Abstract
Ultrastructural changes of a biopsied myocardium were observed by transmission electron microscopy in a patient with cardiomyopathy secondary to systemic triglyceride storage disease with Jordans' anomaly. There were many lipid droplets in the cardiocytes, and lipofuscin and mitochondria were increased. The volume fraction of myofibrils in the cardiocytes decreased because of an abundance of lipid droplets and mitochondriosis. Myocardial contractility may have been reduced by myofibrillar scarcity and low energy production resulting from an abnormality in the metabolism of fatty acids in the cardiocytes.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Ibayashi H, Ideguchi H, Harada N, Ishimoto S, Goto I (1988) Systemic triglyceride storage disease with normal carnitine: a putative defect in long-chain fatty acid metabolism. J Neurol Sci 85:149–159
Jordans GHW (1953) The familial occurrence of fat-contaning vacuoles in the leucocytes diagnosed in two brothers suffering from dystrophia musculorum progressiva (ERB). Acta Med Scand 145:419–423
Ando S, Usui M, Matsumoto T, Egashira K, Takeshita A, Terasaki F, Deguchi H, Kawamura K (1996) Vasospastic angina associated with patients with systemic triglyceride storage disease with Jordans' anomaly and cardiomyopathy. Jpn Circ J 60:124–129
Ghadially FN (1988) Lipid In: Ultrastructural pathology of the cell and matrix, 3rd edn. Butterworths, London, pp 974–977
Maron BJ, Ferrans VJ (1978) Ultrastructural features of hypertrophied human ventricular myocardium. Prog Cardiovasc Dis 21:207–238
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Terasaki, F., Kawamura, K., Okabe, M. et al. Cardiomyopathy secondary to systemic triglyceride storage disease. Med Electron Microsc 30, 88–91 (1997). https://doi.org/10.1007/BF01545087
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01545087