title: Evaluation of Bone Mineral Density in Patients with Type 1 Gaucher Disease in Argentina.
abstract: The purpose of this study was to evaluate the frequency of osteoporosis (OP) in patients with Gaucher disease (GD) in Argentina. GD patients from 28 centers were consecutively included from April 2012 to 2014. Bone mineral density (BMD) was determined by dual X-ray absorptiometry in the lumbar spine and the femoral neck or the total proximal femur for patients ≥20 yr of age, and by whole-body scan in the lumbar spine in patients <20 yr of age. In children, mineral density was calculated using the chronological age and Z height. OP diagnosis was determined following adult and pediatric official position of the International Society for Clinical Densitometry. A total of 116 patients were included, of which 62 (53.5%) were women. The median age was 25.8 yr. All patients received enzyme replacement therapy, with a median time of 9.4 yr. Normal BMD was found in 89 patients (76.7%), whereas low bone mass (LBM) or osteopenia was found in 15 patients (13%) and OP in 12 patients (10.3%). The analysis of the pediatric population revealed that 4 patients (9.3%) had LBM and 3 (7%) had OP (Z-score ≤ -2 + fractures height-adjusted by Z), whereas in the adult population (n = 73), 11 patients (15%) had LBM or osteopenia and 9 (12.3%) had OP. Bone marrow infiltration and the presence of fractures were significantly correlated with the presence of OP (p = 0.04 and <0.001, respectively). This is the first study in Argentina and in the region describing the frequency of OP or LBM in GD patients treated with imiglucerase using the official position of the International Society for Clinical Densitometry.
authors: Departamento de Densitometría ósea, Centro de Diagnóstico E. Rossi, Buenos Aires, Argentina; Departamento de Densitometría ósea, Hospital Cesar Milstein, Buenos Aires, Argentina. Electronic address: mlarroude@cdrossi.com.
authors: M S
authors: MS
authors: Larroudé
authors: Departamento de Densitometría ósea, Centro de Diagnóstico E. Rossi, Buenos Aires, Argentina.
authors: G
authors: G
authors: Aguilar
authors: Departamento de Densitometría ósea, Centro de Diagnóstico E. Rossi, Buenos Aires, Argentina.
authors: I
authors: I
authors: Rossi
authors: Hospital de Niños "Ricardo Gutiérrez," CABA, Buenos Aires, Argentina.
authors: G
authors: G
authors: Drelichman
authors: Hospital de Niños "Ricardo Gutiérrez," CABA, Buenos Aires, Argentina.
authors: N
authors: N
authors: Fernandez Escobar
authors: Hospital de Niños "Ricardo Gutiérrez," CABA, Buenos Aires, Argentina.
authors: N
authors: N
authors: Basack
authors: Department of Hematology, Laboratorio de Neuroquímica "Dr. N.A. Chamoles," Buenos Aires, Argentina.
authors: M
authors: M
authors: Slago
authors: Department of Hematology, Laboratorio de Neuroquímica "Dr. N.A. Chamoles," Buenos Aires, Argentina.
authors: A
authors: A
authors: Schenone
authors: Department of Hematology, Hospital de Niños "Sor María Ludovica," La Plata, Argentina.
authors: A
authors: A
authors: Fynn
authors: Department of Hematology, Hospital de Niños "Sor María Ludovica," La Plata, Argentina.
authors: M F
authors: MF
authors: Cuello
authors: Department of Hematology, Hospital de Niños "Sor María Ludovica," La Plata, Argentina.
authors: R
authors: R
authors: Fernandez
authors: Department of Hematology, Hospital CEpsi Eva Perón, Santiago del Estero, Argentina.
authors: A
authors: A
authors: Ruiz
authors: Department of Hematology, Hospital CEpsi Eva Perón, Santiago del Estero, Argentina.
authors: P
authors: P
authors: Reichel
authors: Department of Hematology, Hospital Provincial de Niños "Santa Trinidad," Córdoba, Argentina.
authors: N
authors: N
authors: Guelbert
authors: Department of Hematology, Hospital Provincial de Niños "Santa Trinidad," Córdoba, Argentina.
authors: H
authors: H
authors: Robledo
authors: Hospital Ramos Mejía, CABA, Buenos Aires, Argentina.
authors: N
authors: N
authors: Watman
authors: Hospital Ramos Mejía, CABA, Buenos Aires, Argentina.
authors: M
authors: M
authors: Bolesina
authors: Hospital de Niños Pedro de Elizalde, CABA, Buenos Aires, Argentina.
authors: G
authors: G
authors: Elena
authors: Hospital de Niños Pedro de Elizalde, CABA, Buenos Aires, Argentina.
