A Measurement-Based Protocol Improves Interrater Agreement and Accuracy of Right Ventricular Systolic Pressure Assessment by Echocardiography in Children: A Call for Quality Improvement.

BACKGROUND: Echocardiographic measurements carry the promise of improving interrater (IR) agreement over subjective assessment. The aim of this study was to assess the effect of implementing a measurement-based protocol on IR agreement and accuracy in reporting of right ventricular (RV) systolic pressure in children. The effect of this reporting protocol on IR agreement in reporting RV dilation, hypertrophy, and systolic function was also evaluated. METHODS: Five echocardiography readers reported their assessment of RV systolic pressure, dilation, hypertrophy, and systolic function on 40 deidentified echocardiograms using their individual accustomed methods and then using an agreed-upon protocol based solely on RV measurements. IR agreement was assessed using κ statistics. Accuracy of RV systolic pressure ratings was assessed using the McNemar test in comparison with hemodynamic data obtained by cardiac catheterization. The reliability of the RV measurements was assessed using intraclass correlation coefficient (ICC) and coefficient of variation. RESULTS: IR agreement and accuracy of RV systolic pressure assessment improved after using the measurement-based protocol (agreement from 0.39 [95% CI, 0.27-0.5] to 0.62 [95% CI, 0.48-0.76] and accuracy from 18 of 40 to 29 of 40 [P = .03]). IR agreement of RV dilation improved (from 0.36 [95% CI, 0.25-0.48] to 0.63 [95% CI, 0.48-0.79]), while IR agreement of RV hypertrophy (from 0.29 [95% CI, 0.17-0.42] to 0.35 [95% CI, 0.15-0.55]) and RV systolic function (from 0.57 [95% CI, 0.45-0.69] to 0.53 [95% CI, 0.41-0.66]) did not improve. The reliability of the measurements was good (ICC > 0.8), except for RV free wall thickness (ICC = 0.62, coefficient of variation = 24%) and RV fractional area change (ICC = 0.47, coefficient of variation = 22%), suggesting a possible reason for the lack of improvement in IR agreement of RV hypertrophy and RV systolic function. Heteroscedasticity was observed in the reliability of RV measurements, with the ICC being significantly lower at larger magnitudes for all RV measurements. CONCLUSIONS: Standardization of reporting protocols using RV measurements in place of subjective assessment improved IR agreement and accuracy of RV systolic pressure assessment. Reliable measurements (RV systolic pressure and dilation) resulted in improvement in IR agreement while unreliable measurements (RV hypertrophy and systolic function) did not. Special attention to measurements' reliability and heteroscedasticity of reliability is required when designing clinical protocols to decrease IR disagreement as a source of error.

Neuropsychological assessment as an objective tool to monitor treatment response in anti-N-methyl-D-aspartate receptor encephalitis.

We report a 1-year follow-up of a young woman with anti-N-methyl-D-aspartate receptor encephalitis. Management of autoimmune encephalitis remains challenging as objective and clinically relevant biomarkers are sought, which allow for the monitoring of treatment response. While further investigation is required, we believe that this case highlights the importance of following a comprehensive neuropsychological profile as a clinically relevant biomarker to guide therapeutic decision-making. By relying on the neuropsychological assessment of the patient, treatment with more toxic medications was avoided and her antiepileptic drug regimen was simplified.

Printing silicone-based hydrophobic barriers on paper for microfluidic assays using low-cost ink jet printers.

Paper-based microfluidic devices exhibit many advantages for biological assays. Normally, the assays are restricted to certain areas of the paper by hydrophobic barriers comprised of wax or alkyl ketene dimers (AKD). Neither hydrophobic barrier is able to constrain aqueous solutions of surfactants, which are frequently used in biological assays. We demonstrate that rapidly curing silicone resins can be inkjet printed onto pure cellulose paper using inexpensive thermal ink-jet printers. The Piers-Rubinsztajn (PR) reaction dominates the cure chemistry leading to cellulose fibers that are surface coated with a silicone resin. The resulting barriers are able to resist penetration by surfactant solutions and even by the lower surface energy solvents DMF and DMSO. The utility of the barrier was demonstrated using a coliform assay based on detection of ß-galactosidase.

Neuropsychological assessment in a case of adult-onset hemophagocytic lymphohistiocytosis (HLH).

We present a case of an individual diagnosed with hemophagocytic lymphohistiocytosis (HLH), an extremely rare and commonly fatal disorder characterized by rapid dysregulation of immune system processes. Typical age of onset is in childhood, with adult-onset occurring less frequently. The pathophysiology of this condition is characterized by a hyperinflammatory response with infiltration of visceral organs, lymph nodes, bone marrow, and the central nervous system. The clinical presentation has been documented in the extant medical literature. However, there appear to be no published reports of neuropsychological functioning in HLH patients. The patient we present here is a 28-year-old woman with 16 years of education who developed HLH subsequent to systemic lupus erythematosus flare-up. She was initially comatose for 3 weeks. Acute MRI reported multiple subcortical abnormalities, including the brainstem. The patient underwent chemotherapy, immunosuppresant, and steroid treatments. She underwent a neuropsychological evaluation at 2.5 and 7 months post initial presentation. Preliminary neuropsychological evaluation found impairments in motor abilities and aspects of executive functions. Subsequent evaluation showed improved executive function and relative sparing of higher-order cognitive abilities, but continued impairment on motor tests. To our knowledge this is the first study to report neuropsychological data for an adult diagnosed with HLH.

Reliability of two-dimensional echocardiography in the assessment of clinically significant abnormal hemidiaphragm motion in pediatric cardiothoracic patients: Comparison with fluoroscopy.

OBJECTIVE: To assess the utility and reliability of echocardiographic assessment of hemidiaphragm motion abnormalities in pediatric cardiothoracic patients. DESIGN: Retrospective observational study, with post hoc blinded assessment of echocardiographic and fluoroscopic results. SETTING: Tertiary care center. PATIENTS: Thirty-six consecutive pediatric cardiothoracic patients with suspected hemidiaphragm paralysis were identified and included in the study. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The results of both echocardiographic and fluoroscopic studies on all patients were included. In addition, blinded review of study results were performed. The sensitivity and specificity of fluoroscopy in identifying hemidiaphragms that needed plication were 100% and 74%, respectively. The positive predictive value was 55%; negative predictive value was 100%. Comparing reported diagnoses with blinded review of the studies showed poor agreement; reviewers agreed with 89% diagnosed as normal, 44% of paralyzed, and 76% of paradoxical hemidiaphragms. The sensitivity and specificity of echo in identifying hemidiaphragms that needed plication were 100% and 81%, respectively. The positive predictive value and negative predictive value were 66% and 100%. Comparing reported diagnoses with blinded review, reviewers agreed with 97% diagnosed as normal, 81% of paralyzed, and 100% of paradoxical hemidiaphragms. Echocardiography was less accurate in discriminating between paralyzed and paradoxical diaphragm motion. Echocardiography was specific for paradoxical motion, since both patients identified by echocardiography were confirmed by fluoroscopy, but it was not sensitive. In nine patients, echo showed paralyzed motion that was identified by fluoroscopy as paradoxical. CONCLUSIONS: This study supports the use of echocardiography in the assessment of diaphragm function. When the diaphragms are clearly visualized by echo, as they are in the majority of cases, the addition of an additional fluoroscopic study adds no clinical value. The differentiation between paralyzed and paradoxical motion is unreliable by both imaging modalities.