Evidence map›Paper›PMID 39470916›Full record

ArticleClinical rheumatology2024

Familial Mediterranean fever in children from central-southern Italy: a multicentric retrospective cohort study.

Saverio La Bella, Roberta Loconte, Marina Attanasi, Mario Muselli, Giulia Di Donato, Armando Di Ludovico, Marco Natale, Violetta Mastrorilli, Andrea Giugno, Santi Papa and 8 more

Abstract readMulticenter Study
PubMed Publisher
In one paragraph

Article in Clinical rheumatology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed, 1 pooled it
–field-weighted citation impact
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 synthesis or guideline pooled it.

  1. Pooled it
  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

18 authors.

Saverio La BellaPediatric Rheumatology Unit, Department of Pediatrics, "G. D'Annunzio" University of Chieti, Chieti, Italy. saveriolabella@outlook.it.ORCID http://orcid.org/0000-0002-1244-0789
Roberta LoconteDepartment of Pediatrics, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
Marina AttanasiPediatric Clinic - Department of Pediatrics, "G. D'Annunzio" University of Chieti, Chieti, Italy.
Mario MuselliDepartment of Life, Health and Environmental Sciences, Public Health Section, University of L'Aquila, L'Aquila, Italy.
Giulia Di DonatoPediatric Rheumatology Unit, Department of Pediatrics, "G. D'Annunzio" University of Chieti, Chieti, Italy.
Armando Di LudovicoPediatric Rheumatology Unit, Department of Pediatrics, "G. D'Annunzio" University of Chieti, Chieti, Italy.
Marco NatalePediatric Rheumatology Unit, Department of Pediatrics, "G. D'Annunzio" University of Chieti, Chieti, Italy.
Violetta MastrorilliDepartment of Pediatrics, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
Andrea GiugnoDepartment of Integrated Maternal-Child and Reproduction Activity, "Policlinico-San Marco" University of Catania, Catania, Italy.
Santi PapaDepartment of Integrated Maternal-Child and Reproduction Activity, "Policlinico-San Marco" University of Catania, Catania, Italy.
Rossella FerranteCenter for Advanced Studies and Technology (CAST), "G. D'Annunzio" University of Chieti, Chieti, Italy.
Carlotta BuccoliniCenter for Advanced Studies and Technology (CAST), "G. D'Annunzio" University of Chieti, Chieti, Italy.
Ivana AntonucciCenter for Advanced Studies and Technology (CAST), "G. D'Annunzio" University of Chieti, Chieti, Italy.
Francesco ChiarelliPediatric Clinic - Department of Pediatrics, "G. D'Annunzio" University of Chieti, Chieti, Italy.
Stefano NecozioneDepartment of Life, Health and Environmental Sciences, Public Health Section, University of L'Aquila, L'Aquila, Italy.
Patrizia BaroneDepartment of Integrated Maternal-Child and Reproduction Activity, "Policlinico-San Marco" University of Catania, Catania, Italy.
Francesco La TorreDepartment of Pediatrics, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
Luciana BredaPediatric Rheumatology Unit, Department of Pediatrics, "G. D'Annunzio" University of Chieti, Chieti, Italy.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionAlthough familial Mediterranean fever (FMF) is a relevant disease in countries surrounding the Mediterranean Sea, there are still few reports from Italy.

methodsWe retrospectively evaluated patients with FMF diagnosed according to the EuroFever/PRINTO classification criteria in three pediatric rheumatology referral centers in central-southern Italy. Logistic regression analysis assessed the associations between age at disease onset and symptoms.

resultsOverall, 48 patients were enrolled (28 females, 20 males), with a median age at onset of 3.3 [3.1] years, and a median follow-up period of 5.1 [10.8] years. The most common MEFV genotype was M694V/- (11 patients, 22.9%), followed by M694V/M694V (6 patients, 12.5%). At onset, recurrent fever was observed in 47 patients (97.9%), with a median time between attacks of 18 [11] days. Overall, recurrent fever was observed in all patients, abdominal pain in 44 (91.7%), and chest pain in 18 (37.5%). At the last follow-up visit, 24 patients were on colchicine (50%), 2 on biologic (4.2%), and 6 on both (12.5%). Canakinumab was the most used biologic drug, in 6 (12.5%) patients. MEFV genotype was associated with disease severity (p = 0.007) and the use of a biological drug (p = 0.01). FMF prevalence in the Abruzzo region was found highly than expected (at least 1:45,000). Differently, we found a relevant gap among FMF patients expected and observed in the Apulia and Sicily regions.

conclusionsFMF is a relevant issue in central-southern Italy. A large epidemiologic study should be performed to better define its prevalence in the country. Key Points • Italian children with familial Mediterranean fever tend to have an early age of onset, primarily manifesting with recurrent fever and characteristic associated symptoms. • Many MEFV gene variants are present in Italian children with familial Mediterranean fever, and these patients are most often heterozygous, exhibiting a mild to moderate phenotype. • The prevalence of familial Mediterranean fever in Italy is still unknown but recently estimated to be around 1:60,000, probably higher in central and southern Italy. • According to our cohort, the prevalence of FMF in the Abruzzo region is at least 1:45,000, higher than expected. Differently, we found lower prevalence rates of the disease in Apulia and Sicily.

Indexed as

ColchicineFamilial Mediterranean FeverGenotypePyrinAdolescentAge of OnsetAntibodies, Monoclonal, HumanizedChildChild, PreschoolFemaleHumansInfantItalyMaleRetrospective StudiesAntibodies, Monoclonal, HumanizedcanakinumabColchicineMEFV protein, humanPyrinAutoinflammatory diseasesFamilial Mediterranean feverFMFItalyPediatric FMF

Identifiers

PMID39470916

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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 OpenQuestion graph.