Document Detail


The association of Buschke-Ollendorf syndrome and nail-patella syndrome.
MedLine Citation:
PMID:  11907523     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Buschke-Ollendorf syndrome and nail-patella syndrome are both rare connective tissue disorders inherited in an autosomal dominant pattern and characterized by cutaneous and bone lesions. We describe a 3-year-old boy and his family who showed clinical features of both Buschke-Ollendorf syndrome and nail-patella syndrome. To our knowledge, this association has not been reported previously, suggesting that these two connective tissue disorders may share the same gene location with different mutations or involve different mutated genes that share downstream segments of their signaling pathways. Furthermore, this young patient is also affected by a chronic idiopathic neutropenia usually not observed in Buschke-Ollendorf syndrome or nail-patella syndrome.
Authors:
Christian Allen Drouin; Hugues Grenon
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of the American Academy of Dermatology     Volume:  46     ISSN:  0190-9622     ISO Abbreviation:  J. Am. Acad. Dermatol.     Publication Date:  2002 Apr 
Date Detail:
Created Date:  2002-03-21     Completed Date:  2002-05-03     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7907132     Medline TA:  J Am Acad Dermatol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  621-5     Citation Subset:  IM    
Affiliation:
Centre hospitalier du Grand-Portage, 75 rue St-Henri, Rivière-du-Loup, Quebec G5R 2A4, Canada.
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MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Connective Tissue Diseases / complications*,  genetics
Humans
Male
Nail-Patella Syndrome / complications*,  genetics
Neutropenia / complications
Osteopoikilosis / complications*,  genetics
Pedigree
Syndrome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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