A Case of Nemaline Myopathy With Associated Dilated Cardiomyopathy and Respiratory Failure
スポンサーリンク
概要
- 論文の詳細を見る
Nemaline myopathy is a representative form of congenital myopathy, and is characterized by nemaline bodies in muscle fibers. Here we report a 47-year-old man with congenital nemaline myopathy complicated with dilated cardiomyopathy-related heart failure, and restrictive respiratory failure. The complication of dilated cardiomyopathy in nemaline myopathy has rarely been reported. In this case, nemaline bodies were detected in the cardiac muscle fibers, demonstrating the presence of underlying disease-related myocardial degeneration. The patient responded to the combination of conventional therapy for heart failure including β-blocker and noninvasive continuous positive-pressure ventilation for respiratory failure. His general condition has been stable during a 10-month follow up period.
著者
-
Kamimura Daisuke
Department Of Cardiology Sagamihara Hospital
-
Uchino Kazuaki
Department Of Cardiology Sagamihara National Hospital
-
Umemura Satoshi
Department Of Internal Medicine Ii Yokohama City University School Of Medicine
-
Shimizu Makoto
Department Of Agricultural Chemistry Faculty Of Engineering The University Of Tokyo
-
Saito Toshihiko
Department Of Internal Medicine Tokyo Medical College
-
Suzuki Yoji
Department Of Biochemistry University Of School Of Pharmaceutical Science
-
Miwa Yoshiyuki
Department of Gastroenterology, Gifu University Graduate School of Medicine
-
Toyama Hideshi
Department of Cardiology, Yaizu City Hospital
-
Nagata Rihito
Department of Cardiology, Yaizu City Hospital
-
Dejima Tohru
Department of Cardiology, Yaizu City Hospital
-
Inada Haruhiko
Department of Cardiology, Yaizu City Hospital
-
Kamimura Daisuke
Department of Cardiology, Yaizu City Hospital
-
Saito Toshihiko
Department of Cardiology, Yaizu City Hospital
-
Uchino Kazuaki
Department of Medical Science and Cardiorenal Medicine, Yokohama City University Graduate School of Medicine
関連論文
- PJ-004 Inflammatory Markers at the Site of Culprit Coronary Plaque in Acute Myocardial Infarction(Acute myocardial infarction, clinical (pathophysiology)-3 (IHD) PJ1,Poster Session (Japanese),The 70th Anniversary Annual Scientific Meeting of the Japanese
- Early, Accurate, Non-Invasive Predictors of Left Main or 3-Vessel Disease in Patients With Non-ST-Segment Elevation Acute Coronary Syndrome
- Effects of angiotension II type 1 receptor blocker on ambulatory blood pressure variability in hypertensive patients with overt diabetic nephropathy
- PJ-635 Effect of Olmesartan on Tissue Expression Balance between Angiotensin II Receptor and Its Inhibitory Binding Molecule(PJ107,Cardiomyopathy/Hypertrophy (Basic, Clinical) (M),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanes
- Effect of LDL Apheresis on Atherosclerosis Goes beyond Its Cholesterol Lowering in Hemodialysis Patients : A Pilot Study
- Behcet's disease complicated by IgA nephropathy with nephrotic syndrome
- OE-254 Overexpression of ATRAP(AT1 receptor-assosiated protein) in cardiomyocytes completely inhibits cardiac hypertrophy induced by Angiotensin(Cardiac hypertrophy, basic/clinical(01)(M),Oral Presentation(English),The 72nd Annual Scientific Meeting of th
- OE-061 Cardiorenal Relationship-A role of Na channel-Nedd4L-proteasome system for essential hypertension and angiotensin II (All) induced transcriptional changes in cardiomyocyte.(Hypertension, basic(01)(H),Oral Presentation(English),The 72nd Annual Scien
