Evidence map›Paper›PMID 39735177›Full record

ReviewJournal of diabetes and metabolic disorders2025

The role of the analysis of sialotransferrin isoforms in the management of hereditary fructose intolerance: a systematic review.

Evelina Maines, Giorgia Gugelmo, Arianna Maiorana, Diego Martinelli, Nicola Vitturi, Livia Lenzini, Giovanni Piccoli, Massimo Soffiati, Roberto Franceschi

Abstract readReview
In one paragraph

Review in Journal of diabetes and metabolic disorders, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

9 authors.

Evelina MainesDivision of Pediatrics, Santa Chiara General Hospital, Azienda Provinciale per i Servizi Sanitari, Largo Medaglie d'oro, 9, 38122 Trento, Italy.ORCID 0000-0002-0445-2974
Giorgia GugelmoDivision of Metabolic Diseases, Department of Medicine, Padova University Hospital, Via Nicolò Giustiniani 2, 35121 Padua, Italy.
Arianna MaioranaDivision of Metabolism and Research Unit of Metabolic Biochemistry, Bambino Gesù Children's Hospital, IRCCS, Piazza Di Sant'Onofrio 4, 00165 Rome, Italy.
Diego MartinelliDivision of Metabolism and Research Unit of Metabolic Biochemistry, Bambino Gesù Children's Hospital, IRCCS, Piazza Di Sant'Onofrio 4, 00165 Rome, Italy.
Nicola VitturiDivision of Metabolic Diseases, Department of Medicine, Padova University Hospital, Via Nicolò Giustiniani 2, 35121 Padua, Italy.
Livia LenziniDepartment of Medicine, Padova University Hospital, Via Nicolò Giustiniani 2, 35121 Padua, Italy.
Giovanni PiccoliCIBIO - Department of Cellular, Computational and Integrative Biology, Università Degli Studi Di Trento, Via Sommarive 9, 38123 Trento, Italy.
Massimo SoffiatiDivision of Pediatrics, Santa Chiara General Hospital, Azienda Provinciale per i Servizi Sanitari, Largo Medaglie d'oro, 9, 38122 Trento, Italy.
Roberto FranceschiDivision of Pediatrics, Santa Chiara General Hospital, Azienda Provinciale per i Servizi Sanitari, Largo Medaglie d'oro, 9, 38122 Trento, Italy.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Untreated patients affected by hereditary fructose intolerance (HFI) present an abnormal transferrin (Tf) glycosylation pattern suggestive of N-hypoglycosylation. Analysis of defects in N-glycosylation is possible by analysis of serum sialotransferrin (sialoTf) pattern. The sialoTf profile is a valuable tool to facilitate the diagnosis of HFI. Its role in the monitoring of the diagnosed patients is less clear and debating. Objectives and methods: We examined the literature for the role of profile of serum sialoTf isoforms in monitoring HFI patients aiming at (1) providing an up-to-date summary of the available evidences on the impact of sialoTf isoforms in the follow-up of HFI patients; 2) evaluating the multifactorial effect of genotype and age at diagnosis on sialoTf isoforms; 3) assessing the relation between sialoTf isoforms and long-term liver complications. We used the GRADE approach to rank the quality of evidence. Results: Nine full papers were identified according to our search criteria. Elevated serum carbohydrate-deficient Tf (CDT) fraction, disialoTf and tetrasialoTf/disialoTf ratio, and the asialoTf, tetrasialoTf and pentasialoTf + hexasialoTf isoforms appeared as the most reliable indicators for a follow up. No clear statistical correlation links sialoTf isoforms and liver damage. Age at diagnosis, potentially related to fructose tolerance, does not overtly impact sialoTf isoforms. Strong genotype-phenotype correlation has not been found so far. Conclusions: There is no consensus about which isoform of sialoTf is more valuable for monitoring HFI patients. No clear correlation links sialoTf isoforms and liver damage, fructose tolerance and genotype. More robust studies are needed to provide conclusive results.

Indexed as

Follow upHereditary fructose intoleranceSialotransferrinSystematic review

Identifiers

PMID39735177
PMCPMC11680511

What Socratic holds

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Registered trials

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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 Socratic graph.