24-h continuous non-invasive multiparameter home monitoring of vitals in patients with Rett syndrome by an innovative wearable technology: evidence of an overlooked chronic fatigue status.

Rett syndrome cardiorespiratory monitoring exercise fatigue sleep–wake cycle wearable devices

Journal

Frontiers in neurology
ISSN: 1664-2295
Titre abrégé: Front Neurol
Pays: Switzerland
ID NLM: 101546899

Informations de publication

Date de publication:
2024
Historique:
received: 19 02 2024
accepted: 13 05 2024
medline: 2 7 2024
pubmed: 2 7 2024
entrez: 2 7 2024
Statut: epublish

Résumé

Sleep is disturbed in Rett syndrome (RTT), a rare and progressive neurodevelopmental disorder primarily affecting female patients (prevalence 7.1/100,000 female patients) linked to pathogenic variations in the X-linked methyl-CpG-binding protein 2 ( A total of 10 female patients (mean age 18.3 ± 9.4 years, range 4.7-35.5 years) with typical RTT and Of the 6,559.79 h of biovital recordings, 5051.03 h (77%) were valid for data interpretation. Sleep and wake hours were 9.0 ± 1.1 h and 14.9 ± 1.1 h, respectively. HRmax % [median: 71.86% (interquartile range 61.03-82%)] and HR/LF [median: 3.75 (interquartile range 3.19-5.05)] were elevated, independent from the wake-sleep cycle. The majority of HRV time- and frequency-domain parameters were significantly higher in the pediatric patients ( Our findings indicate the feasibility of a continuous 24-h non-invasive home monitoring of biovital parameters in RTT. Moreover, for the first time, HRmax% and the HR/LF ratio were identified as potential objective markers of fatigue, illness severity, and disease progression.

Sections du résumé

Background UNASSIGNED
Sleep is disturbed in Rett syndrome (RTT), a rare and progressive neurodevelopmental disorder primarily affecting female patients (prevalence 7.1/100,000 female patients) linked to pathogenic variations in the X-linked methyl-CpG-binding protein 2 (
Methods UNASSIGNED
A total of 10 female patients (mean age 18.3 ± 9.4 years, range 4.7-35.5 years) with typical RTT and
Results UNASSIGNED
Of the 6,559.79 h of biovital recordings, 5051.03 h (77%) were valid for data interpretation. Sleep and wake hours were 9.0 ± 1.1 h and 14.9 ± 1.1 h, respectively. HRmax % [median: 71.86% (interquartile range 61.03-82%)] and HR/LF [median: 3.75 (interquartile range 3.19-5.05)] were elevated, independent from the wake-sleep cycle. The majority of HRV time- and frequency-domain parameters were significantly higher in the pediatric patients (
Conclusion UNASSIGNED
Our findings indicate the feasibility of a continuous 24-h non-invasive home monitoring of biovital parameters in RTT. Moreover, for the first time, HRmax% and the HR/LF ratio were identified as potential objective markers of fatigue, illness severity, and disease progression.

Identifiants

pubmed: 38952469
doi: 10.3389/fneur.2024.1388506
pmc: PMC11215834
doi:

Types de publication

Journal Article

Langues

eng

Pagination

1388506

Informations de copyright

Copyright © 2024 Leoncini, Boasiako, Di Lucia, Beker, Scandurra, Vignoli, Canevini, Prato, Nobili, Nicotera, Di Rosa, Chiarini, Cutrera, Grosso, Lazzeri, Tongiorgi, Morano, Botteghi, Barducci and De Felice.

Déclaration de conflit d'intérêts

The co-author AmB is an advisor of Accyourate Group SpA, a sponsor of the research. PM was employed by Croce Rossa Italiana. AlB was employed by Italian Color Solutions I.C.S. S.r.l. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision.

Auteurs

Silvia Leoncini (S)

Rett Syndrome Trial Center, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Neonatal Intensive Care Unit, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.
U.O.S.A. Programmazione e Ricerca Clinica, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.

Lidia Boasiako (L)

Rett Syndrome Trial Center, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Neonatal Intensive Care Unit, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.

Sofia Di Lucia (S)

Neonatal Intensive Care Unit, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.

Amir Beker (A)

Accyourate Group SpA, L'Aquila, Italy.

Valeria Scandurra (V)

Child Neuropsychiatry Unit, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.

Aglaia Vignoli (A)

Childhood and Adolescence Neurology and Psychiatry Unit, ASST GOM Niguarda, Milan, Italy.

Maria Paola Canevini (MP)

Epilepsy Center - Sleep Medicine Center, Childhood and Adolescence Neuropsychiatry Unit, ASST Santi Paolo e Carlo, San Paolo Hospital, Milan, Italy.

Giulia Prato (G)

Child Neuropsychiatry Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy.

Lino Nobili (L)

Child Neuropsychiatry Unit, IRCCS Istituto Giannina Gaslini, Genova, Italy.
Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics and Maternal and Child Health (DINOGMI), University of Genova, Genova, Italy.

Antonio Gennaro Nicotera (AG)

Child Neuropsychiatry Unit, University Hospital "G. Martino", Messina, Italy.

Gabriella Di Rosa (G)

Child Neuropsychiatry Unit, University Hospital "G. Martino", Messina, Italy.
Department of Biomedical and Dental Sciences and of Morphological and Functional Imaging (BIOMORF), University of Messina, Messina, Italy.

Maria Beatrice Testa Chiarini (MBT)

Pneumology and Cystic Fibrosis Unit, Academic Department of Pediatrics, Bambino Gesù Children's Hospital, Rome, Italy.

Renato Cutrera (R)

Pneumology and Cystic Fibrosis Unit, Academic Department of Pediatrics, Bambino Gesù Children's Hospital, Rome, Italy.

Salvatore Grosso (S)

Department of Molecular and Developmental Medicine, University of Siena, Siena, Italy.
Pediatrics Unit, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.

Giacomo Lazzeri (G)

U.O.S.A. Programmazione e Ricerca Clinica, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Department of Molecular and Developmental Medicine, University of Siena, Siena, Italy.

Enrico Tongiorgi (E)

Department of Life Sciences, University of Trieste, Trieste, Italy.

Pasquale Morano (P)

Croce Rossa Italiana, Rome, Italy.

Matteo Botteghi (M)

Department of Clinical and Molecular Sciences - Experimental Pathology Research Group, Università Politecnica delle Marche, Ancona, Italy.
Medical Physics Activities Coordination Centre - Alma Mater Studiorum - University of Bologna, Bologna, Italy.

Alessandro Barducci (A)

Italian Color Solutions I.C.S. S.r.l., Pistoia, Italy.

Claudio De Felice (C)

Rett Syndrome Trial Center, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Neonatal Intensive Care Unit, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.
Pediatrics Unit, University Hospital Azienda Ospedaliera Universitaria Senese, Siena, Italy.

Classifications MeSH