Publications by authors named "F Johnson"

Article Synopsis
  • Fabry disease is an X-linked lysosomal disorder causing complications like kidney issues and challenges with drug elimination, prompting the need to study migalastat in patients with end-stage renal disease (ESRD) on dialysis.
  • This study tested a 123 mg dose of migalastat in ESRD patients compared to controls with normal kidney function, examining its pharmacokinetics, how well it's cleared during dialysis, and its tolerability.
  • Results showed that migalastat is largely removed by dialysis and suggested dosing regimens for ESRD patients that could maintain effective drug levels while minimizing accumulation in tissues.
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Salivary gland tumor operations are associated with complications including facial nerve dysfunction (FND) and salivary fistula. The objective of this study was to investigate the effect of extracapsular dissection (ECD) and the application of Clostridium botulinum toxin (CBT) in contrast to partial and lateral parotidectomy on complications. All salivary gland tumor operations performed within the last 6 years were retrospectively examined.

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Purpose: Effective therapy for recurrent head and neck squamous cell carcinoma (HNSCC) that is refractory to chemotherapy and immunotherapy is a considerable need. Aurora kinase A inhibition leads to apoptosis and immunogenic cell death in preclinical models of human papilloma virus (HPV)-driven cancers.

Experimental Design: Alisertib was administered orally twice daily on days 1-7 and pembrolizumab on day 1 of a 21-day cycle to adults with advanced solid tumors (phase 1) or with immunotherapy- and platinum-resistant, HPV-positive HNSCC (phase 2).

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Article Synopsis
  • A study examined patient preferences for different types of pacemakers, including new dual-chamber leadless options and traditional transvenous ones, to identify which features are most important to them.
  • Surveying 117 patients, researchers found that half preferred leadless pacemakers while the other half favored conventional ones, highlighting a significant division in choices.
  • Key factors influencing patient decisions included preferred pacemaker type, acceptance of complication and infection risks, and the time since regulatory approval, with many willing to accept higher risks for their preferred options.
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Enzyme replacement therapy (ERT) is the only approved disease-modifying treatment modality for Pompe disease, a rare, inherited metabolic disorder caused by a deficiency in the acid -glucosidase (GAA) enzyme that catabolizes lysosomal glycogen. First-generation recombinant human GAA (rhGAA) ERT (alglucosidase alfa) can slow the progressive muscle degeneration characteristic of the disease. Still, most patients experience diminished efficacy over time, possibly because of poor uptake into target tissues.

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