52 results on '"Matzinger MA"'
Search Results
2. Vertebral Body Reshaping after Fractures: An Important Index of Recovery in Glucocorticoid-Treated Children.
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Ma J, Siminoski K, Jaremko JL, Koujok K, Matzinger MA, Shenouda N, Wilson N, Cheng M, Alos N, Atkinson S, Cummings EA, Ho J, Rodd C, Sbrocchi AM, Stein R, Barr R, Cairney E, Dix DB, Fernandez CV, Grant R, Halton J, Israels S, Laverdière C, Lewis VA, Cabral DA, Huber A, Houghton K, Jurencak R, Lang B, Larché M, LeBlanc CMA, Miettunen P, Roth J, Scuccimarri R, Bell L, Blydt-Hansen T, Filler G, Feber J, Phan V, Smit K, Rauch F, and Ward LM
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- Child, Humans, Glucocorticoids adverse effects, Vertebral Body, Bone Density, Fractures, Bone chemically induced, Spinal Fractures etiology, Spinal Fractures chemically induced, Osteoporotic Fractures chemically induced
- Abstract
Purpose: In this 6-year study we identified factors associated with spontaneous vertebral body reshaping in glucocorticoid (GC)-treated children with leukemia, rheumatic disorders, and nephrotic syndrome., Methods: Subjects were 79 children (mean age 7.4 years) who had vertebral fracture (VF) evaluation on lateral spine radiographs at least 1 year after VF detection. VF were graded using the modified Genant semiquantitative method and fracture burden for individuals was quantified using the spinal deformity index (SDI; sum of grades from T4 to L4)., Results: Sixty-five children (82.3%) underwent complete vertebral body reshaping (median time from VF detection to complete reshaping 1.3 years by Cox proportional hazard modeling). Of 237 VF, the majority (83.1%) ultimately reshaped, with 87.2% reshaping in the thoracic region vs 70.7% in the lumbar region (P = .004). Cox models showed that (1) every g/m2 increase in GC exposure in the first year after VF detection was associated with a 19% decline in the probability of reshaping; (2) each unit increase in the SDI at the time of VF detection was associated with a 19% decline in the probability of reshaping [hazard ratio (HR) = 0.81; 95% confidence interval (CI) = 0.71, 0.92; P = .001]; (3) each additional VF present at the time of VF detection reduced reshaping by 25% (HR = 0.75; 95% CI = 0.62, 0.90; P = .002); and (4) each higher grade of VF severity decreased reshaping by 65% (HR = 0.35; 95% CI = 0.21, 0.57; P < .001)., Conclusion: After experiencing a VF, children with higher GC exposure, higher SDI, more severe fractures, or lumbar VF were at increased risk for persistent vertebral deformity., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
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- 2024
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3. Risk Factors Associated with Incident Vertebral Fractures in Steroid-treated Males with Duchenne Muscular Dystrophy.
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Phung K, McAdam L, Ma J, McMillan HJ, Jackowski S, Scharke M, Matzinger MA, Shenouda N, Koujok K, Jaremko JL, Wilson N, Walker S, Hartigan C, Khan N, Page M, Robinson ME, Saleh DS, Smit K, Rauch F, Siminoski K, and Ward LM
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- Male, Humans, Bone Density, Risk Factors, Glucocorticoids adverse effects, Lumbar Vertebrae diagnostic imaging, Steroids, Muscular Dystrophy, Duchenne complications, Muscular Dystrophy, Duchenne drug therapy, Muscular Dystrophy, Duchenne epidemiology, Spinal Fractures diagnostic imaging, Spinal Fractures epidemiology, Spinal Fractures etiology, Fractures, Bone etiology, Fractures, Bone chemically induced, Osteoporotic Fractures etiology
- Abstract
Purpose: Prevention of fractures is an unmet need in glucocorticoid (GC)-treated Duchenne muscular dystrophy. This study explored factors associated with incident vertebral fractures (VFs) to inform future fracture prevention efforts., Methods: VFs were evaluated prospectively at study baseline and 12 months on lateral spine radiographs in participants aged 4 to 25 years with Duchenne muscular dystrophy. Clinical factors were analyzed for their association with the change in Spinal Deformity Index (sum of the Genant-defined VF grades from T4 to L4) between baseline and 12 months., Results: Thirty-eight males were evaluated (mean ± SD age at baseline 11.0 ± 3.6 years; mean ± SD GC duration at baseline 4.1 ± 3.1 years; 74% ambulatory). Nine of 38 participants (24%) had 17 incident VFs, of which 3/17 VFs (18%) were moderate/severe. Participants with 12-month incident VF had lower mean ± SD baseline lumbar spine areal bone mineral density Z-scores (-2.9 ± 1.0 vs -1.9 ± 1.1; P = .049) and lower total body less head areal bone mineral density Z-scores (-3.1 ± 1.2 vs -1.6 ± 1.7; P = .036). Multivariable linear regression showed that at least 1 VF at baseline (P < .001), a higher number of antecedent non-VF (P < .001), and greater bone age delay at baseline (P = .027) were significant predictors of an increase in the Spinal Deformity Index from baseline to 12 months., Conclusion: The observation that ≥ 1 prevalent VF and/or non-VF were the strongest predictors of incident VFs at 12 months supports the need for prevention of first fractures in this high-risk setting. Bone age delay, a marker of GC exposure, may assist in the prioritization of patients in efforts to prevent first fractures., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
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- 2024
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4. Reductions in Bone Mineral Density Are Apparent Early in Children With Prevalent Osteonecrosis Lesions Following Leukemia Therapy.
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Halton JM, Ma J, Babyn P, Matzinger MA, Kaste SC, Scharke M, Fernandez CV, Miettunen P, Ho J, Alos N, Abish S, Barr R, Cairney E, Dix DB, Grant RM, Israels S, Lewis V, Wilson B, Atkinson S, Cabral D, Cummings E, Rodd C, Stein R, Sbrocchi AM, Jaremko JL, Koujok K, Shenouda N, Rauch F, Siminoski K, and Ward LM
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- Humans, Child, Bone Density, Lumbar Vertebrae, Absorptiometry, Photon methods, Osteoporosis, Leukemia, Osteonecrosis chemically induced, Osteonecrosis diagnostic imaging
- Abstract
Osteonecrosis (ON) is a serious complication of childhood acute lymphoblastic leukemia. We determined the prevalence of osteonecrotic lesions in our patient population by a one-time multisite magnetic resonance imaging (MRI) more than 1 year following leukemia therapy. MRI findings were evaluated in relationship to clinical factors (including longitudinal changes in bone mineral density [BMD]). Eighty-six children enrolled in the Steroid Associated Osteoporosis in the Pediatric Population (STOPP) study were evaluated for ON at 3.1 ± 1.3 years following therapy. Thirty children had a total of 150 confirmed ON lesions (35%). Lumbar spine (LS) BMD Z-scores (mean ± SD) were low at diagnosis and similar between patients with and without ON (-1.09 ± 1.53 versus -1.27 ± 1.25, p = 0.549). LS BMD Z-scores declined from baseline to 12 months in children with ON (-0.31 ± 1.02) but not in those without (0.13 ± 0.82, p = 0.035); the hip BMD Z-scores from baseline to 24 months declined in both groups, but to a greater extent in those with ON (-1.77 ± 1.22) compared to those without (-1.03 ± 1.07, p = 0.045). At the time of the MRI, mean total hip and total body (TB) BMD Z-scores were lower in children with ON (hip -0.98 ± 0.95 versus -0.28 ± 1.06, p = 0.010; TB -1.36 ± 1.10 versus -0.48 ± 1.50, p = 0.018). Pain occurred in 11/30 (37%) with ON versus 20/56 (36%) without, p = 0.841. In multivariable models, older age at diagnosis (odds ratio [OR] 1.57; 95% confidence interval [CI], 1.15-2.13; p = 0.004), and hip BMD Z-score at MRI (OR 2.23; 95% CI, 1.02-4.87; p = 0.046) were independently associated with ON. Overall, one-third of children demonstrated ON after leukemia therapy. Those with ON had greater reductions in spine and hip BMD Z-scores in the first 1 and 2 years of therapy, respectively. Older age and lower hip BMD Z-scores at MRI were significantly associated with prevalent, off-therapy ON. These data assist in identifying children at risk of ON. © 2023 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR)., (© 2023 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).)
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- 2023
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5. Risk factors associated with prevalent vertebral fractures in Duchenne muscular dystrophy.
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Phung K, McAdam L, Ma J, McMillan HJ, Jackowski S, Scharke M, Matzinger MA, Shenouda N, Koujok K, Jaremko JL, Smit K, Walker S, Hartigan C, Khan N, Konji VN, MacLeay L, Page M, Sykes E, Robinson ME, Alos N, Cummings EA, Ho J, Sbrocchi AM, Stein R, Saleh D, Craven BC, Dang UJ, Siminoski K, Rauch F, and Ward LM
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- Male, Adolescent, Humans, Child, Preschool, Child, Young Adult, Adult, Glucocorticoids adverse effects, Cross-Sectional Studies, Bone Density, Risk Factors, Lumbar Vertebrae, Muscular Dystrophy, Duchenne complications, Muscular Dystrophy, Duchenne drug therapy, Spinal Fractures etiology, Spinal Fractures complications, Fractures, Bone complications, Osteoporosis etiology, Osteoporosis chemically induced
- Abstract
Patients with Duchenne muscular dystrophy (DMD) have a high fracture burden due to progressive myopathy and steroid-induced osteoporosis. This study in males with DMD showed that markers of systemic glucocorticoid exposure including shorter stature, greater bone age delay, and lower lumbar spine bone mineral density were associated with spine fragility., Introduction: Fragility fractures are frequent in DMD. The purpose of this study was to identify clinical factors associated with prevalent vertebral fractures (VF) in boys, teens/young adults with Duchenne muscular dystrophy (DMD)., Methods: This was a cross-sectional study of males aged 4-25 years with DMD. VF were evaluated using the modified Genant semi-quantitative method on T4-L4 lateral spine radiographs. Areal bone mineral density (aBMD) was measured at the lumbar spine (LS) and used to estimate volumetric BMD (vBMD). Clinical factors were analyzed for their association with the Spinal Deformity Index (SDI, the sum of the Genant grades)., Results: Sixty participants were enrolled (mean age 11.5 years, range 5.4-19.5). Nineteen participants (32%) had a total of 67 VF; 23/67 VF (34%) were moderate or severe. Participants with VF were shorter (mean height Z-score ± standard deviation: - 3.1 ± 1.4 vs. - 1.8 ± 1.4, p = 0.001), had longer glucocorticoid exposure (mean duration 6.0 ± 3.3 vs. 3.9 ± 3.3 years, p = 0.027), greater bone age (BA) delay (mean BA to chronological age difference - 3.2 ± 3.4 vs. - 1.3 ± 1.2 years, p = 0.035), and lower LSaBMD Z-scores (mean - 3.0 ± 1.0 vs. - 2.2 ± 1.2, p = 0.023). There was no difference in LSvBMD Z-scores. Multivariable Poisson regression showed that every 0.1 mg/kg/day increment in average glucocorticoid daily dose was associated with a 1.4-fold SDI increase (95% confidence interval: 1.1-1.7, p = 0.013). Greater BA delay (p < 0.001), higher weight Z-score (p = 0.004), decreased height Z-score (p = 0.025), and lower LSvBMD Z-score (p = 0.025) were also associated with SDI increase., Conclusion: Readily measurable clinical variables were associated with prevalent VF in males with glucocorticoid-treated DMD. These variables may be useful to identify candidates for primary osteoporosis prevention after glucocorticoid initiation., (© 2022. International Osteoporosis Foundation and Bone Health and Osteoporosis Foundation.)
