Document Detail

Fatal Coronary Artery Disease in an Infant With Severe Mucopolysaccharidosis Type I.
MedLine Citation:
PMID:  21464194     Owner:  NLM     Status:  Publisher    
A previously healthy 10-month-old boy was referred to our hospital because of coarse facial features that were suggestive of lysosomal storage disease. Apart from noisy respiration, there was no medical history. Elevated levels of urinary glycosaminoglycans and complete deficiency of leukocyte α-l-iduronidase indicated severe mucopolysaccharidosis type I. A chest radiograph revealed a markedly enlarged heart, and echocardiography revealed hypertrophic cardiomyopathy. While hematopoietic stem cell transplantation was being planned, progressive cardiac failure developed with a striking hypokinesia of the left-ventricle free wall. In combination with ischemic changes on the electrocardiogram, this was suggestive of coronary artery disease. Results of coronary echo Doppler interrogation were inconclusive, and intravascular ultrasound in this little infant was not feasible. Despite the patient's small size, a successful selective coronary angiography was performed and revealed diffuse narrowing of the left coronary artery with collateral flow from the right coronary artery. Enzyme-replacement therapy was started immediately in an attempt to improve myocardial performance. Evaluation after 3 months, however, revealed complete obliteration of the left coronary main stem with diffuse hypokinesia/akinesia of the left ventricle. At the age of 13 months the boy died of terminal cardiac failure. This case report illustrates the importance of considering early development of coronary artery disease in children with severe mucopolysaccharidosis type I and cardiomyopathy.
Leonie van den Broek; Ad P C M Backx; Hans Coolen; Frits A Wijburg; Ron Wevers; Eva Morava; Chris Neeleman
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-4-4
Journal Detail:
Title:  Pediatrics     Volume:  -     ISSN:  1098-4275     ISO Abbreviation:  -     Publication Date:  2011 Apr 
Date Detail:
Created Date:  2011-4-5     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Department of Pediatrics, Catharina Hospital, Eindhoven, Netherlands;
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