Evidence map›Paper›PMID 19308710›Full record

ArticleJournal of clinical immunology2009

Clinical and molecular characteristics of 35 Chinese children with Wiskott-Aldrich syndrome.

Pamela P W Lee, Tong-Xin Chen, Li-Ping Jiang, Jing Chen, Koon-Wing Chan, Tze-Leung Lee, Marco H K Ho, Shao-Han Nong, Yin Yang, Yong-Jun Fang and 4 more

Abstract read
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In one paragraph

Article in Journal of clinical immunology, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
12citing papers in PubMed, 1 pooled it
0.8field-weighted citation impact, top 30% 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

12 citing papers in PubMed, 1 synthesis or guideline pooled it, 21 citations in OpenAlex.

  1. Pooled it
  2. Article
  3. Myelin oligodendrocyte glycoprotein antibody-associated disease as a novel presentation of central nervous system autoimmunity in a pediatric patient with Wiskott-Aldrich syndrome.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2023
    Article
  4. Article
  5. Review
  6. Article
  7. Autoimmunity in Wiskott-Aldrich Syndrome: Updated Perspectives.The application of clinical genetics · 2021
    Review
  8. Article
  9. Vitiligo following stem-cell transplant.Bone marrow transplantation · 2020
    Review
  10. WhenFrontiers in immunology · 2019
    Article
  11. Article
  12. 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

14 authors at 9 institutions in 2 countries.

Pamela P W LeeDepartment of Paediatrics and Adolescent Medicine, The University of Hong Kong, Hong Kong, SAR, China.
Tong-Xin Chen
Li-Ping Jiang
Jing Chen
Koon-Wing Chan
Tze-Leung Lee
Marco H K Ho
Shao-Han Nong
Yin Yang
Yong-Jun Fang
Qiang Li
Xiao-Chun Wang
Xi-Qiang Yang
Yu-Lung Lau
University of Hong Kong · HKChinese University of Hong Kong · CNNanjing Children's Hospital · CNShanghai Jiao Tong University · CNChildren's Hospital of Chongqing Medical University · CNChildren's Hospital of Fudan University · CNChongqing Medical University · CNGuangdong Provincial People's Hospital · CNSichuan University · CN

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundWiskott-Aldrich syndrome (WAS) is a rare primary immunodeficiency disease, with an incidence of 4/1,000,000 live male births. In China, an estimated number of 35 babies with WAS are born each year, but likely many remain undiagnosed.

objectivesThe objectives of study were to review the clinical and molecular characteristics of a cohort of Chinese children with WAS and to describe the long-term outcome of those who underwent hematopoietic stem cell transplant (HSCT). MATERIALS AND

methodRecords of 35 patients diagnosed with WAS during 1991-2008 were reviewed. Genetic diagnosis was established by direct gene sequencing.

resultsAll patients had classical WAS phenotype. WASP mutations were identified in 33 patients from 29 families. Nine patients underwent HSCT at a mean age of 22.1 months (match-unrelated donor, n = 5; mismatched related donor, n = 2; matched-sibling donor, n = 2). Post-transplant immune hemolytic anemia and thrombocytopenia occurred in three patients with complete resolution. All patients survived without significant long-term complications and had full platelet, T and B lymphocyte recovery within 2 years post-transplant.

conclusionIn the past decade, there has been significant improvement in clinical and genetic diagnosis of WAS in Chinese. We demonstrated excellent long-term survival in patients who underwent HSCT. Early workup for transplant should be advocated for children with classical WAS before they suffer from major disease complications and morbidities.

Indexed as

ChildChinaExonsHematopoietic Stem Cell TransplantationHumansImmunoglobulinsInfantInfant, NewbornIntronsMaleMutationWiskott-Aldrich SyndromeImmunoglobulins

Identifiers

PMID19308710
OpenAlexW2014625236

What OpenQuestion holds

Textmetadata
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.