authors: S E
authors: SE
authors: Veber
authors: Department of Hematology, Hospital "Dr. Julio C. Perrando," Chaco, Argentina.
authors: G
authors: G
authors: Pujal
authors: Department of Hematology, Hospital "Dr. Julio C. Perrando," Chaco, Argentina.
authors: G
authors: G
authors: Galvan
authors: Department of Hematology, Hospital del Niño Jesús, Tucumán, Argentina.
authors: J J
authors: JJ
authors: Chain
authors: Department of Hematology, Hospital Iturraspe, Santa Fe, Argentina.
authors: A
authors: A
authors: Arizo
authors: Department of Hematology, Hospital Iturraspe, Santa Fe, Argentina.
authors: J
authors: J
authors: Bietti
authors: Department of Hematology, Instituto Médico Platense, La Plata, Argentina.
authors: M
authors: M
authors: Aznar
authors: Department of Hematology, Hospital de Oncología "M. Curie," Buenos Aires, Argentina.
authors: M
authors: M
authors: Dragosky
authors: Department of Hematology, Hospital de Oncología "M. Curie," Buenos Aires, Argentina.
authors: M
authors: M
authors: Marquez
authors: Clínica Modelo de Tandil, Pcia, Buenos Aires, Argentina.
authors: L
authors: L
authors: Feldman
authors: Clínica Modelo de Tandil, Pcia, Buenos Aires, Argentina.
authors: K
authors: K
authors: Muller
authors: Department of Hematology, Clínica del Niño del Rosario, Santa Fe, Argentina.
authors: S
authors: S
authors: Zirone
authors: Department of Hematology, Hospital Escuela de Corrientes, Corrientes, Argentina.
authors: G
authors: G
authors: Buchovsky
authors: Hospital Materno Infantil de Mar del Plata, Pcia, Buenos Aires, Argentina.
authors: V
authors: V
authors: Lanza
authors: Hospital de Del Viso, Pcia, Buenos Aires, Argentina.
authors: I
authors: I
authors: Fernandez
authors: Department of Hematology, Hospital de San Martín, La Plata, Argentina.
authors: R
authors: R
authors: Jaureguiberry
authors: Hospital Alemán, CABA, Buenos Aires, Argentina.
authors: M A
authors: MA
authors: Barbieri
authors: Hospital Alemán, CABA, Buenos Aires, Argentina.
authors: A
authors: A
authors: Maro
authors: Hospital Pirovano, CABA, Buenos Aires, Argentina.
authors: G
authors: G
authors: Zarate
authors: Hospital Pirovano, CABA, Buenos Aires, Argentina.
authors: G
authors: G
authors: Fernandez
authors: Hospital de Niños de San Justo, Pcia, Buenos Aires, Argentina.
authors: M
authors: M
authors: Rapetti
authors: Sanatorio General Sarmiento, Pcia, Buenos Aires, Argentina.
authors: A
authors: A
authors: Degano
authors: Hospital Durand, CABA, Buenos Aires, Argentina.
authors: G
authors: G
authors: Kantor
authors: Consultorio Particular, Mar Del Plata, Prov, Buenos Aires, Argentina.
authors: A
authors: A
authors: Albina
authors: Department of Hematology, Sanatorio Allende de Córdoba, Córdoba, Argentina.
authors: M
authors: M
authors: Alvarez Bollea
authors: Hospital Centenario, Gualeguychu, Entre Ríos, Argentina.
authors: H
authors: H
authors: Arrocena
authors: Department of Hematology, Hospital Lagomaggiore, Mendoza, Argentina.
authors: V
authors: V
authors: Bacciedoni
authors: Department of Hematology, Hospital Lagomaggiore, Mendoza, Argentina.
authors: F
authors: F
authors: Del Rio
mesh_terms: D015502:Absorptiometry, Photon / Q000379:methods; D000293:Adolescent; D000328:Adult; D000368:Aged; D001118:Argentina / Q000453:epidemiology; D015519:Bone Density; D001851:Bone Diseases, Metabolic / Q000150:complications / Q000000981:diagnostic imaging / Q000453:epidemiology; D002648:Child; D005260:Female; D005272:Femur Neck / Q000000981:diagnostic imaging; D005776:Gaucher Disease / Q000150:complications* / Q000453:epidemiology; D006801:Humans; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging; D008297:Male; D008875:Middle Aged; D010024:Osteoporosis / Q000150:complications* / Q000000981:diagnostic imaging* / Q000453:epidemiology; D055815:Young Adult
keywords: Argentina; Gaucher disease; bone mineral density; osteoporosis