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- 2023
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6. A Validated Risk Prediction Model for Bone Fragility in Children With Acute Lymphoblastic Leukemia.
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Verwaaijen EJ, Ma J, de Groot-Kruseman HA, Pieters R, van der Sluis IM, van Atteveld JE, Halton J, Fernandez CV, Hartman A, de Jonge R, Lequin MH, Te Winkel ML, Alos N, Atkinson SA, Barr R, Grant RM, Hay J, Huber AM, Ho J, Jaremko J, Koujok K, Lang B, Matzinger MA, Shenouda N, Rauch F, Rodd C, van den Heuvel-Eibrink MM, Pluijm SMF, and Ward LM
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- Absorptiometry, Photon, Bone Density, Canada, Child, Humans, Lumbar Vertebrae diagnostic imaging, Osteoporosis, Precursor Cell Lymphoblastic Leukemia-Lymphoma complications, Precursor Cell Lymphoblastic Leukemia-Lymphoma drug therapy, Precursor Cell Lymphoblastic Leukemia-Lymphoma epidemiology
- Abstract
Although bone fragility may already be present at diagnosis of pediatric acute lymphoblastic leukemia (ALL), routine performance of dual-energy X-ray absorptiometry (DXA) in every child is not universally feasible. The aim of this study was to develop and validate a risk prediction model for low lumbar spine bone mineral density (LS BMD Z-score ≤ -2.0) at diagnosis, as an important indicator for fracture risk and further treatment-related BMD aggravation. Children with ALL (4-18 years), treated according to the Dutch Childhood Oncology Group protocol (DCOG-ALL9; model development; n = 249) and children from the Canadian Steroid-Associated Osteoporosis in the Pediatric Population cohort (STOPP; validation; n = 99) were included in this study. Multivariable logistic regression analyses were used to develop the prediction model and to confirm the association of low LS BMD at diagnosis with symptomatic fractures during and shortly after cessation of ALL treatment. The area under the receiver operating characteristic curve (AUC) was used to assess model performance. The prediction model for low LS BMD at diagnosis using weight (β = -0.70) and age (β = -0.10) at diagnosis revealed an AUC of 0.71 (95% CI, 0.63-0.78) in DCOG-ALL9 and 0.74 (95% CI, 0.63-0.84) in STOPP, and resulted in correct identification of 71% of the patients with low LS BMD. We confirmed that low LS BMD at diagnosis is associated with LS BMD at treatment cessation (OR 5.9; 95% CI, 3.2-10.9) and with symptomatic fractures (OR 1.7; 95% CI, 1.3-2.4) that occurred between diagnosis and 12 months following treatment cessation. In meta-analysis, LS BMD at diagnosis (OR 1.6; 95% CI, 1.1-2.4) and the 6-month cumulative glucocorticoid dose (OR 1.9; 95% CI, 1.1-3.2) were associated with fractures that occurred in the first year of treatment. In summary, a prediction model for identifying pediatric ALL patients with low LS BMD at diagnosis, as an important indicator for bone fragility, was successfully developed and validated. This can facilitate identification of future bone fragility in individual pediatric ALL patients. © 2021 American Society for Bone and Mineral Research (ASBMR)., (© 2021 American Society for Bone and Mineral Research (ASBMR).)
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- 2021
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7. Osteoporotic Fractures and Vertebral Body Reshaping in Children With Glucocorticoid-treated Rheumatic Disorders.
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Ward LM, Ma J, Robinson ME, Scharke M, Ho J, Houghton K, Huber A, Scuccimarri R, Barsalou J, Roth J, Shenouda N, Matzinger MA, Lentle B, Jaremko JL, Koujok K, Watanabe Duffy K, Stein R, Sbrocchi AM, Rodd C, Miettunen PM, LeBlanc CMA, Larche M, Jurencak R, Cummings EA, Couch R, Cabral DA, Atkinson S, Alos N, Sykes E, Konji VN, Rauch F, Siminoski K, and Lang B
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- Adolescent, Canada epidemiology, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Incidence, Infant, Infant, Newborn, Longitudinal Studies, Male, Osteoporosis chemically induced, Osteoporosis pathology, Osteoporotic Fractures chemically induced, Osteoporotic Fractures pathology, Prognosis, Prospective Studies, Rheumatic Diseases pathology, Risk Factors, Spinal Fractures chemically induced, Spinal Fractures pathology, Bone Density, Glucocorticoids adverse effects, Osteoporosis epidemiology, Osteoporotic Fractures epidemiology, Rheumatic Diseases drug therapy, Spinal Fractures epidemiology, Vertebral Body physiopathology
- Abstract
Context: Osteoporotic fractures are an important cause of morbidity in children with glucocorticoid-treated rheumatic disorders., Objective: This work aims to evaluate the incidence and predictors of osteoporotic fractures and potential for recovery over six years following glucocorticoid (GC) initiation in children with rheumatic disorders., Methods: Children with GC-treated rheumatic disorders were evaluated through a prospective inception cohort study led by the Canadian STeroid-induced Osteoporosis in the Pediatric Population (STOPP) Consortium. Clinical outcomes included lumbar spine bone mineral density (LS BMD), vertebral fractures (VF), non-VF, and vertebral body reshaping., Results: A total of 136 children with GC-treated rheumatic disorders were enrolled (mean age 9.9 years, SD 4.4). The 6-year cumulative fracture incidence was 16.3% for VF, and 10.1% for non-VF. GC exposure was highest in the first 6 months, and 24 of 38 VF (63%) occurred in the first 2 years. Following VF, 16 of 19 children (84%) had complete vertebral body reshaping. Increases in disease activity and body mass index z scores in the first year and declines in LS BMD z scores in the first 6 months predicted incident VF over the 6 years, while higher average daily GC doses predicted both incident VF and non-VF. LS BMD z scores were lowest at 6 months (mean -0.9, SD 1.2) and remained low by 6 years even when adjusted for height z scores (-0.6, SD 0.9)., Conclusion: VF occurred early and were more common than non-VF in children with GC-treated rheumatic disorders. Eighty-four percent of children with VF underwent complete vertebral body reshaping, whereas vertebral deformity persisted in the remainder of children. On average, LS BMD z scores remained low at 6 years, consistent with incomplete recovery., (© The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
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- 2021
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8. The Accuracy of Incident Vertebral Fracture Detection in Children Using Targeted Case-Finding Approaches.
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Ma J, Siminoski K, Wang P, Jaremko JL, Koujok K, Matzinger MA, Shenouda N, Lentle B, Alos N, Cummings EA, Ho J, Houghton K, Miettunen PM, Scuccimarri R, Rauch F, and Ward LM
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- Absorptiometry, Photon, Back Pain, Bone Density, Child, Humans, Lumbar Vertebrae diagnostic imaging, Spinal Fractures diagnostic imaging, Spinal Fractures epidemiology
- Abstract
Vertebral fractures are clinically important sequelae of a wide array of pediatric diseases. In this study, we examined the accuracy of case-finding strategies for detecting incident vertebral fractures (IVF) over 2 years in glucocorticoid-treated children (n = 343) with leukemia, rheumatic disorders, or nephrotic syndrome. Two clinical situations were addressed: the prevalent vertebral fracture (PVF) scenario (when baseline PVF status was known), which assessed the utility of PVF and low lumbar spine bone mineral density (LS BMD; Z-score <-1.4), and the non-PVF scenario (when PVF status was unknown), which evaluated low LS BMD and back pain. LS BMD was measured by dual-energy X-ray absorptiometry, vertebral fractures were quantified on spine radiographs using the modified Genant semiquantitative method, and back pain was assessed by patient report. Forty-four patients (12.8%) had IVF. In the PVF scenario, both low LS BMD and PVF were significant predictors of IVF. Using PVF to determine which patients should have radiographs, 11% would undergo radiography (95% confidence interval [CI] 8-15) with 46% of IVF (95% CI 30-61) detected. Sensitivity would be higher with a strategy of PVF or low LS BMD at baseline (73%; 95% CI 57-85) but would require radiographs in 37% of children (95% CI 32-42). In the non-PVF scenario, the strategy of low LS BMD and back pain produced the highest specificity of any non-PVF model at 87% (95% CI 83-91), the greatest overall accuracy at 82% (95% CI 78-86), and the lowest radiography rate at 17% (95% CI 14-22). Low LS BMD or back pain in the non-PVF scenario produced the highest sensitivity at 82% (95% CI 67-92), but required radiographs in 65% (95% CI 60-70). These results provide guidance for targeting spine radiography in children at risk for IVF. © 2021 American Society for Bone and Mineral Research (ASBMR)., (© 2021 American Society for Bone and Mineral Research (ASBMR).)
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- 2021
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9. The Accuracy of Prevalent Vertebral Fracture Detection in Children Using Targeted Case-Finding Approaches.
