Phenotyping Down syndrome: discovery and predictive modelling with electronic medical records.

Background: Individuals with Down syndrome (DS) have a heightened risk for various co‐occurring health conditions, including congenital heart disease (CHD). In this two‐part study, electronic medical records (EMRs) were leveraged to examine co‐occurring health conditions among individuals with DS (S...

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Publicado en:Journal of Intellectual Disability Research Vol. 68; no. 5; pp. 491 - 512
Autores principales: Nguyen, T. Q., Kerley, C. I., Key, A. P., Maxwell‐Horn, A. C., Wells, Q. S., Neul, J. L., Cutting, L. E., Landman, B. A.
Formato: research tables/charts Journal Article
Publicado: Wiley-Blackwell May2024
Acceso en línea:Ver este registro en EBSCOhost
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      dt: May2024
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        atl: Phenotyping Down syndrome: discovery and predictive modelling with electronic medical records.
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        au:
          Nguyen, T. Q.
          Kerley, C. I.
          Key, A. P.
          Maxwell‐Horn, A. C.
          Wells, Q. S.
          Neul, J. L.
          Cutting, L. E.
          Landman, B. A.
        affil: Vanderbilt Brain Institute, Vanderbilt University, Nashville TN,, USA
      sug:
        subj:
          Phenotype
          Down Syndrome Therapy
          Down Syndrome Complications
          Heart Defects, Congenital Risk Factors
          Comorbidity Evaluation
          Risk Assessment
          Electronic Health Records
          Human
          Funding Source
          Male
          Female
          Infant, Newborn
          Infant
          Child
          Adolescence
          Academic Medical Centers
          Heart Failure
          Intellectual Disability
          Prospective Studies
          Descriptive Statistics
          Prediction Models
          Record Review
          Infant, Newborn: birth-1 month
          Infant: 1-23 months
          Child: 6-12 years
          Adolescent: 13-18 years
          Male
          Female
      ab: Background: Individuals with Down syndrome (DS) have a heightened risk for various co‐occurring health conditions, including congenital heart disease (CHD). In this two‐part study, electronic medical records (EMRs) were leveraged to examine co‐occurring health conditions among individuals with DS (Study 1) and to investigate health conditions linked to surgical intervention among DS cases with CHD (Study 2). Methods: De‐identified EMRs were acquired from Vanderbilt University Medical Center and facilitated creating a cohort of N = 2282 DS cases (55% females), along with comparison groups for each study. In Study 1, DS cases were one‐by‐two sex and age matched with samples of case–controls and of individuals with other intellectual and developmental difficulties (IDDs). The phenome‐disease association study (PheDAS) strategy was employed to reveal co‐occurring health conditions in DS versus comparison groups, which were then ranked for how often they are discussed in relation to DS using the PubMed database and Novelty Finding Index. In Study 2, a subset of DS individuals with CHD [N = 1098 (48%)] were identified to create longitudinal data for N = 204 cases with surgical intervention (19%) versus 204 case–controls. Data were included in predictive models and assessed which model‐based health conditions, when more prevalent, would increase the likelihood of surgical intervention. Results: In Study 1, relative to case–controls and those with other IDDs, co‐occurring health conditions among individuals with DS were confirmed to include heart failure, pulmonary heart disease, atrioventricular block, heart transplant/surgery and primary pulmonary hypertension (circulatory); hypothyroidism (endocrine/metabolic); and speech and language disorder and Alzheimer's disease (neurological/mental). Findings also revealed more versus less prevalent co‐occurring health conditions in individuals with DS when comparing with those with other IDDs. Findings with high Novelty Finding Index were abnormal electrocardiogram, non‐rheumatic aortic valve disorders and heart failure (circulatory); acid–base balance disorder (endocrine/metabolism); and abnormal blood chemistry (symptoms). In Study 2, the predictive models revealed that among individuals with DS and CHD, presence of health conditions such as congestive heart failure (circulatory), valvular heart disease and cardiac shunt (congenital), and pleural effusion and pulmonary collapse (respiratory) were associated with increased likelihood of surgical intervention. Conclusions: Research efforts using EMRs and rigorous statistical methods could shed light on the complexity in health profile among individuals with DS and other IDDs and motivate precision‐care development.
      pubtype: Academic Journal
      doctype:
        research
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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