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Autoimmune polyglandular syndrome type 4: experience from a single reference center

PURPOSE: To characterize patients with APS type 4 among those affected by APS diagnosed and monitored at our local Reference Center for Autoimmune Polyglandular Syndromes. METHODS: Monocentric observational retrospective study enrolling patients affected by APS diagnosed and monitored in a Reference...

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Autores principales: Gatta, Elisa, Anelli, Valentina, Cimino, Elena, Di Lodovico, Elena, Piovani, Elda, Zammarchi, Irene, Gozzoli, Giorgia, Maltese, Virginia, Cavadini, Maria, Agosti, Barbara, Delbarba, Andrea, Pirola, Ilenia, Girelli, Angela, Buoso, Caterina, Bambini, Francesca, Alfieri, Daniele, Bremi, Walter, Facondo, Paolo, Lupo, Roberto, Bezzi, Francesco, Fredi, Micaela, Mazzola, Anna Maria, Gandossi, Elena, Saullo, Maura, Marini, Fiorella, Licini, Massimo, Pezzaioli, Letizia Chiara, Pini, Laura, Franceschini, Franco, Ricci, Chiara, Cappelli, Carlo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10627240/
https://www.ncbi.nlm.nih.gov/pubmed/37937054
http://dx.doi.org/10.3389/fendo.2023.1236878
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author Gatta, Elisa
Anelli, Valentina
Cimino, Elena
Di Lodovico, Elena
Piovani, Elda
Zammarchi, Irene
Gozzoli, Giorgia
Maltese, Virginia
Cavadini, Maria
Agosti, Barbara
Delbarba, Andrea
Pirola, Ilenia
Girelli, Angela
Buoso, Caterina
Bambini, Francesca
Alfieri, Daniele
Bremi, Walter
Facondo, Paolo
Lupo, Roberto
Bezzi, Francesco
Fredi, Micaela
Mazzola, Anna Maria
Gandossi, Elena
Saullo, Maura
Marini, Fiorella
Licini, Massimo
Pezzaioli, Letizia Chiara
Pini, Laura
Franceschini, Franco
Ricci, Chiara
Cappelli, Carlo
author_facet Gatta, Elisa
Anelli, Valentina
Cimino, Elena
Di Lodovico, Elena
Piovani, Elda
Zammarchi, Irene
Gozzoli, Giorgia
Maltese, Virginia
Cavadini, Maria
Agosti, Barbara
Delbarba, Andrea
Pirola, Ilenia
Girelli, Angela
Buoso, Caterina
Bambini, Francesca
Alfieri, Daniele
Bremi, Walter
Facondo, Paolo
Lupo, Roberto
Bezzi, Francesco
Fredi, Micaela
Mazzola, Anna Maria
Gandossi, Elena
Saullo, Maura
Marini, Fiorella
Licini, Massimo
Pezzaioli, Letizia Chiara
Pini, Laura
Franceschini, Franco
Ricci, Chiara
Cappelli, Carlo
author_sort Gatta, Elisa
collection PubMed
description PURPOSE: To characterize patients with APS type 4 among those affected by APS diagnosed and monitored at our local Reference Center for Autoimmune Polyglandular Syndromes. METHODS: Monocentric observational retrospective study enrolling patients affected by APS diagnosed and monitored in a Reference Center. Clinical records were retrieved and analyzed. RESULTS: 111 subjects (51 males) were affected by APS type 4, mean age at the onset was 23.1 ± 15.1 years. In 15 patients the diagnosis of APS was performed during the first clinical evaluation, in the other 96 after a latency of 11 years (range 1-46). The most frequent diseases were type I diabetes mellitus and celiac disease, equally distributed among sexes. CONCLUSIONS: The prevalence of APS type 4 is 9:100,000 people. Type I diabetes mellitus was the leading indicator of APS type 4 in 78% subjects and in 9% permitted the diagnosis occurring as second manifestation of the syndrome. Our data, showing that 50% of patients developed APS type 4 within the first ten years, don’t suggest any particular follow-up time and, more importantly, don’t specify any particular disease. It is important to emphasize that 5% of women developed premature ovarian failure.