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Ma J, Siminoski K, Wang P, Alos N, Cummings EA, Feber J, Halton J, Ho J, Houghton K, Lang B, Miettunen PM, Scuccimarri R, Jaremko JL, Koujok K, Lentle B, Matzinger MA, Shenouda N, Rauch F, and Ward LM
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- Absorptiometry, Photon, Back Pain, Bone Density, Child, Humans, Lumbar Vertebrae diagnostic imaging, Spinal Fractures diagnostic imaging, Spinal Fractures epidemiology
- Abstract
Due to concerns about cumulative radiation exposure in the pediatric population, it is not standard practice to perform spine radiographs in most conditions that predispose to vertebral fracture (VF). In this study we examined the accuracy of two clinical predictors, back pain and lumbar spine bone mineral density (LS BMD), to derive four case-finding paradigms for detection of prevalent VF (PVF). Subjects were 400 children at risk for PVF (leukemia 186, rheumatic disorders 135, nephrotic syndrome 79). Back pain was assessed by patient report, LS BMD was measured by dual-energy X-ray absorptiometry, and PVF were quantified on spine radiographs using the modified Genant semiquantitative method. Forty-four patients (11.0%) had PVF. Logistic regression analysis between LS BMD and PVF produced an odds ratio (OR) of 1.9 (95% confidence interval [CI], 1.5 to 2.5) per reduction in Z-score unit, an area under the receiver operating characteristic curve of 0.70 (95% CI, 0.60 to 0.79), and an optimal BMD Z-score cutoff of -1.6. Case identification using either low BMD alone (Z-score < -1.6) or back pain alone gave similar results for sensitivity (55%, 52%, respectively), specificity (78%, 81%, respectively), positive predictive value (PPV; 24%, 25%, respectively), and negative predictive value (NPV; 93%, 93%, respectively). The paradigm using low BMD plus back pain produced lower sensitivity (32%), higher specificity (96%), higher PPV (47%), and similar NPV (92%). The approach using low BMD or back pain had the highest sensitivity (75%), lowest specificity (64%), lowest PPV (20%), and highest NPV (95%). All paradigms had increased sensitivities for higher fracture grades. Our results show that BMD and back pain history can be used to identify children with the highest risk of PVF so that radiography can be used judiciously. The specific paradigm to be applied will depend on the expected PVF rate and the clinical approach to the use of radiography. © 2019 American Society for Bone and Mineral Research., (© 2019 American Society for Bone and Mineral Research.)
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- 2020
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10. Local Tumor Recurrence and Escape from Suppression of Bone Resorption With Denosumab Treatment in Two Adolescents With Giant Cell Tumors of Bone.
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Akel U, Robinson ME, Werier J, Rampersaud R, Rakhra K, Johnston D, Konji VN, Ma J, Pagé M, Matzinger MA, and Ward LM
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Giant cell tumors of bone (GCTB) may be difficult to resect because of size or location. We describe two adolescents who were treated with denosumab and followed for tumoral and biochemical responses. Denosumab was effective in achieving sufficient regression to allow surgical resection and in preserving peritumor cortical bone. Reactivation of bone resorption markers was noted while the patients were receiving monthly denosumab. One patient suffered a local tumor recurrence. Denosumab was safe in enabling surgical resection of GCTB. However, the effect was transient, with an escape of resorption markers and tumor recurrence. © 2019 The Authors. JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research., (© 2019 The Authors. JBMR Plus published by Wiley Periodicals, Inc. on behalf of American Society for Bone and Mineral Research.)
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- 2019
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11. Impact of Vertebral Fractures and Glucocorticoid Exposure on Height Deficits in Children During Treatment of Leukemia.
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Ma J, Siminoski K, Alos N, Halton J, Ho J, Cummings EA, Shenouda N, Matzinger MA, Lentle B, Jaremko JL, Wilson B, Stephure D, Stein R, Sbrocchi AM, Rodd C, Lewis VA, Laverdière C, Israels S, Grant RM, Fernandez CV, Dix DB, Couch R, Cairney E, Barr R, Atkinson S, Abish S, Moher D, Rauch F, and Ward LM
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- Adolescent, Anthropometry methods, Body Height drug effects, Bone Density drug effects, Child, Child, Preschool, Drug Administration Schedule, Female, Follow-Up Studies, Glucocorticoids administration & dosage, Glucocorticoids therapeutic use, Growth Disorders physiopathology, Humans, Infant, Male, Prospective Studies, Risk Factors, Sex Factors, Glucocorticoids adverse effects, Growth Disorders etiology, Precursor Cell Lymphoblastic Leukemia-Lymphoma drug therapy, Spinal Fractures complications
- Abstract
Objective: To assess the effect of vertebral fractures (VF) and glucocorticoid (GC) exposure on height deficits in children during treatment of acute lymphoblastic leukemia (ALL)., Methods: Children with ALL treated without cranial radiation therapy (n = 160; median age, 5.1 years; 58.1% male) were followed prospectively for 6 years. Spinal deformity index (SDI) was used to quantify VF status., Results: Baseline height z score ± SD was 0.3 ± 1.2. It fell by 0.5 ± 0.4 in the first 6 months for boys and by 0.4 ± 0.4 in the first 12 months for girls (P < 0.01 for both) and then subsequently recovered. The prevalence of VF peaked at 1 year (17.6%). Among those with VF, median SDI rose from 2 [interquartile range (IQR): 1, 7] at baseline to 8 (IQR: 1, 8) at 1 year. A mixed model for repeated measures showed that height z score declined by 0.13 (95% CI: 0.02 to 0.24; P = 0.02) for each 5-unit increase in SDI during the previous 12 months. Every 10 mg/m2 increase in average daily GC dose (prednisone equivalent) in the previous 12 months was associated with a height z score decrement of 0.26 (95% CI: 0.20 to 0.32; P < 0.01)., Conclusions: GC likely plays a major role in the observed height decline during therapy for ALL. Because only a minority of children had VF, fractures could not have contributed significantly to the height deficit in the entire cohort but may have been important among the subset with VF.
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- 2019
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12. Bone Morbidity and Recovery in Children With Acute Lymphoblastic Leukemia: Results of a Six-Year Prospective Cohort Study.
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Ward LM, Ma J, Lang B, Ho J, Alos N, Matzinger MA, Shenouda N, Lentle B, Jaremko JL, Wilson B, Stephure D, Stein R, Sbrocchi AM, Rodd C, Lewis V, Israels S, Grant RM, Fernandez CV, Dix DB, Cummings EA, Couch R, Cairney E, Barr R, Abish S, Atkinson SA, Hay J, Rauch F, Moher D, Siminoski K, and Halton J
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- Child, Child, Preschool, Female, Fractures, Bone complications, Fractures, Bone epidemiology, Humans, Incidence, Male, Multivariate Analysis, Precursor Cell Lymphoblastic Leukemia-Lymphoma complications, Prevalence, Proportional Hazards Models, Prospective Studies, Spinal Fractures diagnostic imaging, Spinal Fractures epidemiology, Spine diagnostic imaging, Spine pathology, Bone and Bones pathology, Precursor Cell Lymphoblastic Leukemia-Lymphoma pathology
- Abstract
Osteoporotic fractures are a significant cause of morbidity in acute lymphoblastic leukemia (ALL). Our objective was to determine the incidence and predictors of fractures and recovery from osteoporosis in pediatric ALL over 6 years following glucocorticoid initiation. Vertebral fractures (VF) and vertebral body reshaping were assessed on annual spine radiographs, low-trauma non-VF were recorded at regular intervals and spine bone mineral density (BMD) was captured every 6 months for 4 years and then annually. A total of 186 children with ALL were enrolled (median age 5.3 years; range, 1.3 to 17.0 years). The cumulative fracture incidence was 32.5% for VF and 23.0% for non-VF; 39.0% of children with VF were asymptomatic. No fractures occurred in the sixth year and 71.3% of incident fractures occurred in the first 2 years. Baseline VF, cumulative glucocorticoid dose, and baseline lumbar spine (LS) BMD Z-score predicted both VF and non-VF. Vertebral body reshaping following VF was incomplete or absent in 22.7% of children. Those with residual vertebral deformity following VF were older compared to those without (median age 8.0 years at baseline [interquartile range {IQR}, 5.5 to 9.4] versus 4.8 years [IQR, 3.6 to 6.2], p = 0.04) and had more severe vertebral collapse (median maximum spinal deformity index 3.5 [IQR, 1.0 to 8.0] versus 0.5 [IQR, 0.0 to 1.0], p = 0.01). VF and low LS BMD Z-score at baseline as well as glucocorticoid exposure predicted incident VF and non-VF. Nearly 25% of children had persistent vertebral deformity following VF, more frequent in older children, and in those with more severe collapse. These results suggest the need for trials addressing interventions in the first 2 years of chemotherapy, targeting older children and children with more severe vertebral collapse, because these children are at greatest risk for incident VF and subsequent residual vertebral deformity. © 2018 American Society for Bone and Mineral Research., (© 2018 American Society for Bone and Mineral Research.)
- Published
- 2018
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13. Musculoskeletal health in newly diagnosed children with Crohn's disease.
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Ward LM, Ma J, Rauch F, Benchimol EI, Hay J, Leonard MB, Matzinger MA, Shenouda N, Lentle B, Cosgrove H, Scharke M, Konji VN, and Mack DR
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- Absorptiometry, Photon methods, Adolescent, Bone Density physiology, Child, Crohn Disease physiopathology, Cross-Sectional Studies, Female, Humans, Male, Muscle Strength physiology, Osteoporosis physiopathology, Osteoporotic Fractures diagnostic imaging, Osteoporotic Fractures etiology, Osteoporotic Fractures physiopathology, Radiography, Spinal Fractures diagnostic imaging, Spinal Fractures etiology, Spinal Fractures physiopathology, Tibia physiopathology, Tomography, X-Ray Computed methods, Crohn Disease complications, Osteoporosis etiology
- Abstract
We evaluated the impact of Crohn's disease on muscle and bone strength, mass, density, and geometry in children with newly diagnosed CD and found profound muscle and bone deficits; nevertheless, the prevalence of vertebral fractures at this time point was low., Introduction: Crohn's disease (CD) is an inflammatory condition of the gastrointestinal tract that can affect the musculoskeletal system. The objective of this study was to determine the prevalence of vertebral fractures and the impact of CD on muscle and bone mass, strength, density, and geometry in children with newly diagnosed CD., Methods: Seventy-three children (26 girls) aged 7.0 to 17.7 years were examined within 35 days following CD diagnosis by lateral spine radiograph for vertebral fractures and by jumping mechanography for muscle strength. Bone and muscle mass, density, and geometry were assessed by dual-energy x-ray absorptiometry and peripheral quantitative computed tomography (pQCT)., Results: Disease activity was moderate to severe in 66 (90%) patients. Mean height (Z-score -0.3, standard deviation (SD) 1.1, p = 0.02), weight (Z-score -0.8, SD 1.3, p < 0.01), body mass index (Z-score -1.0, SD 1.3, p < 0.01), lumbar spine areal bone mineral density (BMD; Z-score -1.1, SD 1.0, p < 0.01), total body bone mineral content (Z-score -1.5, SD 1.0, p < 0.01), and total body lean mass (Z-score -2.5, SD 1.1, p < 0.01) were all low for age and gender. pQCT showed reduced trabecular volumetric BMD at the tibial metaphysis, expansion of the bone marrow cavity and thin cortices at the diaphysis, and low calf muscle cross-sectional area. Jumping mechanography demonstrated low muscle power. Only one patient had a vertebral fracture., Conclusions: Children with newly diagnosed CD have profound muscle and bone deficits; nevertheless, the prevalence of vertebral fractures at this time point was low.
