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Evaluation of bone mineral density and microarchitectural parameters by DXA and HR-pQCT in 37 children and adults with X-linked hypophosphatemic rickets.
Osteoporos Int. 2017 05; 28(5):1685-1692.OI

Abstract

In X-linked hypophosphatemic (XLH) rickets, dual-energy X-ray absorptiometry (DXA) measurements must be analyzed with caution. High-resolution peripheral quantitative computed tomography (HR-pQCT) analysis suggested that XLH primarily affects the cancellous compartment, with the tibia more affected than the radius. Effective treatment of XLH appears to positively affect bone mineralization, mainly in the bone cortex.

INTRODUCTION

The purpose of this study is to evaluate bone mineral density (BMD) and microarchitecture in 37 patients (13 children and 24 adults) with XLH confirmed by PHEX mutations from a tertiary center compared to healthy controls.

METHODS

Areal BMD (aBMD) was evaluated by DXA, whereas volumetric BMD (vBMD) and microarchitectural parameters were analyzed by HR-pQCT.

RESULTS

Adult XLH patients had higher lumbar aBMD (p < 0.01) than the controls. At the radius, the vBMD was similar between XLH patients and controls. At the tibia, XLH patients had lower total vBMD (p = 0.04), likely resulting from decreased trabecular vBMD (p < 0.01), and this difference was observed in the children and adult groups. Analysis based on metabolic status showed that the adult XLH patients with non-compensated disease had lower cortical vBMD at the tibia than the compensated XLH patients (p = 0.03). The microarchitectural differences at the radius and tibia included lower trabecular number (p < 0.01), greater trabecular separation (p < 0.01), and higher trabecular network inhomogeneity (p < 0.01) in XLH patients compared to their controls. At the radius, adults exhibited greater trabecular deficits than were seen in children.

CONCLUSIONS

In XLH patients, DXA measurements must be analyzed with caution due to the interference of anatomic and anthropometric factors. HR-pQCT analysis suggested that XLH primarily affects the cancellous compartment, with the tibia more affected than the radius. Effective treatment of XLH appears to positively affect bone mineralization, mainly in the bone cortex.

Authors+Show Affiliations

Osteometabolic Disorders Unit, Endocrinology Division, Hospital das Clínicas da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar, 155 - PAMB, 8° andar, Bloco 3, São Paulo, SP, 05403-900, Brazil. Hormone and Molecular Genetics Laboratory (LIM/42), Endocrinology Division, Hospital das Clínicas da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar, 155 - PAMB, 2° andar, Bloco 6, São Paulo, SP, 05403-900, Brazil.Bone Metabolism Laboratory (LIM/17), Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455, 3° andar, Sala 3193, São Paulo, SP, 01246-903, Brazil.Bone Metabolism Laboratory (LIM/17), Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455, 3° andar, Sala 3193, São Paulo, SP, 01246-903, Brazil.Bone Metabolism Laboratory (LIM/17), Rheumatology Division, Faculdade de Medicina da Universidade de São Paulo, Av. Dr. Arnaldo, 455, 3° andar, Sala 3193, São Paulo, SP, 01246-903, Brazil.Hormone and Molecular Genetics Laboratory (LIM/42), Endocrinology Division, Hospital das Clínicas da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar, 155 - PAMB, 2° andar, Bloco 6, São Paulo, SP, 05403-900, Brazil.Osteometabolic Disorders Unit, Endocrinology Division, Hospital das Clínicas da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar, 155 - PAMB, 8° andar, Bloco 3, São Paulo, SP, 05403-900, Brazil. reginamm@usp.br. Hormone and Molecular Genetics Laboratory (LIM/42), Endocrinology Division, Hospital das Clínicas da Universidade de São Paulo, Av. Dr. Enéas de Carvalho Aguiar, 155 - PAMB, 2° andar, Bloco 6, São Paulo, SP, 05403-900, Brazil. reginamm@usp.br.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

