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Diminished cortical bone density of long bones among children with haemophilic arthropathy

Introduction Children with haemophilia (CwH) have lower bone mineral density in the spine (trabecular bone) than healthy children. There are few studies focusing on bone mineral density in long bones (cortical bone). Aim To evaluate bi‐laterally the distal third of radius and midshaft tibias using q...

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Published in:Haemophilia : the official journal of the World Federation of Hemophilia 2023-01, Vol.29 (1), p.274-281
Main Authors: Tlacuilo‐Parra, Alberto, Soto‐Padilla, Janet, Toro‐Castro, Luis, Gutierrez‐Hermosillo, Violeta, Guevara‐Gutierrez, Elizabeth
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container_title Haemophilia : the official journal of the World Federation of Hemophilia
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creator Tlacuilo‐Parra, Alberto
Soto‐Padilla, Janet
Toro‐Castro, Luis
Gutierrez‐Hermosillo, Violeta
Guevara‐Gutierrez, Elizabeth
description Introduction Children with haemophilia (CwH) have lower bone mineral density in the spine (trabecular bone) than healthy children. There are few studies focusing on bone mineral density in long bones (cortical bone). Aim To evaluate bi‐laterally the distal third of radius and midshaft tibias using quantitative ultrasound (QUS) and assess the speed of sound (SoS). Methods A cross‐sectional study where 91 CwH and 91 age‐matched healthy boys were included. Joint evaluation was determined with the Haemophilia Joint Health Score 2.1 and SoS values. The Z scores were measured with the Sunlight Omnisense 8000 S equipment. Results Ninety‐one CwH (haemophilia A) were evaluated (26 mild form, 26 moderate, and 39 severe). Most patients were treated with on‐demand factor replacement and had higher total HJHS scores according to severity (4.8, 14.8 and 14.1, respectively). Patients with moderate and severe disease showed a statistical difference in SoS values for both radius and tibias compared to controls. QUS Z‐scores ≥‐2 were more frequent in radius and tibias in CwH, but were statistically significant only in tibias when compared to controls (in 30% mild, 46% moderate, 28% severe, respectively). Conclusions There was diminished cortical bone density in radius and tibias of CwH compared to healthy controls. Changes predominated in tibias, more frequently affected according to the severity of haemophilia. Early intervention with factor replacement combined with physical activity are key aspects to promote bone health.
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There are few studies focusing on bone mineral density in long bones (cortical bone). Aim To evaluate bi‐laterally the distal third of radius and midshaft tibias using quantitative ultrasound (QUS) and assess the speed of sound (SoS). Methods A cross‐sectional study where 91 CwH and 91 age‐matched healthy boys were included. Joint evaluation was determined with the Haemophilia Joint Health Score 2.1 and SoS values. The Z scores were measured with the Sunlight Omnisense 8000 S equipment. Results Ninety‐one CwH (haemophilia A) were evaluated (26 mild form, 26 moderate, and 39 severe). Most patients were treated with on‐demand factor replacement and had higher total HJHS scores according to severity (4.8, 14.8 and 14.1, respectively). Patients with moderate and severe disease showed a statistical difference in SoS values for both radius and tibias compared to controls. QUS Z‐scores ≥‐2 were more frequent in radius and tibias in CwH, but were statistically significant only in tibias when compared to controls (in 30% mild, 46% moderate, 28% severe, respectively). Conclusions There was diminished cortical bone density in radius and tibias of CwH compared to healthy controls. Changes predominated in tibias, more frequently affected according to the severity of haemophilia. Early intervention with factor replacement combined with physical activity are key aspects to promote bone health.