ISSN:
1432-2307
Keywords:
Cardiomyopathy
;
Leigh's disease
;
Mitochondriopathy
;
Ultrastructure
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary Clinical and postmortem findings in a female infant, suffering from Leigh's disease and cardiomegaly are described. The cardiac enlargement was due to symmetrical thickening of both ventricular walls and the septum. On light microscopy a widespread fibre disarray with a slight predilection for the ventricular septum was observed. Ultrastructural changes included an extreme reduction in the number of myofibrils and an excess of mitochondria. Abnormalities of the mitochondrial structure with tubular and myelinic transformation of the cristae suggested that a mitochondriopathy is responsible for the cardiomegaly in Leigh's disease.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00701332
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