Evaluation of bone mineral density and microarchitectural parameters by DXA and HR-pQCT in 37 children and adults with X-linked hypophosphatemic rickets

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dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author COLARES NETO, G. P. FMUSP-HC
PEREIRA, R. M. R. FMUSP-HC
ALVARENGA, J. C. FMUSP-HC
TAKAYAMA, L. FMUSP-HC
FUNARI, M. F. A. FMUSP-HC
MARTIN, R. M. FMUSP-HC
dc.date.issued 2017
dc.identifier.citation OSTEOPOROSIS INTERNATIONAL, v.28, n.5, p.1685-1692, 2017
dc.identifier.issn 0937-941X
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/19912
dc.description.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. 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. Areal BMD (aBMD) was evaluated by DXA, whereas volumetric BMD (vBMD) and microarchitectural parameters were analyzed by HR-pQCT. 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. 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.
dc.language.iso eng
dc.publisher SPRINGER LONDON LTD
dc.relation.ispartof Osteoporosis International
dc.rights restrictedAccess
dc.subject Bone microarchitecture; Bone mineral density; DXA; HR-pQCT; X-linked hypophosphatemic rickets
dc.subject.other computed-tomography; absorptiometry; strength
dc.title Evaluation of bone mineral density and microarchitectural parameters by DXA and HR-pQCT in 37 children and adults with X-linked hypophosphatemic rickets
dc.type article
dc.rights.holder Copyright SPRINGER LONDON LTD
dc.description.group LIM/42
dc.description.group LIM/17
dc.identifier.doi 10.1007/s00198-017-3949-8
dc.identifier.pmid 28194480
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author COLARES NETO, G. P.:FM:
hcfmusp.author PEREIRA, R. M. R.:FM:MCM
hcfmusp.author ALVARENGA, J. C.:FM:
hcfmusp.author TAKAYAMA, L.:FM:MCM
hcfmusp.author FUNARI, M. F. A.:HC:ICHC
hcfmusp.author MARTIN, R. M.:HC:ICHC
hcfmusp.origem.id 2-s2.0-85012254043
hcfmusp.origem.id WOS:000399443600019
hcfmusp.publisher.city LONDON
hcfmusp.publisher.country ENGLAND
hcfmusp.relation.reference · Baroncelli GI, 2001, J PEDIATR-US, V138, P236, DOI 10.1067/mpd.2001.108955
· Beck-Nielsen SS, 2013, OSTEOPOROSIS INT, V24, P2215, DOI 10.1007/s00198-013-2286-9
· Beck-Nielsen SS, 2010, CALCIFIED TISSUE INT, V87, P108, DOI 10.1007/s00223-010-9373-0
· Bouxsein Mary L, 2006, Curr Osteoporos Rep, V4, P49, DOI 10.1007/s11914-006-0002-9
· Carpenter TO, 2011, J BONE MINER RES, V26, P1381, DOI 10.1002/jbmr.340
· Cheung AM, 2013, CURR OSTEOPOROS REP, V11, P136, DOI 10.1007/s11914-013-0140-9
· Cheung M, 2013, J CLIN ENDOCR METAB, V98, pE954, DOI 10.1210/jc.2012-4133
· Cohen A, 2010, OSTEOPOROSIS INT, V21, P263, DOI 10.1007/s00198-009-0945-7
· *HYP CONS, 1995, NAT GENET, V11, P130
· Leaf DE, 2013, J CLIN ENDOCR METAB, V98, P887, DOI 10.1210/jc.2012-3473
· Li H, 2011, AM J PHYSIOL-ENDOC M, V300, pE508, DOI 10.1152/ajpendo.00499.2010
· OLIVERI MB, 1991, BONE MINER, V12, P91, DOI 10.1016/0169-6009(91)90038-2
· Rauch F., 2006, Journal of Musculoskeletal & Neuronal Interactions, V6, P142
· REID IR, 1991, AM J MED, V90, P63, DOI 10.1016/0002-9343(91)90507-T
· Shanbhogue VV, 2015, J BONE MINER RES, V30, P176, DOI 10.1002/jbmr.2310
· Shore RM, 2000, SKELETAL RADIOL, V29, P90
· Veilleux LN, 2013, J CLIN ENDOCR METAB, V98, pE990, DOI 10.1210/jc.2012-4146
· Yu XJ, 2005, CYTOKINE GROWTH F R, V16, P221, DOI 10.1016/j.cytogfr.2005.01.002
dc.description.index MEDLINE
dc.identifier.eissn 1433-2965
hcfmusp.citation.scopus 3
hcfmusp.citation.wos 4


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