Document Detail

Perthes-like disease in Alagille syndrome.
MedLine Citation:
PMID:  21642856     Owner:  NLM     Status:  MEDLINE    
We describe a unique case of a bilateral osteochondrosis of the femoral heads, similar to Perthes disease, in a boy affected by Alagille syndrome. This is a rare genetic syndrome, caused by vascular anomalies, and characterized by five main features: hepatic, cardiovascular, ophthalmological, skeletal malformations, and characteristic facial appearance. The most frequent skeletal finding is the 'butterfly vertebra'. We have followed the patient from the age of 5 years to the age of 20 years. We performed two bilateral valgus osteotomies when he was 10 years old to limit the progression of the deformity. We believe that the association of a bilateral osteochondrosis of the femoral heads with Alagille syndrome, a disease characterized by a vascular etiology, supports the hypothesis of angiogenic pathogenesis of Perthes disease.
Marco Massobrio; Giorgio Antonietti; Fabiana Necci; Cristina Esposito
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric orthopedics. Part B     Volume:  20     ISSN:  1473-5865     ISO Abbreviation:  J Pediatr Orthop B     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-08-08     Completed Date:  2011-12-15     Revised Date:  2012-09-17    
Medline Journal Info:
Nlm Unique ID:  9300904     Medline TA:  J Pediatr Orthop B     Country:  United States    
Other Details:
Languages:  eng     Pagination:  299-302     Citation Subset:  IM    
Sapienza, University of Rome, Rome, Italy.
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MeSH Terms
Alagille Syndrome / complications*,  pathology,  surgery
Child, Preschool
Coxa Valga / surgery
Femur Head / pathology
Legg-Calve-Perthes Disease / complications*,  pathology,  surgery
Osteotomy / methods
Vascular Diseases
Young Adult

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

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