Literature DB >> 32989270

(Un)standardized testing: the diagnostic odyssey of children with rare genetic disorders in Alberta, Canada.

Christine Michaels-Igbokwe1,2, Brenda McInnes3, Karen V MacDonald4, Gillian R Currie5,4,6,7, Fadya Omar3, Brittany Shewchuk4, Francois P Bernier3,7, Deborah A Marshall4,6,7.   

Abstract

PURPOSE: We provide a description of the diagnostic odyssey for a cohort of children seeking diagnosis of a rare genetic disorder in terms of the time from initial consultation to most recent visit or receipt of diagnosis, the number of tests per patient, and the types of tests received.
METHODS: Retrospective chart review of 299 children seen at the Alberta Children's Hospital (ACH) Genetics Clinic (GC) for whom the result of at least one single-gene test, gene panel, or chromosome microarray analysis (CMA) was recorded.
RESULTS: Of 299 patients, 90 (30%) received a diagnosis in the period of the review. Patients had an average of 5.4 tests each; 236 (79%) patients received CMA; 172 (58%) patients received single-gene tests and 34 (11%) received gene panels; 167 (56%) underwent imaging/electrical activity studies. The mean observation period was 898 days (95% confidence interval [CI] 791, 1004). Among patients with visits recorded prior to visiting ACH GC, 43% of the total observation time occurred prior to the GC.
CONCLUSION: As genomic technologies expand, the nature of the diagnostic odyssey will change. This study has outlined the current standard of care in the ACH GC, providing a baseline against which future changes can be assessed.

Entities:  

Keywords:  children; diagnostic odyssey; genetic disorders; pediatric

Mesh:

Year:  2020        PMID: 32989270     DOI: 10.1038/s41436-020-00975-0

Source DB:  PubMed          Journal:  Genet Med        ISSN: 1098-3600            Impact factor:   8.822


  5 in total

1.  A Collaborative Psychiatric-Genetics Inpatient Care Delivery Model Improves Access to Clinical Genetic Evaluation, Testing, and Diagnosis for Patients With Neurodevelopmental Disorders.

Authors:  Amelle Shillington; Martine Lamy; Kelli C Dominick; Michael Sorter; Craig A Erickson; Robert Hopkin
Journal:  Front Genet       Date:  2022-06-13       Impact factor: 4.772

2.  Recommendations from the IRDiRC Working Group on methodologies to assess the impact of diagnoses and therapies on rare disease patients.

Authors:  Galliano Zanello; Chun-Hung Chan; David A Pearce
Journal:  Orphanet J Rare Dis       Date:  2022-05-07       Impact factor: 4.303

Review 3.  DNA Methylation Episignatures in Neurodevelopmental Disorders Associated with Large Structural Copy Number Variants: Clinical Implications.

Authors:  Kathleen Rooney; Bekim Sadikovic
Journal:  Int J Mol Sci       Date:  2022-07-16       Impact factor: 6.208

Review 4.  GENE TARGET: A framework for evaluating Mendelian neurodevelopmental disorders for gene therapy.

Authors:  Maya Chopra; Meera E Modi; Kira A Dies; Nancy L Chamberlin; Elizabeth D Buttermore; Stephanie Jo Brewster; Lisa Prock; Mustafa Sahin
Journal:  Mol Ther Methods Clin Dev       Date:  2022-08-29       Impact factor: 5.849

5.  Positioning whole exome sequencing in the diagnostic pathway for rare disease to optimise utility: a protocol for an observational cohort study and an economic evaluation.

Authors:  Robin Z Hayeems; Francois Bernier; Kym M Boycott; Taila Hartley; Christine Michaels-Igbokwe; Deborah A Marshall
Journal:  BMJ Open       Date:  2022-10-10       Impact factor: 3.006

  5 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.