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1.
Artigo em Inglês | MEDLINE | ID: mdl-39126675

RESUMO

Glucocorticoid (GC) therapy remains the cornerstone of treatment for many conditions of childhood and an important cause of skeletal and endocrine morbidity. Here, we discuss cases that bring to life the most important concepts in the management of pediatric GC-induced osteoporosis (pGIO). Given the wide variety of underlying conditions linked to pGIO, we focus on the fundamental clinical-biological principles that provide a blueprint for management in any clinical context. In so doing, we underscore the importance of longitudinal vertebral fracture phenotyping, how knowledge about the timing and risk of fractures influences monitoring, the role of bone mineral density in pGIO assessments, and the impact of growth-mediated "vertebral body reshaping" after spine fractures on the therapeutic approach. Overall, pGIO management is predicated upon early identification of fractures (including vertebral) in those at risk, and timely intervention when there is limited potential for spontaneous recovery. Even a single, low-trauma long bone or vertebral fracture can signal an osteoporotic event in an at-risk child. The most widely used treatments for pediatric osteoporosis, intravenous bisphosphonates, are currently recommended first-line for the treatment of pGIO. It is recognized, however, that even early identification of bone fragility, combined with timely introduction of the most potent bisphosphonate therapies, may not completely prevent osteoporosis progression in all contexts. Therefore, prevention of first-ever fractures in the highest-risk settings is on the horizon, where there is also a need to move beyond anti-resorptives to the study of anabolic agents.

2.
Hand (N Y) ; : 15589447241257705, 2024 Jul 26.
Artigo em Inglês | MEDLINE | ID: mdl-39066492

RESUMO

BACKGROUND: Pediatric scaphoid fractures can be challenging to diagnose on plain radiograph. Rates of missed scaphoid fractures can be as high as 30% to 37% on initial imaging and overall sensitivity ranging from 21% to 97%. Few studies, however, have examined the reliability of radiographs in the diagnosis of scaphoid fractures, and none are specific to the pediatric population. Reliability, both between different specialists and for individual raters, may elucidate some of the diagnostic challenges. METHODS: We conducted a 2-iteration survey of pediatric orthopedic surgeons, plastic surgeons, radiologists, and emergency physicians at a tertiary children's hospital. Participants were asked to assess 10 series of pediatric wrist radiographs for evidence of scaphoid fracture. Inter-rater and intrarater reliability was calculated using the intraclass correlation coefficient of 2.1. RESULTS: Forty-two respondents were included in the first iteration analysis. Inter-rater reliability between surgeons (0.66; 95% confidence interval, 0.43-0.87), radiologists (0.76; 0.55-0.92), and emergency physicians (0.65; 0.46-0.86) was "good" to "excellent." Twenty-six respondents participated in the second iteration for intrarater reliability (0.73; 0.67-0.78). Sensitivity (0.75; 0.69-0.81) and specificity (0.78; 0.71-0.83) of wrist radiographs for diagnosing scaphoid fractures were consistent with results in other studies. CONCLUSIONS: Both inter-rater and intrarater reliability for diagnosing pediatric scaphoid fractures on radiographs was good to excellent. No significant difference was found between specialists. Plain radiographs, while useful for obvious scaphoid fractures, are unable to reliably rule out subtle fractures routinely. Our study demonstrates that poor sensitivity stems from the test itself, and not rater variability.

3.
Bone Rep ; 20: 101725, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38229908

RESUMO

Cutaneous-skeletal hypophosphatemia syndrome (CSHS) is a rare bone disorder featuring fibroblast growth factor-23 (FGF23)-mediated hypophosphatemic rickets. We report a 2-year, 10-month-old girl with CSHS treated with burosumab, a novel human monoclonal antibody targeting FGF23. This approach was associated with rickets healing, improvement in growth and lower limb deformity, and clinically significant benefit to her functional mobility and motor development. This case report provides evidence for the effective use of FGF23-neutralizing antibody therapy beyond the classic FGF23-mediated disorders of X-linked hypophosphatemia and tumor-induced osteomalacia.

4.
J Clin Endocrinol Metab ; 109(3): e1225-e1237, 2024 Feb 20.
Artigo em Inglês | MEDLINE | ID: mdl-37843393

RESUMO

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.


