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Total 224 results found since Jan 2013.

Left Ventricular Noncompaction Cardiomyopathy in Children Listed for Heart Transplant: Analysis from the Pediatric Heart Transplant Study Group
Left ventricular noncompaction cardiomyopathy (LVNC) is a rare cardiomyopathy characterized by hypertrabeculation of the left ventricle with deep intertrabecular recesses and a thin, compacted layer of myocardium. The clinical phenotype can be heterogeneous with a benign or severe course including life-threatening arrhythmias, thromboembolism, skeletal myopathy, and immune deficiencies, most notably neutropenia.1–3 Prior studies of cardiomyopathy outcomes in children listed for heart transplant (HT) did not assess patients with LVNC 4 and a recent evaluation of LVNC outcomes utilizing the United Network for Organ Sharing...
Source: The Journal of Heart and Lung Transplantation - January 6, 2016 Category: Transplant Surgery Authors: Ashwin K. Lal, Elizabeth Pruitt, Borah J. Hong, Kimberly Y. Lin, Brian Feingold Source Type: research

Left ventricular non-compaction cardiomyopathy in children listed for heart transplant: Analysis from the Pediatric Heart Transplant Study Group
Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy characterized by hypertrabeculation of the left ventricle with deep intertrabecular recesses and a thin, compacted layer of myocardium. The clinical phenotype can be heterogeneous with a benign or severe course, including life-threatening arrhythmias, thromboembolism, skeletal myopathy, and immune deficiencies, most notably neutropenia.1–3 Prior studies of cardiomyopathy outcomes in children listed for heart transplant (HT) did not assess patients with LVNC,4 and a recent evaluation of LVNC outcomes using the United Network for Organ Sharing d...
Source: The Journal of Heart and Lung Transplantation - January 6, 2016 Category: Transplant Surgery Authors: Ashwin K. Lal, Elizabeth Pruitt, Borah J. Hong, Kimberly Y. Lin, Brian Feingold Tags: Research Correspondence Source Type: research

Neurological complications in renal transplant patients: A single-center experience
Conclusion We conclude that complications involving the neurological system occur in 10.5% of all transplant patients with 8% involving CNS and 7% involving the PNS. The high mortality rates associated with CNS complications warrant early diagnosis and aggressive treatment in renal transplant recipients.
Source: Indian Journal of Transplantation - November 24, 2015 Category: Transplant Surgery Source Type: research

Idiopathic Inflammatory Myopathies: an Update on Classification and Treatment with Special Focus on Juvenile Forms
Abstract Juvenile inflammatory myopathies represent a heterogeneous group of rare and potentially fatal disorders of unknown aetiology, characterised by inflammation and proximal and symmetric muscle weakness. Beyond many similarities, specific clinical, laboratoristic and histopathologic features underlie different subsets with distinguishing demographic, prognostic and therapeutic peculiarities. Over time, several forms of inflammatory idiopathic myopathies have been described, including macrophagic myofascitis, immune-mediated necrozing myopathy and the spectrum of amyopathic dermatomyositis that include hypomy...
Source: Clinical Reviews in Allergy and Immunology - October 1, 2015 Category: Allergy & Immunology Source Type: research

Clinical Characteristics and Outcomes of Cardiomyopathy in Barth Syndrome: The UK Experience
This study describes the UK national clinical experience and outcome of cardiomyopathy in BTHS. The clinical course and echocardiographic changes of all patients with BTHS in the UK were reviewed from 2004 to 2014. In addition, strain analysis using 2D speckle tracking echocardiography was performed to further assess left ventricular function in the most recent follow-up. At last follow-up, 22 of 27 patients were alive with a median age of 12.6 (2.0–23.8) years; seven underwent cardiac transplantation at a median age of 2 (0.33–3.6) years, and five died (18.5 %) at a median age of 1.8 (0.02–4.22) years. All deaths ...
Source: Mammalian Genome - September 4, 2015 Category: Genetics & Stem Cells Source Type: research

