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Dilated Cardiomyopathy Complicating a Case of Epidermolysis Bullosa Dystrophica
Author(s) -
Brook Michael M.,
Weinhouse Elliott,
Jarenwattana Marisa,
Nudel Dov B.
Publication year - 1989
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1989.tb00261.x
Subject(s) - medicine , epidermolysis bullosa dystrophica , hemosiderosis , cardiomyopathy , dilated cardiomyopathy , heart failure , anemia , epidermolysis bullosa , restrictive cardiomyopathy , dermatology , cardiology , pediatrics
A Child with epidermolysis bullosa dystrophica, recessive type (EBDR) developed significant anemia at 9 years of age and was treated with long‐term transfuslon therapy. At age 17 he had symptoms of congestive heart failure secondary to dilated cardlomyopathy. Treatment with digoxin and vasodilators for the past year has failed to improve his cardiomyopathy significantly. Chronic iron overload and secondary hemosiderosis may have contributed to his problems, and we propose that chelation therapy be used in any child received long‐term transfusion therapy.