</description><identifier>ISSN: 1351-8216</identifier><identifier>EISSN: 1365-2516</identifier><identifier>DOI: 10.1111/hae.14682</identifier><identifier>PMID: 36253885</identifier><language>eng</language><publisher>England: Wiley Subscription Services, Inc</publisher><subject>Arthritis ; Bone and Bones ; Bone Density ; Bone health ; Bone mineral density ; Bones ; Cancellous bone ; Child ; Children ; Cortical Bone ; Cortical bone density ; Cross-Sectional Studies ; Haemophilia joint health score ; Hemophilia ; Hemophilia A - complications ; Humans ; Long bone ; Male ; Physical activity ; Quantitative ultrasound ; Radius ; Radius - diagnostic imaging ; Statistical analysis ; Ultrasonography ; Ultrasound</subject><ispartof>Haemophilia : the official journal of the World Federation of Hemophilia, 2023-01, Vol.29 (1), p.274-281</ispartof><rights>2022 John Wiley &amp; Sons Ltd.</rights><rights>2023 John Wiley &amp; Sons Ltd.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c2432-dc3dd9e4da3a9eb8de98c50ddccd099dc07a8ae416985e1b41ff4cfebada99b83</cites><orcidid>0000-0003-0747-7189</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,780,784,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/36253885$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Tlacuilo‐Parra, Alberto</creatorcontrib><creatorcontrib>Soto‐Padilla, Janet</creatorcontrib><creatorcontrib>Toro‐Castro, Luis</creatorcontrib><creatorcontrib>Gutierrez‐Hermosillo, Violeta</creatorcontrib><creatorcontrib>Guevara‐Gutierrez, Elizabeth</creatorcontrib><title>Diminished cortical bone density of long bones among children with haemophilic arthropathy</title><title>Haemophilia : the official journal of the World Federation of Hemophilia</title><addtitle>Haemophilia</addtitle><description>Introduction Children with haemophilia (CwH) have lower bone mineral density in the spine (trabecular bone) than healthy children. There are few studies focusing on bone mineral density in long bones (cortical bone). Aim To evaluate bi‐laterally the distal third of radius and midshaft tibias using quantitative ultrasound (QUS) and assess the speed of sound (SoS). Methods A cross‐sectional study where 91 CwH and 91 age‐matched healthy boys were included. Joint evaluation was determined with the Haemophilia Joint Health Score 2.1 and SoS values. The Z scores were measured with the Sunlight Omnisense 8000 S equipment. Results Ninety‐one CwH (haemophilia A) were evaluated (26 mild form, 26 moderate, and 39 severe). Most patients were treated with on‐demand factor replacement and had higher total HJHS scores according to severity (4.8, 14.8 and 14.1, respectively). Patients with moderate and severe disease showed a statistical difference in SoS values for both radius and tibias compared to controls. QUS Z‐scores ≥‐2 were more frequent in radius and tibias in CwH, but were statistically significant only in tibias when compared to controls (in 30% mild, 46% moderate, 28% severe, respectively). Conclusions There was diminished cortical bone density in radius and tibias of CwH compared to healthy controls. Changes predominated in tibias, more frequently affected according to the severity of haemophilia. 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There are few studies focusing on bone mineral density in long bones (cortical bone). Aim To evaluate bi‐laterally the distal third of radius and midshaft tibias using quantitative ultrasound (QUS) and assess the speed of sound (SoS). Methods A cross‐sectional study where 91 CwH and 91 age‐matched healthy boys were included. Joint evaluation was determined with the Haemophilia Joint Health Score 2.1 and SoS values. The Z scores were measured with the Sunlight Omnisense 8000 S equipment. Results Ninety‐one CwH (haemophilia A) were evaluated (26 mild form, 26 moderate, and 39 severe). Most patients were treated with on‐demand factor replacement and had higher total HJHS scores according to severity (4.8, 14.8 and 14.1, respectively). Patients with moderate and severe disease showed a statistical difference in SoS values for both radius and tibias compared to controls. 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subjects Arthritis
Bone and Bones
Bone Density
Bone health
Bone mineral density
Bones
Cancellous bone
Child
Children
Cortical Bone
Cortical bone density
Cross-Sectional Studies
Haemophilia joint health score
Hemophilia
Hemophilia A - complications
Humans
Long bone
Male
Physical activity
Quantitative ultrasound
Radius
Radius - diagnostic imaging
Statistical analysis
Ultrasonography
Ultrasound
title Diminished cortical bone density of long bones among children with haemophilic arthropathy
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