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- 2017
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14. The time to and determinants of first fractures in boys with Duchenne muscular dystrophy.
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Ma J, McMillan HJ, Karagüzel G, Goodin C, Wasson J, Matzinger MA, DesClouds P, Cram D, Page M, Konji VN, Lentle B, and Ward LM
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- Adolescent, Bone Density physiology, Child, Child, Preschool, Drug Administration Schedule, Glucocorticoids administration & dosage, Glucocorticoids adverse effects, Glucocorticoids therapeutic use, Humans, Longitudinal Studies, Lumbar Vertebrae diagnostic imaging, Lumbar Vertebrae physiopathology, Male, Muscular Dystrophy, Duchenne physiopathology, Osteoporotic Fractures diagnostic imaging, Osteoporotic Fractures physiopathology, Radiography, Retrospective Studies, Spinal Fractures diagnostic imaging, Spinal Fractures etiology, Spinal Fractures physiopathology, Thoracic Vertebrae diagnostic imaging, Thoracic Vertebrae physiopathology, Time Factors, Muscular Dystrophy, Duchenne complications, Osteoporotic Fractures etiology
- 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.
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- 2017
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15. The Spectrum of Recovery From Fracture-Induced Vertebral Deformity in Pediatric Leukemia.
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Dal Osto LC, Konji VN, Halton J, Matzinger MA, Bassal M, Rauch F, and Ward LM
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- Adolescent, Antineoplastic Combined Chemotherapy Protocols adverse effects, Child, Female, Humans, Male, Precursor Cell Lymphoblastic Leukemia-Lymphoma drug therapy, Radiography, Osteogenesis physiology, Precursor Cell Lymphoblastic Leukemia-Lymphoma complications, Spinal Fractures diagnostic imaging, Spinal Fractures etiology
- Abstract
Vertebral fractures (VF) are a frequent complication of acute lymphoblastic leukemia. Some children with VF undergo vertebral body reshaping to the point of complete restoration of normal vertebral dimensions. Others are left with permanent vertebral deformity if the degree of reshaping has been incomplete by the time of final adult height attainment. In this report, we describe three children with painful VF at leukemia diagnosis or during chemotherapy. Each patient highlights different clinical trajectories in their recovery from VF and underscores the need for osteoporosis intervention trials with the goal to prevent permanent vertebral deformity in selected patients., (© 2016 Wiley Periodicals, Inc.)
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- 2016
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16. The Radiology of Vertebral Fractures in Childhood Osteoporosis Related to Glucocorticoid Administration.
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Lentle B, Ma J, Jaremko JL, Siminoski K, Matzinger MA, Shenouda N, Konji VN, and Ward LM
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- Adult, Child, Humans, Male, Radiography, Glucocorticoids adverse effects, Osteoporosis chemically induced, Osteoporosis complications, Osteoporotic Fractures diagnostic imaging, Osteoporotic Fractures etiology, Spinal Fractures diagnostic imaging, Spinal Fractures etiology
- Abstract
A number of unusual conditions cause decreased bone mass and density in children and these may be associated with low-trauma fractures. However, a series of reports have more recently identified that children with chronic disease sustain vertebral fractures (VFs) much more often than had been suspected. The common denominator involved is glucocorticoid (GC) administration, although other factors such as disease activity come into play. This review will focus on the imaging findings in this form of secondary osteoporosis. Spinal fractures in children have been found to correlate with back pain. At the same time, up to 2/3 of children with VFs in the GC-treated setting are asymptomatic, underscoring the importance of routine surveillance in at-risk children. Other predictors of prevalent and incident VFs include GC exposure (average daily and cumulative dose), declines in lumbar spine bone mineral density Z-scores and increases in body mass index Z-scores, as well as increases in disease activity scores. The imaging diagnosis of osteoporotic VFs in children is made differently from that in adults because immature vertebral bodies continue to ossify during growth. Thus, it is not possible to assess the vertebral end plates or periphery until late, as enchondral ossification extends centripetally within the centrum. Diagnosis, therefore, is much more dependent upon changes in shape than on loss of structural integrity, which may have a more prominent diagnostic role in adults. However, children have a unique ability to model (a growth-dependent process) and thereby reshape previously fractured vertebral bodies. If the underlying disease is successfully treated and the child has sufficient residual growth potential, this means that, on one hand, treatment of the bone disease may be of more limited duration, and, as a last recourse, the diagnosis may be apparent retrospectively., (Copyright © 2015 International Society for Clinical Densitometry. Published by Elsevier Inc. All rights reserved.)
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- 2016
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17. Incident Vertebral Fractures in Children With Leukemia During the Four Years Following Diagnosis.
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Cummings EA, Ma J, Fernandez CV, Halton J, Alos N, Miettunen PM, Jaremko JL, Ho J, Shenouda N, Matzinger MA, Lentle B, Stephure D, Stein R, Sbrocchi AM, Rodd C, Lang B, Israels S, Grant RM, Couch R, Barr R, Hay J, Rauch F, Siminoski K, and Ward LM
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- Antineoplastic Agents therapeutic use, Bone Density, Child, Child, Preschool, Female, Humans, Incidence, Longitudinal Studies, Male, Precursor Cell Lymphoblastic Leukemia-Lymphoma drug therapy, Precursor Cell Lymphoblastic Leukemia-Lymphoma diagnosis, Spinal Fractures epidemiology
- Abstract
Objectives: The purpose of this article was to determine the incidence and predictors of vertebral fractures (VF) during the 4 years after diagnosis in pediatric acute lymphoblastic leukemia (ALL)., Patients and Methods: Children were enrolled within 30 days of chemotherapy initiation, with incident VF assessed annually on lateral spine radiographs according to the Genant method. Extended Cox models were used to assess the association between incident VF and clinical predictors., Results: A total of 186 children with ALL completed the baseline evaluation (median age, 5.3 years; interquartile range, 3.4-9.7 years; 58% boys). The VF incidence rate was 8.7 per 100 person-years, with a 4-year cumulative incidence of 26.4%. The highest annual incidence occurred at 12 months (16.1%; 95% confidence interval [CI], 11.2-22.7), falling to 2.9% at 4 years (95% CI, 1.1-7.3). Half of the children with incident VF had a moderate or severe VF, and 39% of those with incident VF were asymptomatic. Every 10 mg/m(2) increase in average daily glucocorticoid dose (prednisone equivalents) was associated with a 5.9-fold increased VF risk (95% CI, 3.0-11.8; P < .01). Other predictors of increased VF risk included VF at diagnosis, younger age, and lower spine bone mineral density Z-scores at baseline and each annual assessment., Conclusions: One quarter of children with ALL developed incident VF in the 4 years after diagnosis; most of the VF burden was in the first year. Over one third of children with incident VF were asymptomatic. Discrete clinical predictors of a VF were evident early in the patient's clinical course, including a VF at diagnosis.
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- 2015
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18. Incident Vertebral Fractures and Risk Factors in the First Three Years Following Glucocorticoid Initiation Among Pediatric Patients With Rheumatic Disorders.
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LeBlanc CM, Ma J, Taljaard M, Roth J, Scuccimarri R, Miettunen P, Lang B, Huber AM, Houghton K, Jaremko JL, Ho J, Shenouda N, Matzinger MA, Lentle B, Stein R, Sbrocchi AM, Oen K, Rodd C, Jurencak R, Cummings EA, Couch R, Cabral DA, Atkinson S, Alos N, Rauch F, Siminoski K, and Ward LM
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- Adolescent, Arthritis, Juvenile complications, Arthritis, Juvenile drug therapy, Bone Density, Child, Cohort Studies, Dermatomyositis complications, Dermatomyositis drug therapy, Female, Humans, Incidence, Lupus Erythematosus, Systemic complications, Lupus Erythematosus, Systemic drug therapy, Male, Osteoporosis drug therapy, Proportional Hazards Models, Rheumatic Diseases complications, Risk Factors, Scleroderma, Localized complications, Scleroderma, Localized drug therapy, Scleroderma, Systemic complications, Scleroderma, Systemic drug therapy, Spinal Fractures diagnosis, Spinal Fractures epidemiology, Systemic Vasculitis complications, Systemic Vasculitis drug therapy, Glucocorticoids adverse effects, Glucocorticoids therapeutic use, Rheumatic Diseases drug therapy, Spinal Fractures chemically induced
- Abstract
Vertebral fractures are an important yet underrecognized manifestation of osteoporosis in children with chronic, glucocorticoid-treated illnesses. Our goal was to determine the incidence and clinical predictors of vertebral fractures in the 3 years following glucocorticoid initiation among pediatric patients with rheumatic disorders. Incident vertebral fractures were evaluated according to the Genant semiquantitative method on lateral radiographs at baseline and then annually in the 3 years following glucocorticoid initiation. Extended Cox models were used to assess the association between vertebral fractures and clinical risk predictors. A total of 134 children with rheumatic disorders were enrolled in the study (mean ± standard deviation (SD) age 9.9 ± 4.4 years; 65% girls). The unadjusted vertebral fracture incidence rate was 4.4 per 100 person-years, with a 3-year incidence proportion of 12.4%. The highest annual incidence occurred in the first year (6.0%; 95% confidence interval (CI) 2.9% to 11.7%). Almost one-half of the patients with fractures were asymptomatic. Every 0.5 mg/kg increase in average daily glucocorticoid (prednisone equivalents) dose was associated with a twofold increased fracture risk (hazard ratio (HR) 2.0; 95% CI 1.1 to 3.5). Other predictors of increased vertebral fracture risk included: (1) increases in disease severity scores between baseline and 12 months; (2) increases in body mass index Z-scores in the first 6 months of each 12-month period preceding the annual fracture assessment; and (3) decreases in lumbar spine bone mineral density Z-scores in the first 6 months of glucocorticoid therapy. As such, we observed that a clinically significant number of children with rheumatic disorders developed incident vertebral fractures in the 3 years following glucocorticoid initiation. Almost one-half of the children were asymptomatic and thereby would have been undiagnosed in the absence of radiographic monitoring. In addition, discrete clinical predictors of incident vertebral fractures were evident early in the course of glucocorticoid therapy., (© 2015 American Society for Bone and Mineral Research.)
