Peer-reviewed veterinary case report
Heart storage disease causing sudden death in a 12-year-old pug
By Tran, J N S N et al.·Published in Journal of comparative pathology·2017·Department of Biomedical Sciences, United States·View original on PubMed →
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Original publication title: Clinicopathological Features of Cardiac Glycolipid Storage Disease in an Adult Pug.
- Species:
- dog
Plain-English summary
A 12-year-old neutered male pug experienced a sudden cardiac arrest and sadly died during anesthesia while being evaluated for exercise intolerance and breathing problems. A detailed examination of his organs revealed a serious condition called glycolipid storage disease, which caused significant damage to his heart and other organs like the liver and kidneys. This disease is a type of lysosomal storage disease, similar to a condition seen in humans called Fabry disease, where harmful substances build up in the body. Unfortunately, despite the investigation, the pug could not be saved.
People also search for: pug exercise intolerance · pug breathing problems · cardiac arrest in dogs · glycolipid storage disease in dogs
Abstract
A 12-year-old neutered male pug suffered cardiac arrest and died under general anaesthesia during diagnostic imaging for evaluation of exercise intolerance and respiratory crisis. Histopathological evaluation revealed two types of storage material, glycolipid and lipopigment, having differential distributions in multiple organs. The heart was most strikingly affected and other less affected tissues included the liver, brain, kidneys and skin. Cardiomyocytes were swollen with extensive sarcoplasmic vacuolation together with coalescing areas of myocardial fibrosis. Transmission electron microscopy revealed irregular myelin-like structures and complex concentric lamellar bodies dominating the sarcoplasm and displacing myofibrils. These findings were consistent with a lysosomal storage disease (LSD) as the cause of cardiac disease and death. The unique clinical presentation, histomorphology and ultrastructural features of the material suggested a glycolipid storage disease most closely resembling Anderson-Fabry (Fabry) disease in man. Fabry disease is a LSD that can present in later life and is characterized by loss of α-galactosidase A function and, often, accumulation of glycosphingolipids in tissues including the heart, kidneys, vascular endothelium and smooth muscle.
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Search related cases →Original publication on PubMed: https://pubmed.ncbi.nlm.nih.gov/27974158/