Objective: To determine the presentation, diagnosis, progression, and family risk of fibrotic myopathy, a disease with marked breed predisposition in the German Shepherd Dog (GSD).
Animals: 41 dogs prospectively recruited to the University of Wisconsin-Madison Comparative Genetics and Orthopedic Laboratory between November 2019 to August 2022.
Methods: Medical records of dogs diagnosed with fibrotic myopathy were reviewed upon referral. The following data were recorded: sex, age, weight, regio interscapularis (withers) height, date of neutering, coat color and length, and age at fibrotic myopathy diagnosis. A pedigree was also obtained.
Results: In the study population, breeds included 37 GSDs, a Belgian Malinois, a Belgian Malinois cross, and 2 dogs with a GSD phenotype and no pedigree. Mean age at fibrotic myopathy diagnosis was 5.9 ± 2.0 years, and duration of lameness before diagnosis was 5.6 months and ranged from 0.75 to 18 months. Males were overrepresented at 61% of the study population. Inherited familial risk for fibrotic myopathy in the GSD was supported by pedigree analysis.
Clinical Relevance: This was the largest case series of fibrotic myopathy to date, providing a more comprehensive look at presentation and progression of the disease. The longer duration of lameness in bilaterally affected dogs likely represents disease progression rather than a more severe phenotype. Family history data support a genetic contribution to fibrotic myopathy, suggesting that further genetic investigation is warranted.
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http://dx.doi.org/10.2460/javma.23.04.0183 | DOI Listing |
Zhonghua Gan Zang Bing Za Zhi
November 2024
The Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Shanghai201102, China.
Lancet Respir Med
November 2024
Department of Pulmonary and Critical Care Medicine, Integrated Hospital Care Institute, Cleveland Clinic, Cleveland, OH, USA.
Bioeng Transl Med
September 2024
Regenerative Medicine Technologies Laboratory, Laboratories for Translational Research (LRT) Ente Ospedaliero Cantonale (EOC) Bellinzona Switzerland.
Fibrosis is a pathological condition that in the muscular context is linked to primary diseases such as dystrophies, laminopathies, neuromuscular disorders, and volumetric muscle loss following traumas, accidents, and surgeries. Although some basic mechanisms regarding the role of myofibroblasts in the progression of muscle fibrosis have been discovered, our knowledge of the complex cell-cell, and cell-matrix interactions occurring in the fibrotic microenvironment is still rudimentary. Recently, vascular dysfunction has been emerging as a key hallmark of fibrosis through a process called endothelial-mesenchymal transition (EndoMT).
View Article and Find Full Text PDFJ Electrocardiol
December 2024
Hypertrophic Cardiomyopathy Center of Excellence, Division of Cardiology, University of California San Francisco, San Francisco, CA, United States of America. Electronic address:
Background: Signal-averaged electrocardiogram (SAECG) records myocardial depolarization, and can detect inhomogeneous/slow conduction in fibrotic myocardium, which promotes reentrant ventricular arrhythmias (VAs). Hypertrophic cardiomyopathy (HCM) is associated with a high prevalence of cardiac fibrosis and VAs, but abnormal SAECG has low predictive power for VAs. We hypothesized that HCM-specific structural/electrical remodeling underlies this result.
View Article and Find Full Text PDFJ Physiol
December 2024
Department of Mechanical Engineering, University of Washington, Seattle, WA, USA.
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