28194480

Citation

Colares Neto, G P., et al. "Evaluation of Bone Mineral Density and Microarchitectural Parameters By DXA and HR-pQCT in 37 Children and Adults With X-linked Hypophosphatemic Rickets." Osteoporosis International : a Journal Established as Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA, vol. 28, no. 5, 2017, pp. 1685-1692.
Colares Neto GP, Pereira RM, Alvarenga JC, et al. Evaluation of bone mineral density and microarchitectural parameters by DXA and HR-pQCT in 37 children and adults with X-linked hypophosphatemic rickets. Osteoporos Int. 2017;28(5):1685-1692.
Colares Neto, G. P., Pereira, R. M., Alvarenga, J. C., Takayama, L., Funari, M. F., & Martin, R. M. (2017). Evaluation of bone mineral density and microarchitectural parameters by DXA and HR-pQCT in 37 children and adults with X-linked hypophosphatemic rickets. Osteoporosis International : a Journal Established as Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA, 28(5), 1685-1692. https://doi.org/10.1007/s00198-017-3949-8
Colares Neto GP, et al. Evaluation of Bone Mineral Density and Microarchitectural Parameters By DXA and HR-pQCT in 37 Children and Adults With X-linked Hypophosphatemic Rickets. Osteoporos Int. 2017;28(5):1685-1692. PubMed PMID: 28194480.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Evaluation of bone mineral density and microarchitectural parameters by DXA and HR-pQCT in 37 children and adults with X-linked hypophosphatemic rickets. AU - Colares Neto,G P, AU - Pereira,R M R, AU - Alvarenga,J C, AU - Takayama,L, AU - Funari,M F A, AU - Martin,R M, Y1 - 2017/02/13/ PY - 2016/05/16/received PY - 2017/01/26/accepted PY - 2017/2/15/pubmed PY - 2017/10/17/medline PY - 2017/2/15/entrez KW - Bone microarchitecture KW - Bone mineral density KW - DXA KW - HR-pQCT KW - X-linked hypophosphatemic rickets SP - 1685 EP - 1692 JF - Osteoporosis international : a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA JO - Osteoporos Int VL - 28 IS - 5 N2 - : In X-linked hypophosphatemic (XLH) rickets, dual-energy X-ray absorptiometry (DXA) measurements must be analyzed with caution. High-resolution peripheral quantitative computed tomography (HR-pQCT) analysis suggested that XLH primarily affects the cancellous compartment, with the tibia more affected than the radius. Effective treatment of XLH appears to positively affect bone mineralization, mainly in the bone cortex. INTRODUCTION: The purpose of this study is to evaluate bone mineral density (BMD) and microarchitecture in 37 patients (13 children and 24 adults) with XLH confirmed by PHEX mutations from a tertiary center compared to healthy controls. METHODS: Areal BMD (aBMD) was evaluated by DXA, whereas volumetric BMD (vBMD) and microarchitectural parameters were analyzed by HR-pQCT. RESULTS: Adult XLH patients had higher lumbar aBMD (p < 0.01) than the controls. At the radius, the vBMD was similar between XLH patients and controls. At the tibia, XLH patients had lower total vBMD (p = 0.04), likely resulting from decreased trabecular vBMD (p < 0.01), and this difference was observed in the children and adult groups. Analysis based on metabolic status showed that the adult XLH patients with non-compensated disease had lower cortical vBMD at the tibia than the compensated XLH patients (p = 0.03). The microarchitectural differences at the radius and tibia included lower trabecular number (p < 0.01), greater trabecular separation (p < 0.01), and higher trabecular network inhomogeneity (p < 0.01) in XLH patients compared to their controls. At the radius, adults exhibited greater trabecular deficits than were seen in children. CONCLUSIONS: In XLH patients, DXA measurements must be analyzed with caution due to the interference of anatomic and anthropometric factors. HR-pQCT analysis suggested that XLH primarily affects the cancellous compartment, with the tibia more affected than the radius. Effective treatment of XLH appears to positively affect bone mineralization, mainly in the bone cortex. SN - 1433-2965 UR - https://www.unboundmedicine.com/medline/citation/28194480/Evaluation_of_bone_mineral_density_and_microarchitectural_parameters_by_DXA_and_HR_pQCT_in_37_children_and_adults_with_X_linked_hypophosphatemic_rickets_ L2 - https://doi.org/10.1007/s00198-017-3949-8 DB - PRIME DP - Unbound Medicine ER -