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August 1953

ENDOCARDIAL FIBROELASTOSIS: Report of Unusual Case with Impaired Ability to Fabricate Serum Proteins

Author Affiliations

From the Department of Pediatrics, University of Minnesota, Minneapolis, and the Department of Pediatrics, University of California School of Medicine at Los Angeles.

AMA Am J Dis Child. 1953;86(2):186-197. doi:10.1001/archpedi.1953.02050080195007

ENDOCARDIAL fibroelastosis or endocardial sclerosis until recently has been looked upon as a pathologic diagnosis.1 With the growing interest in this condition, attempts have been made to define this as a clinical entity and one which in many cases can be diagnosed ante mortem.2

The postmortem findings of endocardial fibroelastosis have been described in infants from 3 days to 5 years of age; the majority of the deaths have been of infants between 4 and 9 months. Generally speaking, three clinical patterns have been noted, characterized best by their course: (1) sudden death occurring 24 to 48 hours after the onset of symptoms of respiratory distress; (2) prolonged progressive increase in dyspnea, anorexia, irritability, occasional vomiting, cardiac failure, and death; (3) repeated episodes of respiratory distress and cardiac failure which respond well to oxygen and digitalis and which may last months or even years.

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