Assuntos
Fraturas Ósseas , Fraturas por Osteoporose , Fraturas da Coluna Vertebral , Criança , Humanos , Glucocorticoides/efeitos adversos , Corpo Vertebral , Densidade Óssea , Fraturas Ósseas/induzido quimicamente , Fraturas da Coluna Vertebral/etiologia , Fraturas da Coluna Vertebral/induzido quimicamente , Fraturas por Osteoporose/induzido quimicamente
5.
J Clin Endocrinol Metab ; 109(2): 536-548, 2024 Jan 18.
Artigo em Inglês | MEDLINE | ID: mdl-37610420

RESUMO

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.


Assuntos
Fraturas Ósseas , Distrofia Muscular de Duchenne , Fraturas por Osteoporose , Fraturas da Coluna Vertebral , Masculino , Humanos , Densidade Óssea , Distrofia Muscular de Duchenne/complicações , Distrofia Muscular de Duchenne/tratamento farmacológico , Distrofia Muscular de Duchenne/epidemiologia , Fraturas da Coluna Vertebral/diagnóstico por imagem , Fraturas da Coluna Vertebral/epidemiologia , Fraturas da Coluna Vertebral/etiologia , Fraturas Ósseas/etiologia , Fraturas Ósseas/induzido quimicamente , Fatores de Risco , Glucocorticoides/efeitos adversos , Vértebras Lombares/diagnóstico por imagem , Esteroides , Fraturas por Osteoporose/etiologia
7.
J Bone Miner Res ; 38(8): 1104-1115, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37326443

RESUMO

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).


Assuntos
Leucemia , Osteonecrose , Osteoporose , Humanos , Criança , Densidade Óssea , Vértebras Lombares , Osteonecrose/induzido quimicamente , Osteonecrose/diagnóstico por imagem , Absorciometria de Fóton/métodos
8.
Calcif Tissue Int ; 112(5): 613-620, 2023 05.
Artigo em Inglês | MEDLINE | ID: mdl-36867194

RESUMO

Osteogenesis imperfecta (OI) type VI, a recessively inherited form of OI caused by mutations in SERPINF1, is a severe form distinguished by osteomalacia on bone histomorphometry. We describe a boy with severe OI type VI who was initially treated with intravenous (IV) zoledronic acid (ZA) at 1.4 years of age; however, a year later he transitioned to denosumab 1 mg/kg sub-cutaneously every three months in an effort to decrease fracture rates. After two years on denosumab, he presented with symptomatic hypercalcemia due to the denosumab-induced, hyper-resorptive rebound phenomenon. Laboratory parameters at the time of the rebound were as follows: elevated serum ionized calcium (1.62 mmol/L, N 1.16-1.36), elevated serum creatinine due to hypercalcemia-induced muscle catabolism (83 µmol/L, N 9-55), and suppressed parathyroid hormone (PTH) (< 0.7 pmol/L, N 1.3-5.8). The hypercalcemia was responsive to low-dose IV pamidronate, with a rapid decline in serum ionized calcium, and otherwise normalization of the aforementioned parameters within 10 days. To benefit from the powerful, albeit short-term, anti-resorptive effect of denosumab without further rebound episodes, he was treated thereafter with denosumab 1 mg/kg alternating every three months with IV ZA 0.025 mg/kg. Five years later, he remained on dual alternating anti-resorptive therapy without further rebound episodes, and an overall improvement in his clinical status. This novel pharmacological approach of alternating short- and long-term anti-resorptive therapy every three months has not previously been described. Our report suggests this strategy may be an effective method for prevention of the rebound phenomenon in select children for whom denosumab may be beneficial.


Assuntos
Conservadores da Densidade Óssea , Hipercalcemia , Osteogênese Imperfeita , Criança , Masculino , Humanos , Osteogênese Imperfeita/tratamento farmacológico , Osteogênese Imperfeita/genética , Denosumab , Hipercalcemia/tratamento farmacológico , Cálcio/farmacologia , Densidade Óssea , Ácido Zoledrônico/uso terapêutico
9.
Bone Rep ; 18: 101663, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36950254

RESUMO

Osteochondritis dissecans (OCD) is a disease of the joints characterized by idiopathic focal subchondral lesions. Aggrecan, a proteoglycan encoded by the ACAN gene, is important for cartilage structure and function. We describe the clinical evolution of a patient with short stature, multi-focal OCD, and subchondral osteopenia that appeared linked to a novel pathogenic ACAN variant. A multi-disciplinary approach including medical (bisphosphonate) therapy, surgical intervention and rehabilitation were successful in restoring wellness and physical function.

