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Histologic findings of femoral heads from patients with Gaucher disease treated with enzyme replacement.
MedLine Citation:
PMID:  23765538     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Objectives: To assess correlations of patient demographics, including enzyme replacement therapy (ERT) with bone histology, to facilitate decisions of whether and when to perform hip replacement surgery in patients with Gaucher disease. Methods: We examined the histology of surgically removed femoral heads and categorized findings by the presence or extent of osteonecrosis, Gaucher cell (GC) infiltration, and bone regeneration qualifiers using a tripartite histology-based scoring system. Results: Twenty-two patients with 26 bone specimens were evaluated. Seventeen patients (77%) were splenectomized, 16 (73%) received ERT, and 12 (55%) had the putatively milder genotype (N370S/N370S), with the rest putatively at increased risk for skeletal disease (N370S/other). The 3 histology subscores were applicable to all specimens. Osteonecrotic bone was seen in 19 of 26 (73%); osteoarthritis was seen in all cartilage specimens. Gaucher cell infiltration was not correlated with demographics or disease severity. A trend was noted between reduced GC infiltration and ERT (ρ = 0.407), but regeneration qualifiers were not correlated with ERT or other features. Conclusions: Histologic findings of GC infiltration and bone regeneration qualifiers did not correlate with demographics or with exposure to ERT. Most specimens unexpectedly showed good regenerative responses to osteonecrosis despite heavy GC infiltration.
Authors:
Ehud Lebel; Deborah Elstein; Ariel Peleg; Constantine Reinus; Ari Zimran; Gail Amir
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of clinical pathology     Volume:  140     ISSN:  1943-7722     ISO Abbreviation:  Am. J. Clin. Pathol.     Publication Date:  2013 Jul 
Date Detail:
Created Date:  2013-06-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0370470     Medline TA:  Am J Clin Pathol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  91-6     Citation Subset:  AIM; IM    
Affiliation:
Gaucher Clinic/Shaare Zedek Medical Center, PO Box 3235, Jerusalem 91031, Israel; elstein@szmc.org.il.
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