Multicentric Retrospective Evaluation of Five Classic Infantile Pompe Disease Subjects Under Enzyme Replacement Therapy With Early Infratentorial Involvement.

ERT (enzyme replacement therapy) IOPD Pompe disease brain MRI infantile-onset Pompe disease white matter (WM)

Journal

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

Informations de publication

Date de publication:
2020
Historique:
received: 04 06 2020
accepted: 20 10 2020
entrez: 17 12 2020
pubmed: 18 12 2020
medline: 18 12 2020
Statut: epublish

Résumé

White matter (WM) abnormalities and ventricular enlargement in brain MRI are well-known features in infantile-onset Pompe disease (IOPD) in the era of enzyme replacement therapy (ERT). In this multicentric observational retrospective study, we report a small cohort of IOPD subjects under ERT treatment (

Identifiants

pubmed: 33329311
doi: 10.3389/fneur.2020.569153
pmc: PMC7732650
doi:

Types de publication

Journal Article

Langues

eng

Pagination

569153

Informations de copyright

Copyright © 2020 Paoletti, Pichiecchio, Colafati, Conte, Deodato, Gasperini, Menni, Furlan, Rubert, Triulzi and Cinnante.

Références

Orphanet J Rare Dis. 2018 Feb 8;13(1):32
pubmed: 29422078
Mol Genet Metab. 2010 Jan;99(1):26-33
pubmed: 19775921
Dev Med Child Neurol. 2018 Jun;60(6):579-586
pubmed: 29573408
Pediatr Neurosurg. 2015;50(1):12-7
pubmed: 25613691
Neurology. 2013 Mar 19;80(12):1173
pubmed: 23509050
J Inherit Metab Dis. 2016 Mar;39(2):261-71
pubmed: 26497565
Mol Genet Metab. 2018 Feb;123(2):85-91
pubmed: 29050825
J Inherit Metab Dis. 2010 Dec;33(6):751-7
pubmed: 20882352
Pediatr Res. 2006 Sep;60(3):349-52
pubmed: 16857770
J Neuropathol Exp Neurol. 1971 Jul;30(3):412-30
pubmed: 5284681
JIMD Rep. 2015;23:45-54
pubmed: 25763511
Pediatr Neurol. 2010 Jun;42(6):455-8
pubmed: 20472203

Auteurs

Matteo Paoletti (M)

Advanced Imaging and Radiomics Center, Neuroradiology Department, Istituto di Ricovero e Cura a Carattere Scientifico Mondino Foundation, Pavia, Italy.

Anna Pichiecchio (A)

Advanced Imaging and Radiomics Center, Neuroradiology Department, Istituto di Ricovero e Cura a Carattere Scientifico Mondino Foundation, Pavia, Italy.
Department of Brain and Behavioral Sciences, University of Pavia, Pavia, Italy.

Giovanna Stefania Colafati (GS)

Oncological Neuroradiology Unit, Imaging Department, Istituto di Ricovero e Cura a Carattere Scientifico Ospedale Pediatrico Bambino Gesù, Rome, Italy.

Giorgio Conte (G)

Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Neuroradiology Unit, Milan, Italy.

Federica Deodato (F)

Unit of Metabolic Disease, Istituto di Ricovero e Cura a Carattere Scientifico Ospedale Pediatrico Bambino Gesù, Rome, Italy.

Serena Gasperini (S)

Pediatric Rare Diseases Unit, Department of Pediatrics, Fondazione Monza e Brianza per il Bambino e la sua Mamma, San Gerardo Hospital, Monza, Italy.

Francesca Menni (F)

Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, University of Milan, Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Francesca Furlan (F)

Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, University of Milan, Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Laura Rubert (L)

Division of Inherited Metabolic Diseases, Department of Diagnostic Services, University Hospital of Padua, Padua, Italy.

Fabio Maria Triulzi (FM)

Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Neuroradiology Unit, Milan, Italy.
Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.

Claudia Cinnante (C)

Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Neuroradiology Unit, Milan, Italy.

Classifications MeSH