10.
Osteoporos Int ; 34(1): 147-160, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-36342539

RESUMO

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.


Assuntos
Fraturas Ósseas , Distrofia Muscular de Duchenne , Osteoporose , Fraturas da Coluna Vertebral , Masculino , Adolescente , Humanos , Pré-Escolar , Criança , Adulto Jovem , Adulto , Glucocorticoides/efeitos adversos , Distrofia Muscular de Duchenne/complicações , Distrofia Muscular de Duchenne/tratamento farmacológico , Estudos Transversais , Fraturas da Coluna Vertebral/etiologia , Fraturas da Coluna Vertebral/complicações , Fraturas Ósseas/complicações , Osteoporose/etiologia , Osteoporose/induzido quimicamente , Densidade Óssea , Fatores de Risco , Vértebras Lombares
11.
J Bone Miner Res ; 36(12): 2290-2299, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34610647

RESUMO

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 (ß = -0.70) and age (ß = -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).


Assuntos
Osteoporose , Leucemia-Linfoma Linfoblástico de Células Precursoras , Absorciometria de Fóton , Densidade Óssea , Canadá , Criança , Humanos , Vértebras Lombares/diagnóstico por imagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiologia
12.
J Clin Endocrinol Metab ; 106(12): e5195-e5207, 2021 11 19.
Artigo em Inglês | MEDLINE | ID: mdl-34232311

RESUMO

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.


Assuntos
Densidade Óssea , Glucocorticoides/efeitos adversos , Osteoporose/epidemiologia , Fraturas por Osteoporose/epidemiologia , Doenças Reumáticas/tratamento farmacológico , Fraturas da Coluna Vertebral/epidemiologia , Corpo Vertebral/fisiopatologia , Adolescente , Canadá/epidemiologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Incidência , Lactente , Recém-Nascido , Estudos Longitudinais , Masculino , Osteoporose/induzido quimicamente , Osteoporose/patologia , Fraturas por Osteoporose/induzido quimicamente , Fraturas por Osteoporose/patologia , Prognóstico , Estudos Prospectivos , Doenças Reumáticas/patologia , Fatores de Risco , Fraturas da Coluna Vertebral/induzido quimicamente , Fraturas da Coluna Vertebral/patologia
13.
J Bone Miner Res ; 36(7): 1255-1268, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33784410

RESUMO

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).


Assuntos
Fraturas da Coluna Vertebral , Absorciometria de Fóton , Dor nas Costas , Densidade Óssea , Criança , Humanos , Vértebras Lombares/diagnóstico por imagem , Fraturas da Coluna Vertebral/diagnóstico por imagem , Fraturas da Coluna Vertebral/epidemiologia
14.
Childs Nerv Syst ; 37(4): 1229-1236, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33404726

RESUMO

PURPOSE: Tumors affecting peripheral nerves in children are rare. Accurate diagnosis ensures that management is appropriate and timely. A review of intrinsic nerve tumors was completed to differentiate common peripheral nerve lesions based on clinical characteristics and investigations. METHODS: A retrospective review was conducted for children (< 18 years old) diagnosed with an intrinsic tumor affecting peripheral nerve(s) or roots at the Children's Hospital of Eastern Ontario (CHEO) from 2009 to 2019. RESULTS: We report 14 children with perineurioma (N = 6), neurofibroma (N = 4), intraneural ganglion cyst (N = 2), or lipomatosis (N = 2). Mean age of symptom onset was 8.2 years (range 0.3 to 17.3 years). Presenting symptoms included muscle weakness (7/14), painless muscle wasting (2/14), contracture (1/14), pain (1/14), or the identification of a painless mass (3/14). Nerve conduction studies (NCS) or electromyography (EMG) were performed in 11/14 patients. MRI was useful at differentiating between these pediatric nerve tumors. Biopsies were performed in nine patients with additional surgical management pursued in four patients. CONCLUSION: The rare nature of peripheral nerve tumors in children can pose diagnostic challenges. NCS/EMG are important to assist with localization, and MRI is useful to distinguish more benign tumors. Key MRI, clinical, and NCS features can in some cases guide management, potentially avoiding the need for invasive procedures.


