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. OBJECTIVES: Test the reliability and validity of training coders and assigning Nova categories to individual foods collected via 24-h dietary recalls. DESIGN: A secondary analysis of 24-h dietary recalls from 610 children who participated in a randomized controlled trial and were 3-5 y old at baseline was conducted. The Nutrition Data System for Research (NDSR) software was used to collect 2-3 dietary recalls at baseline and yearly for 3 y. Trained and certified coder pairs independently categorized foods into one of 4 Nova categories (minimally processed, processed culinary ingredients, processed, and ultraprocessed). Interrater reliability was assessed by percent concordance between coder pairs and by Cohen's κ coefficient. Construct validity was evaluated by comparing the average daily macronutrient content of foods between Nova categories. RESULTS: In 5546 valid recall days, 3099 unique foods were categorized: minimally processed (18%), processed culinary ingredients (0.4%), processed (15%), and ultraprocessed (67%). Coder concordance = 88.3%, and κ coefficient = 0.75. Descriptive comparisons of macronutrient content across 66,531 diet recall food entries were consistent with expectations. On average, UPFs were 62% (SD 19) of daily calories, and a disproportionally high percentage of daily added sugar (94%; SD 16) and low percentage of daily protein (47%; SD 24). Minimally processed foods were 30% (SD 17) of daily calories, and a disproportionally low percentage of daily added sugar (1%; SD 8) and high percentage of daily protein (43%; SD 24). CONCLUSIONS: This method of Nova classifying NDSR-based 24-h dietary recalls was reliable and valid for identifying individual intake of processed foods, including UPFs.

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. Mixed-effects regressions were used to determine whether IQ was associated with clinical features. Models were controlled for variation in test version, participant identification, and test administrator. RESULTS: Mean verbal, performance, and full-scale IQs were 81.77, 75.98, and 82.02, respectively, with a consistent discrepancy between verbal and performance IQs. Mean (SD) verbal, performance, and full-scale coefficient of variations were 0.07 (0.04), 0.09 (0.05), and 0.06 (0.02), respectively. IQ varied about a mean, with no clear trajectory, indicating no clear patterns of improvement or decline over time. EEG lateralization and behavioral issues were consistently associated with IQ. CONCLUSIONS: The observed variation in IQ in Gaucher disease type 3 across the cohort and within single individuals over time may be characteristic of other neuronopathic diseases. Therefore, to reliably use IQ as an efficacy measure in any clinical trial of neurotherapeutics, a normal variation range must be established to assess the clinical relevance of any IQ change.

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. Our objective was to assess if increasing PN volume in NF1 is associated with increasing PN-related morbidity. Methods: This is a retrospective review of patients enrolled on the NCI NF1 natural history study with ≥7 years of data available. Morbidities including pain, motor dysfunction, vision loss, and PN-related surgery were assessed at time of baseline PN MRI with volumetric analysis and time of MRI with maximum PN volume. Results: Forty-one patients (median age at baseline 8 y) with 57 PN were included. At baseline, 40 PN had at least 1 PN-associated morbidity. During the observation period, 27 PN required increasing pain medication, and these PN grew faster per year (median difference 8.3%; 95% CI: 2.4, 13.8%) than those PN which did not. PN resulting in motor impairment at baseline (n = 11) had larger volumes compared with those that did not (median difference 461 mL; 95% CI: 66.9, 820). Conclusions: Many NF1 PN were associated with clinically significant morbidity at baseline, highlighting the need for longitudinal morbidity evaluations starting at an early age to capture changes in PN-associated morbidities. Prospective evaluation of standardized patient reported and functional outcomes in clinical trials are ongoing and may allow further characterization of the association of PN volume increase or decrease and clinical changes.