Evidence map›Paper›PMID 35945378›Full record

ArticleJournal of clinical immunology2022

A Novel Assay in Whole Blood Demonstrates Restoration of Mitochondrial Activity in Phagocytes After Successful HSCT in Hyperinflamed X-Linked Chronic Granulomatous Disease.

Maddalena Migliavacca, Luca Basso Ricci, Giada Farinelli, Valeria Calbi, Francesca Tucci, Federica Barzaghi, Francesca Ferrua, Maria Pia Cicalese, Silvia Darin, Lina Raffaella Barzaghi and 17 more

Open access · hybridAbstract read
In one paragraph

Article in Journal of clinical immunology, 2022. 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
0.1field-weighted citation impact, top 60% 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, 1 citations in OpenAlex.

  1. 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

27 authors at 5 institutions in 1 country.

Maddalena MigliavaccaSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Luca Basso RicciSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Giada FarinelliFondazione Telethon, Milan, Italy.
Valeria CalbiSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Francesca TucciSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Federica BarzaghiSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Francesca FerruaSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Maria Pia CicaleseSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Silvia DarinSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Lina Raffaella BarzaghiNeurosurgery department, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Fabio GiglioHematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Jacopo PeccatoriHematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Francesca FumagalliSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Roberto NicolettiDepartment of Radiology, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Stefania GiannelliSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Claudia SartiranaSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Alessandro BandieraThoracic surgery, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Maria EspositoS.O.C. Malattie Infettive ASL VC Piemonte, Milan, Italy.
Raffaella MilaniCytometry Laboratory, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Benedetta MazziHLA Laboratory, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Andrea FinocchiDepartment of Paediatrics, Ospedale Pediatrico Bambino Gesù and University of Rome "Tor Vergata", Rome, Italy.
Sarah MarktelHematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Andrea AssanelliHematology and Bone Marrow Transplantation Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Franco LocatelliDepartment of Onco-Haematology and Cell and Gene Therapy, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
Fabio CiceriSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Alessandro AiutiSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy. aiuti.alessandro@hsr.it.ORCID 0000-0002-5398-1717
Maria Ester BernardoSan Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, Pediatric Immunohematology and Bone Marrow Transplantation Unit, San Raffaele Scientific Institute, Via Olgettina, 60, 20123, Milan, Italy.
Vita-Salute San Raffaele University · ITThe San Raffaele Telethon Institute for Gene Therapy · ITBambino Gesù Children's Hospital · ITTelethon Foundation · ITUniversity of Rome Tor Vergata · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

X-linked chronic granulomatous disease is a rare disease caused by mutations in the CYBB gene. While more extensive knowledge is available on genetics, pathogenesis, and possible therapeutic options, mitochondrial activity and its implications on patient monitoring are still not well-characterized. We have developed a novel protocol to study mitochondrial activity on whole blood of XCGD patients before and after transplantation, as well as on XCGD carriers. Here we present results of these analyses and of the restoration of mitochondrial activity in hyperinflamed X-linked Chronic Granulomatous Disease after hematopoietic stem cell transplantation. Moreover, we show a strong direct correlation between mitochondrial activity, chimerism, and DHR monitored before and after transplantation and in XCGD carriers. In conclusion, based on these findings, we suggest testing this new ready-to-use marker to better characterize patients before and after treatment and to investigate disease expression in carriers.

Indexed as

Granulomatous Disease, ChronicHematopoietic Stem Cell TransplantationChimerismHeterozygoteHumansPhagocytesHematopoietic stem cell transplantationMitochondrial activityNovel protocol on whole bloodPhagocytes disordersPrimary Immunodeficiencystrong direct correlation between mitochondrial activity, hematopoietic stem cell transplantation chimerism and DHR monitored before and after transplantation and in XCGD carriersX linked chronic granulomatous diseaseX linked chronic granulomatous disease carriers

Identifiers

PMID35945378
PMCPMC9700633
OpenAlexW4292442875

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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.