left ventricular dysfunction and QT prolongation may lead to life-threatening complications. Systematic analyses of cardiac phenotypes, in particular effects of specific cardiac therapies, are scarce. METHODS:In this longitudinal observational monocentric study (data from 1989 to 2017) ... Read more >>

Mol. Genet. Metab. (Molecular genetics and metabolism)
[2020, :]

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Dilated cardiomyopathy in children: moving beyond traditional pharmacologic therapy.

Jordan A Cohen, Melvin C Almodovar,

PURPOSE OF REVIEW:Dilated cardiomyopathy (DCM) is a rare myocardial disorder characterized by a dilated left ventricle and systolic dysfunction. Globally, it affects around 1 in every 100 000 children. The prognosis is generally poor, with 40% either failing traditional medical therapy within the first 2 years or requiring a heart transplant. ... Read more >>

Curr. Opin. Cardiol. (Current opinion in cardiology)
[2020, 35(1):52-57]

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Desmin-related myopathy characterized by non-compaction cardiomyopathy, cardiac conduction defect, and coronary artery dissection.

Ran Tamiya, Yuki Saito, Daisuke Fukamachi, Koichi Nagashima, Yoshihiro Aizawa, Kimie Ohkubo, Takumi Hatta, Akira Sezai, Masashi Tanaka, Taisuke Ishikawa, Naomasa Makita, Naokata Sumitomo, Yasuo Okumura,

Desmin-related myopathy (DRM) is a rare heritable cardiac and skeletal muscle disease caused by mutations in the desmin gene (DES). DRM is generally characterized by skeletal muscle weakness, conduction disturbance, and dilated cardiomyopathy. However, the clinical cardiac phenotypes of DRM are not yet fully understood. Herein, we report the first ... Read more >>

ESC Heart Fail (ESC heart failure)
[2020, :]

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Proteomic profiling of fatty acid binding proteins in muscular dystrophy.

Paul Dowling, Stephen Gargan, Margit Zweyer, Dieter Swandulla, Kay Ohlendieck,

Introduction: Duchenne muscular dystrophy is a neuromuscular disorder, which is caused by abnormalities in the DMD gene that encodes the membrane cytoskeletal protein dystrophin. Besides progressive skeletal muscle wasting, dystrophinopathy also affects non-skeletal muscle tissues, including cells in the cardio-respiratory system, the central nervous system, the liver and the kidney.Areas ... Read more >>

Expert Rev Proteomics (Expert review of proteomics)
[2020, 17(2):137-148]

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Prediction of postnatal clinical course in primary congenital dilated cardiomyopathy.

Hidenori Yamamoto, Yoshie Fukasawa, Naoki Ohashi, Takehiko Yokoyama, Kazutaka Suzuki, Takaya Ota, Kazushi Yasuda, Kentaro Omoya, Yoshiyuki Takahashi, Taichi Kato,

BACKGROUND:The aim of this study was to investigate the prediction of postnatal prognosis using fetal and perinatal data in patients with primary congenital dilated cardiomyopathy (PCDCM), and to estimate the incidence of this disease. METHODS:We examined correlations between fetal or perinatal data and postnatal clinical course in a multicenter retrospective ... Read more >>

Pediatr Int (Pediatrics international : official journal of the Japan Pediatric Society)
[2019, 61(12):1196-1201]

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Muscle MRI: A biomarker of disease severity in Duchenne muscular dystrophy? A systematic review.

Juliette Ropars, France Gravot, Douraied Ben Salem, François Rousseau, Sylvain Brochard, Christelle Pons,

OBJECTIVE:To assess the evidence of a relationship between muscle MRI and disease severity in Duchenne muscular dystrophy (DMD). METHODS:We conducted a systematic review of studies that analyzed correlations between MRI measurements and motor function in patients with DMD. PubMed, Cochrane, Scopus, and Web of Science were searched using relevant keywords ... Read more >>

Neurology (Neurology)
[2020, 94(3):117-133]

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Clinical exome sequencing revealed that FLNC variants contribute to the early diagnosis of cardiomyopathies in infant patients.

Feifan Xiao, Qiufen Wei, Bingbing Wu, Xu Liu, Aiyao Mading, Lin Yang, Yan Li, Fang Liu, Xinnian Pan, Huijun Wang,

Background:FLNC encodes actin-binding protein and is mainly concentrated in skeletal and cardiac muscle. Mutations in FLNC were found in cardiomyopathies. To date, studies on FLNC-cardiomyopathies have mainly been reported in adults. There are limited studies that have investigated FLNC variants in pediatric patients with cardiomyopathies. Methods:We summarized the patients who ... Read more >>

Transl Pediatr (Translational pediatrics)
[2020, 9(1):21-33]

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Increased dystrophin production with golodirsen in patients with Duchenne muscular dystrophy.

