Studies on Motor Activity Log-28 and Actual Amount of Use Test, Actual Amount of Use Test Inter-rater Reliability in Healthy Individuals: Age Dependence and Handedness

2019 ◽  
Vol 26 (2) ◽  
pp. 24-33
Author(s):  
Kyeong-hyeon Kim ◽  
Yu-mi Shin ◽  
Mi-yu Lim ◽  
Yu-chang Jung ◽  
Ji-eun Oh ◽  
...  
2012 ◽  
Vol 26 (5) ◽  
pp. 507-514 ◽  
Author(s):  
Shuya Chen ◽  
Steven L. Wolf ◽  
Qin Zhang ◽  
Paul A. Thompson ◽  
Carolee J. Winstein

PEDIATRICS ◽  
1976 ◽  
Vol 58 (2) ◽  
pp. 303-303
Author(s):  
N. Matsaniotis

In their interesting article, Dr. Fleisher and his colleagues1 stated that "data on T lymphocytes in young children have not been previously reported." We would like to point out that we have already reported in the British Medical Journal2 data on age dependence of T cells. We studied 171 healthy individuals of different ages; 50 of them were younger than 2 years. E rosettes were very low during the first month of life, both in percentages and absolute numbers.


2021 ◽  
Vol 13 (1) ◽  
Author(s):  
Yosuke Takahashi ◽  
Yuji Fujino ◽  
Kohei Miura ◽  
Ayumi Toida ◽  
Tadamitsu Matsuda ◽  
...  

Abstract Background Ultrasonography (US) is a feasible and accessible method for the measurement of skeletal muscle mass. This technique presents acceptable intra-rater reliability; however, there are a few reports on its inter-rater reliability. Additionally, relative reliability should equally be inspected to determine the presence of systematic errors. Therefore, this study aimed to investigate the intra- and inter-rater reliabilities and absolute reliability of rectus femoris muscle thickness as measured using US. Methods The participants included in our study comprised 12 healthy young men (26.5 ± 3.9 years. Rectus femoris muscle thickness was measured from the right side of the thigh using US by two trained physical therapists. Inter- and intra-rater reliabilities were determined using the intraclass correlation coefficient (ICC) (1, 1) and ICC (2, 1) methods, respectively. Absolute reliability was evaluated using Bland − Altman analysis. Additionally, we calculated the minimal detectable change at the 95% level of confidence (MDC95). Result According to the results of the Bland − Altman analysis, no fixed or proportional errors were present. The ICC (1, 1) was 0.95, and the ICC (2, 1) was 0.70. The MDC95 values of rectus femoris thickness for the intra- and inter-rater reliabilities were 2.0 mm and 4.3 mm, respectively. Conclusions In our study, intra- and inter-rater reliabilities were measured at “excellent” and “moderate” levels in the healthy individuals based on a previously defined scale. Moreover, we determined the measurement error for quantifying rectus femoris thickness. Therefore, the measurement of rectus femoris thickness using US could be considered applicable in clinical research.


2020 ◽  
Author(s):  
Eugen Mengel ◽  
Bruno Bembi ◽  
Mireia del Toro ◽  
Federica Deodato ◽  
Matthias Gautschi ◽  
...  

Abstract BackgroundNiemann–Pick disease type C (NPC) is a rare, progressive, neurodegenerative disease associated with neurovisceral manifestations resulting from lysosomal dysfunction and aberrant lipid accumulation. A multicentre, prospective observational study (ClinicalTrials.gov ID:NCT02435030) of individuals with genetically confirmed NPC1 or NPC2 receiving routine clinical care was conducted, to prospectively characterize and measure NPC disease progression and to investigate potential NPC-related biomarkers versus healthy individuals. Progression was measured using the abbreviated 5-domain NPC Clinical Severity Scale (NPCCSS), 17-domain NPCCSS and NPC clinical database (NPC-cdb) score. Cholesterol esterification and heat shock protein 70 (HSP70) levels were assessed from peripheral blood mononuclear cells (PBMCs), cholestane-3β,5α-,6β-triol (cholestane-triol) from serum, and unesterified cholesterol from both PBMCs and skin biopsy samples. The inter- and intra-rater reliability of the 5-domain NPCCSS was assessed by 13 expert clinicians’ rating of four participants via video recordings, repeated after ≥3 weeks. Intraclass correlation coefficients (ICCs) were calculated.ResultsOf the 36 individuals with NPC (2–18 years) enrolled, 31 (86.1%) completed the 6–14-month observation period; 30/36 (83.3%) were receiving miglustat as part of routine clinical care. A mean (±SD) increase in 5-domain NPCCSS scores of 1.4 (±2.9) was observed, corresponding to an annualized progression rate of 1.5. On the 17-domain NPCCSS, a mean (±SD) progression of 2.7 (±4.0) was reported. Compared with healthy individuals, the NPC population had significantly lower levels of cholesterol esterification (p<0.0001), HSP70 (p<0.0001) and skin unesterified cholesterol (p=0.0006). Cholestane-triol levels were significantly higher in individuals with NPC versus healthy individuals (p=0.008) and correlated with the 5-domain NPCCSS (Spearman’s correlation coefficient=0.265, p=0.0411). The 5-domain NPCCSS showed high ICC agreement in inter-rater reliability (ICC=0.995) and intra-rater reliability (ICC=0.937).ConclusionsProgression rates observed were consistent with other reports on disease progression in NPC. The 5-domain NPCCSS reliability study supports its use as an abbreviated alternative to the 17-domain NPCCSS that focuses on the most relevant domains of the disease. The data support the use of cholestane-triol as a disease monitoring biomarker and the novel methods of measuring unesterified cholesterol could be applicable to support NPC diagnosis. Levels of HSP70 in individuals with NPC were significantly decreased compared with healthy individuals.Trial Registration: CT-ORZY-NPC-001: ClincalTrials.gov NCT02435030, Registered 6 May 2015, https://clinicaltrials.gov/ct2/show/NCT02435030; EudraCT 2014-005194-37, Registered 28 April 2015, https://www.clinicaltrialsregister.eu/ctr-search/trial/2014-005194-37/DE. OR-REL-NPC-01: Unregistered.


