Evidence map›Paper›PMID 37206292›Full record

ReviewEJHaem2023

VHrare study: Prevalence, clinical features and management of severe rare bleeding disorders in a large cohort.

Olga Benítez Hidalgo, Maria Fernanda Martinez Garcia, Irene Corrrales Insa, Mariana Fernández-Caballero, Lorena Ramírez Orihuela, Vicente Cortina Giner, Natàlia Comes Fernández, Juan Carlos Juarez Gimenez

Open access · diamondAbstract readReview
In one paragraph

Review in EJHaem, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
1.7field-weighted citation impact, top 15% 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

2 citing papers in PubMed, 6 citations in OpenAlex.

  1. Article
  2. 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

8 authors at 2 institutions in 1 country.

Olga Benítez HidalgoHematology Department Hospital Universitari Vall d'Hebron, Experimental Hematology, Vall d'Hebron Institute of Oncology (VHIO), Vall d'Hebron Barcelona Hospital Campus Barcelona Spain.ORCID https://orcid.org/0000-0002-2883-9049
Maria Fernanda Martinez GarciaHematology Department Hospital Universitari Vall d'Hebron, Experimental Hematology, Vall d'Hebron Institute of Oncology (VHIO), Vall d'Hebron Barcelona Hospital Campus Barcelona Spain.
Irene Corrrales InsaCongenital Coagulopaties Banc de Sang i Teixits Barcelona Spain.
Mariana Fernández-CaballeroLaboratory of Haematology, ICO-Badalona, Germans Trias i Pujol University Hospital, Institut Josep Carreras Contra la Leucemia Universitat Autònoma de Barcelona Barcelona Spain.
Lorena Ramírez OrihuelaCongenital Coagulopaties Banc de Sang i Teixits Barcelona Spain.
Vicente Cortina GinerHematology Department Hemostasis Laboratory, Vall d´Hebron Hospital Barcelona Spain.
Natàlia Comes FernándezCongenital Coagulopaties Banc de Sang i Teixits Barcelona Spain.
Juan Carlos Juarez GimenezPharmacy Department Hospital Universitari Vall d'Hebron Barcelona Spain.
Universitat Autònoma de Barcelona · ESVall d'Hebron Hospital Universitari · ES

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionRare bleeding disorders (RBD) constitute 5% of total hereditary bleeding disorders, although the number could be higher, due to the presence of undiagnosed asymptomatic patients. The objective of this study was to analyze the prevalence and characteristics of patients with severe RBDs in our area. MATERIAL AND

methodsWe analyzed the patients with RBD followed at a tertiary-level hospital between January 2014 and December 2021.

resultsA total of 101 patients were analyzed, with a median age at diagnosis of 27.67 years (range 0-89), of which 52.47% were male. The most frequent RBD in our population was FVII deficiency. Regarding the diagnostic reason, the most frequent cause was a preoperative test and only 14.8% reported bleeding symptoms at the time of diagnosis. A genetic study was carried out in 63.36% of patients and the most frequent mutation type found was finding a missense mutation.

conclusionsThe distribution of RBDs in our centre is similar to the one reported in the literature. The majority of RBDs were diagnosed from a preoperative test and this allowed preventive treatment prior to invasive procedures to avoid bleeding complications. 83% of patients did not have a pathological bleeding phenotype according to ISTH-BAT.

Indexed as

bleeding phenotypediagnosismanagementrare bleeding disorders

Identifiers

PMID37206292
PMCPMC10188473
OpenAlexW4323661928

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.