PEDIATRICS Vol. 10 No. 2 August 1952, pp. 208-217
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GLYCOGEN DISEASE OF THE HEART

Report of Two Cases Occurring in Siblings

ALFRED W. CHILDS M.D.1, ROBERT F. CROSE M.D.1, and PATRICIA H. HENDERSON M.D.1

1 The Divisions of Pediatrics and Pathology, University of California School of Medicine, San Francisco.

Two cases of siblings with glycogen storage disease of the heart are reported. The clinical picture was characterized by progressive weakness, areflexia, and susceptibility to secondary infections. There was also progressive hepatomegaly and cardiomegaly.

The pathologic findings correlated well with the clinical and were characterized by marked glycogen storage in skeletal, heart, and smooth muscle, the liver, and nerve cells of the central and autonomic nervous systems. In addition, many other tissues showed some glycogen storage. Storage in the skeletal muscle was so marked as to result in degeneration of the muscle fibers.

The familial aspect of this disease is again noted. Suggestions regarding differential diagnosis are made.

Submitted on July 10, 1951




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