Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase
- Autores
- Parisi, Muriel Solange; Mastaglia, Silvina Rosana; Bagur, Alicia Cristina; Goldstein, Griselda; Zeni, Susana Noemi; Oliveri, María Beatriz
- Año de publicación
- 2007
- Idioma
- inglés
- Tipo de recurso
- artículo
- Estado
- versión publicada
- Descripción
- Bone involvement is one of the most disabling complications in patients with type 1 Gaucher disease (GDI) and its pathophysiology is yet to be fully understood. It is well known that body composition is a determinant of bone mass. Previous reports indicating disturbance in glucose and lipid metabolism in GDI patients suggested a posible alteration in body composition in this group of patients. OBJECTIVE: To analyze body composition, bone mass and turnover in young adults with GDI receiving enzyme replacement therapy (ERT). Population: 5 women and 4 men with GDI aged (X +/- SD) 26.9 +/- 6.9 years, receiving imiglucerase in a mean dose of 53 +/- 13 IU/kg/2weeks, during 4.9 +/- 3.9 years; and 145 sex and age matched healthy adults agreed to participate in the study. All control subjects had a body mass index (BMI) between 20 and 25 kg/m2. METHODS: Total body dual X-ray absorptiometry (DXA) was used to measure body composition and bone mass. Serum creatinine, calcium, osteocalcin (BGP), and type I collagen beta carboxy-terminal telopeptide (betaCTX) were determined in patients and controls. In addition, 25 hydroxyvitamin D (25OHD), and chitotriosidase activity were measured in patients. Results: GDI patients presented statistically significant (p<0.01) lower BMI, bone mineral density (BMD), bone mineral content (BMC), lean mass (LM), and fat mass (FM), compared to controls. LM correlated positively with BMC and BMD in both groups (p<0.01). GDI patients receiving the lower dose of ERT (<60 IU/kg/2weeks) presented lower BMD values than those receiving the higher dose (> or =60 IU/kg/2weeks) (0.968 +/- 0.032 vs 1.088 +/- 0.061 g/m2, respectively, p<0.001). Mean BGP levels were similar in patients and controls, whereas betaCTX levels were higher in GDI patients (p<0.02). All patients presented deficiency levels (<30ng/ml) of 25OHD. CONCLUSIONS: Although the patients had been receiving ERT, they presented a significant diminution in all body composition parameters, the decrease was more evident in those receiving the lower dose. The reduction in bone mass was associated with an imbalance in bone turnover (increased bone resorption). The correlation between LM and bone mass, suggests that metabolic disturbance occurring in GDI patients may be indirectly responsible for bone mass reduction in GDI patients, by altering body composition
Fil: Parisi, Muriel Solange. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; Argentina
Fil: Mastaglia, Silvina Rosana. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; Argentina
Fil: Bagur, Alicia Cristina. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina
Fil: Goldstein, Griselda. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina
Fil: Zeni, Susana Noemi. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; Argentina
Fil: Oliveri, María Beatriz. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; Argentina - Materia
-
Gaucher disease
bone turnover markers
bone mineral density
imiglucerase - Nivel de accesibilidad
- acceso abierto
- Condiciones de uso
- https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
- Repositorio
- Institución
- Consejo Nacional de Investigaciones Científicas y Técnicas
- OAI Identificador
- oai:ri.conicet.gov.ar:11336/164592
