Evidence map›Paper›PMID 38078335›Full record

ArticleFrontiers in pediatrics2023

Opinion paper: effectiveness of sirolimus in treating partial Di George syndrome with autoimmune lymphoproliferative syndrome-like features.

Jutte van der Werff Ten Bosch

Open access · goldAbstract read
In one paragraph

Article in Frontiers in pediatrics, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
–field-weighted citation impact, top 59% 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

0 citing papers in PubMed, 0 citations in OpenAlex.

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

1 author at 1 institution in 1 country.

Jutte van der Werff Ten BoschDepartment of Pediatrics, Paola Kinderziekenhuis, Antwerpen, Belgium.
Queen Fabiola Children's University Hospital · BE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Di George syndrome is a disorder well known by pediatricians, caused by deletions in chromosome 22q11.2 and presenting with a wide range of clinical abnormalities, including immunodeficiency. Thymic function is diminished, leading to a decreased output of naive T cells (naïve T-helper cells, naïve T-regulatory cells, naïve cytotoxic T cells) as compared to healthy age matched controls. Immunedysregulation, such as autoimmunity and immunoproliferation are common in these patients, because the lack of a correctly functioning T cell repertoire. treatment of these complication are a challenge in many immunodeficiencies, including Di George syndrome. Gu et al. discuss a case of a patient with 22q11 deletion and lymphoproliferation and treated successfully with Sirolimus. This opinion paper highlights the need to collect information on the treatment of patients with immunoproliferation through sharing of information of individual cases and international cooperation.

Indexed as

ALPSchildrenDi George syndromesirolimustreatment

Identifiers

PMID38078335
PMCPMC10701383
OpenAlexW4388928572

What OpenQuestion 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 OpenQuestion graph.