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Total 224 results found since Jan 2013.
Outcomes of Autologous Hematopoietic Stem Cell Transplant in Sporadic Late Onset Nemaline Myopathy with Associated Monoclonal Gammopathy of Unknown Significance
Background: Sporadic Late Onset Nemaline Myopathy (SLONM) is a rare muscle disorder with subacute progression, characterized by axial and proximal muscle weakness and atrophy, and the presence of nemaline rods in myofibers. When SLONM is associated with a monoclonal gammopathy of undetermined significance (SLONM/MGUS), patients seem to fare worse compared to patients with SLONM and no MGUS. Supportive and immunosuppressive therapies have yielded poor outcomes for most patients with SLONM/MGUS; IVIG have been beneficial in some.
Source: Biology of Blood and Marrow Transplantation - February 1, 2018 Category: Hematology Authors: Ankit J. Kansagra, Francis Buadi, Margherita Milone, Elie Naddaf, Morie Gertz, Angela Dispenzieri, Shaji Kumar, David Dingli, Martha Q. Lacy, Prashant Kapoor, Wilson I. Gonsalves, Rahma Warsame, Eli Muchtar, Robert Kyle, Taxiarchis Kourelis Source Type: research
Chemotherapy with stem cell transplantation is more effective than immunotherapy in sporadic late onset nemaline myopathy with monoclonal gammopathy
Chemotherapy with stem cell transplantation is more effective than immunotherapy in sporadic late onset nemaline myopathy with monoclonal gammopathy, Published online: 24 January 2018; doi:10.1038/s41409-017-0080-6Chemotherapy with stem cell transplantation is more effective than immunotherapy in sporadic late onset nemaline myopathy with monoclonal gammopathy
Source: Bone Marrow Transplantation - January 24, 2018 Category: Hematology Authors: Rouslan Kotchetkov Anna Dyszkiewicz-Korpanty Vishal Kukreti Source Type: research
Sporadic late-onset nemaline myopathy: clinical, pathology and imaging findings in a single center cohort
AbstractSporadic late-onset nemaline myopathy (SLONM) is a rare acquired myopathy characterized by rapid-onset proximal weakness in late adulthood, and the presence of nemaline bodies on muscle biopsy. In recent years, several therapeutic interventions, including immunomodulating agents and autologous stem cell transplantation, have shown variable degrees of efficacy in different patients, but no consensus has been reached to allow an effective tailoring of treatments in this severe disease. We performed a retrospective evaluation of clinical, pathological, laboratory, muscle MRI, and follow-up data of SLONM patients diagn...
Source: Journal of Neurology - January 22, 2018 Category: Neurology Source Type: research
Biventricular Support With Intracorporeal, Continuous Flow, Centrifugal Ventricular Assist Devices
Conclusions The use of durable, intracorporeal, continuous flow centrifugal pumps for management of advanced biventricular heart failure is associated with high morbidity and mortality. Further investigation of this device configuration is warranted.
Source: The Annals of Thoracic Surgery - January 21, 2018 Category: Cardiovascular & Thoracic Surgery Source Type: research
Biventricular Support With Intracorporeal, Continuous Flow, Centrifugal Ventricular Assist Devices.
CONCLUSIONS: The use of durable, intracorporeal, continuous flow centrifugal pumps for management of advanced biventricular heart failure is associated with high morbidity and mortality. Further investigation of this device configuration is warranted.
PMID: 29157741 [PubMed - as supplied by publisher]
Source: The Annals of Thoracic Surgery - November 17, 2017 Category: Cardiovascular & Thoracic Surgery Authors: Arabía FA, Milano CA, Mahr C, McGee EC, Mokadam NA, Rame JE, Moriguchi JD, Ramzy D, Naftel DC, Myers SL, Kirklin JK Tags: Ann Thorac Surg Source Type: research
Heart transplantation in Danon disease: a single family displaying diverse phenotypes
Danon disease (DD) is an X-linked systemic disorder characterized by left ventricular hypertrophy, mental retardation, and skeletal myopathy.1 DD is caused by a deficiency of the lysosome-associated membrane protein (LAMP-2) which leads to an accumulation of autophagic vacuoles in skeletal and heart muscles, liver, and kidneys (Figure 1A).2 Histological analysis shows disorganized cellular architecture and hypertrophic cardiomyocytes with sarcoplasmic vacuoles. Electrocardiogram usually displays a Wolff-Parkinson-White (WPW) pre-excitation pattern.
Source: The Journal of Heart and Lung Transplantation - November 4, 2017 Category: Transplant Surgery Authors: Simon Poignant, Thierry Bourguignon, Fabien Espitalier Source Type: research
Green tea epigallocatechin gallate enhances cardiac function restoration through survival signaling expression in diabetes mellitus rats with autologous adipose tissue-derived stem cells
The present study tests a hypothesis that cardioprotective effects mediated by autologous adipose-derived stem cells (ADSC) in rats afflicted with insulin-dependent diabetes mellitus (IDDM) may be synergistically enhanced by oral treatment with green tea epigallocatechin gallate (EGCG). Wistar rats were divided into sham, DM, DM+ADSC (autologous transplanted 1 x 106 cells per rat), and DM+ADSC+E (E, green tea oral administration EGCG). Heart tissues were isolated from all rats, and investigations were performed after 2-mo treatment. In the sham, DM, and DM+ADSC groups, we found that DM induced cardiac dysfunction (sham and...