- NEDD4L protein truncating variant (v13[G/A] : rs4149601) is associated with essential hypertension in a sample of the Japanese population
- PJ-685 Prognostic Value of APACHE II Score for Patients Suffering Massive Pulmonary Embolism(PJ115,Thromboembolism/Antithrombotic Therapy/Thrombolysis (IHD),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanese Circulation Society)
- Association of SLC6A9 Gene Variants with Human Essential Hypertension
- Novel Method of Predicting the Optimal Atrioventricular Delay in Patients With Complete AV Block, Normal Left Ventricular Function and an Implanted DDD Pacemaker
- Long-Term Follow-up of Neointimal Coverage of Sirolimus-Eluting Stents : Evaluation With Optical Coherence Tomography
- Treatment With Recombinant Placental Growth Factor (PlGF) Enhances Both Angiogenesis and Arteriogenesis and Improves Survival After Myocardial Infarction
- PJ-702 Non-contact Mapping System Accurately Localize Right-sided APs in Ablation of Type B WPW Syndrome(PJ118,Arrhythmia, Others (Clinical/Diagnosis/Treatment) 6 (A),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanese Circulation
- Measurement of Hemodynamics During Postural Changes Using a New Wearable Cephalic Laser Blood Flowmeter
- Plaque Location in the Left Anterior Descending Coronary Artery and Tissue Characteristics in Angina Pectoris : An Integrated Backscatter Intravascular Ultrasound Study
- Left ventricular geometry, risk factors, and outcomes of hospitalized patients with diastolic heart failure in Japan
- A case of giant coronary artery aneurysm and literature review
- PJ-815 Can Integrated Backscatter Intravascular Ultrasound Predict the Thickness of Coronary Calcification as Assessed by Optical Coherence Tomography(PJ137,Intravascular Imagings 1 (I),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The J
- PJ-768 Role of Myocardial Injury and Inflammation in Excess Elevation of N-Terminal ProBrain Natriuretic Peptide in Patients with Coronary Artery Disease(PJ129,Myocardial Ischemia-reperfusion (Basic, Clinical) (IHD),Poster Session (Japanese),The 73rd Annu
- PJ-766 Acute Hyperglycemia Attenuates the Beneficial Effects of Preinfarction Angina in Patients with Acute Myocardial Infarction Treated with Fibrinolysis(PJ129,Myocardial Ischemia-reperfusion (Basic, Clinical) (IHD),Poster Session (Japanese),The 73rd An
- PJ-656 Non-high-density Lipoprotein Cholesterol Levels are Associated with Multiple Complex Coronary Lesions in Patients with Acute Myocardial Infarction(PJ110,ACS/AMI (Clinical/Pathophysiology) 1 (IHD),Poster Session (Japanese),The 73rd Annual Scientific
- PJ-635 Effect of Olmesartan on Tissue Expression Balance between Angiotensin II Receptor and Its Inhibitory Binding Molecule(PJ107,Cardiomyopathy/Hypertrophy (Basic, Clinical) (M),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanes
- PJ-521 Association between Admission Neutrophil to Lymphocyte Ratio and Angiographical Findings in Patients with Non-ST-Segment Elevation Acute Coronary Syndrome(PJ088,ACS/AMI (Clinical/Pathophysiology) 2 (IHD),Poster Session (Japanese),The 73rd Annual Sc
- PJ-485 Ratio of Late Transmitral Velocity to Averaged Mitral Annular Late Diastolic Velocity is Powerful Index after a First ST-Elevation AMI(PJ082,Echo/Doppler (Myocardium) (I),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanese
