Evidence map›Paper›PMID 37690115›Full record

ArticleThe Journal of clinical endocrinology and metabolism2024

Endogenous Glucose Production in Patients With Glycogen Storage Disease Type Ia Estimated by Oral D-[6,6-2H2]-glucose.

Alessandro Rossi, Maaike H Oosterveer, Theo H van Dijk, Aycha Bleeker, Martijn Koehorst, David A Weinstein, Barbara M Bakker, Terry G J Derks

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In one paragraph

Article in The Journal of clinical endocrinology and metabolism, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
0.2field-weighted citation impact, top 39% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed, 1 citations in OpenAlex.

  1. Review
  2. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors at 2 institutions in 3 countries.

Alessandro RossiDepartment of Pediatrics, Section of Metabolic Diseases, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands.
Maaike H OosterveerDepartment of Pediatrics, Laboratory of Pediatrics, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands.
Theo H van DijkDepartment of Laboratory Medicine, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands.
Aycha BleekerDepartment of Pediatrics, Laboratory of Pediatrics, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands.
Martijn KoehorstDepartment of Laboratory Medicine, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands.
David A WeinsteinDepartment of Pediatrics, University of Connecticut School of Medicine, Farmington, CT, USA.
Barbara M BakkerDepartment of Pediatrics, Laboratory of Pediatrics, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands.
Terry G J DerksDepartment of Pediatrics, Section of Metabolic Diseases, Beatrix Children's Hospital, University of Groningen, University Medical Center Groningen, 9713 GZ Groningen, The Netherlands.ORCID 0000-0002-7259-1095
University Medical Center Groningen · NLUniversity of Connecticut · US

Funding

Ultragenyx Pharmaceutical IncUniversity of Groningen
6 · The paper itself

Abstract

contextGlycogen storage disease type Ia (GSDIa) is an inborn metabolic disorder characterized by impaired endogenous glucose production (EGP). Monitoring of patients with GSDIa is prioritized because of ongoing treatment developments. Stable isotope tracers may enable reliable EGP monitoring.

objectiveThe aim of this study was to prospectively assess the rate of appearance of endogenous glucose into the bloodstream (Ra) in patients with GSDIa after a single oral D-[6,6-2H2]-glucose dose.

methodsTen adult patients with GSDIa and 10 age-, sex-, and body mass index-matched healthy volunteers (HVs) were enrolled. For each participant, 3 oral glucose tracer tests were performed: (1) preprandial/fasted, (2) postprandial, and (3) randomly fed states. Dried blood spots were collected before D-[6,6-2H2]-glucose administration and 10, 20, 30, 40, 50, 60, 75, 90, and 120 minutes thereafter.

resultsGlucose Ra in fasted HVs was consistent with previously reported data. The time-averaged glucose Ra was significantly higher in (1) preprandial/fasted patients with GSDIa than HV and (2) postprandial HV compared with fasted HV(P < .05). A progressive decrease in glucose Ra was observed in preprandial/fasted patients with GSDIa; the change in glucose Ra time-course was directly correlated with the change in capillary glucose (P < .05).

conclusionThis is the first study to quantify glucose Ra in patients with GSDIa using oral D-[6,6-2H2] glucose. The test can reliably estimate EGP under conditions in which fasting tolerance is unaffected but does not discriminate between relative contributions of EGP (eg, liver, kidney) and exogenous sources (eg, dietary cornstarch). Future application is warranted for longitudinal monitoring after novel genome based treatments in patients with GSDIa in whom nocturnal dietary management can be discontinued.

Indexed as

GlucoseGlycogen Storage Disease Type IAdultBlood GlucoseGlucose-6-PhosphataseHumansLiverBlood GlucoseGlucoseGlucose-6-Phosphatasedietglycogen storage disease type Iamonitoringprecision medicinestable isotopes

Identifiers

PMID37690115
PMCPMC10795891
OpenAlexW4386586911

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC-ND
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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.