Utilization of cornstarch in glycogen storage disease type Ia
Bibliographic record
Abstract
OBJECTIVE: Uncooked cornstarch (UCCS) is used widely for the treatment of patients with glycogen storage disease type I (GSD-I). Previous studies suggested that glucose absorption may be impaired in GSD-I. In order to measure utilization of UCCS in young adults with GSD-Ia and healthy controls, we used a C-breath test based on the natural enrichment of C in UCCS. DESIGN: Open, not randomized, prospective interventional study. METHODS: Following 1 g/kg UCCS, we studied eight subjects with GSD-Ia (7 males, 1 female; mean age 28.3 years, range 16-42 years) and 15 healthy controls (10 males, 5 females; mean age 23.5 years, range 19-36 years). Breath samples for analysis of CO enrichment were collected at baseline and at 30-min intervals for 6 h or until hypoglycaemia occurred. Indirect calorimetry was used to measure respiratory gas exchange. Intermediate metabolites, lipids and glucose were measured in plasma. Breath H concentrations were measured as an indicator of malabsorption. RESULTS: Cumulative utilization over 6 h was significantly higher in controls (18.35 +/- 6.2% of total carbohydrate intake) than in subjects with GSD-Ia (11.5 +/- 4.7%) (P < 0.02). However, utilization of UCCS was virtually identical up to 2.5 h. Two subjects with GSD-Ia fulfilled the criteria for malabsorption. CONCLUSIONS: Starch digestion and absorption are not impaired in GSD-Ia. However, overall utilization of UCCS appears to be lower in GSD-Ia, which is most likely secondary to perturbed intermediary metabolism. There are important implications for treatment of this disorder. Ways to improve the efficacy of UCCS in GSD-I are needed.
Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.
How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one teacher head, not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".