- PJ-386 E/e' is the Strongest Predictor of Major Adverse Cardio-Cerebrovascular Events in Patients with a First Anterior ST-Elevation Acute Myocardial Infarction(PJ065,ACS/AMI (Clinical/Treatment) 3 (IHD),Poster Session (Japanese),The 73rd Annual Scientifi
- PJ-283 Severely Impaired Left Ventricular Ejection Fraction is a Risk Factor of Pulmonary Toxicity in Patients with Long-term Administration of Amiodarone(PJ048,Arrhythmia, Others (Clinical/Diagnosis/Treatment) 3 (A),Poster Session (Japanese),The 73rd Ann
- Efficacy of Consistent Atrial Pacing Algorithm for Suppression of Atrial Arrhythmias in Patients With Sick Sinus Syndrome and Atrial Fibrillation
- PJ-598 Preferential Decrease of Renal Klotho Expression in Diabetic Nephropathy Correlated with Tissue Hypoxia(PJ100,Kidney/Renal Circulation/CKD 5 (H),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanese Circulation Society)
- PJ-264 Impact of Drug-Eluting Stents on the Peristent Plaque Compositions : A Serial Volumetric Analysis Using Quantitative Integrated Backscatter Intravascular Ultrasound(PJ045,Coronary Revascularization, PCI (DES) 1 (IHD),Poster Session (Japanese),The 7
- PJ-230 Medial Annular E/e' has a Strongest Relationship with Plasma BNP among Echocardiographical Parameters after a First ST-Elevation AMI(PJ039,Cardiac Function (Clinical) 2 (M),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanes
- Smoking cessation is associated with increased plasma adiponectin levels in men
- PJ-257 Clinical Characteristics and Prognosis of Patients with Acute Myocardial Infarction due to Left Main Coronary Artery Disease(PJ043,ACS/AMI (Clinical/Treatment) 2 (IHD),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanese Cir
- PJ-631 Cardiac Autonomic Nervous System is impaired in Churg-Strauss Syndrome(Autonomic Nervous System 5 (H) : PJ106)(Poster Session (Japanese))
- Matrix Metalloprpteinas-2 Levels Changes May Predict Restenosisafter PTCA
- PJ-050 Impact of Pericardial Fat on Coronary Atherosclerosis(PJ009,CT/MRI (Coronary/Vascular) 4 (I),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanese Circulation Society)
- PE-361 Impact of Brain Natriuretic Peptide in Predicting Reperfusion Injury Immediately after Reperfusion in Patients with ST-segment Elevation Acute Myocardial Infarction(PE061,ACS/AMI (Clinical/Pathophysiology) 2 (IHD),Poster Session (English),The 73rd
- PJ-865 Differences in Clinical Courses Between Massive Pulmonary Embolism and Acute Myocardial Infarction Requiring Percutaneous Cardiopulmonary Life Support(Emergency care / CPR(05)(H),Poster Session(Japanese),The 72nd Annual Scientific Meeting of the Ja
- PJ-685 Transmural Myocardial Strain Profile is an Useful Method to Estimate the Regional Myocardial Damage after Reperfused ST-Elevation Acute Myocardial Infarction(Echo / Doppler(17)(I),Poster Session(Japanese),The 72nd Annual Scientific Meeting of the J
- PJ-583 Does Statin Pre-treatment Promote Thrombolysis in Patients with Acute Myocardial Infarction Treated with Thrombolytic Therapy?(Acute myocardial infarction, clinical(diagnosis/treatment)(11)(IHD),Poster Session(Japanese),The 72nd Annual Scientific M
- PJ-088 Impact of Early Reperfusion Therapy on The Regional Left Ventricular Relaxation of ST-elevation Acute Coronary Syndrome : Tissue Strain Imaging Study(Echo/Doppler(12)(I),Poster Session(Japanese),The 72nd Annual Scientific Meeting of the Japanese Ci
