Risk Factors For Developing Avascular Necrosis Or Fractures In Patients With Type 1 Gaucher Disease: Analysis From The Gaucher Registry

2012 ◽  
Vol 105 (2) ◽  
pp. S39-S40 ◽  
Author(s):  
Aneal Khan ◽  
Thomas Hangartner ◽  
Neal Weinreb ◽  
John Taylor ◽  
Pramod Mistry
Keyword(s):  
Risk Factors ◽  
Avascular Necrosis ◽  
Gaucher Disease ◽  
Type 1 Gaucher Disease ◽  
Disease Analysis

149. Avascular Necrosis in type I Gaucher disease: Analysis of risk factors

2009 ◽  
Vol 96 (2) ◽  
pp. S45
Author(s):  
Neal Weinreb ◽  
Patrick Deegan ◽  
Ashok Vellodi ◽  
J. Alexander Cole ◽  
Michael Yeh ◽  
...  
Keyword(s):  
Risk Factors ◽  
Avascular Necrosis ◽  
Gaucher Disease ◽  
Type I ◽  
Disease Analysis

Avascular Necrosis in Untreated Patients with Type 1 Gaucher Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1353-1353
Author(s):  
Pramod K Mistry ◽  
Patrick Deegan ◽  
Ashok Vellodi ◽  
J. Alexander Cole ◽  
Michael Yeh ◽  
...  
Keyword(s):  
Incidence Rate ◽  
Avascular Necrosis ◽  
Gaucher Disease ◽  
Incidence Rates ◽  
Advisory Committees ◽  
Type 1 Gaucher Disease ◽  
Receive Treatment ◽  
To Receive

Abstract Abstract 1353 Poster Board I-375 Objective To determine the incidence of avascular necrosis (AVN) in untreated patients with type 1 Gaucher disease (GD1). Methods All patients with GD1 enrolled in the ICGG Gaucher Registry as of July 2007 were included in this analysis. All GD1 patients who either never received treatment or eventually went on to receive treatment were identified. Follow-up began on each patient's date of earliest reported assessment in the Registry. Among patients who never received treatment, follow-up continued until the last recorded assessment date in the Registry. For patients who eventually went on to receive treatment, follow-up continued until the date of initiation of therapy. Incidence rates (and Poisson exact 95% confidence intervals) of AVN were determined for both groups of patients. AVN was typically ascertained from X-Ray or MRI results. Results As of July 2007, the inclusion criteria were met by 3,497 patients. The incidence rate of AVN among untreated patients was 22.8 per 1,000 person years (95% CI 20.2 to 25.7). Patients with antecedent splenectomy (total or partial) had a higher incidence rate of AVN (46.6 per 1,000 person-years, 95% CI 38.4 to 56.1) compared to patients without a splenectomy (incidence rate 17.0 per 1,000 person-years, 95% CI 14.5 to 19.8). The primary sites where AVN was identified in both groups were the hip and femur. Conclusion This is the first epidemiologic analysis to estimate incidence rates of AVN among untreated patients with GD1. Splenectomy appears to be a risk factor for GD1-associated AVN. Based on the incidence results above and presupposing equal risk distribution, without therapeutic intervention, most patients should theoretically experience at least one episode of AVN at some point in their life. However, because the GD1 population is genotypically and phenotypically heterogeneous, further analyses will attempt to identify characteristics that distinguish untreated patients at high risk of developing AVN from those who are less likely to develop this serious complication. Disclosures Mistry: Genzyme Corporation: Honoraria, Research Funding. Deegan:Genzyme Corporation: Honoraria. Vellodi:Genzyme Corporation: Honoraria, Speakers Bureau. Cole:Genzyme Corporation: Employment. Yeh:Genzyme Corporation: Employment. Weinreb:Genzyme Corporation: Honoraria, Membership on an entity's Board of Directors or advisory committees.

scholarly journals Liver steatosis is highly prevalent and is associated with metabolic risk factors and liver fibrosis in adult patients with type 1 Gaucher disease

2020 ◽  
Vol 40 (12) ◽  
pp. 3061-3070 ◽  
Author(s):  
Fabio Nascimbeni ◽  
Simonetta Lugari ◽  
Elena Cassinerio ◽  
Irene Motta ◽  
Alessia Cavicchioli ◽  
...  
Keyword(s):  
Risk Factors ◽  
Liver Fibrosis ◽  
Gaucher Disease ◽  
Liver Steatosis ◽  
Metabolic Risk Factors ◽  
Adult Patients ◽  
Metabolic Risk ◽  
Type 1 Gaucher Disease

Bone Marker Alterations in Patients with Type 1 Gaucher Disease

2003 ◽  
Vol 72 (3) ◽  
pp. 185-189 ◽  
Author(s):  
G. Ciana ◽  
C. Martini ◽  
A. Leopaldi ◽  
G. Tamaro ◽  
F. Katouzian ◽  
...  
Keyword(s):  
Gaucher Disease ◽  
Bone Marker ◽  
Type 1 Gaucher Disease
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