2020 ◽  
Author(s):  
Eugen Mengel ◽  
Bruno Bembi ◽  
Mireia del Toro ◽  
Federica Deodato ◽  
Matthias Gautschi ◽  
...  

Abstract BackgroundNiemann–Pick disease type C (NPC) is a rare, progressive, neurodegenerative disease associated with neurovisceral manifestations resulting from lysosomal dysfunction and aberrant lipid accumulation. A multicentre, prospective observational study (ClinicalTrials.gov ID:NCT02435030) of individuals with genetically confirmed NPC1 or NPC2 receiving routine clinical care was conducted, to prospectively characterize and measure NPC disease progression and to investigate potential NPC-related biomarkers versus healthy individuals. Progression was measured using the abbreviated 5-domain NPC Clinical Severity Scale (NPCCSS), 17-domain NPCCSS and NPC clinical database (NPC-cdb) score. Cholesterol esterification and heat shock protein 70 (HSP70) levels were assessed from peripheral blood mononuclear cells (PBMCs), cholestane-3β,5α-,6β-triol (cholestane-triol) from serum, and unesterified cholesterol from both PBMCs and skin biopsy samples. The inter- and intra-rater reliability of the 5-domain NPCCSS was assessed by 13 expert clinicians’ rating of four participants via video recordings, repeated after ≥3 weeks. Intraclass correlation coefficients (ICCs) were calculated.ResultsOf the 36 individuals with NPC (2–18 years) enrolled, 31 (86.1%) completed the 6–14-month observation period; 30/36 (83.3%) were receiving miglustat as part of routine clinical care. A mean (±SD) increase in 5-domain NPCCSS scores of 1.4 (±2.9) was observed, corresponding to an annualized progression rate of 1.5. On the 17-domain NPCCSS, a mean (±SD) progression of 2.7 (±4.0) was reported. Compared with healthy individuals, the NPC population had significantly lower levels of cholesterol esterification (p<0.0001), HSP70 (p<0.0001) and skin unesterified cholesterol (p=0.0006). Cholestane-triol levels were significantly higher in individuals with NPC versus healthy individuals (p=0.008) and correlated with the 5-domain NPCCSS (Spearman’s correlation coefficient=0.265, p=0.0411). The 5-domain NPCCSS showed high ICC agreement in inter-rater reliability (ICC=0.995) and intra-rater reliability (ICC=0.937). ConclusionsProgression rates observed were consistent with other reports on disease progression in NPC. The 5-domain NPCCSS reliability study supports its use as an abbreviated alternative to the 17-domain NPCCSS that focuses on the most relevant domains of the disease. The data support the use of cholestane-triol as a disease monitoring biomarker and the novel methods of measuring unesterified cholesterol could be applicable to support NPC diagnosis. Levels of HSP70 in individuals with NPC were significantly decreased compared with healthy individuals.


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Eugen Mengel ◽  
Bruno Bembi ◽  
Mireia del Toro ◽  
Federica Deodato ◽  
Matthias Gautschi ◽  
...  

