Publications by authors named "L C Hoffman"

Background: Elexacaftor/tezacaftor/ivacaftor (ETI) is a highly effective therapy that improves lung disease in people with cystic fibrosis (pwCF), but its effect on glucose tolerance and insulin secretion is unclear.

Methods: PROMISE is a multicenter prospective, observational study of ETI in pwCF ≥12 years and at least one F508del allele. The PROMISE Endocrine sub-study (PROMISE-ENDO) enrolled participants at 10 CF Centers where hemoglobin A1c (HbA1c) was collected and 3-hour oral glucose tolerance tests (OGTT) conducted to examine glucose tolerance, glucose excursions, insulin secretory rates (deconvolution of C-peptide) and sensitivity (oral minimal model) prior to ETI and 12-18 months (mos) and 24-30 mos following ETI initiation.

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Though it has no catalytic activity toward GM2 ganglioside, the GM2 activator protein (GM2A) is essential for ganglioside hydrolysis by facilitating the action of lysosomal ß-N-acetylhexosaminidase. GM2A deficiency results in death in early childhood due to rapid central nervous system deterioration similar to the related GM2 gangliosidoses, Tay-Sachs disease and Sandhoff disease. This manuscript further characterizes a feline model of GM2A deficiency with a focus on clinical and biochemical parameters that may be useful as benchmarks for translational therapeutic research.

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Metallic nanoparticle photocatalysts have been developed in various catalytic systems over the past few decades, including diverse noble and non-noble metals with plasmonic properties. The hot-carrier-induced mechanism is one of the most appealing pathways as it can provide energetic electrons or holes for driving thermodynamically unfavorable reactions or increasing the reaction rate. In this work, we evaluate the photocatalytic performance of semimetallic bismuth nanoparticles and offer detailed mechanistic interpretations in terms of hot carriers and interband transitions.

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Purpose: TRK fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients.

Experimental Design: We conducted an international retrospective cohort study of patients with TRK fusion-driven CNS tumors.

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The transition from jail to community is a high-risk time for individuals experiencing substance use disorders (SUD), with elevated risks of overdose and other substance-related harms, as well as high recidivism rates and re-incarceration. Gains made from successful treatment in prison are often lost in this transition. The current paper evaluates this process for one justice programme, Chesterfield HARP, a Therapeutic Community based in the Chesterfield County jail that continues supporting former inmates through their release by examining engagement and retention rates and changes in recovery capital among incarcerated individuals before incarceration and after transition to recovery housing in the community.

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