Recessive MYH6 Mutations in Hypoplastic Left Heart With Reduced Ejection Fraction Original Articles
Conclusions— In contrast to dominant MYH6 mutations with variable penetrance identified in other congenital heart defects and dilated cardiomyopathy, this study reveals compound heterozygosity for recessive MYH6 mutations in patients with hypoplastic left heart and reduced systemic right ventricular ejection fraction. These findings implicate a shared molecular basis for the developmental arrest and latent myopathy of left and right ventricles, respectively.
Source: Circulation: Cardiovascular Genetics - August 18, 2015 Category: Cardiology Authors: Theis, J. L., Zimmermann, M. T., Evans, J. M., Eckloff, B. W., Wieben, E. D., Qureshi, M. Y., O'Leary, P. W., Olson, T. M. Tags: Contractile function, Clinical genetics, Cardiac development Original Articles Source Type: research

Colchicine toxicity in renal patients - Are we paying attention?
We report two cases of colchicine toxicity marked by severe neuromyopathy in a diabetic with stage 4 chronic kidney disease (CKD) and a renal transplant recipient. Both patients presented with diarrhea, acute on chronic kidney injury and progressive muscle weakness while on colchicine for several weeks or longer. In addition to kidney disease, risk factors for colchicine toxicity included maintenance therapy with simvastatin in the first patient and cyclosporine in the second. Creatine phosphokinase (CPK) was elevated in both cases at presentation and neurophysiologic studies showed a pattern of severe myopathy with axonal...
Source: Clinical Nephrology - August 7, 2015 Category: Urology & Nephrology Authors: Medani S, Wall C Tags: Clin Nephrol Source Type: research

Liver transplantation for treatment of severe S-adenosylhomocysteine hydrolase deficiency
Publication date: Available online 19 June 2015 Source:Molecular Genetics and Metabolism Author(s): Kevin A. Strauss , Carlos Ferreira , Teodoro Bottiglieri , Xueqing Zhao , Erland Arning , Shucha Zhang , Steven H. Zeisel , Maria L. Escolar , Nancy Presnick , Erik G. Puffenberger , Oliver Vugrek , Lucija Kovacevic , Conrad Wagner , George V. Mazariegos , S. Harvey Mudd , Kyle Soltys A child with severe S-adenosylhomocysteine hydrolase (AHCY) deficiency (AHCY c.428A>G, p.Tyr143Cys; c.982T>G, p.Tyr328Asp) presented at 8months of age with growth failure, microcephaly, global developmental delay, myopathy, hepa...
Source: Molecular Genetics and Metabolism - June 27, 2015 Category: Genetics & Stem Cells Source Type: research

Cystinosis associated vasculopathy masquerading as atypical migraine headache (P1.032)
Conclusions: Cystinosis, especially if untreated, can lead to neurological complications, more commonly distal myopathy and, rarely as our patient suffered, cerebral vasculopathy. Identifying this vasculopathy is essential and can be treatable, thus preventing further neurologic deterioration.Disclosure: Dr. Smith has nothing to disclose. Dr. Kushlaf has nothing to disclose.
Source: Neurology - April 8, 2015 Category: Neurology Authors: Smith, D., Kushlaf, H. Tags: Cerebrovascular Disease and Interventional Neurology: Case Reports Source Type: research

Extraocular muscle fatigue evaluation in Mitochondrial Neurogastrointestinal Encephalomyopathy (MNGIE) (P5.316)
CONCLUSIONS:The difference in eye movement characteristics, possibly related to the different response to fatigue, among treated and untreated subjects, have been discussed taking into consideration the anatomical peculiarities of extraocular muscles and their diverse dependence to mitochondrial metabolism. We suggest that the evaluation of adducting and abducting saccades (even clinically) in this and probably in other mitochondrial diseases, may add information on the severity of the disease or its response to treatment. Study Supported by: EC FP7-PEOPLE-IRSES-CERVISO 269263Disclosure: Dr. Vinciguerra has nothing to disc...
Source: Neurology - April 8, 2015 Category: Neurology Authors: Vinciguerra, C., Federighi, P., Rosini, F., Pretegiani, E., Cardaioli, E., Dotti, M. T., Sicurelli, F., Federico, A., Rufa, A. Tags: Neuro-ophthalmology/Neuro-otology II Source Type: research