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- 2015
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19. Common normal variants of pediatric vertebral development that mimic fractures: a pictorial review from a national longitudinal bone health study.
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Jaremko JL, Siminoski K, Firth GB, Matzinger MA, Shenouda N, Konji VN, Roth J, Sbrocchi AM, Reed MH, O'Brien MK, Nadel H, McKillop S, Kloiber R, Dubois J, Coblentz C, Charron M, and Ward LM
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- Adolescent, Canada epidemiology, Child, Child, Preschool, False Positive Reactions, Female, Glucocorticoids therapeutic use, Humans, Infant, Longitudinal Studies, Male, Reference Values, Reproducibility of Results, Sensitivity and Specificity, Glucocorticoids adverse effects, Osteoporotic Fractures epidemiology, Osteoporotic Fractures pathology, Spinal Fractures epidemiology, Spinal Fractures pathology, Spine growth & development
- Abstract
Children with glucocorticoid-treated illnesses are at risk for osteoporotic vertebral fractures, and growing awareness of this has led to increased monitoring for these fractures. However scant literature describes developmental changes in vertebral morphology that can mimic fractures. The goal of this paper is to aid in distinguishing between normal variants and fractures. We illustrate differences using lateral spine radiographs obtained annually from children recruited to the Canada-wide STeroid-Associated Osteoporosis in the Pediatric Population (STOPP) observational study, in which 400 children with glucocorticoid-treated leukemia, rheumatic disorders, and nephrotic syndrome were enrolled near glucocorticoid initiation and followed prospectively for 6 years. Normal variants mimicking fractures exist in all regions of the spine and fall into two groups. The first group comprises variants mimicking pathological vertebral height loss, including not-yet-ossified vertebral apophyses superiorly and inferiorly, which can lead to a vertebral shape easily over-interpreted as anterior wedge fracture, physiological beaking, or spondylolisthesis associated with shortened posterior vertebral height. The second group includes variants mimicking other radiologic signs of fractures: anterior vertebral artery groove resembling an anterior buckle fracture, Cupid's bow balloon disk morphology, Schmorl nodes mimicking concave endplate fractures, and parallax artifact resembling endplate interruption or biconcavity. If an unexpected vertebral body contour is detected, careful attention to its location, detailed morphology, and (if available) serial changes over time may clarify whether it is a fracture requiring change in management or simply a normal variant. Awareness of the variants described in this paper can improve accuracy in the diagnosis of pediatric vertebral fractures.
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- 2015
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20. Periorbital ecchymosis ("raccoon eyes") on a bone scan.
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Johnston DL, Story E, Matzinger MA, and Halton J
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- Female, Frontal Bone diagnostic imaging, Humans, Infant, Orbital Neoplasms diagnostic imaging, Radionuclide Imaging, Skull Neoplasms diagnostic imaging, Skull Neoplasms secondary, Bone Neoplasms diagnostic imaging, Bone Neoplasms secondary, Ecchymosis diagnostic imaging, Ecchymosis etiology, Kidney Neoplasms pathology, Neuroblastoma diagnostic imaging, Neuroblastoma pathology, Orbital Neoplasms secondary
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- 2014
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21. Observer agreement in pediatric semiquantitative vertebral fracture diagnosis.
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Siminoski K, Lentle B, Matzinger MA, Shenouda N, and Ward LM
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- Child, Preschool, Diagnosis, Differential, Female, Humans, Infant, Male, Observer Variation, Radiography, Reproducibility of Results, Precursor Cell Lymphoblastic Leukemia-Lymphoma complications, Spinal Fractures diagnostic imaging, Spinal Fractures etiology
- Abstract
Background: The Genant semiquantitative (GSQ) method has been a standard procedure for diagnosis of vertebral fractures in adults but has only recently been shown to be of clinical utility in children. Observer agreement using the GSQ method in this age group has not been described., Objective: To evaluate observer agreement on vertebral readability and vertebral fracture diagnosis using the GSQ method in pediatric vertebral morphometry., Materials and Methods: Spine radiographs of 186 children with acute lymphoblastic leukemia were evaluated independently by three radiologists using the same GSQ methodology as in adults. A subset of 100 radiographs was evaluated on two occasions., Results: An average of 4.7% of vertebrae were unreadable for the three radiologists. Intraobserver Cohen's kappa (κ) on readability ranged from 0.434 to 0.648 at the vertebral level and from 0.416 to 0.611 at the patient level, while interobserver κ for readability had a range of 0.330 to 0.504 at the vertebral level and 0.295 to 0.467 at the patient level. Intraobserver κ for the presence of vertebral fracture had a range of 0.529 to 0.726 at the vertebral level and was 0.528 to 0.767 at the patient level. Interobserver κ for fracture at the vertebral level ranged from 0.455 to 0.548 and from 0.433 to 0.486 at the patient level., Conclusion: Most κ values for both intra- and interobserver agreement in applying the GSQ method to pediatric spine radiographs were in the moderate to substantial range, comparable to the performance of the technique in adult studies. The GSQ method should be considered for use in pediatric research and clinical practice.
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- 2014
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22. The use of intravenous bisphosphonate therapy to treat vertebral fractures due to osteoporosis among boys with Duchenne muscular dystrophy.
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Sbrocchi AM, Rauch F, Jacob P, McCormick A, McMillan HJ, Matzinger MA, and Ward LM
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- Adolescent, Back Pain drug therapy, Back Pain etiology, Back Pain physiopathology, Biopsy, Bone Density drug effects, Bone Density Conservation Agents administration & dosage, Bone Density Conservation Agents adverse effects, Child, Diphosphonates administration & dosage, Diphosphonates adverse effects, Drug Evaluation methods, Glucocorticoids adverse effects, Humans, Ilium pathology, Infusions, Intravenous, Male, Muscular Dystrophy, Duchenne drug therapy, Osteoporosis drug therapy, Osteoporosis pathology, Osteoporosis physiopathology, Osteoporotic Fractures etiology, Osteoporotic Fractures physiopathology, Retrospective Studies, Spinal Fractures etiology, Spinal Fractures physiopathology, Treatment Outcome, Bone Density Conservation Agents therapeutic use, Diphosphonates therapeutic use, Muscular Dystrophy, Duchenne complications, Osteoporotic Fractures drug therapy, Spinal Fractures drug therapy
- Abstract
Unlabelled: The impact of intravenous bisphosphonate treatment to treat painful vertebral fractures in boys with DMD has not been documented. In this retrospective observational study of seven boys, 2 years of intravenous bisphosphonate therapy was associated with back pain improvement and stabilization or increases in the height ratios of fractured vertebrae., Introduction: Boys with Duchenne muscular dystrophy (DMD) are at risk for vertebral fractures. We studied the impact of intravenous bisphosphonate therapy for the treatment of painful vertebral fractures in DMD., Methods: This was a retrospective observational study in seven boys with DMD (median 11.6 years, range 8.5 to 14.3) treated with intravenous pamidronate (9 mg/kg/year) or zoledronic acid (0.1 mg/kg/year) for painful vertebral fractures., Results: At baseline, 27 vertebral fractures were evident in the seven boys. After 2 years of bisphosphonate therapy, 17 of the fractures had an increase in the most severely affected vertebral height ratio, 10 vertebrae stabilized, and none showed a decrease in height ratio. Back pain resolved completely (N = 3) or improved (N = 4). The median change in lumbar spine volumetric bone mineral density Z-score was 0.5 standard deviations (interquartile range, -0.3 to 1.7). Two boys had three incident vertebral fractures in previously normal vertebral bodies that developed over the observation period. There was a decline in the trabecular bone formation rate on trans-iliac bone biopsy but no evidence of osteomalacia. First-dose side effects included fever and malaise (N = 4), hypocalcemia (N = 2), and vomiting (N = 1); there were no side effects with subsequent infusions., Conclusions: Intravenous bisphosphonate therapy was associated with improvements in back pain and stabilization to improvement in vertebral height ratios of previously fractured vertebral bodies. At the same time, such therapy does not appear to completely prevent the development of new vertebral fractures in this context.
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- 2012
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23. Anatomical distribution of vertebral fractures: comparison of pediatric and adult spines.
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Siminoski K, Lee KC, Jen H, Warshawski R, Matzinger MA, Shenouda N, Charron M, Coblentz C, Dubois J, Kloiber R, Nadel H, O'Brien K, Reed M, Sparrow K, Webber C, Lentle B, and Ward LM
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- Adolescent, Age Distribution, Age Factors, Aged, Child, Child, Preschool, Glucocorticoids adverse effects, Humans, Kyphosis complications, Lordosis complications, Lumbar Vertebrae injuries, Middle Aged, Osteoporosis chemically induced, Osteoporosis etiology, Osteoporotic Fractures chemically induced, Osteoporotic Fractures etiology, Osteoporotic Fractures pathology, Spinal Fractures etiology, Thoracic Vertebrae injuries, Trauma Severity Indices, Spinal Fractures pathology
- Abstract
Summary: We compared the distribution of vertebral fractures in adults and children and found that fractures occurred in different locations in the two age groups. This likely relates to the different shape of the immature spine., Introduction: We hypothesized that the anatomical distribution of vertebral fractures (VF) would be different in children compared to adults., Methods: We compared the distribution of VF defined using the Genant semi-quantitative method (GSQ method) in adults (N = 221; 545 fractures) and in children early in the course of glucocorticoid therapy (N = 44; 94 fractures)., Results: The average age in the adult cohort was 62.9 years (standard deviation (SD), 13.4 years), 26% was male, the mean lumbar spine Z-score was -1.0 (SD, 1.5), and the corresponding T-score was -2.4 (SD, 1.4). The pediatric cohort median age was 7.7 years (range, 2.1-16.6 years), the mean lumbar spine Z-score was -1.7 (SD, 1.5), 52% was male, and disease categories were acute lymphoblastic leukemia (66%), rheumatological conditions (21%), and nephrotic syndrome (14%). The VF distribution was biphasic in both populations, but the peaks differed in location. In adults, the peaks were at T7/T8 and at T12/L1. In children, the focus was higher in the thoracic spine, at T6/T7, and lower in the lumbar spine, at L1/L2. When children were assessed in two age-defined sub-groups, a biphasic VF distribution was seen in both, but the upward shift of the thoracic focus to T6 was observed only in the older group, with the highest rates of fracture present between ages 7 and 10 years., Conclusions: These results suggest that the anatomical distribution of VF differs between children and adults, perhaps relating to the different shape of the immature spine, notably the changing ratio of kyphosis to lordosis.