- PE-269 Does Tissue Characteristics Differ Between Proximal and Distal Segment of Left Anterior Descending Coronary Artery? : Integrated Backscatter Intravascular Ultrasound Study(Atherosclerosis, clinical(06)(IHD),Poster Session(English),The 72nd Annual S
- PE-245 Impact of Plaque Rupture on Infarct Size in ST-Segment Elevation Anterior Acute Myocardial Infarction (Final Results)(Acute myocardial infarction, clinical(pathophysiology)(02)(IHD),Poster Session(English),The 72nd Annual Scientific Meeting of the
- OJ-259 Impact of Hyperinsulinemia on Tissue Characteristics of Non-culprit Plaque in Non-diabetic Patients with Acute Coronary Syndrome(Diabetes / Obesity / Metabolic syndrome(03)(H),Oral Presentation (Japanese),The 72nd Annual Scientific Meeting of the J
- FRS-098 Strain-rate by Tissue Strain Imaging is Powerful Tool to Evaluate Ischemic Diastolic Stunning Course after Reperfused ST-Elevation Acute Coronary Syndrome(Leading Edge in Echocardiographic Technologies(2)(I),Featured Research Session,The 72nd Annu
- FRS-044 Deep Ultrasound Attenuation Detected by Intravascular Ultrasound Impacts Quantitative Coronary Flow during Percutaneous Coronary Intervention in Acute Myocardial Infarction(New Clinical Approach for Acute Myocardial Infarction(IHD),Featured Resear
- PJ-477 Transmural Myocardial Strain Imaging is a Powerful Tool for Evaluating the Myocardial Stunning in Reperfused ST Elevation Acute Coronary Syndrome(Echo/Doppler-19, The 71st Annual Scientific Meeting of the Japanese Circulation Society)
- PJ-643 Tissue-Specific Regulation of ATRAP, A Novel Interacting Molecule with AT1 Receptor, in Spontaneously Hypertensive Rats(Hypertension, basic-3, The 71st Annual Scientific Meeting of the Japanese Circulation Society)
- Effects of Right Atrial Pacing Preference in Prevention of Paroxysmal Atrial Fibrillation : Atrial Pacing Preference Study (APP Study)
- PJ-219 Novel Method of Predicting Optimal Atrioventricular Delay using Phonocardiogram in Patients with Cardiac Resynchronization Therapy(Arrhythmia, therapy(24)(A),Poster Session(Japanese),The 72nd Annual Scientific Meeting of the Japanese Circulation So
- PJ-380 Prevalence of Sleep Disorderd Breathing and Effect of Biventricular Pacing in Patients with Heart Failure(Heart failure, clinical-11, The 71st Annual Scientific Meeting of the Japanese Circulation Society)
- 3 Assessment of Regional Wall Motion during Pacing Therapy using Strain Doppler Image(Identification Strategy and Treatment Approach of the Non-responder Group in Patients Withcardiac Resynchronization Therapy using biventricular Pacing, The 71st Annual S
- Reverse Perfusion-Metabolism Mismatch Predicts Good Prognosis in Patients Undergoing Cardiac Resynchronization Therapy : A Pilot Study
- Daily Shock Impedance Measured by Implantable Cardioverter Defibrillator is Useful in the Management of Congestive Heart Failure
- Suppression of Atrial Fibrillation by Atrial Pacing
- PJ-461 Daily Shock Impedance Measured by Implantable Cardioverter Defibrillator is Useful in the Management of Congestive Heart Failure(Arrhythmia, therapy-16 (A) PJ78,Poster Session (Japanese),The 70th Anniversary Annual Scientific Meeting of the Japanes
- Changes of Sympathetic Activity in Patient with Chronic Atrial Fibrillation and Severe Congestive Heart Failure Treated with Biventricular Pacing
- Inverse Relation between Condition of Heart Failure and Intrathoracic Impedance Measured by Implantable Cardioverter Defibrillator : A case report
