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  • 1
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    Online Resource
    Benzodiazepine administration patterns before escalation to second‐line medications in pediatric refractory convulsive status epilepticus
    Wiley ; 2021
    In:  Epilepsia Vol. 62, No. 11 ( 2021-11), p. 2766-2777
    Details
    In: Epilepsia, Wiley, Vol. 62, No. 11 ( 2021-11), p. 2766-2777
    Abstract: This study was undertaken to evaluate benzodiazepine (BZD) administration patterns before transitioning to non‐BZD antiseizure medication (ASM) in pediatric patients with refractory convulsive status epilepticus (rSE). Methods This retrospective multicenter study in the United States and Canada used prospectively collected observational data from children admitted with rSE between 2011 and 2020. Outcome variables were the number of BZDs given before the first non‐BZD ASM, and the number of BZDs administered after 30 and 45 min from seizure onset and before escalating to non‐BZD ASM. Results We included 293 patients with a median (interquartile range) age of 3.8 (1.3–9.3) years. Thirty‐six percent received more than two BZDs before escalating, and the later the treatment initiation was after seizure onset, the less likely patients were to receive multiple BZD doses before transitioning (incidence rate ratio [IRR] = .998, 95% confidence interval [CI] = .997–.999 per minute, p  = .01). Patients received BZDs beyond 30 and 45 min in 57.3% and 44.0% of cases, respectively. Patients with out‐of‐hospital seizure onset were more likely to receive more doses of BZDs beyond 30 min (IRR = 2.43, 95% CI = 1.73–3.46, p   〈  .0001) and beyond 45 min (IRR = 3.75, 95% CI = 2.40–6.03, p   〈  .0001) compared to patients with in‐hospital seizure onset. Intermittent SE was a risk factor for more BZDs administered beyond 45 min compared to continuous SE (IRR = 1.44, 95% CI = 1.01–2.06, p  = .04). Forty‐seven percent of patients ( n  = 94) with out‐of‐hospital onset did not receive treatment before hospital arrival. Among patients with out‐of‐hospital onset who received at least two BZDs before hospital arrival ( n  = 54), 48.1% received additional BZDs at hospital arrival. Significance Failure to escalate from BZDs to non‐BZD ASMs occurs mainly in out‐of‐hospital rSE onset. Delays in the implementation of medical guidelines may be reduced by initiating treatment before hospital arrival and facilitating a transition to non‐BZD ASMs after two BZD doses during handoffs between prehospital and in‐hospital settings.
    Type of Medium: Online Resource
    ISSN: 0013-9580 , 1528-1167
    URL: Issue
    URL: Article
    DOI: 10.1111/epi.v62.11
    DOI: 10.1111/epi.17043
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2002194-X
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  • 2
    Online Resource
    Online Resource
    Familial rhabdoid tumour ' avant la lettre '-from pathology review to exome sequencing and back again : Familial rhabdoid tumours masquerading as teratomas
    Wiley ; 2013
    In:  The Journal of Pathology Vol. 231, No. 1 ( 2013-09), p. 35-43
    Details
    In: The Journal of Pathology, Wiley, Vol. 231, No. 1 ( 2013-09), p. 35-43
    Type of Medium: Online Resource
    ISSN: 0022-3417
    URL: Article
    DOI: 10.1002/path.4225
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2013
    detail.hit.zdb_id: 1475280-3
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  • 3
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    Online Resource
    Design and implementation of electronic health record common data elements for pediatric epilepsy: Foundations for a learning health care system
    Wiley ; 2021
    In:  Epilepsia Vol. 62, No. 1 ( 2021-01), p. 198-216
    Details
    In: Epilepsia, Wiley, Vol. 62, No. 1 ( 2021-01), p. 198-216
    Abstract: Common data elements (CDEs) are standardized questions and answer choices that allow aggregation, analysis, and comparison of observations from multiple sources. Clinical CDEs are foundational for learning health care systems, a data‐driven approach to health care focused on continuous improvement of outcomes. We aimed to create clinical CDEs for pediatric epilepsy. Methods A multiple stakeholder group (clinicians, researchers, parents, caregivers, advocates, and electronic health record [EHR] vendors) developed clinical CDEs for routine care of children with epilepsy. Initial drafts drew from clinical epilepsy note templates, CDEs created for clinical research, items in existing registries, consensus documents and guidelines, quality metrics, and outcomes needed for demonstration projects. The CDEs were refined through discussion and field testing. We describe the development process, rationale for CDE selection, findings from piloting, and the CDEs themselves. We also describe early implementation, including experience with EHR systems and compatibility with the International League Against Epilepsy classification of seizure types. Results Common data elements were drafted in August 2017 and finalized in January 2020. Prioritized outcomes included seizure control, seizure freedom, American Academy of Neurology quality measures, presence of common comorbidities, and quality of life. The CDEs were piloted at 224 visits at 10 centers. The final CDEs included 36 questions in nine sections (number of questions): diagnosis (1), seizure frequency (9), quality of life (2), epilepsy history (6), etiology (8), comorbidities (2), treatment (2), process measures (5), and longitudinal history notes (1). Seizures are categorized as generalized tonic‐clonic (regardless of onset), motor, nonmotor, and epileptic spasms. Focality is collected as epilepsy type rather than seizure type. Seizure frequency is measured in nine levels (all used during piloting). The CDEs were implemented in three vendor systems. Early clinical adoption included 1294 encounters at one center. Significance We created, piloted, refined, finalized, and implemented a novel set of clinical CDEs for pediatric epilepsy.
