Evidence map›Paper›PMID 34477998›Full record

Trial reportJournal of clinical immunology2021

Simple Measurement of IgA Predicts Immunity and Mortality in Ataxia-Telangiectasia.

Stefan Zielen, Ruth Pia Duecker, Sandra Woelke, Helena Donath, Sharhzad Bakhtiar, Aileen Buecker, Hermann Kreyenberg, Sabine Huenecke, Peter Bader, Nizar Mahlaoui and 43 more

2 registry-linked trialsOpen access · hybridAbstract readClinical Trial
PubMed Full text at PMC Publisher
In one paragraph

Trial report in Journal of clinical immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 2 registered trials, which are not on this map. Cited by 13 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed, 1 pooled it
1.5field-weighted citation impact, top 17% 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.

NCT02345135 nacompletednot on this map

Susceptibility to Infections in Patients With Ataxia Telangiectasia : A Prospective Follow-up Study

TypeinterventionalSponsorJohann Wolfgang Goethe University HospitalRan2012 to 2016Enrolled41ConditionsAtaxia Telangiectasia, Infections, Immunodeficiency, Lung DiseaseArmsBlood collection, Lung function measurement, Symptom diary
NCT03357978 naunknown statusnot on this map

Susceptibility to Infections, Tumor Risk and Liver Disease in Patients With Ataxia Telangiectasia With and Without Substitution of Immunoglobulin G: a Prospective Observational Study

TypeinterventionalSponsorJohann Wolfgang Goethe University HospitalRan2016 to 2019Enrolled30ConditionsAtaxia TelangiectasiaArmsbioelectrical impedance analysis, blood draw, transient elastography (FibroScan), ataxia score, Five-Times-Sit-to-Stand Test
3 · Its place in the literature

Who cites it

13 citing papers in PubMed, 1 synthesis or guideline pooled it, 25 citations in OpenAlex.

  1. Pooled it
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  11. Importance of Population-Based Cancer Risk Information in the Care of Patients With Rare Genetic Disorders.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2022
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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

53 authors at 20 institutions in 18 countries.