Assuntos
Neoplasias de Bainha Neural , Neoplasias do Sistema Nervoso Periférico , Adolescente , Criança , Pré-Escolar , Hospitais Pediátricos , Humanos , Lactente , Imageamento por Ressonância Magnética , Neoplasias de Bainha Neural/diagnóstico por imagem , Nervos Periféricos , Neoplasias do Sistema Nervoso Periférico/diagnóstico por imagem , Estudos Retrospectivos , Atenção Terciária à Saúde
15.
Can Assoc Radiol J ; 71(4): 505-513, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32054306

RESUMO

Infantile hemangiomas are the most frequent vascular soft tissue lumps in the pediatric population. The clinical presentation and evolution of these lesions is characteristic, while the sonographic appearance is classic but not specific. This pictorial essay illustrates the different vascular soft tissue lumps on ultrasound that may mimic infantile hemangiomas. Awareness of these mimics is crucial to avoid misdiagnosis. Clinical and sonographic discriminators for each lesion are presented.


Assuntos
Hemangioma/diagnóstico por imagem , Neoplasias de Tecidos Moles/irrigação sanguínea , Neoplasias de Tecidos Moles/diagnóstico por imagem , Ultrassonografia/métodos , Criança , Diagnóstico Diferencial , Humanos
16.
J Bone Miner Res ; 35(3): 460-468, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31742768

RESUMO

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.


Assuntos
Fraturas da Coluna Vertebral , Absorciometria de Fóton , Dor nas Costas , Densidade Óssea , Criança , Humanos , Vértebras Lombares/diagnóstico por imagem , Fraturas da Coluna Vertebral/diagnóstico por imagem , Fraturas da Coluna Vertebral/epidemiologia
17.
J Magn Reson Imaging ; 49(7): e241-e249, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30609174

RESUMO

BACKGROUND: Noncontrast MRI has been shown to be feasible in children with postappendectomy abscesses and helps guide clinical management, but its role in preoperative appendiceal abscesses is unclear. PURPOSE: To determine the cost-effectiveness and impact on clinical management of noncontrast MRI in pediatric patients with suspected appendiceal abscess, both pre- and postappendectomy. STUDY TYPE: Retrospective cohort study. POPULATION: In all, 82 children under the age of 18 years with suspected appendiceal abscess on ultrasound. FIELD STRENGTH/SEQUENCE: Diffusion-weighted imaging and T2 -weighted single-shot fast spin-echo imaging of the abdomen and pelvis at 1.5T and 3T. ASSESSMENT: The presence, location, size, and apparent diffusion coefficient (ADC) of fluid collections and the presence of a drainage path was noted by three pediatric radiologists. Imaging time, completeness of the exam, and impact on clinical management was recorded. The incremental cost-effectiveness ratio was calculated for MRI relative to CT, taking into account hospital charges, radiation exposure, and risk of adverse reaction to iodinated contrast. STATISTICAL TESTS: Descriptive statistics were used. Intraclass correlation coefficient and Fleiss' kappa were used to assess interobserver variation. Proportions were compared using Fisher's exact test (statistical significance at P < 0.05). RESULTS: MRI confirmed the presence of collections in most cases, with alternative diagnosis established in 10 patients (Tubo-ovarian abscess n = 7, Crohn's disease, ileal anastomotic leak, and Birkitts lymphoma each n = 1). MRI showed the presence of a safe drainage pathway in 92-97% of pelvic abscesses and 86-98% of abdominal abscesses compared with 7-10% and 75-81%, respectively, for ultrasound. MR was cost-effective compared with CT, taking into account the direct charges, risk of radiation induced cancer, and adverse reaction to iodinated contrast. DATA CONCLUSION: Noncontrast MR is cost-effective and affects clinical management in a significant proportion of children with suspected appendiceal abscesses. LEVEL OF EVIDENCE: 5 Technical Efficacy: Stage 6 J. Magn. Reson. Imaging 2019.


Assuntos
Abscesso/diagnóstico por imagem , Apêndice/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Adolescente , Apendicectomia , Apendicite/diagnóstico por imagem , Apendicite/cirurgia , Criança , Pré-Escolar , Meios de Contraste , Análise Custo-Benefício , Diagnóstico por Computador , Feminino , Humanos , Masculino , Variações Dependentes do Observador , Período Pré-Operatório , Estudos Retrospectivos
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