Evidence map›Paper›PMID 30204261›Full record

ArticleEuropean journal of haematology2018

Rheological properties of sickle erythrocytes in patients with sickle-cell anemia: The effect of hydroxyurea, fetal hemoglobin, and α-thalassemia.

Samir K Ballas, Philippe Connes, Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia

Abstract read
In one paragraph

Article in European journal of haematology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
12citing papers in PubMed, 2 pooled it
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

12 citing papers in PubMed, 2 syntheses or guidelines pooled it, 27 citations in OpenAlex.

  1. Pooled it
  2. Hydroxyurea (hydroxycarbamide) for sickle cell disease.The Cochrane database of systematic reviews · 2022
    Pooled it
  3. Article
  4. Article
  5. Article
  6. Article
  7. Article
  8. Article
  9. Article
  10. The Evolving Pharmacotherapeutic Landscape for the Treatment of Sickle Cell Disease.Mediterranean journal of hematology and infectious diseases · 2020
    Review
  11. Article
  12. 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

3 authors at 2 institutions in 2 countries.

Samir K BallasDepartment of Medicine, Cardeza Foundation for Hematologic Research, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, Pennsylvania.
Philippe ConnesLaboratoire LIBM EA7424, Equipe "Biologie Vasculaire et du Globule Rouge", Université Claude Bernard Lyon 1, Villeurbanne, France.ORCID http://orcid.org/0000-0002-9232-0268
Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia
Thomas Jefferson University · USUniversité Claude Bernard Lyon 1 · FR

Funding

UTILIZATION OF TRANSGENIC MICE FOR STUDIES IN SICKLE CELL DISEASEP60HL038632 · NHLBI · CHILDREN'S HOSPITAL OF PHILADELPHIA · PI OHENE-FREMPONG, KWAKU · 1988 to 2002
$6.4M
MULTICENTER STUDY OF HYDROXYUREA IN SICKLE CELL ANEMIAU01HL045692 · NHLBI · JOHNS HOPKINS UNIVERSITY · PI MOORE, RICHARD DOUGLAS · 1991 to 1995
–
NHLBI NIH HHS P60 HL038632NHLBI NIH HHS U01 HL045692NIH Comprehensive Sickle Cell Center HL38632NIH Multicenter Study of Hydroxyurea in Sickle Cell Anaemia Grant HL 45692
6 · The paper itself

Abstract

objectiveDetermine the effect of fetal hemoglobin (HbF) and α-thalassemia on red blood cell (RBC) deformability of patients with sickle-cell anemia (SCA) with and without hydroxyurea (HU).

methodsAdult patients were enrolled in the Sickle Cell Program of the Cardeza Foundation (Thomas Jefferson University) and were followed up prospectively during the period in which the Multicenter Study of Hydroxyurea (MSH) in patients with SCA was conducted. Ninety-one patients did not receive HU, 20 patients were enrolled in MSH, and 10 patients were enrolled in an open-label study of HU in SCA. Of the 20 patients enrolled in MSH, 11 took HU and nine took placebo. Control group included 113 normal individuals. Red blood cell deformability index (DI) was measured by ektacytometry.

resultsPatients with SCA taking HU (n = 21) had higher DI than those taking placebo (n = 9) or who were not taking this therapy (n = 91). In patients without therapy, those with α-thalassemia (n = 31) had higher DI than those without. We showed a significant positive correlation between the level of HbF and DI. SCA patients without α-thalassemia and HbF <10% (n = 48) had lower DI than patients with α-thalassemia and HbF <10% (n = 23) and patients with (n = 8) or without α-thalassemia but with HbF >10% (n = 12). DI measured in patients without α-thalassemia and HbF >10% was higher than in the three other subgroups.

conclusionElevated levels of HbF with or without HU and α-thalassemia improve sickle RBC rheology, which, in turn, improve the clinical picture of SCA.

Indexed as

alpha-Globinsalpha-ThalassemiaAnemia, Sickle CellBiomarkersErythrocyte IndicesErythrocytes, AbnormalFetal HemoglobinGenotypeHemorheologyHumansHydroxyureaTreatment Outcomealpha-GlobinsBiomarkersFetal HemoglobinHydroxyureahydroxyureared blood cell deformabilitysickle-cell anemiaα-thalassemia

Identifiers

PMID30204261
PMCPMC6224298
OpenAlexW2891600339

What OpenQuestion holds

Textmetadata
LicenceTDM
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.