    Type of Medium: Online Resource
    ISSN: 0013-9580 , 1528-1167
    URL: Issue
    URL: Article
    DOI: 10.1111/epi.v62.1
    DOI: 10.1111/epi.16733
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2021
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  • 4
    Online Resource
    Online Resource
    Performance of FRAX in Women with Breast Cancer Initiating Aromatase Inhibitor Therapy: A Registry‐Based Cohort Study
    Wiley ; 2019
    In:  Journal of Bone and Mineral Research Vol. 34, No. 8 ( 2019-08), p. 1428-1435
    Details
    In: Journal of Bone and Mineral Research, Wiley, Vol. 34, No. 8 ( 2019-08), p. 1428-1435
    Abstract: FRAX was developed to predict 10‐year probability of major osteoporotic fracture (MOF) and hip fracture in the general population. Aromatase inhibitors (AI) used in breast cancer induce loss in bone mineral density (BMD) and are reported to increase fracture risk. AI exposure is not a direct input to FRAX but is captured under “secondary osteoporosis”. To inform use of FRAX in women treated with AI, we used a population‐based registry for the Province of Manitoba, Canada, to identify women aged ≥40 years initiating AI for breast cancer with at least 12 months’ AI exposure ( n = 1775), women with breast cancer not receiving AI ( n = 1016), and women from the general population ( n = 34,205). Among AI users, fracture probability estimated without BMD (AI use coded as secondary osteoporosis) significantly overestimated risk (10‐year observed/predicted ratio 0.56, 95% confidence interval [CI] 0.45–0.68; 10‐year hip fracture observed/predicted ratio 0.33, 95% CI 0.18–0.49). However, when BMD was included in the fracture probability, there was no significant difference between observed and predicted fracture risk. In Cox proportional hazards models, FRAX stratified risk of MOF, hip, and any fracture equally well in all subgroups ( p ‐interaction 〉 0.1). When adjusted for FRAX score without BMD, with AI use coded as secondary osteoporosis, AI users were at significantly lower risk for MOF (hazard ratio [HR] = 0.78, 95% CI 0.64–0.95), hip fracture (HR = 0.46, 95% CI 0.29–0.73) and any fracture (HR = 0.75, 95% CI 0.63–0.89). AI use was no longer significantly associated with fractures when AI use was not entered as secondary osteoporosis in FRAX without BMD or when BMD was included in the FRAX calculation. In conclusion, FRAX scores stratify fracture risk equally well in women receiving AI therapy as in non‐users, but including secondary osteoporosis as a risk factor for AI users overestimates fracture risk. Our results call this practice into question. © 2019 American Society for Bone and Mineral Research.
