The usefulness of an endomyocardial biopsy in XXXX of unknown etiology.

@entity1 @entity2125 @entity632 @entity296 @entity82 @entity6249 @entity2806 @entity384 @entity2314 @entity24881 @entity299 @entity1121 @entity2401 @entity68
Light-, electron microscopic and enzyme histochemical examinations (phosphorylase, LDH, @entity2125 :TR, @entity24881 and 3-HBDH) were performed on endomyocardial biopsies of 26 @entity1 with @entity299 of unknown etiology. On the basis of the clinical findings the @entity1 were grouped into @entity1121 @entity1 ), @entity384 (8 @entity1 ), latent @entity296 and @entity6249 (11 @entity1 ) and @entity2401 (4 @entity1 ). Morphologic changes which might characterize the pathogenesis, were found in 7 @entity1 : @entity6249 in 3 @entity1 , nonspecific @entity2401 in 1, @entity68 storage disease in 1, @entity632 @entity296 in 1 and @entity296 with inclusions typical of @entity2314 in 1 @entity1 . In the other @entity1 the morphologic changes were not sufficiently characteristic to be indicative of an etiopathogenesis. Several pathologic alterations did, nonetheless, appear to have a certain prognostic value such as endocardial and @entity82 , myofibrillolysis, myolysis, mitochondrial degeneration and increased lipid content in the muscle fibers. The frequency of these changes was evaluated partly semiquantitatively, partly by means of the point-counting method and graded with 1-3 points. Three @entity1 with @entity384 scored at least 7 points. Two of them died within 8 weeks, 1 @entity1 with @entity632 @entity296 recovered after discontinuation of the therapy but he died 4 years after the biopsy. Six to 50 months after the biopsy (mean 31.5, median 6.5) the score was less than 7 in the other @entity1 and all these @entity1 were still alive. The histochemical changes manifested as an increase and/or a decrease of the enzymatic activities, involving scattered muscle fibers or their segments. A decrease of the activities of all dehydrogenases examined appeared to be prognostically ominous, correlating with a score of 7 or higher. A decrease of @entity24881 activity in 7 cases, in combination with a decrease of the HBDH activity in 4 of them, was indicative of a disturbance in the Krebs cycle and lipid metabolism in the absence of @entity2806 . The alterations in the phosphorylase activity did not, however, appear to have a prognostic significance. Normal activity of the phosphorylase seemed to be prognostically favorable.
