RESUMO
Childhood dementia is a devastating and under-recognized group of disorders with a high level of unmet need. Typically monogenic in origin, this collective of individual neurodegenerative conditions are defined by a progressive impairment of neurocognitive function, presenting in childhood and adolescence. This scoping review aims to clarify definitions and conceptual boundaries of childhood dementia and quantify the collective disease burden. A literature review identified conditions that met the case definition. An expert clinical working group reviewed and ratified inclusion. Epidemiological data were extracted from published literature and collective burden modelled. One hundred and seventy genetic childhood dementia disorders were identified. Of these, 25 were analysed separately as treatable conditions. Collectively, currently untreatable childhood dementia was estimated to have an incidence of 34.5 per 100 000 (1 in 2900 births), median life expectancy of 9 years and prevalence of 5.3 per 100 000 persons. The estimated number of premature deaths per year is similar to childhood cancer (0-14 years) and approximately 70% of those deaths will be prior to adulthood. An additional 49.8 per 100 000 births are attributable to treatable conditions that would cause childhood dementia if not diagnosed early and stringently treated. A relational database of the childhood dementia disorders has been created and will be continually updated as new disorders are identified (https://knowledgebase.childhooddementia.org/). We present the first comprehensive overview of monogenic childhood dementia conditions and their collective epidemiology. Unifying these conditions, with consistent language and definitions, reinforces motivation to advance therapeutic development and health service supports for this significantly disadvantaged group of children and their families.
Assuntos
Demência , Neoplasias , Doenças Neurodegenerativas , Criança , Adolescente , Humanos , Efeitos Psicossociais da Doença , Prevalência , Demência/epidemiologiaAssuntos
Financiamento Governamental , Serviços de Saúde para Idosos/economia , Instituições Residenciais/economia , Idoso , Austrália , Serviços de Saúde para Idosos/tendências , Serviços de Assistência Domiciliar/economia , Serviços de Assistência Domiciliar/tendências , Instituição de Longa Permanência para Idosos/economia , Humanos , Casas de Saúde/economiaRESUMO
The Australian Technical Advisory Group on Immunisation (ATAGI) 2023 Annual Statement on Immunisation is the third publication in this series. It highlights the key successes, trends and challenges in the use of vaccines and control of vaccine preventable diseases (VPDs) in Australia in 2022. It also signals ATAGI's priority actions for addressing key issues for 2023 and beyond.