Evidence map›Paper›PMID 16722529›Full record

ReviewOrphanet journal of rare diseases2006

Glanzmann thrombasthenia.

Alan T Nurden

Open access · goldAbstract readReview
In one paragraph

Review in Orphanet journal of rare diseases, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 93 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
93citing papers in PubMed, 1 pooled it
4.9field-weighted citation impact, top 4% 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

93 citing papers in PubMed, 1 synthesis or guideline pooled it, 254 citations in OpenAlex.

  1. Pooled it
  2. Article
  3. Review
  4. Article
  5. Review
  6. Article
  7. Article
  8. European Management of Glanzmann's Thrombasthenia: A Survey of Current Clinical Practice.Haemophilia : the official journal of the World Federation of Hemophilia · 2025
    Article
  9. Optogenetic induction of subcellular CaCommunications biology · 2025
    Article
  10. Glanzmann's thrombasthenia: A nightmare for hernia surgeons.Journal of minimal access surgery · 2025
    Article
  11. Outcomes of recombinant activated factor VIIa (NovoSeven) therapy in glanzmann thrombasthenia: two case reports.Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis · 2025
    Article
  12. Ablation of CD38 Alleviates the Secondary Aggregation of Platelets.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2025
    Article
  13. Article
  14. Review
  15. Review
  16. The Impact of Single Nucleotide Polymorphisms and Other Mechanisms on Aspirin Resistance.Cardiovascular & hematological disorders drug targets · 2025
    Review
  17. Article
  18. Article
  19. Article
  20. Article

33 more citing papers are in PubMed but not listed here.

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

1 author at 1 institution in 1 country.

Alan T NurdenIFR No4/CRPP, Laboratoire d'Hématologie, Hôpital Cardiologique, 33604 Pessac, France. Alan.Nurden@cnrshl.u-bordeaux2.fr
Hôpital Cardiologique du Haut-Lévêque · FR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding syndrome affecting the megakaryocyte lineage and characterized by lack of platelet aggregation. The molecular basis is linked to quantitative and/or qualitative abnormalities of alphaIIb beta3 integrin. This receptor mediates the binding of adhesive proteins that attach aggregating platelets and ensure thrombus formation at sites of injury in blood vessels. GT is associated with clinical variability: some patients have only minimal bruising while others have frequent, severe and potentially fatal hemorrhages. The site of bleeding in GT is clearly defined: purpura, epistaxis, gingival hemorrhage, and menorrhagia are nearly constant features; gastrointestinal bleeding and hematuria are less common. In most cases, bleeding symptoms manifest rapidly after birth, even if GT is occasionally only diagnosed in later life. Diagnosis should be suspected in patients with mucocutaneous bleeding with absent platelet aggregation in response to all physiologic stimuli, and a normal platelet count and morphology. Platelet alphaIIb beta3 deficiency or nonfunction should always be confirmed, for example by flow cytometry. In order to avoid platelet alloimmunisation, therapeutic management must include, if possible, local hemostatic procedures and/or desmopressin (DDAVP) administration. Transfusion of HLA-compatible platelet concentrates may be necessary if these measures are ineffective, or to prevent bleeding during surgery. Administration of recombinant factor VIIa is an increasingly used therapeutic alternative. GT can be a severe hemorrhagic disease, however the prognosis is excellent with careful supportive care.

Indexed as

Blood PlateletsFactor VIIaHemorrhageHumansIntegrin beta3Platelet TransfusionThrombastheniaFactor VIIaIntegrin beta3

Identifiers

PMID16722529
PMCPMC1475837
OpenAlexW4248176343

What OpenQuestion holds

Textmetadata
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.