Neurobehavioral Concerns among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network

Kristin Caspers Conway, Katherine D. Mathews, Pangaja Paramsothy, Joyce Oleszek, Christina Trout, Ying Zhang, Paul A. Romitti

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

Objective: To describe the occurrence of selected neurobehavioral concerns among males with a dystrophinopathy and to explore the associations with corticosteroid or supportive device use. Methods: Medical record abstraction of neurobehavioral concerns was conducted for 857 affected males from 765 families, born since 1982 and followed through 2011, and enrolled in the population-based Muscular Dystrophy Surveillance, Tracking, and Research Network. Cumulative probabilities for attention-deficit hyperactivity disorder (ADHD), behavior problems, and depressed mood were calculated from Kaplan-Meier estimates for the subsample of oldest affected males (n 765). Hazard ratios (HRs) and 95% confidence intervals (95% CIs) for corticosteroid and supportive device use were estimated from Cox regression models with time-dependent covariates. Results: Of the 857 affected males, 375 (44%) had at least 1 of the 3 selected neurobehavioral concerns; a similar percentage (45%) was found among the 765 oldest affected males. The estimated cumulative probabilities among these oldest affected males were 23% for ADHD, 43% for behavior problems, and 51% for depressed mood. Corticosteroid (HR 2.35, 95% CI 1.75-3.16) and mobility device (HR 1.53, 95% CI 1.06-2.21) use were associated with behavior problems. Use of a mobility device (HR 3.53, 95% CI 2.13-5.85), but not corticosteroids, was associated with depressed mood. ADHD was not significantly associated with corticosteroid or mobility device use. Respiratory assist device use was not examined due to low numbers of users before onset of neurobehavioral concerns. Conclusion: Selected neurobehavioral concerns were common among males with a dystrophinopathy. Reported associations highlight the importance of increased monitoring of neurobehavioral concerns as interventions are implemented and disease progresses.

Original languageEnglish (US)
Pages (from-to)455-463
Number of pages9
JournalJournal of Developmental and Behavioral Pediatrics
Volume36
Issue number6
DOIs
StatePublished - Jul 20 2015

Fingerprint

Population Surveillance
Muscular Dystrophies
Adrenal Cortex Hormones
Equipment and Supplies
Research
Attention Deficit Disorder with Hyperactivity
Confidence Intervals
Kaplan-Meier Estimate
Proportional Hazards Models
Medical Records

Keywords

  • ADHD
  • dystrophinopathy
  • neurobehavioral
  • neuromuscular disorder

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Developmental and Educational Psychology
  • Psychiatry and Mental health

Cite this

Neurobehavioral Concerns among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network. / Conway, Kristin Caspers; Mathews, Katherine D.; Paramsothy, Pangaja; Oleszek, Joyce; Trout, Christina; Zhang, Ying; Romitti, Paul A.

In: Journal of Developmental and Behavioral Pediatrics, Vol. 36, No. 6, 20.07.2015, p. 455-463.

Research output: Contribution to journalArticle

Conway, Kristin Caspers ; Mathews, Katherine D. ; Paramsothy, Pangaja ; Oleszek, Joyce ; Trout, Christina ; Zhang, Ying ; Romitti, Paul A. / Neurobehavioral Concerns among Males with Dystrophinopathy Using Population-Based Surveillance Data from the Muscular Dystrophy Surveillance, Tracking, and Research Network. In: Journal of Developmental and Behavioral Pediatrics. 2015 ; Vol. 36, No. 6. pp. 455-463.
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abstract = "Objective: To describe the occurrence of selected neurobehavioral concerns among males with a dystrophinopathy and to explore the associations with corticosteroid or supportive device use. Methods: Medical record abstraction of neurobehavioral concerns was conducted for 857 affected males from 765 families, born since 1982 and followed through 2011, and enrolled in the population-based Muscular Dystrophy Surveillance, Tracking, and Research Network. Cumulative probabilities for attention-deficit hyperactivity disorder (ADHD), behavior problems, and depressed mood were calculated from Kaplan-Meier estimates for the subsample of oldest affected males (n 765). Hazard ratios (HRs) and 95{\%} confidence intervals (95{\%} CIs) for corticosteroid and supportive device use were estimated from Cox regression models with time-dependent covariates. Results: Of the 857 affected males, 375 (44{\%}) had at least 1 of the 3 selected neurobehavioral concerns; a similar percentage (45{\%}) was found among the 765 oldest affected males. The estimated cumulative probabilities among these oldest affected males were 23{\%} for ADHD, 43{\%} for behavior problems, and 51{\%} for depressed mood. Corticosteroid (HR 2.35, 95{\%} CI 1.75-3.16) and mobility device (HR 1.53, 95{\%} CI 1.06-2.21) use were associated with behavior problems. Use of a mobility device (HR 3.53, 95{\%} CI 2.13-5.85), but not corticosteroids, was associated with depressed mood. ADHD was not significantly associated with corticosteroid or mobility device use. Respiratory assist device use was not examined due to low numbers of users before onset of neurobehavioral concerns. Conclusion: Selected neurobehavioral concerns were common among males with a dystrophinopathy. Reported associations highlight the importance of increased monitoring of neurobehavioral concerns as interventions are implemented and disease progresses.",
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AU - Paramsothy, Pangaja

AU - Oleszek, Joyce

AU - Trout, Christina

AU - Zhang, Ying

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AB - Objective: To describe the occurrence of selected neurobehavioral concerns among males with a dystrophinopathy and to explore the associations with corticosteroid or supportive device use. Methods: Medical record abstraction of neurobehavioral concerns was conducted for 857 affected males from 765 families, born since 1982 and followed through 2011, and enrolled in the population-based Muscular Dystrophy Surveillance, Tracking, and Research Network. Cumulative probabilities for attention-deficit hyperactivity disorder (ADHD), behavior problems, and depressed mood were calculated from Kaplan-Meier estimates for the subsample of oldest affected males (n 765). Hazard ratios (HRs) and 95% confidence intervals (95% CIs) for corticosteroid and supportive device use were estimated from Cox regression models with time-dependent covariates. Results: Of the 857 affected males, 375 (44%) had at least 1 of the 3 selected neurobehavioral concerns; a similar percentage (45%) was found among the 765 oldest affected males. The estimated cumulative probabilities among these oldest affected males were 23% for ADHD, 43% for behavior problems, and 51% for depressed mood. Corticosteroid (HR 2.35, 95% CI 1.75-3.16) and mobility device (HR 1.53, 95% CI 1.06-2.21) use were associated with behavior problems. Use of a mobility device (HR 3.53, 95% CI 2.13-5.85), but not corticosteroids, was associated with depressed mood. ADHD was not significantly associated with corticosteroid or mobility device use. Respiratory assist device use was not examined due to low numbers of users before onset of neurobehavioral concerns. Conclusion: Selected neurobehavioral concerns were common among males with a dystrophinopathy. Reported associations highlight the importance of increased monitoring of neurobehavioral concerns as interventions are implemented and disease progresses.

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