Stefan ZielenDivision of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Ruth Pia DueckerDivision of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany. RuthPia.Duecker@kgu.de.
Sandra WoelkeDivision of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Helena DonathDivision of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Sharhzad BakhtiarDivision for Stem Cell Transplantation, Immunology and Intensive Care Unit, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Aileen BueckerDivision of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Hermann KreyenbergDivision for Stem Cell Transplantation, Immunology and Intensive Care Unit, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Sabine HueneckeDivision for Stem Cell Transplantation, Immunology and Intensive Care Unit, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Peter BaderDivision for Stem Cell Transplantation, Immunology and Intensive Care Unit, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Nizar MahlaouiPediatric Immunology-Hematology and Rheumatology Unit, French National Reference Center for Primary Immune Deficiencies (CEREDIH), Necker Children's University Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France.
Stephan EhlInstitute for Immunodeficiency, Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Sabine M El-HelouInstitute for Immunodeficiency, Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Barbara PietruchaDepartment of Immunology, The Children's Memorial Health Institute, Av. Dzieci Polskich 20, 04-730, Warsaw, Poland.
Alessandro PlebaniPediatrics Clinic and Institute for Molecular Medicine A. Nocivelli, Department of Clinical and Experimental Sciences, University of Brescia and ASST-Spedali Civili di Brescia, Brescia, Italy.
Michiel van der FlierSection Pediatric Infectious Diseases, Laboratory of Medical Immunology, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, the Netherlands.
Koen van AerdeDepartment of Pediatrics, Amalia's Children Hospital, Radboud University Medical Center, Nijmegen, the Netherlands.
Sara S KilicDepartment of Pediatric Immunology and Rheumatology, the School of Medicine, Uludag University, Bursa, Turkey.
Shereen M RedaDepartment of Pediatrics, Children's Hospital, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Larysa KostyuchenkoCenter of Pediatric Immunology, Western Ukrainian Specialized Children's Medical Centre, Lviv, Ukraine.
Elizabeth McDermottClinical Immunology and Allergy Unit, Nottingham University Hospitals, Nottingham, UK.
Nermeen GalalDepartment of Pediatrics, Cairo University Specialized Pediatric Hospital, Cairo, Egypt.
Claudio PignataDepartment of Translational Medical Sciences, Section of Pediatrics, Federico II University, Naples, Italy.
Juan Luis Santos PérezInfectious Diseases and Immunodeficiencies Unit, Service of Pediatrics, Hospital Universitario Virgen de Las Nieves, Granada, Spain.
Hans-Juergen LawsDepartment of Pediatric Oncology, Hematology and Clinical Immunology, Medical Faculty, Center of Child and Adolescent Health, Heinrich-Heine University, Duesseldorf, Germany.
Tim NiehuesCentre for Child and Adolescent Health, Helios Klinikum Krefeld, Krefeld, Germany.
Necil KutukculerFaculty of Medicine, Department of Pediatric Immunology, Ege University, Izmir, Turkey.
Markus G SeidelResearch Unit for Pediatric Hematology and Immunology, Division of Pediatric Hemato-Oncology, Department of Pediatrics and Adolescent Medicine, Medical University Graz, Graz, Austria.
Laura MarquesPediatric Department, Infectious Diseases and Immunodeficiencies Unit, Porto Hospital Center, Porto, Portugal.
Peter CiznarPediatric Department, Faculty of Medicine, Children University Hospital in Bratislava, Comenius University in Bratislava, Bratislava, Slovakia.
John David M EdgarThe Royal Hospitals & Queen's University, Belfast, UK.
Pere Soler-PalacínPediatric Infectious Diseases and Immunodeficiencies Unit, Vall D'Hebron Research Institute, Hospital Universitari Vall D'Hebron, Universitat Autònoma de Barcelona, Barcelona, Catalonia, Spain.
Horst von BernuthDepartment of Pediatric Pneumology, Immunology and Intensive Care, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Renate KruegerDepartment of Pediatric Pneumology, Immunology and Intensive Care, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Isabelle MeytsDepartment of Pediatrics, University Hospitals Leuven, and the Laboratory for Inborn Errors of Immunity, Department of Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium.
Ulrich BaumannDepartment of Paediatric Pulmonology, Allergy and Neonatology, Hannover Medical School, Hannover, Germany.
Maria KanariouDepartment of Immunology and Histocompatibility, Centre for Primary Immunodeficiencies, "Aghia Sophia" Children's Hospital, Athens, Greece.
Bodo GrimbacherInstitute for Immunodeficiency, Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Fabian HauckDepartment of Pediatrics, Dr. Von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-University Munich, Munich, Germany.
Dagmar GrafMVZ Dr. Reising-Ackermann Und Kollegen, Leipzig, Germany.
Luis Ignacio Gonzalez GranadoPrimary Immunodeficiencies Unit, Pediatrics, Hospital 12 Octubre, Complutense University School of Medicine, Madrid, Spain.
Seraina PraderDivision of Immunology and Children's Research Center, University Children's Hospital Zurich, Zurich, Switzerland.
Ismail ReisliDepartment of Pediatrics, Division of Pediatric Immunology and Allergy, Meram Medical Faculty, Necmettin Erbakan University, Konya, Turkey.
Mary SlatterPrimary Immunodeficiency Group, Paediatric Immunology and Haematopoietic Stem Cell Transplantation, Translational and Clinical Research Institute, Great North Childrens' Hospital, Newcastle University, Newcastle upon Tyne, UK.
Carlos Rodríguez-GallegoDepartment of Immunology, Dr. Negrin University Hospital of Gran Canaria, University Fernando Pessoa Canarias, Las Palmas de Gran Canaria, Spain.
Peter D ArkwrightLydia Becker Institute of Immunology and Inflammation, University of Manchester and Royal Manchester Children's Hospital, Manchester, UK.
Claire BethuneUniversity Hospital Plymouth NHS Trust, Plymouth, UK.
Elena DeripapaNational Medical Research Center of Pediatric Hematology, Oncology and Immunology, Moscow, Russia.
Svetlana O SharapovaResearch Department, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Minsk region, Minsk, Belarus.
Kai LehmbergDivision for Pediatric Stem Cell Transplantation and Immunology, Clinic for Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
E Graham DaviesGreat Ormond Street Hospital and UCL Great Ormond Street Institute of Child Health, London, UK.
Catharina SchuetzDepartment of Pediatrics, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
Gerhard KindleInstitute for Immunodeficiency, Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.
Ralf SchubertDivision of Allergology, Pulmonology and Cystic Fibrosis, Department for Children and Adolescents, Goethe University, Frankfurt, Germany.
Goethe University Frankfurt · DEUniversity of Freiburg · DECharité - Universitätsmedizin Berlin · DEAin Shams University Hospital · EGAssistance Publique – Hôpitaux de Paris · FRAzienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia · ITBelarusian Research Center For Pediatric Oncology and Hematology · BYBursa Uludağ Üni̇versi̇tesi̇ · TRCairo University · EGChildren's Hospital Agia Sophia · GRChildren's Memorial Health Institute · PLComenius University Bratislava · SKCUF Porto Hospital · PTEge University · TRFederico II University Hospital · ITGreat North Children's Hospital · GBHeinrich Heine University Düsseldorf · DEHelios Klinikum Krefeld · DEHospital Universitario Virgen de las Nieves · ESKU Leuven · BE

