Evidence map›Paper›PMID 25584041›Full record

ArticlePediatric rheumatology online journal2014

Neonatal treatment of CINCA syndrome.

Yan Paccaud, Gerald Berthet, Annette Von Scheven-Gête, Bernard Vaudaux, Yvan Mivelaz, Michael Hofer, Matthias Roth-Kleiner

Open access · goldAbstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Pediatric rheumatology online journal, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.

0numbers the graph read from it
0cells of the map it votes in
10citing papers in PubMed
1.1field-weighted citation impact, top 22% 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

10 citing papers in PubMed, 23 citations in OpenAlex.

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  8. Primary immunodeficiency diseases in the newborn.Northern clinics of Istanbul · 2021
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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

7 authors at 4 institutions in 1 country.

Yan PaccaudClinic of Neonatology, Department of Pediatrics, University Hospital and University of Lausanne, Lausanne, Switzerland.
Gerald BerthetClinic of Pediatrics, Cantonal Hospital of Aarau, Aarau, Switzerland.
Annette Von Scheven-GêteUnit of Pediatric Rheumatology of Western Switzerland, Departments of Pediatrics, University Hospital and University of Lausanne, and University Hospital of Geneva, Geneva, Switzerland.
Bernard VaudauxPediatric Infectiology Unit, Department of Pediatrics, University Hospital and University of Lausanne, Lausanne, Switzerland.
Yvan MivelazPediatric Cardiology Unit, Department of Pediatrics, University Hospital and University of Lausanne, Lausanne, Switzerland.
Michael HoferUnit of Pediatric Rheumatology of Western Switzerland, Departments of Pediatrics, University Hospital and University of Lausanne, and University Hospital of Geneva, Geneva, Switzerland.
Matthias Roth-KleinerClinic of Neonatology, Department of Pediatrics, University Hospital and University of Lausanne, Lausanne, Switzerland ; Clinic of Neonatology, Centre Hospitalier Universitaire Vaudois, CHUV, Av Pierre Decker 2, CH-1011 Lausanne, Switzerland.
University of Lausanne · CHKantonsspital Aarau · CHUniversity Hospital of Geneva · CHUniversity Hospital of Lausanne · CH

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

unlabelledChronic Infantile Neurological Cutaneous Articular (CINCA) syndrome, also called Neonatal Onset Multisystem Inflammatory Disease (NOMID) is a chronic disease with early onset affecting mainly the central nervous system, bones and joints and may lead to permanent damage. We report two preterm infants with severe CINCA syndrome treated by anti-interleukin-1 in the neonatal period, although, so far, no experience with this treatment in infants younger than three months of age has been reported. A review of the literature was performed with focus on treatment and neonatal features of CINCA syndrome. CASE REPORT: Two cases suspected to have CINCA syndrome were put on treatment with anakinra in the early neonatal period due to severe clinical presentation. We observed a rapid and persistent decline of clinical signs and systemic inflammation and good drug tolerance. Diagnosis was confirmed in both cases by mutations in the NLRP3/CIAS1-gene coding for cryopyrin. As particular neonatal clinical signs polyhydramnios and endocardial overgrowth are to be mentioned.

conclusionWe strongly suggest that specific treatment targeting interleukin-1 activity should be started early. Being well tolerated, it can be introduced already in neonates presenting clinical signs of severe CINCA syndrome in order to rapidly control inflammation and to prevent life-long disability.

Indexed as

Antirheumatic AgentsCarrier ProteinsCryopyrin-Associated Periodic SyndromesFemaleHumansInfant, NewbornInfant, PrematureInterleukin-1Interleukin 1 Receptor Antagonist ProteinMaleMutationNLR Family, Pyrin Domain-Containing 3 ProteinTime FactorsTreatment OutcomeAntirheumatic AgentsCarrier ProteinsInterleukin-1Interleukin 1 Receptor Antagonist ProteinNLR Family, Pyrin Domain-Containing 3 ProteinNLRP3 protein, humanAnakinraCINCAIL-1 receptorIL-1βNeonatal treatmentNOMID

Identifiers

PMID25584041
PMCPMC4290099
OpenAlexW2149383126

What OpenQuestion holds

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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.