Development and Validation of a Diagnostic Algorithm for Down Syndrome Using Birth Certificate and International Classification of Diseases Codes

Objective: We aimed to develop an algorithm that accurately identifies children with Down syndrome (DS) using administrative data. Methods: We identified a cohort of children born between 2000 and 2017, enrolled in the Tennessee Medicaid Program (TennCare), who either had DS coded on their birth cer...

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Main Authors: Lin Ammar, Kristin Bird, Hui Nian, Angela Maxwell-Horn, Rees Lee, Tan Ding, Corinne Riddell, Tebeb Gebretsadik, Brittney Snyder, Tina Hartert, Pingsheng Wu
Format: Article
Language:English
Published: MDPI AG 2024-10-01
Series:Children
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Online Access:https://www.mdpi.com/2227-9067/11/10/1271
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author Lin Ammar
Kristin Bird
Hui Nian
Angela Maxwell-Horn
Rees Lee
Tan Ding
Corinne Riddell
Tebeb Gebretsadik
Brittney Snyder
Tina Hartert
Pingsheng Wu
author_facet Lin Ammar
Kristin Bird
Hui Nian
Angela Maxwell-Horn
Rees Lee
Tan Ding
Corinne Riddell
Tebeb Gebretsadik
Brittney Snyder
Tina Hartert
Pingsheng Wu
author_sort Lin Ammar
collection DOAJ
description Objective: We aimed to develop an algorithm that accurately identifies children with Down syndrome (DS) using administrative data. Methods: We identified a cohort of children born between 2000 and 2017, enrolled in the Tennessee Medicaid Program (TennCare), who either had DS coded on their birth certificate or had a diagnosis listed using an International Classification of Diseases (ICD) code (suspected DS), and who received care at Vanderbilt University Medical Center, a comprehensive academic medical center, in the United States. Children with suspected DS were defined as having DS if they had (a) karyotype-confirmed DS indicated on their birth certificate; (b) karyotype-pending DS indicated on their birth certificate (or just DS if test type was not specified) and at least two healthcare encounters for DS during the first 6 years of life; or (c) at least three healthcare encounters for DS, with the first and last encounter separated by at least 30 days, during the first six years of life. The positive predictive value (PPV) of the algorithm and 95% confidence interval (CI) were reported. Results: Of the 411 children with suspected DS, 354 (86.1%) were defined as having DS by the algorithm. According to medical chart review, the algorithm correctly identified 347 children with DS (PPV = 98%, 95%CI: 96.0–99.0%). Of the 57 children the algorithm defined as not having DS, 50 (97.7%, 95%CI: 76.8–93.9%) were confirmed as not having DS by medical chart review. Conclusions: An algorithm that accurately identifies individuals with DS using birth certificate data and/or ICD codes provides a valuable tool to study DS using administrative data.
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spelling doaj-art-8fe8ab867b5d430eab07b73f01d80d4c2025-08-20T02:11:03ZengMDPI AGChildren2227-90672024-10-011110127110.3390/children11101271Development and Validation of a Diagnostic Algorithm for Down Syndrome Using Birth Certificate and International Classification of Diseases CodesLin Ammar0Kristin Bird1Hui Nian2Angela Maxwell-Horn3Rees Lee4Tan Ding5Corinne Riddell6Tebeb Gebretsadik7Brittney Snyder8Tina Hartert9Pingsheng Wu10Division of Epidemiology, Vanderbilt University School of Medicine, Nashville, TN 37232, USADepartment of Pediatrics, Vanderbilt University Medical Center, Nashville, TN 37232, USADepartment of Biostatistics, Vanderbilt University Medical Center, Nashville, TN 37232, USADepartment of Pediatrics, Vanderbilt University Medical Center, Nashville, TN 37232, USADepartment of Pediatrics, College of Medicine, University of Arizona, Tucson, AZ 85721, USADepartment of Biostatistics, Vanderbilt University Medical Center, Nashville, TN 37232, USADivision of Biostatistics and Epidemiology, School of Public Health, University of California, Berkeley, CA 94720, USADepartment of Biostatistics, Vanderbilt University Medical Center, Nashville, TN 37232, USADepartment of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232, USADepartment of Pediatrics, Vanderbilt University Medical Center, Nashville, TN 37232, USADepartment of Biostatistics, Vanderbilt University Medical Center, Nashville, TN 37232, USAObjective: We aimed to develop an algorithm that accurately identifies children with Down syndrome (DS) using administrative data. Methods: We identified a cohort of children born between 2000 and 2017, enrolled in the Tennessee Medicaid Program (TennCare), who either had DS coded on their birth certificate or had a diagnosis listed using an International Classification of Diseases (ICD) code (suspected DS), and who received care at Vanderbilt University Medical Center, a comprehensive academic medical center, in the United States. Children with suspected DS were defined as having DS if they had (a) karyotype-confirmed DS indicated on their birth certificate; (b) karyotype-pending DS indicated on their birth certificate (or just DS if test type was not specified) and at least two healthcare encounters for DS during the first 6 years of life; or (c) at least three healthcare encounters for DS, with the first and last encounter separated by at least 30 days, during the first six years of life. The positive predictive value (PPV) of the algorithm and 95% confidence interval (CI) were reported. Results: Of the 411 children with suspected DS, 354 (86.1%) were defined as having DS by the algorithm. According to medical chart review, the algorithm correctly identified 347 children with DS (PPV = 98%, 95%CI: 96.0–99.0%). Of the 57 children the algorithm defined as not having DS, 50 (97.7%, 95%CI: 76.8–93.9%) were confirmed as not having DS by medical chart review. Conclusions: An algorithm that accurately identifies individuals with DS using birth certificate data and/or ICD codes provides a valuable tool to study DS using administrative data.https://www.mdpi.com/2227-9067/11/10/1271Down syndromeadministrative databasesInternational Classification of Diseasesbirth certificate
spellingShingle Lin Ammar
Kristin Bird
Hui Nian
Angela Maxwell-Horn
Rees Lee
Tan Ding
Corinne Riddell
Tebeb Gebretsadik
Brittney Snyder
Tina Hartert
Pingsheng Wu
Development and Validation of a Diagnostic Algorithm for Down Syndrome Using Birth Certificate and International Classification of Diseases Codes
Children
Down syndrome
administrative databases
International Classification of Diseases
birth certificate
title Development and Validation of a Diagnostic Algorithm for Down Syndrome Using Birth Certificate and International Classification of Diseases Codes
title_full Development and Validation of a Diagnostic Algorithm for Down Syndrome Using Birth Certificate and International Classification of Diseases Codes
title_fullStr Development and Validation of a Diagnostic Algorithm for Down Syndrome Using Birth Certificate and International Classification of Diseases Codes
title_full_unstemmed Development and Validation of a Diagnostic Algorithm for Down Syndrome Using Birth Certificate and International Classification of Diseases Codes
title_short Development and Validation of a Diagnostic Algorithm for Down Syndrome Using Birth Certificate and International Classification of Diseases Codes
title_sort development and validation of a diagnostic algorithm for down syndrome using birth certificate and international classification of diseases codes
topic Down syndrome
administrative databases
International Classification of Diseases
birth certificate
url https://www.mdpi.com/2227-9067/11/10/1271
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