Evidence map›Paper›PMID 37891621›Full record

Observational studyOrphanet journal of rare diseases2023

A unicentric cross-sectional observational study on chronic intestinal inflammation in total colonic aganglionosis: beware of an underestimated condition.

M Erculiani, F Poluzzi, G Mottadelli, E Felici, Novi Ml, M Caraccia, A Grandi, S Casella, L Giacometti, G Montobbio and 8 more

Open access · goldAbstract readObservational Study
PubMed Full text at PMC Publisher
In one paragraph

Observational study in Orphanet journal of rare diseases, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
1.6field-weighted citation impact, top 16% 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

1 citing paper in PubMed, 5 citations in OpenAlex.

  1. Review
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

18 authors at 5 institutions in 1 country.

M ErculianiUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
F PoluzziUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
G MottadelliUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
E FeliciUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
Novi MlUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
M CaracciaUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
A GrandiUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
S CasellaUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
L GiacomettiUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
G MontobbioUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy.
I CeccheriniUOSD Laboratory of Genetics and Genomics of Rare Diseases, IRCCS Istituto Giannina Gaslini, University of Genoa, Genoa, Italy.
E Di MarcoCentral Laboratory, Giannina Gaslini Institute, Genoa, Italy.
C BonarettiMolecular Diagnostic, Giannina Gaslini Institute, Genoa, Italy.
R BiassoniMolecular Diagnostic, Giannina Gaslini Institute, Genoa, Italy.
M SquillarioIRCCS Ospedale Policlinico San Martino Genoa, Genoa, Italy.
A PietrantoniInstitute of Pathology, ASST-Spedali Civili, University of Brescia, Brescia, Italy.
V VillanacciInstitute of Pathology, ASST-Spedali Civili, University of Brescia, Brescia, Italy.
A Pini PratoUmberto Bosio Center for Digestive Diseases, AO SS Antonio e Biagio e Cesare Arrigo, Alessandria, Italy. a.piniprato@gmail.com.ORCID 0000-0003-3080-7481
Azienda Ospedaliera Nazionale SS. Antonio e Biagio e Cesare Arrigo · ITIstituto Giannina Gaslini · ITUniversity of Brescia · ITOspedale Policlinico San Martino · ITUniversità degli Studi del Piemonte Orientale “Amedeo Avogadro” · IT

Funding

Compagnia di San Paolo 2019.0880 - ID ROL 32612Ministero della Salute Ricerca Corrente
6 · The paper itself

Abstract

backgroundInflammatory Bowel Diseases (IBD) are known to occur in association with Hirschsprung disease (HSCR). Most of cases are represented by Crohn Disease (CD) occurring in patients with Total Colonic Aganglionosis (TCSA) with an estimated prevalence of around 2%. Based on these considerations and on a number of provisional data belonging to our Center for Digestive Diseases, we developed a unicentric cross-sectional observational study aimed at describing phenotype, genotype, pathology and metagenomics of all patients with TCSA and Crohn-like lesions.

resultsOut of a series of 62 eligible TCSA patients, 48 fulfilled inclusion criteria and were enrolled in the study. Ten patients did not complete the study due to non-compliance or withdrawal of consent and were subsequently dropped out. A total of 38 patients completed the study. All patients were tested for chronic intestinal inflammation by a combination of fecal calprotectine (FC) or occult fecal blood (OFB) and underwent fecal metagenomics. Nineteen (50%) tested positive for FC, OFB, or both and subsequently underwent retrograde ileoscopy. Fourteen patients (36.8%) presented Crohn-like lesions, occurring after a median of 11.5 years after surgery (range 8 months - 21.5 years). No statistically significant differences regarding demographic, phenotype and genotype were observed comparing patients with and without lesions, except for need for blood transfusion that was more frequent in those with lesions. Faecal microbiome of patients with lesions (not that of caregivers) was less biodiverse and characterized by a reduction of Bacteroidetes, and an overabundance of Proteobacteria. FC tested negative in 3/14 patients with lesions (21%).

conclusionsOur study demonstrated an impressive 10-folds higher incidence of chronic inflammation in TCSA. Up to 50% of patients may develop IBD-like lesions postoperatively. Nonetheless, we failed in identifying specific risk factors to be used to implement prevention strategies. Based on the results of our study, we suggest screening all TCSA patients with retrograde ileoscopy regardless of FC/OFB values. The frequency of endoscopic assessments and the role of FC/OFB screening in prompting endoscopy is yet to be determined.

Indexed as

Hirschsprung DiseaseInflammatory Bowel DiseasesCross-Sectional StudiesHumansInflammationSalicylanilides3,3',4',5-tetrachlorosalicylanilideSalicylanilidesCrohnEndoscopyEnterocolitisHirschsprungInflammationInflammatory Bowel DiseasesTotal colonic aganglionosis

Identifiers

PMID37891621
PMCPMC10612252
OpenAlexW4387969446

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

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.