    Type of Medium: Online Resource
    ISSN: 0884-0431 , 1523-4681
    URL: Issue
    URL: Article
    DOI: 10.1002/jbmr.v34.8
    DOI: 10.1002/jbmr.3726
    RVK:
    XA 52230
    Language: English
    Publisher: Wiley
    Publication Date: 2019
    detail.hit.zdb_id: 2008867-X
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  • 5
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    Online Resource
    The Effect of Fracture Recency on Observed 10‐Year Fracture Probability: A Registry‐Based Cohort Study
    Wiley ; 2022
    In:  Journal of Bone and Mineral Research Vol. 37, No. 5 ( 2022-05), p. 848-855
    Details
    In: Journal of Bone and Mineral Research, Wiley, Vol. 37, No. 5 ( 2022-05), p. 848-855
    Abstract: FRAX estimates 10‐year fracture major osteoporotic fracture (MOF) and hip fracture probability from multiple risk factors. FRAX does not consider prior fracture site or time since fracture. Fracture risk is greater in the initial 2‐year post‐fracture period (imminent risk), implying that FRAX may underestimate risk in this setting. We used the population‐based Manitoba Bone Mineral Density (BMD) Program registry to examine the effect of fracture recency and site on incident fracture risk predictions using FRAX. We identified women aged 40 years or older with baseline BMD and FRAX scores. Observed fracture outcomes to 10 years were compared with predicted 10‐year fracture probability stratified by prior fracture status: none, recent ( 〈 2 years [median 0.3 years]), and remote (≥2 years [median 10.6 years] ). For women with recent fractures, we also examined proposed multipliers to adjust FRAX for the effect of fracture recency and site. The cohort comprised 33,465 women aged 40 to 64 years (1897 recent fracture, 2120 remote fracture) and 33,806 women aged ≥65 years (2365 fracture, 4135 remote fracture). Observed fracture probability was consistent with predicted probability in most analyses. In women aged 40 to 64 years, there was a significant effect of recent vertebral and humerus fracture on MOF (observed to predicted 1.61 and 1.48, respectively), but these effects were still lower than the proposed multipliers (2.32 and 1.67, respectively). No significant effect of fracture recency was found after hip or forearm fracture in either age group. Our findings contribute to accumulating evidence of the importance of recent fracture. The effect of fracture recency was not consistent across fracture sites and with a lower magnitude than previously reported. Further quantification of effect size and specificity in additional independent cohorts is warranted to validate and refine recent‐fracture multipliers in fracture risk assessment. © 2022 American Society for Bone and Mineral Research (ASBMR).
    Type of Medium: Online Resource
    ISSN: 0884-0431 , 1523-4681
    URL: Issue
    URL: Article
    DOI: 10.1002/jbmr.v37.5
    DOI: 10.1002/jbmr.4526
    RVK:
    XA 52230
    Language: English
    Publisher: Wiley
    Publication Date: 2022
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  • 6
    Online Resource
    Online Resource
    Performance of FRAX in Men With Prostate Cancer: A Registry‐Based Cohort Study
    Wiley ; 2023
    In:  Journal of Bone and Mineral Research Vol. 38, No. 5 ( 2023-05), p. 659-664
    Details
    In: Journal of Bone and Mineral Research, Wiley, Vol. 38, No. 5 ( 2023-05), p. 659-664
    Abstract: The Fracture Risk Assessment Tool (FRAX®) was created to predict major osteoporotic fractures (MOF) and hip fractures in the general population. Whether FRAX accurately predicts fractures in men with prostate cancer is unknown. Our objective was to assess the performance of FRAX for predicting incident fractures in men with prostate cancer. Men from the Manitoba Bone Mineral Density (BMD) Registry (1996–2018) with prostate cancer diagnoses in the 3 years prior to dual‐energy X‐ray absorptiometry (DXA) were identified. FRAX scores with and without BMD were calculated. From population‐based healthcare data we identified incident MOF, hip fracture, any osteoporotic fracture and death from the date of BMD testing to March 31, 2018. Cox regression was performed to estimate hazard ratios (HRs) with 95% confidence intervals (95% CIs) per standard deviation increase in FRAX score. Observed 10‐year probability (estimated with competing risk of mortality) was compared with 10‐year FRAX‐predicted fracture probability to assess calibration. The study population included 684 men with prostate cancer (mean age 74.6 years) and 8608 men without prostate cancer (mean age 65.5 years). FRAX stratified risk for MOF (HR 1.91, 95% CI 1.48–2.45 with BMD; HR 1.96, 95% CI 1.43–2.69 without BMD) and hip fracture (HR 3.37, 95% CI 1.90–6.01 with BMD; HR 4.58, 95% CI 2.17–9.67 without BMD) in men with prostate cancer. There was no effect modification observed with prostate cancer status or current androgen deprivation therapy. Observed 10‐year fracture probability in men with prostate cancer showed good agreement with FRAX with and without BMD included in the calculation (observed/predicted calibration ratios MOF 0.97, hip 1.00 with BMD; MOF 0.92, hip 0.93 with BMD). In conclusion, FRAX reliably predicts incident fractures in men with prostate cancer. © 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).
