Evidence map›Paper›PMID 31618931›Full record

ReviewJournal of clinical medicine2019

Vaso-Occlusion in Sickle Cell Disease: Is Autonomic Dysregulation of the Microvasculature the Trigger?

Saranya Veluswamy, Payal Shah, Christopher C Denton, Patjanaporn Chalacheva, Michael C K Khoo, Thomas D Coates

Open access · goldAbstract readReview
In one paragraph

Review in Journal of clinical medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.

0numbers the graph read from it
0cells of the map it votes in
21citing papers in PubMed
3.0field-weighted citation impact, top 8% 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

21 citing papers in PubMed, 42 citations in OpenAlex.

  1. Trial
  2. Article
  3. Crosstalk Between Sickle Cell Disease and Ferroptosis.International journal of molecular sciences · 2025
    Review
  4. Article
  5. Article
  6. Article
  7. Review
  8. Article
  9. Article
  10. Article
  11. Precision, integrative medicine for pain management in sickle cell disease.Frontiers in pain research (Lausanne, Switzerland) · 2023
    Review
  12. Review
  13. Topical Vapocoolant-Associated Vaso-occlusive Event in a 10-year-old with Sickle Cell Disease.Pain management nursing : official journal of the American Society of Pain Management Nurses · 2021
    Article
  14. Article
  15. Sickle cell vaso-occlusion: The dialectic between red cells and white cells.Experimental biology and medicine (Maywood, N.J.) · 2021
    Review
  16. Review
  17. Article
  18. Review
  19. Article
  20. [Pain management in sickle cell disease].Schmerz (Berlin, Germany) · 2020
    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

6 authors at 2 institutions in 1 country.

Saranya VeluswamyHematology Section, Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, 4650 Sunset boulevard, Los Angeles, CA 90027, USA. sveluswamy@chla.usc.edu.
Payal ShahHematology Section, Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, 4650 Sunset boulevard, Los Angeles, CA 90027, USA. pmshah@chla.usc.edu.
Christopher C DentonHematology Section, Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, 4650 Sunset boulevard, Los Angeles, CA 90027, USA. chdenton@chla.usc.edu.
Patjanaporn ChalachevaDepartment of Biomedical Engineering, University of Southern California, 1042 Downey Way, Los Angeles, CA 90089, USA. pchalach@andrew.cmu.edu.
Michael C K KhooDepartment of Biomedical Engineering, University of Southern California, 1042 Downey Way, Los Angeles, CA 90089, USA.
Thomas D CoatesHematology Section, Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, 4650 Sunset boulevard, Los Angeles, CA 90027, USA. tcoates@chla.usc.edu.ORCID 0000-0001-9878-6029
Children's Hospital of Los Angeles · USUniversity of Southern California · US

Funding

TrainingP41EB001978 · NIBIB · UNIVERSITY OF SOUTHERN CALIFORNIA · PI KHOO, MICHAEL C K · 2003 to 2017
$18.1M
Neuromodulatory Mechanisms and Regional Autonomic Dysregulation in the Development of Pain in Sickle Cell Disease PatientsU01HL117718 · NHLBI · CHILDREN'S HOSPITAL OF LOS ANGELES · PI COATES, THOMAS DUANE, KHOO, MICHAEL C K · 2013 to 2017
$9.6M
NHLBI NIH HHS U01 HL117718NIBIB NIH HHS P41 EB001978
6 · The paper itself

Abstract

Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by polymerization of hemoglobin S upon deoxygenation that results in the formation of rigid sickled-shaped red blood cells that can occlude the microvasculature, which leads to sudden onsets of pain. The severity of vaso-occlusive crises (VOC) is quite variable among patients, which is not fully explained by their genetic and biological profiles. The mechanism that initiates the transition from steady state to VOC remains unknown, as is the role of clinically reported triggers such as stress, cold and pain. The rate of hemoglobin S polymerization after deoxygenation is an important determinant of vaso-occlusion. Similarly, the microvascular blood flow rate plays a critical role as fast-moving red blood cells are better able to escape the microvasculature before polymerization of deoxy-hemoglobin S causes the red cells to become rigid and lodge in small vessels. The role of the autonomic nervous system (ANS) activity in VOC initiation and propagation has been underestimated considering that the ANS is the major regulator of microvascular blood flow and that most triggers of VOC can alter the autonomic balance. Here, we will briefly review the evidence supporting the presence of ANS dysfunction in SCD, its implications in the onset of VOC, and how differences in autonomic vasoreactivity might potentially contribute to variability in VOC severity.

Indexed as

autonomic nervous system dysfunctionmicrovascular blood flowpainsickle cell diseasevaso-occlusive crisis

Identifiers

PMID31618931
PMCPMC6832215
OpenAlexW2981140282

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.