- Beneficial Effects of Biatrial Pacing on Cardiac Function in Patients With Bradycardia-Tachycardia Syndrome
- The Assessment of Inter-atrial Contraction Delay by Using Strain Doppler Imaging(Arrhythmia, Diagnosis/Pathophysiology/EPS 11 (A), The 69th Annual Scientific Meeting of the Japanese Circulation Society)
- Biventricular and Right-ventricular Bifocal Pacing Improve Severe Heart Failure by Different Mechanisms(Heart Failure, Clinical 3 (M), The 69th Annual Scientific Meeting of the Japanese Circulation Society)
- Reverse Perfusion-Metabolism Mismatch is the Predictor of Good Prognosis of Patients Traeted with Cardiac Resynchronization Therapy(Advances in Nuclear Cardiology (I), The 69th Annual Scientific Meeting of the Japanese Circulation Society)
- Long-Term Follow-up of Atrioventricular Delay Optimization in Patients With Biventricular Pacing
- Diagnostic Use of T2-Weighted Inversion-Recovery Magnetic Resonance Imaging in Acute Coronary Syndromes Compared With ^Tc-Pyrophosphate, ^I-BMIPP and ^TlCl Single Photon Emission Computed Tomography
- Clinical usefulness of ECG-gated ^F-FDG PET combined with ^Tc-MIBI gated SPECT for evaluating myocardial viability and function
- Prevalence of Right Bundle-Branch Block and Right Precordial ST-Segment Elevation (Brugada-Type Electrocardiogram) in Japanese Children
- PJ-346 Anti-Tachycardia Therapy Altered Myocardiac Glucose Metabolism of the Patients with Tachycardia-Induced Cardiomyopathy(Nuclear Cardiology 10 (I) : PJ58)(Poster Session (Japanese))
- PJ-204 Assessment of regional wall motion of atrium during biatrial pacing using Strain Doppler Imaging(Arrhythmia, Non-Pharmacological Therapy 7 (A) : PJ34)(Poster Session (Japanese))
- FRS-119 The Benefit of Biventricular Pacing on Glucose Metabolism, Perfusion and Wall Thickening Assessed By Gated FOG-PET and Gated MIBI SPECT(Nuclear Cardiology (I) : FRS14)(Featured Research Session (English))
- Benefical Effects of Biatrial Pacing for Cardiac Function
- PJ-227 Diastolic Wall Strain can Noninvasively Predict Hemodynamic Deterioration Unassociated with Systolic Dysfunction in Hypertensive Hearts(PJ038,Echo/Doppler (New Technology) 1 (I),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Ja
- PJ-170 Why is Diabetic Retinopathy the Risk Factor of Heart Failure?(PJ029,Diabetes 2 (H),Poster Session (Japanese),The 73rd Annual Scientific Meeting of The Japanese Circulation Society)
- DPJ-031 Different Contributions of Diabetes Mellitus to the Progressions of Aortic Valve Calcification and Valvular Steonsis(DPJ06,Valvular Heart Disease/Pericarditis/Cardiac Tumor (M),Digital Poster Session (Japanese),The 73rd Annual Scientific Meeting o
- OE-376 Diastolic Wall Strain Reflects the Myocardial Fibrosis in Heart Transplanted Patients(OE64,Heart Failure (Diagnosis) (M),Oral Presentation (English),The 73rd Annual Scientific Meeting of The Japanese Circulation Society)
- OE-276 Transcriptional Regulation of Human Nedd4L Gene Isoform I and Development of Essential Hypertension(Hypertension, basic-1, The 71st Annual Scientific Meeting of the Japanese Circulation Society)
- ARBs or ACEIs, That Is the Question
- PE-400 Common Variant of Human NEDD4L Activates a Cryptic Splice Site to Form a Frameshift Transcript(Hypertension, basic-2 (H) PE67,Poster Session (English),The 70th Anniversary Annual Scientific Meeting of the Japanese Circulation Society)
