Publications by authors named "J E Hoppe"
Article Synopsis
- Vanzacaftor-tezacaftor-deutivacaftor is a new CFTR modulator showing safety and effectiveness in phase 2 trials for adults with cystic fibrosis, leading to a study evaluating its use in children aged 6-11.
- This phase 3 trial, called RIDGELINE, involved participants from 33 clinical sites across eight countries, focusing on children with specific CFTR variants and stable health conditions.
- The study aimed to assess the drug's safety, tolerability, and efficacy over 24 weeks, with primary outcomes evaluated through various health metrics and participant feedback.
As cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies including elexacaftor/tezacaftor/ivacaftor (ETI) have become widely used in eligible patients with cystic fibrosis (CF), the use of these medications in pregnant people has become a critical area of investigation. Since these medications appear generally safe to both mother and fetus when taken by pregnant people with CF, interest has pivoted to the use of ETI in CF carrier mothers to decrease morbidity and mortality from meconium ileus (MI) in fetuses with cystic fibrosis. Here we discuss three infants at our institution with ultrasound findings of MI who were exposed to prenatal ETI through CF carrier mothers for the purposes of treating MI and lowering risk of intestinal complications from this severe manifestation of CF.
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- - A 20-year-old woman with type I hypoplastic amelogenesis imperfecta experienced aesthetic issues and sensitivity, prompting a complete mouth rehabilitation using a fully digital workflow.
- - An interdisciplinary approach included a multigene panel analysis and digital scans to guide minimally invasive dental preparations, resulting in interim restorations made of high-performance polycarbonate and final restorations in translucent zirconia.
- - This efficient treatment plan not only addressed her dental concerns but also enhanced her understanding of her genetic condition, highlighting the importance of genetics in her treatment, with a strong follow-up plan to ensure long-term success.
Background: Coprescribing naloxone with opioid analgesics is a Centers for Disease Control and Prevention (CDC) best practice to mitigate the risk of fatal opioid overdose, yet coprescription by emergency medicine clinicians is rare, occurring less than 5% of the time it is indicated. Clinical decision support (CDS) has been associated with increased naloxone prescribing; however, key CDS design characteristics and pragmatic outcome measures necessary to understand replicability and effectiveness have not been reported.
Objective: This study aimed to rigorously evaluate and quantify the impact of CDS designed to improve emergency department (ED) naloxone coprescribing.
Background: Aortic valve disease (AVD) is associated with high mortality and morbidity. To date, there is no pharmacological therapy available to prevent AVD progression. Because valve calcification is the hallmark of AVD and S1P (sphingosine-1-phosphate) plays an important role in osteogenic signaling, we examined the role of S1P signaling in aortic stenosis disease.
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