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spelling pubmed-106272402023-11-07 Autoimmune polyglandular syndrome type 4: experience from a single reference center Gatta, Elisa Anelli, Valentina Cimino, Elena Di Lodovico, Elena Piovani, Elda Zammarchi, Irene Gozzoli, Giorgia Maltese, Virginia Cavadini, Maria Agosti, Barbara Delbarba, Andrea Pirola, Ilenia Girelli, Angela Buoso, Caterina Bambini, Francesca Alfieri, Daniele Bremi, Walter Facondo, Paolo Lupo, Roberto Bezzi, Francesco Fredi, Micaela Mazzola, Anna Maria Gandossi, Elena Saullo, Maura Marini, Fiorella Licini, Massimo Pezzaioli, Letizia Chiara Pini, Laura Franceschini, Franco Ricci, Chiara Cappelli, Carlo Front Endocrinol (Lausanne) Endocrinology PURPOSE: To characterize patients with APS type 4 among those affected by APS diagnosed and monitored at our local Reference Center for Autoimmune Polyglandular Syndromes. METHODS: Monocentric observational retrospective study enrolling patients affected by APS diagnosed and monitored in a Reference Center. Clinical records were retrieved and analyzed. RESULTS: 111 subjects (51 males) were affected by APS type 4, mean age at the onset was 23.1 ± 15.1 years. In 15 patients the diagnosis of APS was performed during the first clinical evaluation, in the other 96 after a latency of 11 years (range 1-46). The most frequent diseases were type I diabetes mellitus and celiac disease, equally distributed among sexes. CONCLUSIONS: The prevalence of APS type 4 is 9:100,000 people. Type I diabetes mellitus was the leading indicator of APS type 4 in 78% subjects and in 9% permitted the diagnosis occurring as second manifestation of the syndrome. Our data, showing that 50% of patients developed APS type 4 within the first ten years, don’t suggest any particular follow-up time and, more importantly, don’t specify any particular disease. It is important to emphasize that 5% of women developed premature ovarian failure. Frontiers Media S.A. 2023-10-23 /pmc/articles/PMC10627240/ /pubmed/37937054 http://dx.doi.org/10.3389/fendo.2023.1236878 Text en Copyright © 2023 Gatta, Anelli, Cimino, Di Lodovico, Piovani, Zammarchi, Gozzoli, Maltese, Cavadini, Agosti, Delbarba, Pirola, Girelli, Buoso, Bambini, Alfieri, Bremi, Facondo, Lupo, Bezzi, Fredi, Mazzola, Gandossi, Saullo, Marini, Licini, Pezzaioli, Pini, Franceschini, Ricci and Cappelli https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Gatta, Elisa
Anelli, Valentina
Cimino, Elena
Di Lodovico, Elena
Piovani, Elda
Zammarchi, Irene
Gozzoli, Giorgia
Maltese, Virginia
Cavadini, Maria
Agosti, Barbara
Delbarba, Andrea
Pirola, Ilenia
Girelli, Angela
Buoso, Caterina
Bambini, Francesca
Alfieri, Daniele
Bremi, Walter
Facondo, Paolo
Lupo, Roberto
Bezzi, Francesco
Fredi, Micaela
Mazzola, Anna Maria
Gandossi, Elena
Saullo, Maura
Marini, Fiorella
Licini, Massimo
Pezzaioli, Letizia Chiara
Pini, Laura
Franceschini, Franco
Ricci, Chiara
Cappelli, Carlo
Autoimmune polyglandular syndrome type 4: experience from a single reference center
title Autoimmune polyglandular syndrome type 4: experience from a single reference center
title_full Autoimmune polyglandular syndrome type 4: experience from a single reference center
title_fullStr Autoimmune polyglandular syndrome type 4: experience from a single reference center
title_full_unstemmed Autoimmune polyglandular syndrome type 4: experience from a single reference center
title_short Autoimmune polyglandular syndrome type 4: experience from a single reference center
title_sort autoimmune polyglandular syndrome type 4: experience from a single reference center
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10627240/
https://www.ncbi.nlm.nih.gov/pubmed/37937054
http://dx.doi.org/10.3389/fendo.2023.1236878
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