- PE-399 Expression of Angiotensinogen in Proximal Tubule as a Function of Glomerular Filtrate-role of Tubular RAS for Development of Essential Hypertension(Hypertension, basic-2 (H) PE67,Poster Session (English),The 70th Anniversary Annual Scientific Meeti
- Effect of Right Atrial Pacing Preference for the Prevention of Paroxysmal Atrial Fibrillation : Atrial Pacing Preference Study (APP Study)(Arrhythmia, Non-pharmacological Therapy 2 (A), The 69th Annual Scientific Meeting of the Japanese Circulation Societ
- I-123 MIBG Imaging is Useful in Assessment of Cardiac Involvement in Churg-Strauss Syndrome (Nuclear Cardiology 11(I), The 69th Annual Scientific Meeting of the Japanese Circulation Society)
- ATRAP May Inhibit Cardiomyocyte Hypertrophy through the p38-MAPK Pathway (Molecular Biology, Myocardium 2 (M), The 69th Annual Scientific Meeting of the Japanese Circulation Society)
- OJ-521 The Angiotensin II Type 1 (AT1) Receptor-Associated Protein, ATRAP, strictly regulates AT1 Receptor-Mediated Signaling in Cardiac Myocytes(Molecular Biology, Myocardium 2 (M) : OJ64)(Oral Presentation (Japanese))
- The point mutation of tyrosine 759 of the IL-6 family cytokine receptor gp130 synergizes with HTLV-1 pX in promoting rheumatoid arthritis-like arthritis
- -P16- PLATELET ALPHA-2 ADRENOCEPTORS IN PATIENTS WITH ANGINA PECTORIS : Coronary Thrombosis : FREE COMMUNICATIONS(I) : PROCEEDINGS OF THE 51th ANNUAL SCIENTIFIC MEETING OF THE JAPANESE CIRCULATION SOCIETY
- Tubular RAS(Renin-Angiotensin System)-Its Implications for Pathogenesis and Development of Essential Hypertension(EH)(Hypertension, Basic 1 (H), The 69th Annual Scientific Meeting of the Japanese Circulation Society)
- OJ-022 Sarcalumenin Plays a Critical Role in SERCA2a Stability and Adaptation to Sustained Biomechanical Stresses in the Failing Heart(Heart failure, basic-2 (M) OJ4,Oral Presentation (Japanese),The 70th Anniversary Annual Scientific Meeting of the Japane
- Sarcalumenin Deficiency Induced Cardiac Dysfunction in Mice(Excitation-contraction Coupling/Ion Channel 1 (A), The 69th Annual Scientific Meeting of the Japanese Circulation Society)
- Acute Eosinophilic Myocarditis in a Young Woman
- Clinical value of I-123 BMIPP myocardium SPECT for predicting the cardiac death among the patients of chronic heart failure
- OJ-018 Left-sided Pressure Overload Down-regulated the Expression of Sarcolipin mRNA Preferentially in the Left Atrium(Excitation-Contraction Coupling/Ion Channel (A) : OJ3)(Oral Presentation (Japanese))
- Utility and feasibility of a new programmable home blood pressure monitoring device for the assessment of nighttime blood pressure
- Altered Platelet α_2-Adrenoceptors in Patients With Ischemic Heart Disease
- Clinical Usefulness of Additional Treatment With Ezetimibe in Patients With Coronary Artery Disease on Statin Therapy : From the Viewpoint of Cholesterol Metabolism
- Long-term Effect of Dual-chamber Pacing on Pressure Gradient at Left Ventricular Outflow Tract in Hypertrophic Obstructive Cardiomyopathy
- A Case of Nemaline Myopathy With Associated Dilated Cardiomyopathy and Respiratory Failure
- Properties of Densification by Surface Rolling and Load Bearing Capacity of 1.5Cr-0.2Mo High Density Sintered Steel Rollers and Gears
- Double-Blind, Placebo-Controlled Clinical Trial With a Rho-Kinase Inhibitor in Pulmonary Arterial Hypertension:– A Pilot Efficacy Trial –
- Double-Blind, Placebo-Controlled Clinical Trial With a Rho-Kinase Inhibitor in Pulmonary Arterial Hypertension : A Pilot Efficacay Trial