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Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with ImigluceraseParisi, Muriel SolangeMastaglia, Silvina RosanaBagur, Alicia CristinaGoldstein, GriseldaZeni, Susana NoemiOliveri, María BeatrizGaucher diseasebone turnover markersbone mineral densityimiglucerasehttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3Bone involvement is one of the most disabling complications in patients with type 1 Gaucher disease (GDI) and its pathophysiology is yet to be fully understood. It is well known that body composition is a determinant of bone mass. Previous reports indicating disturbance in glucose and lipid metabolism in GDI patients suggested a posible alteration in body composition in this group of patients. OBJECTIVE: To analyze body composition, bone mass and turnover in young adults with GDI receiving enzyme replacement therapy (ERT). Population: 5 women and 4 men with GDI aged (X +/- SD) 26.9 +/- 6.9 years, receiving imiglucerase in a mean dose of 53 +/- 13 IU/kg/2weeks, during 4.9 +/- 3.9 years; and 145 sex and age matched healthy adults agreed to participate in the study. All control subjects had a body mass index (BMI) between 20 and 25 kg/m2. METHODS: Total body dual X-ray absorptiometry (DXA) was used to measure body composition and bone mass. Serum creatinine, calcium, osteocalcin (BGP), and type I collagen beta carboxy-terminal telopeptide (betaCTX) were determined in patients and controls. In addition, 25 hydroxyvitamin D (25OHD), and chitotriosidase activity were measured in patients. Results: GDI patients presented statistically significant (p<0.01) lower BMI, bone mineral density (BMD), bone mineral content (BMC), lean mass (LM), and fat mass (FM), compared to controls. LM correlated positively with BMC and BMD in both groups (p<0.01). GDI patients receiving the lower dose of ERT (<60 IU/kg/2weeks) presented lower BMD values than those receiving the higher dose (> or =60 IU/kg/2weeks) (0.968 +/- 0.032 vs 1.088 +/- 0.061 g/m2, respectively, p<0.001). Mean BGP levels were similar in patients and controls, whereas betaCTX levels were higher in GDI patients (p<0.02). All patients presented deficiency levels (<30ng/ml) of 25OHD. CONCLUSIONS: Although the patients had been receiving ERT, they presented a significant diminution in all body composition parameters, the decrease was more evident in those receiving the lower dose. The reduction in bone mass was associated with an imbalance in bone turnover (increased bone resorption). The correlation between LM and bone mass, suggests that metabolic disturbance occurring in GDI patients may be indirectly responsible for bone mass reduction in GDI patients, by altering body compositionFil: Parisi, Muriel Solange. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; ArgentinaFil: Mastaglia, Silvina Rosana. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; ArgentinaFil: Bagur, Alicia Cristina. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; ArgentinaFil: Goldstein, Griselda. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; ArgentinaFil: Zeni, Susana Noemi. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; ArgentinaFil: Oliveri, María Beatriz. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; ArgentinaHolzapfel Verlag2007-12info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/164592Parisi, Muriel Solange; Mastaglia, Silvina Rosana; Bagur, Alicia Cristina; Goldstein, Griselda; Zeni, Susana Noemi; et al.; Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase; Holzapfel Verlag; European Journal Of Medical Research; 12-2007; 1-80949-2321CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://pubmed.ncbi.nlm.nih.gov/18226995/info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-09-03T09:55:00Zoai:ri.conicet.gov.ar:11336/164592instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-09-03 09:55:00.912CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse |
dc.title.none.fl_str_mv |
Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase |
title |
Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase |
spellingShingle |
Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase Parisi, Muriel Solange Gaucher disease bone turnover markers bone mineral density imiglucerase |
title_short |
Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase |
title_full |
Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase |
title_fullStr |
Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase |
title_full_unstemmed |
Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase |
title_sort |
Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase |
dc.creator.none.fl_str_mv |