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- 2012
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24. Iliac bone histomorphometry in children with newly diagnosed inflammatory bowel disease.
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Ward LM, Rauch F, Matzinger MA, Benchimol EI, Boland M, and Mack DR
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- Adolescent, Biopsy, Bone Density physiology, Bone Remodeling physiology, Child, Cross-Sectional Studies, Female, Humans, Inflammatory Bowel Diseases pathology, Inflammatory Bowel Diseases physiopathology, Lumbar Vertebrae physiopathology, Male, Osteoporosis pathology, Osteoporosis physiopathology, Prospective Studies, Ilium pathology, Inflammatory Bowel Diseases complications, Osteoporosis etiology
- Abstract
Unlabelled: Children with inflammatory bowel disease (IBD) manifest low bone mass; the cause remains unclear. We performed transilial bone biopsies in 20 IBD children at diagnosis and found a mild cortical bone deficit and slow bone turnover. It is possible that low mechanical stimulation due to inadequate muscle mass contributes to the bone deficit., Introduction: Children with newly diagnosed IBD can have low bone mineral density and disturbed bone metabolism, but the tissue level characteristics of the bone involvement in pediatric IBD have not been elucidated., Methods: In the present study, we evaluated the skeletal status, including static histomorphometry on transiliac bone samples, in 20 patients (age range 8.4 to 17.7 years, 12 boys) with newly diagnosed IBD and compared results to published normative data., Results: Despite normal height (mean Z-score 0.04, SD 1.2), areal bone mineral density at the lumbar spine was moderately low (mean age- and sex-specific Z-score -0.8, SD 1.1). Total body bone mineral content and lean mass were low for age and sex as well (mean Z-scores -1.2, SD 0.9 and -2.0, SD 0.9, respectively). Biochemical bone markers indicated low bone formation and resorption activity. Bone histomorphometry revealed a slightly low cortical width (mean 23%, SD 25%, below the result expected for age) but a normal amount of trabecular bone. The percentage of trabecular bone surface covered by osteoid or osteoclasts was low, suggesting that both bone formation and bone resorption were suppressed., Conclusions: Our results indicate that young patients manifest a mild cortical bone deficit at the iliac crest and slow trabecular bone turnover even at diagnosis, in the setting of IBD.
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- 2010
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25. Spinal cord injuries without radiographic abnormality at two pediatric trauma centers in Ontario.
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Trigylidas T, Yuh SJ, Vassilyadi M, Matzinger MA, and Mikrogianakis A
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- Adolescent, Child, Child, Preschool, Databases, Factual, Female, Hospitals, Pediatric statistics & numerical data, Humans, Incidence, Male, Ontario epidemiology, Prognosis, Retrospective Studies, Spinal Cord diagnostic imaging, Spinal Cord pathology, Spinal Cord Injuries epidemiology, Trauma Centers statistics & numerical data, Magnetic Resonance Imaging statistics & numerical data, Spinal Cord Injuries diagnostic imaging, Spinal Cord Injuries pathology, Tomography, X-Ray Computed statistics & numerical data
- Abstract
Background: Spinal cord injury without radiographic abnormality (SCIWORA) represents a traumatic myelopathy, either transient or permanent, that is not associated with visible vertebral fractures or ligamentous abnormalities on plain radiographs or CT. MRI has become essential in the diagnosis and evaluation of trauma patients and in predicting the long-term neurological outcome., Methods: The medical charts of 578 children with vertebral trauma at the Children's Hospital of Eastern Ontario and the Hospital for Sick Children were retrospectively reviewed., Results: Forty-five patients were identified as having traumatic myelopathy. Three of these patients had SCIWORA. Two sustained thoracic spinal cord injuries as a result of motor vehicle accidents and permanent neurological deficits. The third was involved in a sports-related injury and sustained a cervical spine injury that improved in 48 h., Conclusions: SCIWORA is uncommon. The serious neurological sequelae that can result from a missed diagnosis merit more attention in identifying a spinal lesion in children with traumatic myelopathy. MRI has played a valuable role in this respect and may be even more predictive of outcome than the presenting neurological findings. The SCIWORA acronym can be modified to SCIWONA (spinal cord injury without neuroimaging abnormality) in order to highlight the importance of MRI in the prognosis., (Copyright © 2010 S. Karger AG, Basel.)
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- 2010
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26. Brainstem compression: a predictor of postoperative cerebellar mutism.
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McMillan HJ, Keene DL, Matzinger MA, Vassilyadi M, Nzau M, and Ventureyra EC
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- Adolescent, Astrocytoma pathology, Astrocytoma surgery, Child, Child, Preschool, Ependymoma pathology, Ependymoma surgery, Female, Humans, Infant, Infratentorial Neoplasms pathology, Magnetic Resonance Imaging, Male, Medulloblastoma pathology, Medulloblastoma surgery, Organ Size, Retrospective Studies, Brain Diseases pathology, Infratentorial Neoplasms surgery, Mutism etiology, Pons pathology, Postoperative Complications
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Purpose: Cerebellar mutism is a common complication of posterior fossa tumor resection. We observed marked, preoperative brainstem compression on MR imaging, among patients who developed postoperative mutism. This study was designed to investigate if an association was indeed present., Materials and Methods: Patients (18 months-18 years) undergoing resection of a midline, posterior fossa tumor were retrospectively reviewed. Demographic data, tumor pathology, mutism onset and duration, and postoperative complications were obtained from hospital records. Pre- and postoperative MR images were studied to assess tumor size and the severity of pons compression (an estimate of the mechanical and distortional forces imparted by the tumor)., Results: Patients with mutism showed greater preoperative pons compression and a greater increase in postoperative pons diameter., Conclusion: We predict that brainstem compression may represent white-matter injury from (1) surgical manipulation and traction, and (2) axonal damage caused by the release of the tumor's compressive force and ensuing axon distortion and dysfunction. The results provide support that mutism may be largely caused by white-matter damage disrupted axon integrity and function.
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- 2009
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27. Factors associated with epilepsy in children with periventricular leukomalacia.
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Humphreys P, Deonandan R, Whiting S, Barrowman N, Matzinger MA, Briggs V, Hurteau J, and Wallace E
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- Cerebral Palsy etiology, Child, Preschool, Cohort Studies, Epilepsy pathology, Female, Humans, Infant, Infant, Newborn, Leukomalacia, Periventricular pathology, Male, Retrospective Studies, Risk Factors, Tomography, X-Ray Computed methods, Epilepsy epidemiology, Epilepsy etiology, Leukomalacia, Periventricular complications, Leukomalacia, Periventricular epidemiology
- Abstract
Children with cerebral palsy associated with periventricular leukomalacia frequently develop unprovoked epileptic seizures. This article reports an analysis of risk factors for epilepsy in children with radiologically confirmed periventricular leukomalacia. This cohort was screened for epilepsy and for an array of clinical and demographic factors. Of 154 subjects with radiologically confirmed periventricular leukomalacia, 40 (26.0%) had epilepsy. In the epileptic group, radiologic pathology other than periventricular leukomalacia was uncommon. Significant associations were found between epilepsy and cerebral palsy patterns other than spastic diparesis, mental handicap, cortical visual impairment, neonatal seizures, and severe periventricular leukomalacia. Only the presence of neonatal seizures was significantly associated with epilepsy once other risk factors were controlled in the regression model. Some previous studies have shown an association between neonatal seizures and later epilepsy for cerebral palsy in general. This is the first report of such an association for a single predominant type of cerebral pathology.
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- 2007
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28. Radioisotope shuntograms at the Children's Hospital of Eastern Ontario.
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Vassilyadi M, Tataryn ZL, Matzinger MA, Briggs V, and Ventureyra EC
- Subjects
- Adolescent, Adult, Cerebrospinal Fluid Shunts adverse effects, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Hydrocephalus diagnostic imaging, Hydrocephalus surgery, Male, Medical Records, Ontario, Radioisotopes adverse effects, Radionuclide Imaging, Retrospective Studies, Technetium Tc 99m Pentetate, Cerebrospinal Fluid Shunts methods, Hospitals
- Abstract
Introduction: Nuclear medicine shuntograms using the radiotracer technetium-99m diethylenetriaminepentaacetic acid have been used for many years as an additional method to assess shunt patency and performance., Methods: The medical records of all children who had shuntograms performed at the Children's Hospital of Eastern Ontario between January 13, 2000, and January 10, 2003, were retrospectively reviewed. There was a total of 68 procedures in 56 patients with an average age of 9 years 3 months. During the same period there were 123 shunt revisions., Results: Forty-two shuntograms were reported as normal. Of these, ten were identified to be false negative. Two children with fractured shunts had shuntograms performed in order to assess for cerebrospinal fluid flow patency; the shuntograms were identified to be normal, confirming shunt dependence, and the shunt replaced. Twenty-six shuntograms were reported as abnormal in 24 patients; 17 went on to have surgery and the shunt malfunction was confirmed. Seven patients did not require surgery: five were declared shunt independent, two patients clinically improved after severe constipation was treated., Conclusions: Approximately one fourth of all shuntograms reported as normal are not (false-negative rate=25%). Review of five other major studies between 1980 and 2003 have reported false-negative rates between 2 and 36%, which may be explained by variations in shuntogram protocols. A standardized method is proposed.
- Published
- 2006
- Full Text
- View/download PDF
29. Unilateral hyperechoic renal pyramids in a child with renal artery stenosis.
- Author
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Navarro OM, Daneman A, and Matzinger MA
- Subjects
- Angioplasty, Balloon, Child, Diagnosis, Differential, Female, Humans, Renal Artery Obstruction therapy, Ultrasonography, Kidney Medulla diagnostic imaging, Renal Artery Obstruction diagnostic imaging
- Published
- 2006
- Full Text
- View/download PDF
30. Cognitive changes in children treated for acute lymphoblastic leukemia with chemotherapy only according to the Pediatric Oncology Group 9605 protocol.
