J Ma1,2, H J McMillan3, G Karagüzel4, C Goodin1, J Wasson1, M A Matzinger5, P DesClouds1, D Cram1, M Page1, V N Konji1, B Lentle6, L M Ward7,8. 1. Pediatric Bone Health Clinical Research Program, Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada. 2. School of Epidemiology, Public Health and Preventive Medicine, University of Ottawa, Ottawa, ON, Canada. 3. Department of Pediatrics, and Division of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada. 4. Department of Pediatric Endocrinology, Karadeniz Technical University School of Medicine, Trabzon, Turkey. 5. Department of Medical Imaging, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada. 6. Department of Radiology, University of British Columbia, Vancouver, BC, Canada. 7. Pediatric Bone Health Clinical Research Program, Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada. lward@cheo.on.ca. 8. Department of Pediatrics, and Division of Endocrinology and Metabolism, Children's Hospital of Eastern Ontario, University of Ottawa, 401 Smyth Rd, Ottawa, ON, K1H 8L1, Canada. lward@cheo.on.ca.
Abstract
Boys with vertebral fractures (VF) identified through routine spine radiographs had milder, less symptomatic, and fewer VF compared to those diagnosed with VF following consultation for back pain. Spontaneous (i.e., medication-unassisted) reshaping of fractured vertebral bodies was absent. Long bone fractures were present even before Duchenne muscular dystrophy (DMD) diagnosis in some boys. INTRODUCTION: The objective of the study was to determine the time to and characteristics of first fractures in Duchenne muscular dystrophy. METHODS: This study was a retrospective longitudinal study of 30 boys with DMD <18 years. Boys were classified into four groups according to their first fracture: those with VF identified on routine lateral spine radiographs, those with VF diagnosed following consultation for back pain, those with long bone fractures, and those without fractures. RESULTS: Compared to boys diagnosed with VF as their initial fracture following consultation for back pain, those with VF surveillance radiographs had shorter durations of glucocorticoid (GC) therapy at the time of VF diagnosis (median 1.6 versus 5.3 years, p < 0.01), higher areal (mean ± standard deviation -1.4 ± 0.7 versus -3.1 ± 0.8, p = 0.01), and volumetric (-0.3 ± 0.5 versus -2.6 ± 0.8, p < 0.01) lumbar spine bone mineral density Z-scores, as well as fewer VF (median 1.4 versus 5.2 per person, p < 0.01) and a lower median spinal deformity index (median 1.5 versus 9.5, p < 0.01). Vertebral body reshaping following VF was not observed. Ten boys sustained a long bone fracture as their first fracture at a mean age of 8.9 ± 4.0 years; four of these boys later sustained a total of 27 incident VF. CONCLUSIONS: Routine lateral spine radiographs led to detection of VF in their earlier stages, vertebral body reshaping following VF was absent, and VF were frequent after the first long bone fracture. These results support the inclusion of a lateral spine radiograph starting at the time of GC initiation as part of routine bone health monitoring in DMD.
Boys with vertebral fractures (VF) identified through routine spine radiographs had milder, less symptomatic, and fewer VF compared to those diagnosed with VF following consultation for back pain. Spontaneous (i.e., medication-unassisted) reshaping of fractured vertebral bodies was absent. Long bone fractures were present even before Duchenne muscular dystrophy (DMD) diagnosis in some boys. INTRODUCTION: The objective of the study was to determine the time to and characteristics of first fractures in Duchenne muscular dystrophy. METHODS: This study was a retrospective longitudinal study of 30 boys with DMD <18 years. Boys were classified into four groups according to their first fracture: those with VF identified on routine lateral spine radiographs, those with VF diagnosed following consultation for back pain, those with long bone fractures, and those without fractures. RESULTS: Compared to boys diagnosed with VF as their initial fracture following consultation for back pain, those with VF surveillance radiographs had shorter durations of glucocorticoid (GC) therapy at the time of VF diagnosis (median 1.6 versus 5.3 years, p < 0.01), higher areal (mean ± standard deviation -1.4 ± 0.7 versus -3.1 ± 0.8, p = 0.01), and volumetric (-0.3 ± 0.5 versus -2.6 ± 0.8, p < 0.01) lumbar spine bone mineral density Z-scores, as well as fewer VF (median 1.4 versus 5.2 per person, p < 0.01) and a lower median spinal deformity index (median 1.5 versus 9.5, p < 0.01). Vertebral body reshaping following VF was not observed. Ten boys sustained a long bone fracture as their first fracture at a mean age of 8.9 ± 4.0 years; four of these boys later sustained a total of 27 incident VF. CONCLUSIONS: Routine lateral spine radiographs led to detection of VF in their earlier stages, vertebral body reshaping following VF was absent, and VF were frequent after the first long bone fracture. These results support the inclusion of a lateral spine radiograph starting at the time of GC initiation as part of routine bone health monitoring in DMD.
