Evidence map›Paper›PMID 42169998›Full record

ArticleJournal of human immunity2026

Quality of life of X-linked agammaglobulinemia patients in the United Kingdom.

Ben Shillitoe, Helen Bourne, Catherine Stroud, Terry Flood, Matthew Buckland, Winnie Ip, Austen Worth, Scott Hackett, Archana Herwadkar, Tanya Coulter and 6 more

Abstract read
In one paragraph

Article in Journal of human immunity, 2026. 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
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.

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

16 authors.

Ben ShillitoeSheffield Children's NHS Foundation Trust, Sheffield, UK.ORCID https://orcid.org/0000-0003-4407-5998
Helen BourneRoyal Victoria Infirmary, Newcastle upon Tyne, UK.ORCID https://orcid.org/0009-0002-6872-2010
Catherine StroudRoyal Victoria Infirmary, Newcastle upon Tyne, UK.ORCID https://orcid.org/0000-0003-2774-7625
Terry FloodGreat North Children's Hospital, Newcastle upon Tyne, UK.ORCID https://orcid.org/0009-0009-1938-6189
Matthew BucklandInstitute of Child Health, UCL, London, UK.ORCID https://orcid.org/0000-0002-5646-4707
Winnie IpDepartment of Immunology and Gene Therapy, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.ORCID https://orcid.org/0000-0002-1550-4856
Austen WorthDepartment of Immunology and Gene Therapy, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.ORCID https://orcid.org/0000-0001-6803-7385
Scott HackettUniversity Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.ORCID https://orcid.org/0000-0002-7043-6067
Archana HerwadkarSalford Care Organisation, Northern Care Alliance NHS Trust, Salford, UK.ORCID https://orcid.org/0009-0008-9384-1162
Tanya CoulterBelfast Health and Social Care Trust, Belfast, UK.ORCID https://orcid.org/0000-0001-8182-9854
Stephen JollesImmunodeficiency Centre for Wales, University Hospital of Wales, Cardiff, UK.ORCID https://orcid.org/0000-0002-7394-6804
Tomaz GarcezManchester University NHS Foundation Trust, Manchester, UK.ORCID https://orcid.org/0000-0002-8106-0480
Eduardo MoyaBradford Teaching Hospitals NHS Foundation Trust, Bradford, UK.ORCID https://orcid.org/0009-0003-6024-5894
Saul N FaustNIHR Southampton Clinical Research Facility and Biomedical Research Centre, University Hospital Southampton NHS Foundation Trust, Southhampton, UK.ORCID https://orcid.org/0000-0003-3410-7642
Mark S PearcePopulation Health Sciences Institute, Newcastle University, Newcastle, UK.ORCID https://orcid.org/0000-0002-0583-3779
Andrew R GenneryGreat North Children's Hospital, Newcastle upon Tyne, UK.ORCID https://orcid.org/0000-0002-6218-1324

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Due to limitations in current therapies (immunoglobulin replacement therapy), complications in X-linked agammaglobulinemia (XLA) such as bronchiectasis may continue to occur, with subsequent significant impacts on health-related quality of life (HRQoL). There were no significant differences in PedsQL 4.0 total scores against UK healthy norms (self 80.98, P = 0.277; parent 79.76, P = 0.465). There were no significant differences in the SF36v2 physical component score (PCS) against UK healthy norms (49.10, P = 0.712). However, XLA patients with bronchiectasis had significantly worse PCS than patients without (47.88 versus 55.14, P < 0.001) and significantly worse PCS than UK healthy norms (P = 0.004). In the absence of bronchiectasis, HRQoL is comparable to UK healthy norms. However, XLA patients with bronchiectasis have significantly worse HRQoL than patients without bronchiectasis and UK healthy norms. These data demonstrate that further work into novel therapies are needed to prevent bronchiectasis to enable XLA patients to have a normal quality of life.

Identifiers

PMID42169998
PMCPMC13177391

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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.