Evidence map›Paper›PMID 34114123›Full record

ArticleJournal of clinical immunology2021

Single-Center Study of 72 Patients with Severe Combined Immunodeficiency: Clinical and Laboratory Features and Outcomes.

Ozlem Bayram, Sule Haskologlu, Deniz Bayrakoğlu, Sevgi Kostel Bal, Candan Islamoglu, Funda Erol Cipe, Tanil Kendirli, Nazmiye Kursun, Sukru Nail Guner, Alisan Yildiran and 8 more

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In one paragraph

Article in Journal of clinical immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed, 1 pooled it
0.7field-weighted citation impact, top 32% 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

5 citing papers in PubMed, 1 synthesis or guideline pooled it, 11 citations in OpenAlex.

  1. Pooled it
  2. Article
  3. Review
  4. Article
  5. 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

18 authors at 8 institutions in 2 countries.

Ozlem BayramDepartment of Pediatric Immunology and Allergy, BMT Unit, Ankara University Medical School, Mamak Street, Dikimevi, 06100, Ankara, Turkey. drbayram.ozlem@gmail.com.ORCID http://orcid.org/0000-0002-3298-5567
Sule HaskologluDepartment of Pediatric Immunology and Allergy, BMT Unit, Ankara University Medical School, Mamak Street, Dikimevi, 06100, Ankara, Turkey.
Deniz BayrakoğluDepartment of Pediatric Immunology and Allergy, BMT Unit, Ankara University Medical School, Mamak Street, Dikimevi, 06100, Ankara, Turkey.
Sevgi Kostel BalDepartment of Pediatric Immunology and Allergy, BMT Unit, Ankara University Medical School, Mamak Street, Dikimevi, 06100, Ankara, Turkey.
Candan IslamogluDepartment of Pediatric Immunology and Allergy, BMT Unit, Ankara University Medical School, Mamak Street, Dikimevi, 06100, Ankara, Turkey.
Funda Erol CipeDepartment of Pediatrics, Istinye University Faculty of Medicine, Istanbul, Turkey.
Tanil KendirliDepartment of Pediatric Intensive Care Unit, Ankara University Medical School, Ankara, Turkey.
Nazmiye KursunDepartment of Biostatistics, Ankara University Medical School, Ankara, Turkey.
Sukru Nail GunerDepartment of Pediatric Immunology and Allergy, Necmettin Erbakan University Meram Medical School, Konya, Turkey.
Alisan YildiranDepartment of Pediatric Immunology and Allergy, Ondokuz Mayis University Medical School, Samsun, Turkey.
Gunseli BozdoganDepartment of Pediatric Immunology and Allergy, Acibadem University, Istanbul, Turkey.
Mutlu YuksekDepartment of Pediatric Immunology and Allergy, Bulent Ecevit University Medical School, Zonguldak, Turkey.
Ismail ReisliDepartment of Pediatric Immunology and Allergy, Necmettin Erbakan University Meram Medical School, Konya, Turkey.
Klara DalvaDepartment of Hematology, Stem Cell Institute, Ankara University Medical School, Ankara, Turkey.
Caner AytekinDepartment of Pediatric Immunology, Dr. Sami Ulus Maternity and Children's, Research and Educational Hospital, Ankara, Turkey.
Kaan BoztugLudwig Boltzmann Institute for Rare and Undiagnosed Diseases, Vienna, Austria.
Figen Dogu *Department of Pediatric Immunology and Allergy, BMT Unit, Ankara University Medical School, Mamak Street, Dikimevi, 06100, Ankara, Turkey.
Aydan Ikinciogullari *Department of Pediatric Immunology and Allergy, BMT Unit, Ankara University Medical School, Mamak Street, Dikimevi, 06100, Ankara, Turkey.
Ankara University · TRNecmettin Erbakan University · TRAcıbadem University · TRAustrian Academy of Sciences · ATBülent Ecevit University · TRIstinye University · TRMinistry of Health · TROndokuz Mayıs University · TR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Severe combined immunodeficiency is an inborn error of immunity characterized by impairments in the numbers and functions of T and B lymphocytes due to various genetic causes, and if it remains untreated, patients succumb to infections during the first 2 years of life. PURPOSE AND

methodsThis study reported retrospective data from 72 infants diagnosed with SCID including their major clinical features, HSCT characteristics, and outcomes over a 20-year period (1997-2017).

resultsSixty-one of 72 SCID patients in the study underwent HSCT from 1997 to 2017. Median ages at the time of diagnosis and transplantation were 3.5 months and 5 months, respectively. Consanguinity was present in 68% of the patients, and T - B - NK + phenotype was predominantly identified. The overall survival was 80.3% over a 20-year period. However, the patients transplanted during an active infection had a lower survival rate of 73.9% compared to 100% for patients transplanted infection-free or with a previous infection that had resolved. The survival rate was significantly higher among recipients of HLA-identical transplants (92.9%), compared to recipients of mismatched related transplants (70%). The overall survival increased from 50 (1997-2006) to 85% (2007-2017) during the last 10 years.

conclusionsThis is one of the largest single-center studies in Turkey with extensive experience about SCID patients. Early diagnosis of SCID patients before the onset of an infection and early transplantation are shown to be extremely important factors affecting the outcome and increasing the survival regardless of the donor type based on the results of this study.

Indexed as

Severe Combined ImmunodeficiencyB-LymphocytesFemaleHematopoietic Stem Cell TransplantationHumansInfantKaplan-Meier EstimateKiller Cells, NaturalMaleRetrospective StudiesT-LymphocytesTreatment OutcomeTurkeyclinical featuresHematopoietic stem cell transplantaion (HSCT)outcomesSevere combined immunodeficiency (SCID)

Identifiers

PMID34114123
OpenAlexW3166641614

What OpenQuestion holds

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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.