Liver Assessment in Patients with Ataxia-Telangiectasia: Transient Elastography Detects Early Stages of Steatosis and Fibrosis.

Background: Ataxia-telangiectasia (A-T) is a rare autosomal-recessive multisystem disorder characterized by pronounced cerebellar ataxia, telangiectasia, cancer predisposition, and altered body composition. Liver diseases with steatosis, fibrosis, and hepatocellular carcinoma are frequent findings in older patients but sensitive noninvasive diagnostic tools are lacking. Objectives: To determine the sensitivity of transient elastography (TE) as a screening tool for early hepatic tissue changes and serum biomarkers for liver disease. Methods: Thirty-one A-T patients aged 2 to 25 years were examined prospectively from 2016-2018 by TE. In addition, we evaluated the diagnostic performance of liver biomarkers for steatosis and necroinflammatory activity (SteatoTest and ActiTest, Biopredictive, Paris) compared to TE. For calculation and comparison, patients were divided into two groups (<12, >12 years of age). Results: TE revealed steatosis in 2/21 (10%) younger patients compared to 9/10 (90%) older patients. Fibrosis was present in 3/10 (30%) older patients as assessed by TE. We found a significant correlation of steatosis with SteatoTest, alpha-fetoprotein (AFP), HbA1c, and triglycerides. Liver stiffness correlated significantly with SteatoTest, ActiTest, HbA1c, and triglycerides. Conclusion: Liver disease is a common finding in older A-T patients. TE is an objective measure to detect early stages of steatosis and fibrosis. SteatoTest and ActiTest are a good diagnostic assessment for steatosis and necroinflammatory activity in patients with A-T and confirmed the TE results.

Assessment of gunshot bullet injuries with the use of magnetic resonance imaging.

BACKGROUND: Magnetic resonance imaging (MRI) is rarely used for preoperative assessment of shotgun injuries because of concerns of displacing the possibly ferromagnetic foreign body within the surrounding tissue. METHODS: A total of 56 different projectiles underwent MRI testing for ferromagnetism and imaging quality in vitro and in pig carcasses with a commercially available 1.5-MRI scan. Image quality was compared with that of computed tomographic scans. RESULTS: Projectiles with ferromagnetic properties can be distinguished easily from nonferromagnetic ones by pretesting the motion of an identical projectile within the MRI coil. When ferromagnetic projectiles were excluded, MRI yielded the more precise images compared with other imaging techniques. Projectile localization and associated soft tissue injuries were visualized without artifacts in all cases. CONCLUSIONS: When ferromagnetic foreign bodies are excluded by pretesting their properties within the MRI with a comparative projectile, MRI portends an excellent imaging procedure for assessing the extent of injury and planning the removal by surgery.

Transformations for within-subject designs: a Monte Carlo investigation.

We explored the use of transformations to improve power in within-subject designs in which multiple observations are collected for each S in each condition, such as reaction time and psychophysiological experiments. Often, the multiple measures within a treatment are simply averaged to yield a single number, but other transformations have been proposed. Monte Carlo simulations were used to investigate the influence of those transformations on the probabilities of Type I and Type II errors. With normally distributed data, Z and range correction transformations led to substantial increases in power over simple averages. With highly skewed distributions, the optimal transformation depended on several variables, but Z and range correction performed well across conditions. Correction for outliers was useful in increasing power, and trimming was more effective than eliminating all points beyond a criterion.