    Type of Medium: Online Resource
    ISSN: 0884-0431 , 1523-4681
    URL: Issue
    URL: Article
    DOI: 10.1002/jbmr.v38.5
    DOI: 10.1002/jbmr.4793
    RVK:
    XA 52230
    Language: English
    Publisher: Wiley
    Publication Date: 2023
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  • 7
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    Online Resource
    Intervention Thresholds and the Diagnosis of Osteoporosis
    Wiley ; 2015
    In:  Journal of Bone and Mineral Research Vol. 30, No. 10 ( 2015-10), p. 1747-1753
    Details
    In: Journal of Bone and Mineral Research, Wiley, Vol. 30, No. 10 ( 2015-10), p. 1747-1753
    Abstract: A position paper of the National Bone Health Alliance recently recommended that diagnostic criteria for osteoporosis be redefined. We review the merits and demerits of this proposal and argue that the operational bone mineral density (BMD)‐based definition be retained while clarity is brought to bear on the distinction between diagnostic and intervention thresholds. © 2015 American Society for Bone and Mineral Research.
    Type of Medium: Online Resource
    ISSN: 0884-0431 , 1523-4681
    URL: Issue
    URL: Article
    DOI: 10.1002/jbmr.v30.10
    DOI: 10.1002/jbmr.2531
    RVK:
    XA 52230
    Language: English
    Publisher: Wiley
    Publication Date: 2015
    detail.hit.zdb_id: 2008867-X
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  • 8
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    Online Resource
    A Meta‐Analysis of Trabecular Bone Score in Fracture Risk Prediction and Its Relationship to FRAX
    Wiley ; 2016
    In:  Journal of Bone and Mineral Research Vol. 31, No. 5 ( 2016-05), p. 940-948
    Details
    In: Journal of Bone and Mineral Research, Wiley, Vol. 31, No. 5 ( 2016-05), p. 940-948
    Abstract: Trabecular bone score (TBS) is a gray‐level textural index of bone microarchitecture derived from lumbar spine dual‐energy X‐ray absorptiometry (DXA) images. TBS is a bone mineral density (BMD)‐independent predictor of fracture risk. The objective of this meta‐analysis was to determine whether TBS predicted fracture risk independently of FRAX probability and to examine their combined performance by adjusting the FRAX probability for TBS. We utilized individual‐level data from 17,809 men and women in 14 prospective population‐based cohorts. Baseline evaluation included TBS and the FRAX risk variables, and outcomes during follow‐up (mean 6.7 years) comprised major osteoporotic fractures. The association between TBS, FRAX probabilities, and the risk of fracture was examined using an extension of the Poisson regression model in each cohort and for each sex and expressed as the gradient of risk (GR; hazard ratio per 1 SD change in risk variable in direction of increased risk). FRAX probabilities were adjusted for TBS using an adjustment factor derived from an independent cohort (the Manitoba Bone Density Cohort). Overall, the GR of TBS for major osteoporotic fracture was 1.44 (95% confidence interval [CI] 1.35–1.53) when adjusted for age and time since baseline and was similar in men and women ( p 〉 0.10). When additionally adjusted for FRAX 10‐year probability of major osteoporotic fracture, TBS remained a significant, independent predictor for fracture (GR = 1.32, 95% CI 1.24–1.41). The adjustment of FRAX probability for TBS resulted in a small increase in the GR (1.76, 95% CI 1.65–1.87 versus 1.70, 95% CI 1.60–1.81). A smaller change in GR for hip fracture was observed (FRAX hip fracture probability GR 2.25 vs. 2.22). TBS is a significant predictor of fracture risk independently of FRAX. The findings support the use of TBS as a potential adjustment for FRAX probability, though the impact of the adjustment remains to be determined in the context of clinical assessment guidelines. © 2015 American Society for Bone and Mineral Research.
