Publications by authors named "J L Bernat"

This letter responds to letters by Garson Leder and by Harrison Lee in the same issue, September-October 2024, of the Hastings Center Report.

View Article and Find Full Text PDF
Article Synopsis
  • Pegunigalsidase alfa is a new enzyme replacement therapy for Fabry disease, showing a longer half-life and administered every 4 weeks instead of the typical 2-week schedule.
  • The BRIGHT study involved 30 adult patients who switched from another ERT to pegunigalsidase alfa, revealing good safety results with mostly mild side effects and no new anti-drug antibodies.
  • Although the treatment showed acceptable tolerance, more research is needed due to the small sample size, but it suggests that this 4-week regimen could be a viable new option for managing Fabry disease.
View Article and Find Full Text PDF

Objective: Metachromatic leukodystrophy (MLD) is a rare neurodegenerative disorder. Emerging therapies are most effective in the presymptomatic phase, and thus defining this window is critical. We hypothesize that early development delay may precede developmental plateau.

View Article and Find Full Text PDF

Organ donation after the circulatory determination of death requires the permanent cessation of circulation while organ donation after the brain determination of death requires the irreversible cessation of brain functions. The unified brain-based determination of death connects the brain and circulatory death criteria for circulatory death determination in organ donation as follows: permanent cessation of systemic circulation causes permanent cessation of brain circulation which causes permanent cessation of brain perfusion which causes permanent cessation of brain function. The relevant circulation that must cease in circulatory death determination is that to the brain.

View Article and Find Full Text PDF

In transplant medicine, the use of normothermic regional perfusion (NRP) in donation after circulatory determination of death raises ethical difficulties. NRP is objectionable because it restores the donor's circulation, thus invalidating a death declaration based on the permanent cessation of circulation. NRP's defenders respond with arguments that are tortuous and factually inaccurate and depend on introducing extraneous concepts into the law.

View Article and Find Full Text PDF