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Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease

Gaucher disease (GD) is one of the most common lysosomal storage disorders. Bone complications are the most critical irreversible consequence of GD. Osteonecrosis (ON) of the femoral head inevitably leads to osteoarthritis and may be managed by hip arthroplasty. The introduction and worldwide use of...

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Autores principales: Cohen, Daniel, Levy, Yadin, Bar-Ziv, Yaron, Revel-Vilk, Shoshana, Zimran, Ari, Lebel, Ehud
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10220706/
https://www.ncbi.nlm.nih.gov/pubmed/37240780
http://dx.doi.org/10.3390/life13051135
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author Cohen, Daniel
Levy, Yadin
Bar-Ziv, Yaron
Revel-Vilk, Shoshana
Zimran, Ari
Lebel, Ehud
author_facet Cohen, Daniel
Levy, Yadin
Bar-Ziv, Yaron
Revel-Vilk, Shoshana
Zimran, Ari
Lebel, Ehud
author_sort Cohen, Daniel
collection PubMed
description Gaucher disease (GD) is one of the most common lysosomal storage disorders. Bone complications are the most critical irreversible consequence of GD. Osteonecrosis (ON) of the femoral head inevitably leads to osteoarthritis and may be managed by hip arthroplasty. The introduction and worldwide use of therapeutic agents (specifically enzyme replacement therapies (ERT)) lowered the prevalence of osteonecrosis events per patient. We present the cases of two female patients who sustained simultaneous bilateral femoral head osteonecrosis after receiving ERT for long periods while exposed to concomitant risk factors related to femoral head ON. Both patients suffered severe pain and deterioration of their daily activity capabilities, and thus, were offered bilateral hip arthroplasty. Surgery was performed in both hip joints during the same procedure. The current report highlights several key aspects of femoral head ON in young patients with GD.
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spelling pubmed-102207062023-05-28 Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease Cohen, Daniel Levy, Yadin Bar-Ziv, Yaron Revel-Vilk, Shoshana Zimran, Ari Lebel, Ehud Life (Basel) Case Report Gaucher disease (GD) is one of the most common lysosomal storage disorders. Bone complications are the most critical irreversible consequence of GD. Osteonecrosis (ON) of the femoral head inevitably leads to osteoarthritis and may be managed by hip arthroplasty. The introduction and worldwide use of therapeutic agents (specifically enzyme replacement therapies (ERT)) lowered the prevalence of osteonecrosis events per patient. We present the cases of two female patients who sustained simultaneous bilateral femoral head osteonecrosis after receiving ERT for long periods while exposed to concomitant risk factors related to femoral head ON. Both patients suffered severe pain and deterioration of their daily activity capabilities, and thus, were offered bilateral hip arthroplasty. Surgery was performed in both hip joints during the same procedure. The current report highlights several key aspects of femoral head ON in young patients with GD. MDPI 2023-05-06 /pmc/articles/PMC10220706/ /pubmed/37240780 http://dx.doi.org/10.3390/life13051135 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Cohen, Daniel
Levy, Yadin
Bar-Ziv, Yaron
Revel-Vilk, Shoshana
Zimran, Ari
Lebel, Ehud
Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease
title Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease
title_full Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease
title_fullStr Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease
title_full_unstemmed Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease
title_short Simultaneous Bilateral Femoral Osteonecrosis in Gaucher Disease
title_sort simultaneous bilateral femoral osteonecrosis in gaucher disease
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10220706/
https://www.ncbi.nlm.nih.gov/pubmed/37240780
http://dx.doi.org/10.3390/life13051135
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