Improving the care of children with GENetic Rare disease: Observational Cohort study (GenROC)-a study protocol

BMJ Open. 2024 May 16;14(5):e085237. doi: 10.1136/bmjopen-2024-085237.

Abstract

Introduction: Around 2000 children are born in the UK per year with a neurodevelopmental genetic syndrome with significantly increased morbidity and mortality. Often little is known about expected growth and phenotypes in these children. Parents have responded by setting up social media groups to generate data themselves. Given the significant clinical evidence gaps, this research will attempt to identify growth patterns, developmental profiles and phenotypes, providing data on long-term medical and educational outcomes. This will guide clinicians when to investigate, monitor or treat symptoms and when to search for additional or alternative diagnoses.

Methods and analysis: This is an observational, multicentre cohort study recruiting between March 2023 and February 2026. Children aged 6 months up to 16 years with a pathogenic or likely pathogenic variant in a specified gene will be eligible. Children will be identified through the National Health Service and via self-recruitment. Parents or carers will complete a questionnaire at baseline and again 1 year after recruitment. The named clinician (in most cases a clinical geneticist) will complete a clinical proforma which will provide data from their most recent clinical assessment. Qualitative interviews will be undertaken with a subset of parents partway through the study. Growth and developmental milestone curves will be generated through the DECIPHER website (https://deciphergenomics.org) where 5 or more children have the same genetic syndrome (at least 10 groups expected).

Ethics and dissemination: The results will be presented at national and international conferences concerning the care of children with genetic syndromes. Results will also be submitted for peer review and publication.

Keywords: community child health; paediatric clinical genetics & dysmorphology; paediatric neurology.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cohort Studies
  • Female
  • Genetic Diseases, Inborn / therapy
  • Humans
  • Infant
  • Male
  • Multicenter Studies as Topic
  • Neurodevelopmental Disorders / genetics
  • Observational Studies as Topic
  • Parents
  • Quality Improvement
  • Rare Diseases* / genetics
  • Rare Diseases* / therapy
  • Research Design
  • United Kingdom