Evidence map›Paper›PMID 31526690›Full record

ArticlePediatric neurology2019

Quality of Life in Children With Sturge-Weber Syndrome.

Kelly A Harmon, Alyssa M Day, Adrienne M Hammill, Anna L Pinto, Charles E McCulloch, Anne M Comi, National Institutes of Health Rare Disease Clinical Research Consortium (RDCRN) Brain and Vascular Malformation Consortium (BVMC) SWS Investigator Group

Open access · greenAbstract read
In one paragraph

Article in Pediatric neurology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.

0numbers the graph read from it
0cells of the map it votes in
16citing papers in PubMed
2.7field-weighted citation impact, top 11% 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

16 citing papers in PubMed, 29 citations in OpenAlex.

  1. Review
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  10. Development of the PROMIS pediatric stigma and extension to the PROMIS pediatric stigma: skin item banks.Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation · 2024
    Article
  11. Review
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  16. Sturge-Weber syndrome: an update on the relevant issues for neurosurgeons.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2020
    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

7 authors at 4 institutions in 1 country.

Kelly A HarmonDepartment of Neurology, Kennedy Krieger Institute, Baltimore, Maryland.
Alyssa M DayDepartment of Neurology, Kennedy Krieger Institute, Baltimore, Maryland.
Adrienne M HammillDivision of Hematology, Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Anna L PintoDepartment of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts.
Charles E McCullochDepartment of Epidemiology and Biostatistics, University of California, San Francisco, San Francisco, California.
Anne M ComiDepartment of Neurology, Kennedy Krieger Institute, Baltimore, Maryland; Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland; Department of Pediatrics, Johns Hopkins School of Medicine, Baltimore, Maryland. Electronic address: comi@kennedykrieger.org.
National Institutes of Health Rare Disease Clinical Research Consortium (RDCRN) Brain and Vascular Malformation Consortium (BVMC) SWS Investigator Group
Kennedy Krieger Institute · USUniversity of California, San Francisco · USHarvard University · USUniversity of Cincinnati Medical Center · US

Funding

The Impact of COVID-19 on People Living with Rare Diseases and Their FamiliesU2CTR002818 · NCATS · CINCINNATI CHILDRENS HOSP MED CTR · PI Michal Kouril · 2019 to 2026
$51.2M
VCRC Administration UnitU54NS065705 · NINDS · UNIVERSITY OF CALIFORNIA, SAN FRANCISCO · PI KIM, HELEN · 2009 to 2023
$20.8M
NCATS NIH HHS U2C TR002818NINDS NIH HHS U54 NS065705
6 · The paper itself

Abstract

aimWe assessed the utilization of the National Institutes of Health Quality of Life in Neurological Disorders (Neuro-QoL) in pediatric patients with Sturge-Weber syndrome, a rare neurovascular disorder which frequently results in seizures, brain atrophy, calcification, and a range of neurological impairments.

methodsSubjects were seen clinically and consented for research. All 22 patients filled out the Pediatric Neuro-QoL. The Neuro-QoL subscores were converted to T-scores to compare with the referenced control population. Twenty-one participants also filled out the Brain Vascular Malformation Consortium Database Questionnaire containing data pertaining to Sturge-Weber syndrome-related medical history, medications, comorbidities, and family history. All data were analyzed with a significance threshold of P < 0.05.

resultsCognitive function quality of life was significantly lower (P < 0.001) in pediatric patients with Sturge-Weber syndrome compared with referenced control subjects. Male gender (P = 0.02) was associated with lower cognitive function Neuro-QoL. The extent of skin (R = -0.46, P = 0.04), total eyelid port-wine birthmark (R = -0.56, P = 0.007), eye (R = -0.58, P = 0.005), and total Sturge-Weber syndrome involvement (R = -0.63, P = 0.002) were negatively correlated with cognitive function Neuro-QoL. A younger age at seizure onset was associated with lower cognitive function Neuro-QoL (hazard ratio = 0.90, P = 0.004) even after controlling for extent of brain, skin, or eye involvement. Antidepressant use was associated with lower cognitive function Neuro-QoL (P = 0.005), and cognitive function Neuro-QoL was negatively correlated with depression Neuro-QoL; however, after adjusting for depression this relationship was no longer significant.

conclusionsThe results suggest targeting cognitive function Neuro-QoL in treatment trials and reiterate the prognostic value of early seizure onset. In addition, sex-related differences were noted, which should be further studied.

Indexed as

AdolescentAnticonvulsantsChildCognitionFemaleHealth SurveysHumansMaleQuality of LifeSex FactorsSturge-Weber SyndromeAnticonvulsantsG protein subunit alpha qLeptomeningeal enhancementNational Institutes of Health ToolboxPort-wine birthmarkQuality of life in neurological disordersSturge-Weber syndromeVascular malformation

Identifiers

PMID31526690
PMCPMC7288751
OpenAlexW2941319874

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.