Document Detail


Complex conotruncal heart defect, severe bleeding disorder and 22q11 deletion: a new case of Bernard-Soulier syndrome and of 22q11 deletion syndrome?
MedLine Citation:
PMID:  11453587     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A patient with a deletion in the DiGeorge/velocardiofacial chromosomal region in 22q11, underwent cardiac repair for truncus arteriosus with a separate origin of the pulmonary arteries. This patient presented with a severe coagulation disorder similar to that described in the Bernard-Soulier syndrome. Additional features included minor facial anomalies, transient hypocalcemia and renal failure. To the best of our knowledge, this is the third case of a severe bleeding disorder associated with 22q 11 deletion reported in the literature.
Authors:
M R Lascone; M Sacchelli; S Vittorini; S Giusti
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Italian heart journal : official journal of the Italian Federation of Cardiology     Volume:  2     ISSN:  1129-471X     ISO Abbreviation:  Ital Heart J     Publication Date:  2001 Jun 
Date Detail:
Created Date:  2001-07-16     Completed Date:  2001-12-04     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  100909716     Medline TA:  Ital Heart J     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  475-7     Citation Subset:  IM    
Affiliation:
Molecular Biology Laboratory, G. Pasquinucci Hospital, Massa, Italy.
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MeSH Terms
Descriptor/Qualifier:
Bernard-Soulier Syndrome / complications,  congenital
Chromosome Deletion
Chromosomes, Human, Pair 22*
Family Health
Heart Defects, Congenital / complications*
Hemorrhage / complications*
Humans
Infant, Newborn
Male

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