Evidence map›Paper›PMID 32812413›Full record

ArticleTurkish journal of haematology : official journal of Turkish Society of Haematology2020

Clinical Features and Outcomes of 23 Patients with Wiskott-Aldrich Syndrome: A Single-Center Experience

Şule Haskoloğlu, Ayşenur Öztürk, Gökcan Öztürk, Sevgi Kostel Bal, Candan İslamoğlu, Kübra Baskın, Serdar Ceylaner, Lale Tufan Satıroğlu, Figen Doğu, Aydan İkincioğulları

Open access · diamondAbstract read
In one paragraph

Article in Turkish journal of haematology : official journal of Turkish Society of Haematology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
11citing 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

11 citing papers in PubMed, 1 synthesis or guideline pooled it, 12 citations in OpenAlex.

  1. Pooled it
  2. Review
  3. Article
  4. Review
  5. Article
  6. Article
  7. Wiskott-Aldrich syndrome: A new synonym mutation in the WAS gene.Intractable & rare diseases research · 2024
    Article
  8. Article
  9. Autoimmunity in Wiskott-Aldrich Syndrome: Updated Perspectives.The application of clinical genetics · 2021
    Review
  10. Article
  11. Review
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

10 authors at 2 institutions in 1 country.

Şule HaskoloğluAnkara University School of Medicine, Department of Pediatrics, Division of Immunology and Allergy, Ankara, TurkeyORCID 0000-00002-2668-0441
Ayşenur ÖztürkAnkara University School of Medicine, Department of Pediatrics, Division of Genetic Diseases, Ankara, Turkey
Gökcan ÖztürkAnkara University School of Medicine, Department of Pediatrics, Ankara, Turkey
Sevgi Kostel BalAnkara University School of Medicine, Department of Pediatrics, Division of Immunology and Allergy, Ankara, TurkeyORCID 0000-0002-3718-5323
Candan İslamoğluAnkara University School of Medicine, Department of Pediatrics, Division of Immunology and Allergy, Ankara, Turkey
Kübra BaskınAnkara University School of Medicine, Department of Pediatrics, Division of Immunology and Allergy, Ankara, Turkey
Serdar CeylanerIntergen Genetic Diagnosis Center, Ankara, TurkeyORCID 0000-0003-2786-1911
Lale Tufan SatıroğluAnkara University School of Medicine, Department of Pediatrics, Division of Genetic Diseases, Ankara, TurkeyORCID 0000-0001-9399-0960
Figen DoğuAnkara University School of Medicine, Department of Pediatrics, Division of Immunology and Allergy, Ankara, TurkeyORCID 0000-0002-7869-4941
Aydan İkincioğullarıAnkara University School of Medicine, Department of Pediatrics, Division of Immunology and Allergy, Ankara, Turkey
Ankara University · TRIntergen (Turkey) · TR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Objective: Wiskott-Aldrich syndrome (WAS) is an X-linked primary immune deficiency characterized by microthrombocytopenia, eczema, and recurrent infections. We aimed to evaluate the clinical features and outcomes of a WAS cohort. Materials and Methods: We retrospectively evaluated the clinical courses, immunological features, treatments, and outcomes in a total of 23 WAS patients together with data related to 11 transplanted cases among them between 1982 and 2019. Results: Before admission, 11 patients (48%) were misdiagnosed with immune thrombocytopenia. WAS scores were mostly 4 or 5. Eleven patients were transplanted and they had an overall survival rate of 100% during a median follow-up period of 8.5 years (range: 8 months to 20 years). Five patients who were not transplanted died at a median of 7 years (range: 2-26 years). Nontransplanted patients had high morbidity due to organ damage, mostly caused by autoimmunity, bleeding, and infections. Two novel mutations were also defined. Conclusion: All male babies with microthrombocytopenia should be evaluated for WAS. Hematopoietic stem cell transplantation should be performed at the earliest age with the best possible donors.

Indexed as

PhenotypeAdolescentBiomarkersChildChild, PreschoolCombined Modality TherapyDiagnosis, DifferentialDisease ManagementDisease SusceptibilityFemaleHematopoietic Stem Cell TransplantationHumansImmunoglobulins, IntravenousInfantInfant, NewbornMaleBiomarkersImmunoglobulins, IntravenousHematopoietic stem cell transplantationMicrothrombocytopeniaOutcomeWiskott-Aldrich syndrome

Identifiers

PMID32812413
PMCPMC7702658
OpenAlexW3055487170

What OpenQuestion holds

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LicenceCC BY
Read underepoch 390

Registered trials

None linked

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.