Diane E Frank, Frederick J Schnell, Cody Akana, Saleh H El-Husayni, Cody A Desjardins, Jennifer Morgan, Jay S Charleston, Valentina Sardone, Joana Domingos, George Dickson, Volker Straub, Michela Guglieri, Eugenio Mercuri, Laurent Servais, Francesco Muntoni, ,

OBJECTIVE:To report safety, pharmacokinetics, exon 53 skipping, and dystrophin expression in golodirsen-treated patients with Duchenne muscular dystrophy (DMD) amenable to exon 53 skipping. METHODS:Part 1 was a randomized, double-blind, placebo-controlled, 12-week dose titration of once-weekly golodirsen; part 2 is an ongoing, open-label evaluation. Safety and pharmacokinetics were primary and secondary ... Read more >>

Neurology (Neurology)
[2020, :]

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Paediatric cardiomyopathies: echocardiographic diagnosis, clinical profile, and demographic characteristics: the experience of a tertiary referral centre for Latin American paediatric cardiology.

Víctor M Huertas-Quiñones, Camilo F Mestra, Valeria Peña-Trujillo, Sebastián Gallo-Bernal, Mariana Villaveces, Laura C Alarcón-Forero,

BACKGROUND:Although multiple studies have been conducted in the adult population, there is a vast knowledge gap regarding the epidemiologic characteristics of cardiomyopathies in the paediatric population. This issue is even more crucial when the precarious situation of medical research in Latin America is considered. Given the potential impact that these ... Read more >>

Cardiol Young (Cardiology in the young)
[2020, :1-6]

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Neurodevelopmental, behavioral, and emotional symptoms common in Duchenne muscular dystrophy.

Andrew J Darmahkasih, Irina Rybalsky, Cuixia Tian, Karen C Shellenbarger, Paul S Horn, Joshua T Lambert, Brenda L Wong,

INTRODUCTION:We studied neurodevelopmental and behavioral/emotional symptoms in patients with Duchenne muscular dystrophy (DMD). METHODS:Retrospective case series of neurodevelopmental and behavioral/emotional symptoms obtained through review of systems of 700 DMD patients in relation to dystrophin gene mutations. RESULTS:The most common symptoms encountered were emotional/behavioral dysregulation (38.7%), inattention/hyperactive features (31.4%), obsessive and ... Read more >>

Muscle Nerve (Muscle & nerve)
[2020, 61(4):466-474]

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Cecal Volvulus in a child with congenital dilated cardiomyopathy: A case report.

Ahmed E Shehata, Mohamed A Helal, EzzElDien A Ibrahim, Basma Magdy, Mohamed El Seoudy, Muayad Shaban, Heba Taher,

INTRODUCTION:Cecal volvulus is an extremely rare cause of intestinal obstruction in the pediatric age group and its incidence is unknown. PRESENTATION OF CASE:We present a unique case of cecal volvulus in a 3 year old female with congenital dilated cardiomyopathy. DISCUSSION:Cecal mobility due to malfixation and malrotation is the main ... Read more >>

Int J Surg Case Rep (International journal of surgery case reports)
[2020, 66:30-32]

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Childhood-onset Takayasu Arteritis.

Florence A. Aeschlimann, Marinka Twilt, Rae S. M. Yeung,

Childhood-onset Takayasu Arteritis (cTAK) is a rare, large-vessel type of vasculitis seen in children, mainly affecting the aorta and its major branches. Clinical manifestations are often severe and arise as a result of systemic and local inflammation, along with end-organ ischemia. Disease flares are common and the disease burden is ... Read more >>

(European Journal of Rheumatology)
[2020, 7(Suppl 1):S58-S66]

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Risk Factors for Cardiac and Non-cardiac Causes of Death in Males with Duchenne Muscular Dystrophy.

Carol A Wittlieb-Weber, Kenneth R Knecht, Chet R Villa, Chentel Cunningham, Jennifer Conway, Matthew J Bock, Katheryn E Gambetta, Ashwin K Lal, Kurt R Schumacher, Sabrina P Law, Shriprasad R Deshpande, Shawn C West, Joshua M Friedland-Little, Irene D Lytrivi, Michael A McCulloch, Ryan J Butts, David R Weber, Jonathan N Johnson,

As survival and neuromuscular function in Duchenne muscular dystrophy (DMD) have improved with glucocorticoid (GC) therapy and ventilatory support, cardiac deaths are increasing. Little is known about risk factors for cardiac and non-cardiac causes of death in DMD. A multi-center retrospective cohort study of 408 males with DMD, followed from ... Read more >>

Pediatr Cardiol (Pediatric cardiology)
[2020, :]

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