- Author
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Montour-Proulx I, Kuehn SM, Keene DL, Barrowman NJ, Hsu E, Matzinger MA, Dunlap H, and Halton JM
- Subjects
- Anti-Inflammatory Agents administration & dosage, Antimetabolites, Antineoplastic administration & dosage, Antimetabolites, Antineoplastic adverse effects, Brain pathology, Child, Child, Preschool, Cohort Studies, Cytarabine administration & dosage, Female, Hematinics administration & dosage, Humans, Hydrocortisone administration & dosage, Leucovorin administration & dosage, Male, Methotrexate administration & dosage, Methotrexate adverse effects, Neuropsychological Tests, Precursor Cell Lymphoblastic Leukemia-Lymphoma pathology, Retrospective Studies, Wechsler Scales, Antineoplastic Combined Chemotherapy Protocols adverse effects, Cognition Disorders chemically induced, Precursor Cell Lymphoblastic Leukemia-Lymphoma drug therapy
- Abstract
The purpose of this study was to examine cognitive functioning and neuroimaging in children with leukemia treated with the Pediatric Oncology Group 9605 protocol at the Children's Hospital of Eastern Ontario. Mean age at diagnosis was 4.88 +/- 2.54 years. The mean (n = 24) Wechsler Verbal and Performance IQ fell in the low-average range (87.33 +/- 15.69 and 84.83 +/- 19.11, respectively). Mean (n = 20) Verbal and Visual Memory Indexes of 82.95 +/- 15.46 and 88.30+/- 10.80, respectively, were obtained. The proportion of scores on measures of intelligence and memory falling > 1 SD below the normative mean was substantially higher than expected. Paired t-test suggested that Wechsler Verbal IQ and memory remained stable, whereas Wechsler Performance IQ declined significantly. The results of growth curve analyses replicated these findings and suggested a significant adverse effect of cumulative dosage of intrathecal methotrexate on estimated Wechsler Performance IQ. Although only two children experienced seizures, 78% of the group showed leukoencephalopathy on at least one magnetic resonance image. Reliance on seizures as a predictor of leukoencephalopathy might underestimate the incidence of neurotoxicity.
- Published
- 2005
- Full Text
- View/download PDF
31. Resolution of severe, adolescent-onset hypophosphatemic rickets following resection of an FGF-23-producing tumour of the distal ulna.
- Author
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Ward LM, Rauch F, White KE, Filler G, Matzinger MA, Letts M, Travers R, Econs MJ, and Glorieux FH
- Subjects
- Base Sequence, Bone Neoplasms complications, Bone Neoplasms metabolism, Child, DNA Primers, Female, Fibroblast Growth Factor-23, Humans, Hypophosphatemia, Familial etiology, Bone Neoplasms surgery, Fibroblast Growth Factors biosynthesis, Hypophosphatemia, Familial therapy, Ulna pathology
- Abstract
Oncogenic hypophosphatemic osteomalacia (OHO) is an uncommon hypophosphatemic syndrome characterized by bone pain, proximal muscle weakness and rickets. It has been postulated that OHO results from overproduction of a humoral phosphaturic factor by an occult tumour. Recently, some OHO tumours have been shown to elaborate fibroblast growth factor-23 (FGF-23), which causes renal phosphate wasting when administered to mice. The purpose of this study was to undertake detailed investigations to confirm the diagnosis of OHO in a pediatric patient and to document the biochemical, radiographic and bone histological phenotype before and after tumour removal. We describe an 11-year-old, previously healthy girl with significant pain and functional disability associated with hypophosphatemic rickets. Circulating 1,25-(OH)(2) vitamin D was very low (14 pM; N: 40-140) while the FGF-23 serum level was markedly elevated [359.5 reference units (RU)/ml, N: 33-105]. An iliac bone biopsy revealed severe osteomalacia, but periosteocytic lesions, as are typical for X-linked hypophosphatemic rickets, were not seen. Sequence analyses of the PHEX and FGF23 genes were normal. A radiographic skeletal survey revealed a small exostosis of the left, distal ulnar metaphysis. A tumour was subsequently removed from this site and the pathology was consistent with benign, fibro-osseous tissue. Serum FGF-23 was normal when measured at 7 h post-operatively, while serum phosphate reached the low-normal range at 16 days following surgery. An iliac bone biopsy taken 5 months after the operation showed improvement, but not yet resolution, of the osteomalacia. Biochemical parameters of bone and mineral metabolism suggested that complete resolution of the osteomalacia was not achieved until 12 months following surgery. One year after tumour removal, the patient was pain-free and had resumed a normal level of activity. The rapid normalization of FGF-23 levels following removal of a benign tumour and the subsequent improvement in the biochemical and histological parameters of bone and mineral metabolism suggest that FGF-23 played a key role in this girl's disease.
- Published
- 2004
- Full Text
- View/download PDF
32. Residents' corner. Answer to case of the month #84. Primary hyperoxaluria type I.
- Author
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Matzinger MA, Filler G, Dunlap H, Briggs V, Sly L, and Udjus K
- Subjects
- Female, Humans, Infant, Radiography, Ultrasonography, Hyperoxaluria, Primary diagnostic imaging
- Published
- 2001
33. Cerebral vascular events associated with ulcerative colitis in children.
- Author
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Keene DL, Matzinger MA, Jacob PJ, and Humphreys P
- Subjects
- Adolescent, Brain pathology, Child, Child, Preschool, Female, Humans, Magnetic Resonance Imaging, Male, Cerebrovascular Disorders diagnosis, Cerebrovascular Disorders etiology, Colitis, Ulcerative complications, Colitis, Ulcerative diagnosis
- Abstract
Although peripheral vascular thrombic events are recognized as a serious extra-intestinal complication of inflammatory bowel disease, the occurrence of cerebral vascular events in association with acute exacerbations of this group of diseases is rare. In this article, relevant literature is reviewed and three children, 5, 12, and 13 years of age, who presented with clinical and magnetic resonance imaging evidence of an acute cerebrovascular event in association with an acute exacerbation of their inflammatory bowel disease are described. Except for the presence of anemia, hematologic and coagulation studies were unremarkable, and a search for evidence of a systemic vasculitis proved negative.
- Published
- 2001
- Full Text
- View/download PDF
34. Temporal lobe focal cortical dysplasia: MRI imaging using FLAIR shows lesions consistent with neoplasia.
- Author
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Modha A, Vassilyadi M, Keene D, Jimenez C, Michaud J, Matzinger MA, and Ventureyra EC
- Subjects
- Brain Diseases pathology, Brain Diseases physiopathology, Brain Neoplasms diagnosis, Child, Diagnosis, Differential, Epilepsies, Partial pathology, Epilepsy, Temporal Lobe pathology, Female, Hippocampus pathology, Humans, Male, Sclerosis, Temporal Lobe physiopathology, Brain Diseases diagnosis, Epilepsies, Partial etiology, Epilepsy, Temporal Lobe etiology, Magnetic Resonance Imaging methods, Temporal Lobe abnormalities, Temporal Lobe pathology
- Abstract
Focal cortical dysplasia (FCD), a form of neuronal migration disorder, is a malformative lesion of the neocortex that occurs during development of the brain. It can cause partial and generalized epilepsy. Seizures occur at an early age and are often resistant to medication. Surgical resection has been found to be beneficial in these patients. Dual pathology, in the form of mesial temporal sclerosis, has been associated with FCD. At the Children's Hospital of Eastern Ontario, four patients with temporal lobe FCD have recently, been identified. This paper discusses how these children presented and how they were managed, with particular emphasis on their MRI findings and differential diagnoses. In three of the four patients neuroimaging studies showed lesions consistent with a neoplastic process because of the large volume and mass effect. Radiologically, FCD may mimic the MRI appearance of tumors, such as dysembryoplastic neuroepithelial tumors, primitive neuroectodermal tumors, gangliogliomas, oligodendrogliomas, and astrocytomas. These lesions are best visualized on fluid-attenuated inversion recovery (FLAIR) imaging, a technique that has recently become applicable in the clinical setting, as we help demonstrate in this series. With better MRI capability, milder forms of FCD and microdysplasia may be distinguished.
- Published
- 2000
- Full Text
- View/download PDF
35. Primary ciliary dyskinesia associated with a novel microtubule defect in a child with Down's syndrome.
- Author
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Kovesi T, Sinclair B, MacCormick J, Matzinger MA, and Carpenter B
- Subjects
- Biopsy, Child, Ciliary Motility Disorders diagnostic imaging, Ciliary Motility Disorders genetics, Ciliary Motility Disorders pathology, Diagnosis, Differential, Down Syndrome genetics, Humans, Male, Radionuclide Imaging, Ciliary Motility Disorders complications, Down Syndrome complications, Microtubules pathology
- Abstract
We present a child with Down's syndrome, bilateral lower lobe bronchiectasis, sinusitis, and severe ear disease who was found to have a novel ciliary defect, with a frequent, partial absence of the walls of the A subunits of some peripheral doublets. The defect caused the A subunits to be "U-shaped" rather than "O-shaped." A nuclear nasal mucociliary transport study confirmed that this defect was associated with abnormal mucociliary transport. The ciliary defect was not observed in a biopsy performed in a second patient who had Down's syndrome.
- Published
- 2000
- Full Text
- View/download PDF
36. Bone marrow metastasis in astrocytic gliomata.
- Author
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Hsu E, Keene D, Ventureyra E, Matzinger MA, Jimenez C, Wang HS, and Grimard L
- Subjects
- Adolescent, Antineoplastic Combined Chemotherapy Protocols therapeutic use, Astrocytoma drug therapy, Bone Marrow Neoplasms diagnostic imaging, Bone Marrow Neoplasms drug therapy, Brain Neoplasms diagnosis, Brain Neoplasms pathology, Carmustine administration & dosage, Cisplatin administration & dosage, Humans, Magnetic Resonance Imaging, Male, Radionuclide Imaging, Tomography, X-Ray Computed, Astrocytoma secondary, Bone Marrow Neoplasms secondary
- Abstract
With the increasing survival time of many pediatric patients with malignancies, unexpected symptoms or signs require diligent search for rare complications or second cancers related to the disease or treatment. We recently encountered a patient with extensive glioblastoma multiforme who developed pancytopenia six months after completion of treatment with craniospinal radiation and chemotherapy with etoposide and cyclophosphamide. Bone marrow aspirate and biopsy confirmed bone marrow metastasis from the brain tumor. He showed good partial remission with chemotherapy with carmustine and cis-platinum as demonstrated by serial bone marrow aspirate for cytology and cytogenetics and enjoyed good quality of life for eight months. 14 other patients with astrocytic glioma, two of whom are children, are reported in the literature to have diffuse bone marrow metastasis. Therefore, in patients with malignant astrocytic tumor, bone marrow metastasis, though not common, should be considered when bone pain or cytopenias occur, especially when prolonged.