Parisi, Muriel Solange Mastaglia, Silvina Rosana Bagur, Alicia Cristina Goldstein, Griselda Zeni, Susana Noemi Oliveri, María Beatriz |
author |
Parisi, Muriel Solange |
author_facet |
Parisi, Muriel Solange Mastaglia, Silvina Rosana Bagur, Alicia Cristina Goldstein, Griselda Zeni, Susana Noemi Oliveri, María Beatriz |
author_role |
author |
author2 |
Mastaglia, Silvina Rosana Bagur, Alicia Cristina Goldstein, Griselda Zeni, Susana Noemi Oliveri, María Beatriz |
author2_role |
author author author author author |
dc.subject.none.fl_str_mv |
Gaucher disease bone turnover markers bone mineral density imiglucerase |
topic |
Gaucher disease bone turnover markers bone mineral density imiglucerase |
purl_subject.fl_str_mv |
https://purl.org/becyt/ford/3.2 https://purl.org/becyt/ford/3 |
dc.description.none.fl_txt_mv |
Bone involvement is one of the most disabling complications in patients with type 1 Gaucher disease (GDI) and its pathophysiology is yet to be fully understood. It is well known that body composition is a determinant of bone mass. Previous reports indicating disturbance in glucose and lipid metabolism in GDI patients suggested a posible alteration in body composition in this group of patients. OBJECTIVE: To analyze body composition, bone mass and turnover in young adults with GDI receiving enzyme replacement therapy (ERT). Population: 5 women and 4 men with GDI aged (X +/- SD) 26.9 +/- 6.9 years, receiving imiglucerase in a mean dose of 53 +/- 13 IU/kg/2weeks, during 4.9 +/- 3.9 years; and 145 sex and age matched healthy adults agreed to participate in the study. All control subjects had a body mass index (BMI) between 20 and 25 kg/m2. METHODS: Total body dual X-ray absorptiometry (DXA) was used to measure body composition and bone mass. Serum creatinine, calcium, osteocalcin (BGP), and type I collagen beta carboxy-terminal telopeptide (betaCTX) were determined in patients and controls. In addition, 25 hydroxyvitamin D (25OHD), and chitotriosidase activity were measured in patients. Results: GDI patients presented statistically significant (p<0.01) lower BMI, bone mineral density (BMD), bone mineral content (BMC), lean mass (LM), and fat mass (FM), compared to controls. LM correlated positively with BMC and BMD in both groups (p<0.01). GDI patients receiving the lower dose of ERT (<60 IU/kg/2weeks) presented lower BMD values than those receiving the higher dose (> or =60 IU/kg/2weeks) (0.968 +/- 0.032 vs 1.088 +/- 0.061 g/m2, respectively, p<0.001). Mean BGP levels were similar in patients and controls, whereas betaCTX levels were higher in GDI patients (p<0.02). All patients presented deficiency levels (<30ng/ml) of 25OHD. CONCLUSIONS: Although the patients had been receiving ERT, they presented a significant diminution in all body composition parameters, the decrease was more evident in those receiving the lower dose. The reduction in bone mass was associated with an imbalance in bone turnover (increased bone resorption). The correlation between LM and bone mass, suggests that metabolic disturbance occurring in GDI patients may be indirectly responsible for bone mass reduction in GDI patients, by altering body composition Fil: Parisi, Muriel Solange. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; Argentina Fil: Mastaglia, Silvina Rosana. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; Argentina Fil: Bagur, Alicia Cristina. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina Fil: Goldstein, Griselda. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina Fil: Zeni, Susana Noemi. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; Argentina Fil: Oliveri, María Beatriz. Universidad de Buenos Aires. Facultad de Medicina. Hospital de Clínicas General San Martín; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay; Argentina |
description |