    Type of Medium: Online Resource
    ISSN: 0884-0431 , 1523-4681
    URL: Issue
    URL: Article
    DOI: 10.1002/jbmr.v31.5
    DOI: 10.1002/jbmr.2734
    RVK:
    XA 52230
    Language: English
    Publisher: Wiley
    Publication Date: 2016
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  • 9
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    Online Resource
    Effect of BMI‐Discordant Abdominal Tissue Thickness on Fracture Probability: A Registry‐Based Study
    Wiley ; 2023
    In:  Journal of Bone and Mineral Research
    Details
    In: Journal of Bone and Mineral Research, Wiley
    Abstract: FRAX®, which is used to assess fracture probability, considers body mass index (BMI) but BMI may not reflect individual variation in body composition and distribution. We examined the effect of BMI‐discordant abdominal thickness on FRAX‐derived fracture probability for major osteoporotic fracture (MOF) and hip fracture. We studied 73,105 individuals, mean age 64.2 years. During mean 8.7 years, 7048 (9.6%) individuals sustained incident MOF, including 2155 (3.0%) hip fractures. We defined abdominal thickness index (ATI) as the difference between abdominal thickness measured by DXA and thickness predicted by BMI using sex‐stratified regression. ATI was categorized from lower ( 〈 −2 cm, −2 to −1 cm) to higher (1 to 2 cm, 〉 +2 cm) with referent around zero (−1 to +1 cm). Adjusted for FRAX probability, increasing ATI was associated with incident MOF and hip fracture (p  〈  0.001). For the highest ATI category, MOF risk was increased (HR 1.23, 95% CI 1.12–1.35) independent of FRAX probability. Similar findings were noted for hip fracture probability (HR 1.28, 95% CI 1.09–1.51). There was significant age‐interaction with much larger effects prior to age 65 years (HR 1.44, 95% CI 1.23–1.69 for MOF; 2.29, 95% CI 1.65–3.18 for hip fracture). In contrast, for the subset of individuals with diabetes there was also increased risk for those in the lowest ATI category (HR 1.73, 95% CI 1.12–2.65 for MOF, 2.81, 95% CI 1.59–4.97 for hip fracture). Calibration plots across ATI categories demonstrated deviation from the line of identity in women (calibration slope 2.26 for MOF, 2.83 for hip fracture). An effect of ATI was not seen in men, but this was inconclusive as the sex‐interaction terms did not show significant effect modification. In conclusion, these data support the need to investigate increased abdominal thickness beyond that predicted by BMI and sex as a FRAX‐independent risk factor for fracture. This article is protected by copyright. All rights reserved.
    Type of Medium: Online Resource
    ISSN: 0884-0431 , 1523-4681
    URL: Article
    DOI: 10.1002/jbmr.4919
    RVK:
    XA 52230
    Language: English
    Publisher: Wiley
    Publication Date: 2023
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  • 10
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    Online Resource
    Heterogeneity of quadriceps muscle phenotype in chronic obstructive pulmonary disease ( Copd ); implications for stratified medicine?
    Wiley ; 2013
    In:  Muscle & Nerve Vol. 48, No. 4 ( 2013-10), p. 488-497
    Details
    In: Muscle & Nerve, Wiley, Vol. 48, No. 4 ( 2013-10), p. 488-497
    Abstract: Quadriceps muscle dysfunction is common in COPD. Determining, and, if possible, predicting quadriceps phenotype in COPD is important for patient stratification for therapeutic trials. Methods In biopsies from 114 COPD patients and 30 controls, we measured fiber size and proportion and assessed the relationship with quadriceps function (strength and endurance), clinical phenotype (lung function, physical activity, fat‐free mass) and exercise performance. In a subset ( n  = 40) we measured muscle mid‐thigh cross‐sectional area by computed tomography. Results Normal ranges for fiber proportions and fiber cross‐sectional area were defined from controls; we found isolated fiber shift in 31% of patients, isolated fiber (predominantly type II) atrophy in 20%, both shift and atrophy in 25%, and normal fiber parameters in 24%. Clinical parameters related poorly to muscle biopsy appearances. Conclusions Quadriceps morphology is heterogeneous in COPD and cannot be predicted without biopsy, underlining the need for biomarkers. Muscle Nerve 48 : 488–497, 2013
    Type of Medium: Online Resource
    ISSN: 0148-639X , 1097-4598
    URL: Issue
    URL: Article
    DOI: 10.1002/mus.v48.4
    DOI: 10.1002/mus.23784
    RVK:
    XA 10000
    Language: English
    Publisher: Wiley
    Publication Date: 2013
    detail.hit.zdb_id: 1476641-3
    SSG: 12
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