OBJECTIVE: Evaluate clinical outcome of early cyclic intravenous pamidronate treatment in children with moderate-to-severe osteogenesis imperfecta (OI), commenced before three years of age.METHODS: A retrospective review of 17 patients with moderate-to-severe OI. Development, anthropometry, fracture history, bone mineral density (BMD) and biochemistry were collected at baseline, 12 and 24 months.RESULTS: Four had OI type I, eleven had type III, one OI-FKBP10 type and one OI type V. Mean age at start of pamidronate was 14 ± 11 months. Pamidronate ranged from 6 to 12 mg/kg/year. No adverse reaction apart from fever and vomiting was noted. Long bone fracture decreased from a mean of 10.4/year to 1.2/year after 12 months and 1.4/year after 24 months (p = 0.02). Lumbar spine age- and height-matched BMD Z-scores increased (p < 0.005). Sixteen with vertebral compression fractures at baseline all showed improved vertebral shape (p < 0.001). Concavity index, likewise, improved (p < 0.005). Motor milestones compared to historical data show earlier attainment in rolling over, crawling, pulling to stand and walking independently but not sitting.CONCLUSION: Cyclic intravenous pamidronate, started under 3 years of age in children with moderate-to-severe OI, was well tolerated and associated with an increase in lumbar spine BMD, reduced fracture frequency, vertebral remodelling and attainment of motor milestones at an earlier age.
BACKGROUND: Observation of movement quality (MQ) is an indelible element in the process of clinical reasoning for patients with non-specific low back pain (NS-LBP). However, the observation and evaluation of MQ in common daily activities are not standardized within allied health care. This study aims to describe how Dutch allied health care professionals (AHCPs) observe and assess MQ in patients with NS-LBP and whether AHCPs feel the need to have a specific outcome measure for assessing MQ in patients with NS-LBP.METHODS: In this cross-sectional digital survey study, Dutch primary care AHCPs (n = 114) answered one open and three closed questions about MQ in NS-LBP management. Qualitative and quantitative analyses were applied.RESULTS: Qualitative analyses of the answers to the open questions revealed four main themes: 1) movement pattern features, 2) motor control features, 3) environmental influences and 4) non-verbal expressions of pain and exertion. Quantitative analyses clearly indicated that AHCPs observe MQ in the diagnostic (92%), therapeutic (91%) and evaluation phases (86%), that they do not apply any objective measurement of MQ and that 63% of the AHCPs consider it important to have a specific outcome measure to assess MQ. The AHCPs expressed added benefits and critical notes regarding clinical reasoning and quality of care.CONCLUSION: AHCPs recognize the importance of observing MQ in the assessment and management of LBP in a standardized way. However, there is no consensus amongst AHCPs how MQ should be standardized. Prior to standardization, it will be important to develop a theoretical framework to determine which observable and measurable dimensions of MQ are most valid and relevant for patients with NS-LBP to include in the assessment.
Objectives: The aim of this study was to assess the predictive value of PMA measurement for mortality. Background: Current surgical risk stratification have limited predictive value in the transcatheter aortic valve implantation (TAVI) population. In TAVI workup, a CT scan is routinely performed but body composition is not analyzed. Psoas muscle area (PMA) reflects a patient's global muscle mass and accordingly PMA might serve as a quantifiable frailty measure. Methods: Multi-slice computed tomography scans (between 2010 and 2016) of 583 consecutive TAVI patients were reviewed. Patients were divided into equal sex-specific tertiles (low, mid, and high) according to an indexed PMA. Hazard ratios (HR) and their confidence intervals (CI) were determined for cardiac and all-cause mortality after TAVI. Results: Low iPMA was associated with cardiac and all-cause mortality in females. One-year adjusted cardiac mortality HR in females for mid-iPMA and high-iPMA were 0.14 [95%CI, 0.05–0.45] and 0.40 [95%CI, 0.15–0.97], respectively. Similar effects were observed for 30-day and 2-years cardiac and all-cause mortality. In females, adding iPMA to surgical risk scores improved the predictive value for 1-year mortality. C-statistics changed from 0.63 [CI = 0.54–0.73] to 0.67 [CI: 0.58–0.75] for EuroSCORE II and from 0.67 [CI: 0.59–0.77] to 0.72 [CI: 0.63–0.80] for STS-PROM. Conclusions: Particularly in females, low iPMA is independently associated with an higher all-cause and cardiac mortality. Prospective studies should confirm whether PMA or other body composition parameters should be extracted automatically from CT-scans to include in clinical decision making and outcome prediction for TAVI.