Abstract Background Niemann–Pick disease type C (NPC) is a rare, progressive, neurodegenerative disease associated with neurovisceral manifestations resulting from lysosomal dysfunction and aberrant lipid accumulation. A multicentre, prospective observational study (Clinical Trials.gov ID: NCT02435030) of individuals with genetically confirmed NPC1 or NPC2 receiving routine clinical care was conducted, to prospectively characterize and measure NPC disease progression and to investigate potential NPC-related biomarkers versus healthy individuals. Progression was measured using the abbreviated 5-domain NPC Clinical Severity Scale (NPCCSS), 17-domain NPCCSS and NPC clinical database (NPC-cdb) score. Cholesterol esterification and heat shock protein 70 (HSP70) levels were assessed from peripheral blood mononuclear cells (PBMCs), cholestane-3β,5α-,6β-triol (cholestane-triol) from serum, and unesterified cholesterol from both PBMCs and skin biopsy samples. The inter- and intra-rater reliability of the 5-domain NPCCSS was assessed by 13 expert clinicians’ rating of four participants via video recordings, repeated after ≥ 3 weeks. Intraclass correlation coefficients (ICCs) were calculated. Results Of the 36 individuals with NPC (2–18 years) enrolled, 31 (86.1%) completed the 6–14-month observation period; 30/36 (83.3%) were receiving miglustat as part of routine clinical care. A mean (± SD) increase in 5-domain NPCCSS scores of 1.4 (± 2.9) was observed, corresponding to an annualized progression rate of 1.5. On the 17-domain NPCCSS, a mean (± SD) progression of 2.7 (± 4.0) was reported. Compared with healthy individuals, the NPC population had significantly lower levels of cholesterol esterification (p < 0.0001), HSP70 (p < 0.0001) and skin unesterified cholesterol (p = 0.0006). Cholestane-triol levels were significantly higher in individuals with NPC versus healthy individuals (p = 0.008) and correlated with the 5-domain NPCCSS (Spearman’s correlation coefficient = 0.265, p = 0.0411). The 5-domain NPCCSS showed high ICC agreement in inter-rater reliability (ICC = 0.995) and intra-rater reliability (ICC = 0.937). Conclusions Progression rates observed were consistent with other reports on disease progression in NPC. The 5-domain NPCCSS reliability study supports its use as an abbreviated alternative to the 17-domain NPCCSS that focuses on the most relevant domains of the disease. The data support the use of cholestane-triol as a disease monitoring biomarker and the novel methods of measuring unesterified cholesterol could be applicable to support NPC diagnosis. Levels of HSP70 in individuals with NPC were significantly decreased compared with healthy individuals. Trial registration CT-ORZY-NPC-001: ClincalTrials.gov NCT02435030, Registered 6 May 2015, https://clinicaltrials.gov/ct2/show/NCT02435030; EudraCT 2014–005,194-37, Registered 28 April 2015, https://www.clinicaltrialsregister.eu/ctr-search/trial/2014-005194-37/DE. OR-REL-NPC-01: Unregistered.


2019 ◽  
Vol 5 ◽  
pp. 205520761988557
Author(s):  
Chelsea Zhang ◽  
Alexandra Talaber ◽  
Melanie Truong ◽  
Bert B Vargas

Background and objective Subjective grade-based scoring balance assessments tend to be lengthy and have demonstrated poor repeatability and reliability. This study examined the reliability of a mobile balance assessment tool and differences in balance measurements between individuals at risk for a balance deficit secondary to a diagnosed neurological or musculoskeletal condition and a control group of healthy individuals. Methods Objective balance testing was measured using K-D Balance on a compatible iPhone. Seventy-seven participants were enrolled (control group, n =  44; group at risk for balance deficits, n =  33). Mean and standard deviation of K-D Balance were recorded for each stance. Intra-rater reliability was calculated by repeating the trial. Results Overall balance scores were superior for the control group compared with the group at risk for balance deficits in double leg stance (mean (SD): 0.15 (0.12) versus 0.18 (0.13), p =  0.260), tandem stance right leg (mean (SD): 0.27 (0.17) versus 0.45 (0.49), p =  0.028), and tandem stance left leg (mean (SD): 0.26 (0.17) versus 0.35 (0.35), p =  0.136). Intra-rater reliability was good to excellent for K-D Balance double leg stance (intra-class correlation coefficient (ICC) = 0.80, 95% confidence interval (CI) 0.58–1.03), tandem stance right leg (ICC = 0.96, 95% CI 0.86–1.06) and tandem stance left leg (ICC = 0.98, 95% CI 0.95–1.0). Conclusions K-D Balance revealed differences in balance performance between healthy individuals compared with individuals with neurological or musculoskeletal impairment. Objective balance measures may improve the accuracy and reliability of clinical balance assessment by detecting subtle differences in balance and aid in early detection of diseases that impair balance.


Author(s):  
B. J. Panessa-Warren ◽  
J. B. Warren ◽  
H. W. Kraner

Our previous studies have demonstrated that abnormally high amounts of calcium (Ca) and zinc (Zn) can be accumulated in human retina-choroid under pathological conditions and that barium (Ba), which was not detected in the eyes of healthy individuals, is deposited in the retina pigment epithelium (RPE), and to a lesser extent in the sensory retina and iris. In an attempt to understand how these cations can be accumulated in the vertebrate eye, a morphological and microanalytical study of the uptake and loss of specific cations (K, Ca,Ba,Zn) was undertaken with incubated Rana catesbiana isolated retina and RPE preparations. Large frogs (650-800 gms) were dark adapted, guillotined and their eyes enucleated in deep ruby light. The eyes were hemisected behind the ora serrata and the anterior portion of the eye removed. The eyecup was bisected along the plane of the optic disc and the two segments of retina peeled away from the RPE and incubated.


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