ArticlePediatric pulmonology2025
Reliability of Composite Autonomic Symptom Score (COMPASS)-31 in Congenital Central Hypoventilation Syndrome.
Article in Pediatric pulmonology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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.
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.
Who cites it
2 citing papers in PubMed.
- Redefining PHOX2B-Related Neurodevelopmental Disorders as Circuitopathies: A New Paradigm in Autonomic Network Medicine.Neuroscience bulletin · 2026Review
- International Congenital Central Hypoventilation Syndrome (CCHS) Registry: Analysis of Patient-Reported Symptoms by PHOX2B Variant.Pediatric pulmonology · 2026Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors.
Funding
Abstract
rationaleCongenital central hypoventilation syndrome (CCHS) is a rare disorder characterized by alveolar hypoventilation and variable autonomic nervous system (ANS) dysfunction (ANSD) due to mutations in PHOX2B, a gene crucial for ANS neural crest lineage differentiation. OBJECTIVES AND
methodsOur prospective study aims were twofold: to (1) assess the relationships between the subjective Composite Autonomic Symptom Score (COMPASS)-31 and objective indices of ANSD obtained from heart rate variability analyses, ambulatory blood pressure (BP) monitoring, and CO
resultsThirty-two PHOX2B mutation-confirmed subjects (median [range] age 9.2 years (4.4; 18.0), 15 girls) were enrolled. COMPASS-31 was assessed in 32 matched (sex and age, range: 4.3; 18.9 years) healthy controls. As compared to healthy controls, children with CCHS had increased vasomotor (p = 0.001), secretomotor (p = 0.021), gastrointestinal (p = 0.002) and pupillomotor (p = 0.028) scores and decreased orthostatic intolerance scores (p = 0.050). There was no difference in overall COMPASS-31 score between CCHS and controls (p = 0.083). However, in CCHS, overall COMPASS-31 scores correlated with high frequencies (HF) normalized (cardiac parasympathetic modulation: R = -0.53; p = 0.002), low frequencies (LF)/HF ratio (R = 0.56; p< 0.001), and both systolic and diastolic nighttime BP dipping (R = 0.45, p = 0.012 and R = 0.40, p = 0.028, respectively). No significant relationships between COMPASS-31 scores and chemosensitivity testing, PedsQL scores, or PHOX2B genotype were identified.
conclusionsCOMPASS-31 identified some aspects of CCHS-related ANSD, and scores correlate with objective ANS function measures, supporting the potential utility of COMPASS-31 in CCHS.
Indexed as
Identifiers
What OpenQuestion holds
Registered trials
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.