Telbivudine in liver transplant recipients: Renal protection does not overcome the risk of polyneuropathy and myopathy
In conclusion, although it seems to have a favorable effect in the improvement of renal function and to be effective in the prevention of HBV recurrence, the high risk of polyneuropathy and myopathy hamper the use of telbivudine in LT recipients. This article is protected by copyright. All rights reserved.
Source: Liver Transplantation - April 2, 2015 Category: Transplant Surgery Authors: Ilker Turan, Suna Yapali, Fikret Bademkiran, Timur Kose, Soner Duman, Murat Sozbilen, Fulya Gunsar, Galip Ersoz, Ulus Salih Akarca, Omer Ozutemiz, Zeki Karasu Tags: Original Articles Source Type: research

Transthyretin V122I amyloidosis with clinical and histological evidence of amyloid neuropathy and myopathy
• V122I ATTR is the commonest cause of familial amyloid cardiomyopathy• Neuromusclar amyloidosis has not been previous described with this mutation• Extra-cardiac involvement may reduce risk associated with tissue diagnosis• Cardiac transplant is precluded by extra-cardiac disease• New treatments for hereditary transthyretin amyloidosis are promising
Source: Neuromuscular Disorders - February 13, 2015 Category: Neurology Authors: AS Carr, AL Pelayo-Negro, Z Jaunmuktane, RS Scalco, D Hutt, MRB Evans, E Heally, S Brandner, J Holton, J Blake, CJ Whelan, AD Wechalekar, JD Gillmore, PN Hawkins, MM Reilly Tags: Case report Source Type: research

Nemaline myopathy and heart failure: Role of ivabradine; a case report
Conclusions: HF is a rare feature of NM, but it can negatively influence prognosis. Conventional HF therapy and/or heart transplant are the only reasonable strategy in these patients. Ivabradine is a useful, effective and safe drug for therapy in NM patients with HF and should be considered when resting HR remains high despite beta-blockers' full titration or beta-blockers' underdosing due to intolerance or side effects.
Source: BMC Cardiovascular Disorders - January 19, 2015 Category: Cardiology Authors: Filippo SarulloGiuseppe VitaleAntonino Di FrancoSilvia SarulloYlenia SalernoLaura VassalloEmanuela BavieraStefania MaraziaGiorgio MandalàGaetano Lanza Source Type: research

Danon disease: a phenotypic expression of LAMP-2 deficiency
Abstract Danon disease is an X-linked disorder clinically characterized by the triad of hypertrophic cardiomyopathy, myopathy, and intellectual disability. Cardiomyopathy is a severe and life-threatening problem, for which cardiac transplantation is the only therapeutic option. The most striking finding in muscle biopsy samples is small basophilic granules scattered in myofibers, which are in fact small autophagic vacuoles surrounded by membranes with sarcolemmal features characterized by the recruitment of sarcolemmal proteins and acetylcholine esterase and by the presence of basal lamina on its luminal side. The...
Source: Acta Neuropathologica - January 15, 2015 Category: Neurology Source Type: research

Sporadic late-onset nemaline myopathy with MGUS: Long-term follow-up after melphalan and SCT
Conclusions: This case series shows the positive effect of HDM-SCT in this rare disorder. Factors that may portend an unfavorable outcome are a long disease course before the hematologic treatment and a poor hematologic response. Age at onset, level and type of M protein ( vs ), and severity of muscle weakness were not associated with a specific outcome. Classification of evidence: This study provides Class IV evidence that for patients with SLONM-MGUS, HDM-SCT increases the probability of survival and functional improvement.
Source: Neurology - December 1, 2014 Category: Neurology Authors: Voermans, N. C., Benveniste, O., Minnema, M. C., Lokhorst, H., Lammens, M., Meersseman, W., Delforge, M., Kuntzer, T., Novy, J., Pabst, T., Bouhour, F., Romero, N., Leblond, V., Bergh, P. v. d., Vekemans, M.-C., van Engelen, B. G., Eymard, B. Tags: All Immunology, All Neuromuscular Disease, Muscle disease, Class IV ARTICLE Source Type: research