Evidence map›Paper›PMID 35451701›Full record

ArticleJournal of clinical immunology2022

Clinical and Laboratory Factors Affecting the Prognosis of Severe Combined Immunodeficiency.

Elif Ozturk, Mehmet Cihangir Catak, Ayca Kiykim, Dilek Baser, Sevgi Bilgic Eltan, Koray Yalcin, Nurhan Kasap, Ercan Nain, Alper Bulutoglu, Gamze Akgun and 9 more

Erratum issuedAbstract read
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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. An erratum has been issued. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing 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

5 citing papers in PubMed.

  1. Article
  2. Article
  3. Article
  4. Review
  5. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

19 authors.

Elif OzturkFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Mehmet Cihangir CatakFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Ayca KiykimFaculty of Medicine, Pediatric Allergy and Immunology, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Dilek BaserFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Sevgi Bilgic EltanFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Koray YalcinPediatric Bone Marrow Transplantation Unit, Medical Park Goztepe Hospital, Istanbul, Turkey.
Nurhan KasapFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Ercan NainFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Alper BulutogluFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Gamze AkgunFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Yasemin CanFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Asena Pinar SeferFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Royala BabayevaFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Suar Caki-KilicDivision of Pediatric Hematology, Umraniye Education and Research Hospital, University of Health Sciences, Istanbul, Turkey.
Gulsun Tezcan KarasuPediatric Bone Marrow Transplantation Unit, Medical Park Goztepe Hospital, Istanbul, Turkey.
Akif YesilipekPediatric Bone Marrow Transplantation Unit, Medical Park Goztepe Hospital, Istanbul, Turkey.
Ahmet OzenFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Elif Karakoc-AydinerFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey.
Safa BarisFaculty of Medicine, Division of Pediatric Allergy and Immunology, Marmara University, Fevzi Çakmak Mah. No: 41, Pendik, Istanbul, Turkey. safabaris@hotmail.com.ORCID http://orcid.org/0000-0002-4730-9422

Funding

Marmara Üniversitesi SAG-C-TUP-250919-0288
6 · The paper itself

Abstract

purposeSevere combined immunodeficiency (SCID) is one of the most severe forms of inborn errors of immunity characterized by absence or loss of function in T cells. The long-term outcomes of all forms of SCID have been evaluated in a limited number of studies. We aimed to evaluate the pre- and post-transplant manifestations of SCID patients and determine the factors affecting the survival of patients.

methodsWe included 54 SCID patients (classical SCID, Omenn syndrome, atypical SCID (AS)) in this study. We evaluated the clinical presentation, infections, and outcome of hematopoietic stem cell transplantation (HSCT). Lymphocyte subsets and T-cell receptor (TCR) repertoire were analyzed by flow cytometry.

resultsThe median age at diagnosis was 5 (range: 3-24) months and follow-up time was 25 (range: 5-61) months. Symptom onset and diagnostic ages were significantly higher in AS compared to others (p = 0.001; p < 0.001). The most common SCID phenotype was T-B-NK + , and mutations in recombination-activating genes (RAG1/2) were the prominent genetic defect among patients. The overall survival (OS) rate was 83.3% after HSCT, higher than in non-transplanted patients (p = 0.001). Peripheral blood stem cell sources and genotypes other than RAG had a significant favorable impact on CD4

conclusionThis study identifies diagnostic and therapeutic approaches predictive of favorable outcomes for patients with SCID.

Indexed as

Hematopoietic Stem Cell TransplantationSevere Combined ImmunodeficiencyChild, PreschoolHumansInfantPrognosisReceptors, Antigen, T-CellTransplantation ConditioningReceptors, Antigen, T-CellBone marrow transplantationImmune reconstitutionPrognosisSevere combined immunodeficiencyT-cell receptor repertoire

Identifiers

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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.