- Published
- 1998
- Full Text
- View/download PDF
37. Residents' corner. Answer to case of the month #21. Transient osteoporosis of the hip.
- Author
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Rhodes I, Matzinger F, and Matzinger MA
- Subjects
- Adult, Diagnosis, Differential, Female, Humans, Pregnancy, Radiography, Radionuclide Imaging, Hip Joint diagnostic imaging, Osteoporosis diagnostic imaging, Pregnancy Complications diagnostic imaging
- Published
- 1993
38. Prevalence of hypertension in children with primary vesicoureteral reflux.
- Author
-
Wolfish NM, Delbrouck NF, Shanon A, Matzinger MA, Stenstrom R, and McLaine PN
- Subjects
- Adolescent, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Hypertension, Renal etiology, Male, Prevalence, Retrospective Studies, Time Factors, Urinary Tract Infections complications, Urinary Tract Infections epidemiology, Vesico-Ureteral Reflux epidemiology, Hypertension, Renal epidemiology, Vesico-Ureteral Reflux complications
- Abstract
This study was undertaken to determine the prevalence of hypertension in children with primary, uncomplicated vesicoureteral reflux (VUR) and to evaluate the relationship between blood pressure (BP), grade and duration of reflux, and renal scarring. Subjects were identified retrospectively during a 17-year period; of 146 subjects who agreed to participate, 129 (88.4%) were female. Mean age at diagnosis was 5.0 years (range, 1 month to 16 years), and at follow-up was 14.4 years (range, 5 months to 21 years). Mean duration of follow-up was 9.6 years. Renal scarring was detected in 34.3% of patients by intravenous pyelogram, ultrasonography, or both. The BP at diagnosis was linearly related to the grade of reflux, but values were not higher than expected norms for age. At follow-up, mean systolic and diastolic BP were at the 41.6 percentile and the 18.7 percentile, respectively. No patient's BP was above the 55th percentile. After a mean follow-up period of 10 years, we conclude that primary, uncomplicated VUR, regardless of the number of documented urinary tract infections, duration and severity of reflux, modality of therapy, presence of renal scarring, and duration of follow-up, is not associated with the development of hypertension. Hypertension does not appear to be a complication of VUR and urinary tract infection unless there is preexisting dysplasia.
- Published
- 1993
- Full Text
- View/download PDF
39. Residents' corner. Case of the month #21.
- Author
-
Rhodes I, Matzinger F, and Matzinger MA
- Subjects
- Adult, Diagnosis, Differential, Female, Humans, Joint Diseases diagnosis, Pregnancy, Radiography, Radionuclide Imaging, Hip Joint diagnostic imaging, Pelvic Bones diagnostic imaging, Pregnancy Complications diagnostic imaging
- Published
- 1993
40. Antenatal and postnatal findings in intra-abdominal pulmonary sequestration.
- Author
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Matzinger MA, Matzinger FR, Matzinger KE, and Black MD
- Subjects
- Abdominal Neoplasms diagnostic imaging, Adult, Diagnosis, Differential, Female, Humans, Neuroblastoma diagnostic imaging, Pregnancy, Tomography, X-Ray Computed, Ultrasonography, Prenatal, Bronchopulmonary Sequestration diagnostic imaging
- Abstract
A hyperechoic mass in the right upper quadrant of the abdomen was demonstrated in a fetus by antenatal ultrasonography (US) at 20 and 33 weeks' gestation. Postnatal US, myelography and computed tomography were performed preoperatively; the findings were judged consistent with neuroblastoma. Surgical excision and pathological examination revealed bronchopulmonary sequestration.
- Published
- 1992
41. Intrapulmonary bronchogenic cyst: spontaneous pneumothorax as the presenting symptom.
- Author
-
Matzinger MA, Matzinger FR, and Sachs HJ
- Subjects
- Adolescent, Bronchogenic Cyst diagnostic imaging, Female, Humans, Tomography, X-Ray Computed, Bronchogenic Cyst complications, Pneumothorax etiology
- Published
- 1992
- Full Text
- View/download PDF
42. Evaluation of renal scars by technetium-labeled dimercaptosuccinic acid scan, intravenous urography, and ultrasonography: a comparative study.
- Author
-
Shanon A, Feldman W, McDonald P, Martin DJ, Matzinger MA, Shillinger JF, McLaine PN, and Wolfish N
- Subjects
- Adolescent, Child, Child, Preschool, Cicatrix diagnostic imaging, Female, Humans, Kidney diagnostic imaging, Male, Observer Variation, Prospective Studies, Radionuclide Imaging, Sensitivity and Specificity, Technetium Tc 99m Dimercaptosuccinic Acid, Ultrasonography, Urography, Cicatrix diagnosis, Kidney pathology, Organotechnetium Compounds, Succimer
- Abstract
The objective of our prospective study was to compare the sensitivity and specificity of ultrasonography, intravenous pyelography, and dimercaptosuccinic acid scan in detecting scarred kidneys. Twenty-seven consecutive subjects with recurrent urinary tract infections, vesicoureteral reflux, scarred kidneys, or a combination of these problems had all three imaging procedures performed. With the total number of scars serving as the gold standard, the sensitivity (94%) and specificity (100%) in identifying renal scars in children were highest for the DMSA scan. Intraobserver (95%) and interobserver (90%) reliability were also high for the DMSA scan. However, the clinical interpretation of the increased sensitivity of the DMSA scan is unknown. Changes on the scan not identified by intravenous urography may not represent true scars. Research into the long-term significance of these scars is indicated.
- Published
- 1992
- Full Text
- View/download PDF
43. Plain film and CT observations in prostaglandin-induced bone changes.
- Author
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Matzinger MA, Briggs VA, Dunlap HJ, Udjus K, Martin DJ, and McDonald P
- Subjects
- Alprostadil therapeutic use, Humans, Hyperostosis diagnostic imaging, Infant, Newborn, Male, Periosteum drug effects, Periostitis diagnostic imaging, Tomography, X-Ray Computed, Alprostadil adverse effects, Bone and Bones diagnostic imaging, Heart Defects, Congenital drug therapy, Hyperostosis chemically induced, Periostitis chemically induced
- Abstract
Prostaglandin E1 intravenous infusion is used in infants with ductal-dependent congenital heart disease to maintain ductal patency and prolong life until palliative or corrective surgery is feasible. Complications of prostaglandin administration include fever, diarrhoea, hypotension, apnoea, bradycardia, pseudowidening of the cranial sutures, underossification of the calvarial bones, periostitis, and skin edema [1-3]. This paper presents dramatic plain radiographic features of prostaglandin-induced bone disease, including periosteal proliferation and the unusual bone-within-bone appearance, and provides the previously unpublished CT correlation.
- Published
- 1992
- Full Text
- View/download PDF
44. Sonography of milk of calcium in a calyceal diverticulum: a case report.
- Author
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Leekam RN, Matzinger MA, and Keresteci AG
- Subjects
- Adult, Humans, Kidney Diseases, Cystic diagnosis, Male, Diverticulum diagnosis, Kidney Calices, Kidney Pelvis, Ultrasonography
- Abstract
Ultrasonography is the modality of choice in differentiating between cystic and solid renal lesions. It is somewhat less effective in demonstrating renal calculi. We report here a patient referred for investigation of a calcified renal mass, in whom ultrasonography permitted the diagnosis of milk of calcium in a calyceal diverticulum.
- Published
- 1984
45. Cirrhosis: an unusual pattern of enhancement on CT.
- Author
-
Daneman A, Matzinger MA, and Martin DJ
- Subjects
- Barium Sulfate administration & dosage, False Negative Reactions, Female, Humans, Infant, Injections, Intravenous, Radiographic Image Enhancement, Time Factors, Tomography, X-Ray Computed, Liver diagnostic imaging, Liver Cirrhosis diagnostic imaging
- Abstract
A previously unreported pattern of enhancement of the liver on CT in a 7-month-old female with cirrhosis is illustrated. Precontrast scans showed the liver to have a normal homogeneous density throughout. Scans performed immediately after rapid bolus injection of contrast showed an uneven enhancement of the entire liver. Bands of enhancing tissue (thought to represent vascular fibrous tissue) surrounded more extensive areas that enhanced less markedly. Delayed post-injection scans revealed again an even level of density throughout the liver.
- Published
- 1983
- Full Text
- View/download PDF
46. Gastric amyloidosis simulating antral malignancy on ultrasound.
- Author
-
Leekam RN, Matzinger MA, and Gray RR
- Subjects
- Diagnosis, Differential, Female, Humans, Middle Aged, Pyloric Antrum, Stomach Neoplasms diagnosis, Amyloidosis diagnosis, Stomach Diseases diagnosis, Ultrasonography
- Published
- 1985
- Full Text
- View/download PDF
47. Ultrasound demonstration of a circumaortic left renal vein.
- Author
-
Leekam RN, Gray RR, Matzinger MA, and Grosman H
- Subjects
- Adult, Humans, Male, Renal Veins abnormalities, Ultrasonography
- Published
- 1985
- Full Text
- View/download PDF
48. The sonography of renal columnar hypertrophy.
- Author
-
Leekam RN, Matzinger MA, Brunelle M, Gray RR, and Grosman H
- Subjects
- Diagnosis, Differential, Humans, Hypertrophy diagnosis, Retrospective Studies, Kidney Cortex pathology, Ultrasonography
- Abstract
An enlarged Column of Bertin is a frequent cause of renal pseudotumor. In a retrospective study of the ultrasound findings in 11 patients with columnar hypertrophy, we have attempted to establish objective sonographic criteria of this entity. Columnar hypertrophy appears as a renal mass with the following characteristics: (1) indents the renal sinus laterally; (2) clearly defined from the renal sinus; (3) largest dimension of less than 3 cm; (4) continuous or contiguous with the renal cortex; and (5) Echogenicity close to that of the cortex. Two minor criteria are also presented, and the present appropriate use of the criteria is suggested.
- Published
- 1983
- Full Text
- View/download PDF
49. Argyrosis of the urinary tract.
- Author
-
Matzinger MA, Gray RR, Leekam RN, Grosman H, and St Louis EL
- Subjects
- Adult, Cystoscopy, Humans, Male, Tomography, X-Ray Computed, Ultrasonography, Urography, Argyria diagnosis, Urologic Diseases diagnosis
- Published
- 1985
- Full Text
- View/download PDF
50. Esophageal involvement in eosinophilic gastroenteritis.
- Author
-
Matzinger MA and Daneman A
- Subjects
- Adolescent, Anemia, Hypochromic etiology, Biopsy, Eosinophilia diagnosis, Eosinophilia pathology, Epithelium pathology, Esophagitis diagnostic imaging, Esophagitis pathology, Esophagoscopy, Gastroenteritis diagnostic imaging, Gastroenteritis pathology, Humans, Hypersensitivity complications, Male, Radiography, Eosinophilia complications, Esophagitis etiology, Gastroenteritis complications
- Published
- 1983
- Full Text
- View/download PDF
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