Reliability and Validity of a Clinical Assessment Tool for Measuring Scapular Motion in All 3 Anatomical Planes

ContextA single clinical assessment device that can be used to objectively measure scapular motion in each anatomical plane is not currently available. The development of a novel electric goniometer would allow scapular motion in all 3 anatomical planes to be quantified.ObjectiveTo investigate the reliability and validity of an electric goniometer for measuring scapular motion in each anatomical plane during upper extremity elevation.DesignCross-sectional study.SettingLaboratory.Patients or Other ParticipantsSixty participants (29 women, 31 men; age = 30 ± 14 years, height = 1.73 ± 0.10 m, mass = 75.32 ± 16.90 kg) recruited from the general population.Intervention(s)An electric goniometer was used to record clinical measurements of scapular position at rest and total arc of motion (excursion) during active upper extremity elevation in 2 testing sessions separated by several days. Measurements were recorded independently by 2 examiners. In 1 session, scapular motion was recorded simultaneously using a 14-camera, 3-dimensional optical motion-capture system.Main Outcome Measure(s)Reliability analysis included examination of clinical measurements for scapular position at rest and excursion during each condition. Both the intrarater reliability between testing sessions and the interrater reliability recorded in the same session were assessed using intraclass correlation coefficients (ICCs [2,3]). The criterion validity was examined by comparing the mean excursion values of each condition recorded using the electric goniometer and the 3-dimensional optical motion-capture system. Validity was assessed by evaluating the average difference and root mean square error.ResultsThe between-sessions intrarater reliability was moderate to good (ICC [2,3] range = 0.628–0.874). The within-session interrater reliability was moderate to excellent (ICC [2,3] range = 0.545–0.912). The average difference between total excursion values recorded using the electric goniometer and the 3-dimensional optical motion-capture system ranged from −7° to 4°, and the root mean square error ranged from 7° to 10°.ConclusionsThe reliability of scapular measurements was best when a standard operating procedure was used. The electric goniometer provided an accurate measurement of scapular excursions in all 3 anatomical planes during upper extremity elevation.     

Longitudinal MRI brain volume changes over one year in children with mucopolysaccharidosis types IIIA and IIIB

ObjectiveTo quantify changes in segmented brain volumes over 12 months in children with mucopolysaccharidosis types IIIA and IIIB (MPS IIIA and IIIB).MethodsIn order to establish suitable outcome measures for clinical trials, twenty-five children greater than 2 years of age were enrolled in a prospective natural history study of MPS IIIA and IIIB at Nationwide Children's Hospital. Data from sedated non-contrast brain 3 T MRIs and neuropsychological measures were reviewed from the baseline visit and at 12-month follow-up. No intervention beyond standard clinical care was provided. Age- and sex-matched controls were gathered from the National Institute of Mental Health Data Archive. Automated brain volume segmentation with longitudinal processing was performed using FreeSurfer.ResultsOf the 25 subjects enrolled with MPS III, 17 children (4 females, 13 males) completed at least one MRI with interpretable volumetric data. The ages ranged from 2.8 to 13.7 years old (average 7.2 years old) at enrollment, including 8 with MPS IIIA and 9 with MPS IIIB. At baseline, individuals with MPS III demonstrated reduced cerebral white matter and corpus callosum volumes, but greater volumes of the lateral ventricles, cerebellar cortex, and cerebellar white matter compared to controls. Among the 13 individuals with MPS III with two interpretable MRIs, there were annualized losses or plateaus in supratentorial brain tissue volumes (cerebral cortex ?42.10 ± 18.52 cm³/year [mean ± SD], cerebral white matter ?4.37 ± 11.82 cm³/year, subcortical gray matter ?6.54 ± 3.63 cm³/year, corpus callosum ?0.18 ± 0.62 cm³/yr) and in cerebellar cortex (?0.49 ± 12.57 cm³/year), with a compensatory increase in lateral ventricular volume (7.17 ± 6.79 cm³/year). Reductions in the cerebral cortex and subcortical gray matter were more striking in individuals younger than 8 years of age. Greater cerebral cortex volume was associated with higher fine and gross motor functioning on the Mullen Scales of Early Learning, while greater subcortical gray matter volume was associated with higher nonverbal functioning on the Leiter International Performance Scale. Larger cerebellar cortex was associated with higher receptive language performance on the Mullen, but greater cerebellar white matter correlated with worse adaptive functioning on the Vineland Adaptive Behavioral Scales and visual problem-solving on the Mullen.ConclusionsLoss or plateauing of supratentorial brain tissue volumes may serve as longitudinal biomarkers of MPS III age-related disease progression compared to age-related growth in typically developing controls. Abnormally increased cerebellar white matter in MPS III, and its association with worse performance on neuropsychological measures, suggest the possibility of pathophysiological mechanisms distinct from neurodegeneration-associated atrophy that warrant further investigation.     

Validation of forensic facial comparison by morphological analysis in photographic and CCTV samples

International Journal of Legal Medicine - Between the ever-increasing availability of surveillance evidence and expert-based forensic facial comparison being considered admissible in court,...     

Meta-analysis of telomere length in 19?713 subjects reveals high heritability,stronger maternal inheritance and a paternal age effect

Telomere length (TL) has been associated with aging and mortality, but individual differences are also influenced by genetic factors, with previous studies reporting heritability estimates ranging from 34 to 82%. Here we investigate the heritability, mode of inheritance and the influence of parental age at birth on TL in six large, independent cohort studies with a total of 19 713 participants. The meta-analysis estimate of TL heritability was 0.70 (95% CI 0.64–0.76) and is based on a pattern of results that is highly similar for twins and other family members. We observed a stronger mother–offspring (r=0.42; P-value=3.60 × 10⁻⁶¹) than father–offspring correlation (r=0.33; P-value=7.01 × 10⁻⁵), and a significant positive association with paternal age at offspring birth (β=0.005; P-value=7.01 × 10⁻⁵). Interestingly, a significant and quite substantial correlation in TL between spouses (r=0.25; P-value=2.82 × 10⁻³⁰) was seen, which appeared stronger in older spouse pairs (mean age ≥55 years; r=0.31; P-value=4.27 × 10⁻²³) than in younger pairs (mean age<55 years; r=0.20; P-value=3.24 × 10⁻¹⁰). In summary, we find a high and very consistent heritability estimate for TL, evidence for a maternal inheritance component and a positive association with paternal age.     

The oral health status and treatment needs of children with fetal alcohol spectrum disorder

Clinical Oral Investigations - To investigate the oral health status and treatment needs of children with fetal alcohol spectrum disorder (FASD). In this retrospective study, the records of...     

The BAFF Receptor Transduces Survival Signals by Co-opting the B Cell Receptor Signaling Pathway

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Highlights? Inducible loss of the Syk tyrosine kinase results in death of follicular B cells ? Syk transduces survival signals from BAFFR to the ERK and PI3 kinase-PDK1 pathways ? BAFFR signaling results in phosphorylation of Igα and Syk ? BAFFR transduces signals via the BCR to activation of Syk