Bone involvement is one of the most disabling complications in patients with type 1 Gaucher disease (GDI) and its pathophysiology is yet to be fully understood. It is well known that body composition is a determinant of bone mass. Previous reports indicating disturbance in glucose and lipid metabolism in GDI patients suggested a posible alteration in body composition in this group of patients. OBJECTIVE: To analyze body composition, bone mass and turnover in young adults with GDI receiving enzyme replacement therapy (ERT). Population: 5 women and 4 men with GDI aged (X +/- SD) 26.9 +/- 6.9 years, receiving imiglucerase in a mean dose of 53 +/- 13 IU/kg/2weeks, during 4.9 +/- 3.9 years; and 145 sex and age matched healthy adults agreed to participate in the study. All control subjects had a body mass index (BMI) between 20 and 25 kg/m2. METHODS: Total body dual X-ray absorptiometry (DXA) was used to measure body composition and bone mass. Serum creatinine, calcium, osteocalcin (BGP), and type I collagen beta carboxy-terminal telopeptide (betaCTX) were determined in patients and controls. In addition, 25 hydroxyvitamin D (25OHD), and chitotriosidase activity were measured in patients. Results: GDI patients presented statistically significant (p<0.01) lower BMI, bone mineral density (BMD), bone mineral content (BMC), lean mass (LM), and fat mass (FM), compared to controls. LM correlated positively with BMC and BMD in both groups (p<0.01). GDI patients receiving the lower dose of ERT (<60 IU/kg/2weeks) presented lower BMD values than those receiving the higher dose (> or =60 IU/kg/2weeks) (0.968 +/- 0.032 vs 1.088 +/- 0.061 g/m2, respectively, p<0.001). Mean BGP levels were similar in patients and controls, whereas betaCTX levels were higher in GDI patients (p<0.02). All patients presented deficiency levels (<30ng/ml) of 25OHD. CONCLUSIONS: Although the patients had been receiving ERT, they presented a significant diminution in all body composition parameters, the decrease was more evident in those receiving the lower dose. The reduction in bone mass was associated with an imbalance in bone turnover (increased bone resorption). The correlation between LM and bone mass, suggests that metabolic disturbance occurring in GDI patients may be indirectly responsible for bone mass reduction in GDI patients, by altering body composition |
publishDate |
2007 |
dc.date.none.fl_str_mv |
2007-12 |
dc.type.none.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://purl.org/coar/resource_type/c_6501 info:ar-repo/semantics/articulo |
format |
article |
status_str |
publishedVersion |
dc.identifier.none.fl_str_mv |
http://hdl.handle.net/11336/164592 Parisi, Muriel Solange; Mastaglia, Silvina Rosana; Bagur, Alicia Cristina; Goldstein, Griselda; Zeni, Susana Noemi; et al.; Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase; Holzapfel Verlag; European Journal Of Medical Research; 12-2007; 1-8 0949-2321 CONICET Digital CONICET |
url |
http://hdl.handle.net/11336/164592 |
identifier_str_mv |
Parisi, Muriel Solange; Mastaglia, Silvina Rosana; Bagur, Alicia Cristina; Goldstein, Griselda; Zeni, Susana Noemi; et al.; Body Composition and Bone Metabolism in Young Gaucher Disease Type I Patients Treated with Imiglucerase; Holzapfel Verlag; European Journal Of Medical Research; 12-2007; 1-8 0949-2321 CONICET Digital CONICET |
dc.language.none.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
info:eu-repo/semantics/altIdentifier/url/https://pubmed.ncbi.nlm.nih.gov/18226995/ |
dc.rights.none.fl_str_mv |
info:eu-repo/semantics/openAccess https://creativecommons.org/licenses/by-nc-sa/2.5/ar/ |
eu_rights_str_mv |
openAccess |
rights_invalid_str_mv |
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/ |
dc.format.none.fl_str_mv |
application/pdf application/pdf application/pdf |
dc.publisher.none.fl_str_mv |
Holzapfel Verlag |
publisher.none.fl_str_mv |
Holzapfel Verlag |
dc.source.none.fl_str_mv |
reponame:CONICET Digital (CONICET) instname:Consejo Nacional de Investigaciones Científicas y Técnicas |
reponame_str |
CONICET Digital (CONICET) |
collection |
CONICET Digital (CONICET) |
instname_str |
Consejo Nacional de Investigaciones Científicas y Técnicas |
repository.name.fl_str_mv |
CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas |
repository.mail.fl_str_mv |
dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar |
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1842269318192037888 |
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13.13397 |