Source: Journal of Applied Physiology - November 2, 2017 Category: Physiology Authors: Chen, T.-S., Liou, S.-Y., Kuo, C.-H., Pan, L.-F., Yeh, Y.-L., Liou, J., Padma, V. V., Yao, C.-H., Kuo, W.-W., Huang, C.-Y. Tags: RESEARCH ARTICLE Source Type: research
Pulmonary Pathologic Manifestations of Anti-Alanyl-tRNA Synthetase (Anti-PL-12)-Related Inflammatory Myopathy.
CONCLUSIONS: - Lung disease is often the first manifestation of anti-PL-12 ARS. There are no pathognomonic histopathologic features to distinguish anti-PL-12 ARS-related lung disease from idiopathic variants of diffuse interstitial lung disease. Increased inflammation, lymphoid aggregates, and nonspecific interstitial pneumonia-like areas in a biopsy, as well as clinical features of mechanic's hands, Raynaud phenomenon, arthritis, and fever, should prompt pathologists to suggest involvement by ARS.
PMID: 28967806 [PubMed - as supplied by publisher]
Source: Archives of Pathology and Laboratory Medicine - October 2, 2017 Category: Laboratory Medicine Authors: Schneider F, Yousem SA, Oddis CV, Aggarwal R Tags: Arch Pathol Lab Med Source Type: research
Acetazolamide for Hypoventilation in a Congenital Myopathy Patient with Advanced Heart Failure after a Heart Transplant; a Case Report
A 61-year-old man was admitted to our hospital for further treatment of worsening congestive heart failure with a mechanical ventilation. He had undergone a heart transplant due to dilated cardiomyopathy complicated with congenital myopathy at the age of 43. He had been suffering from cardiac allograft vasculopathy and had a history of right ventricular infarction complicated with acute inferior myocardial infarction when he was 59. Since then, refractory right-sided heart failure occurred and additional diuretics were required.
Source: Journal of Cardiac Failure - September 20, 2017 Category: Cardiology Authors: Chiharuko Iio, Koichi Tamita, Tomohiko Shimizu, Mika Maeda, Jun Yamamuro Source Type: research
Cyclophosphamide, thalidomide, and dexamethasone as alternative treatment regimen for sporadic late onset nemaline myopathy associated with monoclonal gammopathy of undetermined significance
We report a case of 33-year-old man with a 5-month history of proximal muscle weakness who later develop neck weakness and require tracheostomy.
Source: Neuromuscular Disorders - September 10, 2017 Category: Neurology Authors: J. Tanboon, T. Kumutpongpanich, W. Owattanapanich, T. Sangruchi, K. Boonyapisit, I. Nishino Source Type: research
Increased cardiac muscle autophagy in a child with restrictive cardiomyopathy, proximal myopathy and neuropathy due to a mutation in the BAG3 (Pro209Leu) gene
We report an 8-year-old boy with nocturnal hypoventilation since age 5 years, who underwent heart transplantation (HTX) due to severe RCM at age 7 years.
Source: Neuromuscular Disorders - September 10, 2017 Category: Neurology Authors: A. Sch änzer, S. Rupp, G. Mall, H. Akintürk, D. Schramz, L. Gulatz, J. Thul, N. Mazhari, A. Hahn Source Type: research
Sporadic late-onset nemaline myopathy with monoclonal gammopathy of undetermined significance
Purpose of review: Sporadic late-onset nemaline myopathy (SLONM) with monoclonal gammopathy of undetermined significance (MGUS) is a rare subacute progressive muscle disease. The prognosis is poor due to severe respiratory insufficiency. Recently, however, autologous stem-cell transplantation following high-dose melphalan has been shown to be effective unless there is delay before the treatment. Therefore, early recognition of the disease is important. This review gives an overview of recent advances in SLONM–MGUS, which could help to understand clinical and pathological features and treatment.
Recent findings: Efficacy...
Source: Current Opinion in Neurology - September 1, 2017 Category: Neurology Tags: NERVE, NEURO-MUSCULAR JUNCTION AND MOTOR NEURON DISEASES: Edited by Jean-Marc Leger Source Type: research
Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy
Conclusion On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia.
Source: Brazilian Journal of Anesthesiology - July 19, 2017 Category: Anesthesiology Source Type: research
Atypical reaction to anesthesia in Duchenne/Becker muscular dystrophy.
CONCLUSION: On preanesthetic assessment, the history of delayed motor development, as well as clinical and/or laboratory signs of myopathy, should encourage neurological evaluation, aiming at diagnosing subclinical myopathies and planning the necessary care to prevent anesthetic complications. Duchenne/Becker muscular dystrophy, although it does not increase susceptibility to MH, may lead to atypical fatal reactions in anesthesia.
PMID: 28576279 [PubMed - as supplied by publisher]
Source: Revista Brasileira de Anestesiologia - June 5, 2017 Category: Anesthesiology Tags: Rev Bras Anestesiol Source Type: research
Case of hepatocellular carcinoma in a patient with hereditary tyrosinemia in the post-newborn screening era.
We present a case report of a 7-year-old patient with HT-1 who was born prior to the addition of tyrosinemia to the newborn screening in her birth area. At her time of diagnosis, the patient had developed many of the symptoms associated with her disease, including chronic kidney disease, rickets, and myopathy that left her non-ambulatory. During her initial evaluation, she was also noted to have hepatocellular carcinoma. With cadaveric liver transplantation and nutritional support, her symptoms all either resolved or stabilized. Her case illustrates the severity of the disease if left untreated, the need for vigilance in p...
Source: World Journal of Hepatology - April 12, 2017 Category: Gastroenterology Tags: World J Hepatol Source Type: research
