Reliability and validity of assigning ultraprocessed food categories to 24-h dietary recall data.

Background: The Nova classification system categorizes foods into 4 processing levels, including ultraprocessed foods (UPFs). Consumption of UPFs is extensive in the United States, and high UPF consumption is associated with chronic disease risk. A reliable and valid method to Nova-categorize foods would advance understanding of UPF consumption and its relationship to health outcomes.

Variation in cognitive function over time in Gaucher disease type 3.

Objective: To identify relevant efficacy parameters essential in designing clinical trials for brain-penetrant therapies for Gaucher disease, we evaluated cognitive function longitudinally in 34 patients with Gaucher disease type 3 seen at the NIH Clinical Center.

Methods: Individuals were tested with age-appropriate Wechsler Intelligence Scales administered between 1 and 18 times over 29 years. Variation in all IQ domains was not linear with time and was best characterized with the coefficient of variation (SD/mean) for each individual.

Association of plexiform neurofibroma volume changes and development of clinical morbidities in neurofibromatosis 1.

Background: Plexiform neurofibromas (PN) in neurofibromatosis 1 (NF1) can cause substantial morbidities. Clinical trials targeting PN have recently described decreases in PN volumes. However, no previous study has assessed the association between changes in PN volumes and PN-related morbidities.