Funding

Department of Health
6 · The paper itself

Abstract

Patients with ataxia-telangiectasia (A-T) suffer from progressive cerebellar ataxia, immunodeficiency, respiratory failure, and cancer susceptibility. From a clinical point of view, A-T patients with IgA deficiency show more symptoms and may have a poorer prognosis. In this study, we analyzed mortality and immunity data of 659 A-T patients with regard to IgA deficiency collected from the European Society for Immunodeficiencies (ESID) registry and from 66 patients with classical A-T who attended at the Frankfurt Goethe-University between 2012 and 2018. We studied peripheral B- and T-cell subsets and T-cell repertoire of the Frankfurt cohort and survival rates of all A-T patients in the ESID registry. Patients with A-T have significant alterations in their lymphocyte phenotypes. All subsets (CD3, CD4, CD8, CD19, CD4/CD45RA, and CD8/CD45RA) were significantly diminished compared to standard values. Patients with IgA deficiency (n = 35) had significantly lower lymphocyte counts compared to A-T patients without IgA deficiency (n = 31) due to a further decrease of naïve CD4 T-cells, central memory CD4 cells, and regulatory T-cells. Although both patient groups showed affected TCR-ß repertoires compared to controls, no differences could be detected between patients with and without IgA deficiency. Overall survival of patients with IgA deficiency was significantly diminished. For the first time, our data show that patients with IgA deficiency have significantly lower lymphocyte counts and subsets, which are accompanied with reduced survival, compared to A-T patients without IgA deficiency. IgA, a simple surrogate marker, is indicating the poorest prognosis for classical A-T patients. Both non-interventional clinical trials were registered at clinicaltrials.gov 2012 (Susceptibility to infections in ataxia-telangiectasia; NCT02345135) and 2017 (Susceptibility to Infections, tumor risk and liver disease in patients with ataxia-telangiectasia; NCT03357978).

Indexed as

AdolescentAdultAtaxia TelangiectasiaB-LymphocytesChildChild, PreschoolFemaleHumansIgA DeficiencyIgG DeficiencyImmunoglobulin AImmunoglobulin GImmunoglobulin MInfantLymphocyte CountMaleImmunoglobulin AImmunoglobulin GImmunoglobulin MAtaxia-telangiectasiaIgA deficiencyImmunodeficiencyImmunoglobulinsLymphopeniaMortality

Identifiers

PMID34477998
PMCPMC8604875
OpenAlexW3198181838

What OpenQuestion holds

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LicenceCC BY
Read underepoch 390

Registered trials

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.