Evaluation of Bone Mineral Density in Patients with Type 1 Gaucher Disease in Argentina

被引：1

作者：

Larroude, M. S. ^{[1
,2
]}

Aguilar, G. ^{[1
]}

Rossi, I. ^{[1
]}

Drelichman, G. ^{[3
]}

Fernandez Escobar, N. ^{[3
]}

Basack, N. ^{[3
]}

Slago, M. ^{[4
]}

Schenone, A. ^{[4
]}

Fynn, A. ^{[5
]}

Cuello, M. F. ^{[5
]}

Fernandez, R. ^{[5
]}

Ruiz, A. ^{[6
]}

Reichel, P. ^{[6
]}

Guelbert, N. ^{[7
]}

Robledo, H. ^{[7
]}

Watman, N. ^{[8
]}

Bolesina, M. ^{[8
]}

Elena, G. ^{[9
]}

Veber, S. E. ^{[9
]}

Pujal, G. ^{[10
]}

Galvan, G. ^{[10
]}

Chain, J. J. ^{[11
]}

Arizo, A. ^{[12
]}

Bietti, J. ^{[12
]}

Aznar, M. ^{[13
]}

Dragosky, M. ^{[14
]}

Marquez, M. ^{[14
]}

Feldman, L. ^{[15
]}

Muller, K. ^{[15
]}

Zirone, S. ^{[16
]}

Buchovsky, G. ^{[17
]}

Lanza, V. ^{[18
]}

Fernandez, I. ^{[19
]}

Jaureguiberry, R. ^{[20
]}

Barbieri, M. A. ^{[21
]}

Maro, A. ^{[21
]}

Zarate, G. ^{[22
]}

Fernandez, G. ^{[22
]}

Rapetti, M. ^{[23
]}

Degano, A. ^{[24
]}

Kantor, G. ^{[25
]}

Albina, A. ^{[26
]}

Alvarez Bollea, M. ^{[27
]}

Arrocena, H. ^{[28
]}

Bacciedoni, V. ^{[29
]}

Del Rio, F. ^{[29
]}

机构：

[1] Ctr Diagnost E Rossi, Dept Densitometria Osea, Prudan 422, RA-1872 Buenos Aires, DF, Argentina

[2] Hosp Cesar Milstein, Dept Densitometria Osea, Buenos Aires, DF, Argentina

[3] Hosp Ninos Dr Ricardo Gutierrez, CABA, Buenos Aires, DF, Argentina

[4] Lab Neuroquim Dr NA Chamoles, Dept Hematol, Buenos Aires, DF, Argentina

[5] Hosp Ninos Sor Maria Ludovica, Dept Hematol, La Plata, Buenos Aires, Argentina

[6] Hosp CEpsi Eva Peron, Dept Hematol, Santiago Del Estero, Argentina

[7] Hosp Prov Ninos Santa Trinidad, Dept Hematol, Cordoba, Argentina

[8] Hosp Ramos Mejia, CABA, Buenos Aires, DF, Argentina

[9] Hosp Ninos Pedro de Elizalde, CABA, Buenos Aires, DF, Argentina

[10] Hosp Dr Julio C Perrando, Dept Hematol, Chaco, Argentina

[11] Hosp Nino Jesus, Dept Hematol, San Miguel De Tucuman, Argentina

[12] Hosp Iturraspe, Dept Hematol, Santa Fe, Argentina

[13] Inst Med Platense, Dept Hematol, La Plata, Buenos Aires, Argentina

[14] Hosp Oncol M Curie, Dept Hematol, Buenos Aires, DF, Argentina

[15] Clin Modelo Tandil, Buenos Aires, DF, Argentina

[16] Clin Nino Rosario, Dept Hematol, Santa Fe, Argentina

[17] Hosp Escuela Corrientes, Dept Hematol, Corrientes, Argentina

[18] Hosp Materno Infantil Mar del Plata, Buenos Aires, DF, Argentina

[19] Hosp Del Viso, Buenos Aires, DF, Argentina

[20] Hosp San Martin, Dept Hematol, La Plata, Buenos Aires, Argentina

[21] Hosp Aleman, CABA, Buenos Aires, DF, Argentina

[22] Hosp Pirovano, CABA, Buenos Aires, DF, Argentina

[23] Hosp Ninos San Justo, Buenos Aires, DF, Argentina

[24] Sanatorio Gen Sarmiento, Buenos Aires, DF, Argentina

[25] Hosp Durand, CABA, Buenos Aires, DF, Argentina

[26] Consultorio Particular, Buenos Aires, DF, Argentina

[27] Sanatorio Allende Cordoba, Dept Hematol, Cordoba, Argentina

[28] Hosp Centenario, Gualeguychu, Entre Rios, Argentina

[29] Hosp Lagomaggiore, Dept Hematol, Mendoza, Argentina

来源：

JOURNAL OF CLINICAL DENSITOMETRY | 2016年 / 19卷 / 04期

关键词：

Argentina; bone mineral density; Gaucher disease; osteoporosis; PEDIATRIC OFFICIAL POSITIONS; ENZYME REPLACEMENT THERAPY; FRACTURE PREDICTION; CHILDREN; OSTEOPOROSIS; DENSITOMETRY; ADOLESCENTS; BMD;

D O I：

10.1016/j.jocd.2016.07.003

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：444 / 449

页数：6

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