Entities:
Keywords:
Duchenne muscular dystrophy; First fractures; Glucocorticoids; Osteoporosis
Authors: J Feber; I Gaboury; A Ni; N Alos; S Arora; L Bell; T Blydt-Hansen; C Clarson; G Filler; J Hay; D Hebert; B Lentle; M Matzinger; J Midgley; D Moher; M Pinsk; F Rauch; C Rodd; N Shenouda; K Siminoski; L M Ward Journal: Osteoporos Int Date: 2011-04-15 Impact factor: 4.507
Authors: Celia Rodd; Bianca Lang; Timothy Ramsay; Nathalie Alos; Adam M Huber; David A Cabral; Rosie Scuccimarri; Paivi M Miettunen; Johannes Roth; Stephanie A Atkinson; Robert Couch; Elizabeth A Cummings; Peter B Dent; Janet Ellsworth; John Hay; Kristin Houghton; Roman Jurencak; Maggie Larché; Claire LeBlanc; Kiem Oen; Claire Saint-Cyr; Robert Stein; David Stephure; Shayne Taback; Brian Lentle; Maryann Matzinger; Nazih Shenouda; David Moher; Frank Rauch; Kerry Siminoski; Leanne M Ward Journal: Arthritis Care Res (Hoboken) Date: 2012-01 Impact factor: 4.794
Authors: Jinhui Ma; Kerry Siminoski; Nathalie Alos; Jacqueline Halton; Josephine Ho; Brian Lentle; MaryAnn Matzinger; Nazih Shenouda; Stephanie Atkinson; Ronald Barr; David A Cabral; Robert Couch; Elizabeth A Cummings; Conrad V Fernandez; Ronald M Grant; Celia Rodd; Anne Marie Sbrocchi; Maya Scharke; Frank Rauch; Leanne M Ward Journal: J Clin Endocrinol Metab Date: 2014-12-11 Impact factor: 5.958
Authors: Katharine Bushby; Richard Finkel; David J Birnkrant; Laura E Case; Paula R Clemens; Linda Cripe; Ajay Kaul; Kathi Kinnett; Craig McDonald; Shree Pandya; James Poysky; Frederic Shapiro; Jean Tomezsko; Carolyn Constantin Journal: Lancet Neurol Date: 2009-11-27 Impact factor: 44.182
Authors: W M King; R Ruttencutter; H N Nagaraja; V Matkovic; J Landoll; C Hoyle; J R Mendell; J T Kissel Journal: Neurology Date: 2007-05-08 Impact factor: 9.910
Authors: Laurie S Conklin; Jesse M Damsker; Eric P Hoffman; William J Jusko; Panteleimon D Mavroudis; Benjamin D Schwartz; Laurel J Mengle-Gaw; Edward C Smith; Jean K Mah; Michela Guglieri; Yoram Nevo; Nancy Kuntz; Craig M McDonald; Mar Tulinius; Monique M Ryan; Richard Webster; Diana Castro; Richard S Finkel; Andrea L Smith; Lauren P Morgenroth; Adrienne Arrieta; Maya Shimony; Mark Jaros; Phil Shale; John M McCall; Yetrib Hathout; Kanneboyina Nagaraju; John van den Anker; Leanne M Ward; Alexandra Ahmet; Michaelyn R Cornish; Paula R Clemens Journal: Pharmacol Res Date: 2018-09-13 Impact factor: 7.658
Authors: David R Weber; Alison Boyce; Catherine Gordon; Wolfgang Högler; Heidi H Kecskemethy; Madhusmita Misra; Diana Swolin-Eide; Peter Tebben; Leanne M Ward; Halley Wasserman; Christopher Shuhart; Babette S Zemel Journal: J Clin Densitom Date: 2019-07-10 Impact factor: 2.617
Authors: David J Birnkrant; Katharine Bushby; Carla M Bann; Benjamin A Alman; Susan D Apkon; Angela Blackwell; Laura E Case; Linda Cripe; Stasia Hadjiyannakis; Aaron K Olson; Daniel W Sheehan; Julie Bolen; David R Weber; Leanne M Ward Journal: Lancet Neurol Date: 2018-02-03 Impact factor: 44.182
Authors: David R Weber; Shiny Thomas; Stephen W Erickson; Deborah Fox; Joyce Oleszek; Shree Pandya; Yedatore Venkatesh; Christina Westfield; Emma Ciafaloni Journal: J Neuromuscul Dis Date: 2018