Evidence mapPaperPMID 34215305Full record

ArticleItalian journal of pediatrics2021

Crohn disease-like enterocolitis remission after empagliflozin treatment in a child with glycogen storage disease type Ib: a case report.

Alessandro Rossi, Erasmo Miele, Simona Fecarotta, Maria Veiga-da-Cunha, Massimo Martinelli, Carmine Mollica, Maria D'Armiento, Enza Mozzillo, Pietro Strisciuglio, Terry G J Derks and 2 more

Open access · goldAbstract readCase Reports
In one paragraph

Article in Italian journal of pediatrics, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
29citing papers in PubMed, 1 pooled it
6.3field-weighted citation impact, top 3% 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

29 citing papers in PubMed, 1 synthesis or guideline pooled it, 41 citations in OpenAlex.

  1. Pooled it
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  5. Empagliflozin as treatment in glycogen storage disease type IB patients.Molecular genetics and metabolism reports · 2025
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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

12 authors at 4 institutions in 3 countries.

Alessandro RossiDepartment of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy. alessandro.rossi@unina.it.ORCID http://orcid.org/0000-0002-1689-4948
Erasmo MieleDepartment of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy.
Simona FecarottaDepartment of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy.
Maria Veiga-da-CunhaGroupe de Recherches Metaboliques, de Duve Institute, UC Louvain (Université Catholique de Louvain), B-1200, Brussels, Belgium.
Massimo MartinelliDepartment of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy.
Carmine MollicaSection of Medical Imaging, Department of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy.
Maria D'ArmientoSection of Pathology, Department of Advanced Biomedical Sciences, University of Naples Federico II, Naples, Italy.
Enza MozzilloDepartment of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy.
Pietro StrisciuglioDepartment of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy.
Terry G J DerksSection of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, P.O. Box 30.001, 9700 RB, Groningen, The Netherlands.
Annamaria StaianoDepartment of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy.
Giancarlo ParentiDepartment of Translational Medical Sciences, Section of Pediatrics, University of Naples "Federico II", Naples, Italy.
University of Naples Federico II · ITUniversity Medical Center Groningen · NLde Duve Institute · BETelethon Institute Of Genetics And Medicine · IT

Funding

Associazione Italiana Glicogenosi 01/2020
6 · The paper itself

Abstract

backgroundBesides major clinical/biochemical features, neutropenia and inflammatory bowel disease (IBD) constitute common complications of Glycogen storage disease type Ib (GSD Ib). However, their management is still challenging. Although previous reports have shown benefit of empagliflozin administration on neutropenia, no follow-up data on bowel (macro/microscopic) morphology are available. We herein present for the first time longitudinal assessment of bowel morphology in a GSD Ib child suffering from Crohn disease-like enterocolitis treated with empagliflozin. CASE PRESENTATION: A 14-year-old boy with GSD Ib and severe IBD was (off-label) treated with empagliflozin (20 mg/day) after informed oral and written consent was obtained from the patient's parents. No adverse events were noted. Clinical symptoms and stool frequency improved within the first week of treatment. Pediatric Crohn disease activity index (PCDAI) normalised within the first month of treatment. Abdomen magnetic resonance imaging (MRI) performed 3 months after treatment initiation showed dramatic decrease in disease activity and length. Similar findings were reported on histology at 5.5 months. At 7.5 months hemoglobin levels normalised and fecal calprotectin almost normalised. Improved neutrophil count, metabolic control and quality of life were also noted. G-CSF dose was decreased by 33% and the patient was partly weaned from tube feeding.

conclusionsThis is the first report presenting extensive gastrointestinal morphology follow-up in a GSD Ib patient receiving empagliflozin. The present case suggests that empagliflozin can be safe and effective in inducing IBD remission in GSD Ib patients and can even postpone surgery. Future studies are required to confirm its effect over time and assess its benefit in various disease stages. The development of an international collaborating networks for systematic data collection is worthy.

Indexed as

AdolescentBenzhydryl CompoundsCrohn DiseaseEnterocolitisGlucosidesGlycogen Storage Disease Type IGranulocyte Colony-Stimulating FactorHumansMaleRemission InductionSodium-Glucose Transporter 2 InhibitorsBenzhydryl CompoundsempagliflozinGlucosidesGranulocyte Colony-Stimulating FactorSodium-Glucose Transporter 2 Inhibitors1,5-anhydroglucitolContinuous glucose monitoringEmpagliflozinGlycogen storage disease type IbInflammatory bowel diseaseNeutropenia

Identifiers

PMID34215305
PMCPMC8254289
OpenAlexW3176982619

What Socratic holds

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LicenceCC BY
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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.