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Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry
Background: Intracranial hemorrhage (ICH) is a highly serious event in patients with haemophilia (PWH) which leads to disability and in some cases to death. ICH occurs among all ages but is particularly frequent in newborns. Aim: The primary aim was to assess the incidence and mortality due to ICH i...
Autores principales: | , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8999820/ https://www.ncbi.nlm.nih.gov/pubmed/35407576 http://dx.doi.org/10.3390/jcm11071969 |
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author | Zanon, Ezio Pasca, Samantha Demartis, Francesco Tagliaferri, Annarita Santoro, Cristina Cantori, Isabella Molinari, Angelo Claudio Biasoli, Chiara Coppola, Antonio Luciani, Matteo Sottilotta, Gianluca Ricca, Irene Pollio, Berardino Borchiellini, Alessandra Tosetto, Alberto Peyvandi, Flora Frigo, Anna Chiara Simioni, Paolo |
author_facet | Zanon, Ezio Pasca, Samantha Demartis, Francesco Tagliaferri, Annarita Santoro, Cristina Cantori, Isabella Molinari, Angelo Claudio Biasoli, Chiara Coppola, Antonio Luciani, Matteo Sottilotta, Gianluca Ricca, Irene Pollio, Berardino Borchiellini, Alessandra Tosetto, Alberto Peyvandi, Flora Frigo, Anna Chiara Simioni, Paolo |
author_sort | Zanon, Ezio |
collection | PubMed |
description | Background: Intracranial hemorrhage (ICH) is a highly serious event in patients with haemophilia (PWH) which leads to disability and in some cases to death. ICH occurs among all ages but is particularly frequent in newborns. Aim: The primary aim was to assess the incidence and mortality due to ICH in an Italian population of PWH. Secondary aims were to evaluate the risk factors for ICH, the role of prophylaxis, and the clinical management of patients presenting ICH. Methods: A retrospective-prospective registry was established in the network of the Italian Association of Haemophilia Centers to collect all ICHs in PWH from 2009 to 2019 reporting clinical features, treatments, and outcomes. Results: Forty-six ICHs were collected from 13 Centers. The ICHs occurred in 15 children (10 < 2 years), and in 31 adults, 45.2% of them with mild hemophilia. Overall, 60.9% patients had severe haemophilia (15/15 children). Overall ICH incidence (×1000 person/year) was 0.360 (0.270–0.480 95% CI), higher in children <2 years, 1.995 (1.110–3.442 95% CI). Only 7/46 patients, all with severe haemophilia, had received a prophylactic regimen before the ICH, none with mild. Inhibitors were present in 10.9% of patients. In adult PWHs 17/31 suffered from hypertension; 85.7% of the mild subjects and 29.4% of the moderate/severe ones (p < 0.05). ICH was spontaneous in the 69.6% with lower rate in children (46.7%). Surgery was required in 21/46 patients for cerebral hematoma evacuation. Treatment with coagulation factor concentrates for at least three weeks was needed in 76.7% of cases. ICH was fatal in 30.4% of the cases. Of the survivors, 50.0% became permanently disabled. Only one-third of adult patients received long term prophylaxis after the acute treatment. Conclusion: The results from our Registry confirm the still high incidence of ICH in infants <2 years and in adults, particularly in mild PWHs presenting hypertension and its unfavorable outcomes. The majority of PWHs were treated on-demand before ICH occurred, suggesting the important role of prophylaxis in preventing such life-threatening bleeding. |
format | Online Article Text |
id | pubmed-8999820 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-89998202022-04-12 Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry Zanon, Ezio Pasca, Samantha Demartis, Francesco Tagliaferri, Annarita Santoro, Cristina Cantori, Isabella Molinari, Angelo Claudio Biasoli, Chiara Coppola, Antonio Luciani, Matteo Sottilotta, Gianluca Ricca, Irene Pollio, Berardino Borchiellini, Alessandra Tosetto, Alberto Peyvandi, Flora Frigo, Anna Chiara Simioni, Paolo J Clin Med Article Background: Intracranial hemorrhage (ICH) is a highly serious event in patients with haemophilia (PWH) which leads to disability and in some cases to death. ICH occurs among all ages but is particularly frequent in newborns. Aim: The primary aim was to assess the incidence and mortality due to ICH in an Italian population of PWH. Secondary aims were to evaluate the risk factors for ICH, the role of prophylaxis, and the clinical management of patients presenting ICH. Methods: A retrospective-prospective registry was established in the network of the Italian Association of Haemophilia Centers to collect all ICHs in PWH from 2009 to 2019 reporting clinical features, treatments, and outcomes. Results: Forty-six ICHs were collected from 13 Centers. The ICHs occurred in 15 children (10 < 2 years), and in 31 adults, 45.2% of them with mild hemophilia. Overall, 60.9% patients had severe haemophilia (15/15 children). Overall ICH incidence (×1000 person/year) was 0.360 (0.270–0.480 95% CI), higher in children <2 years, 1.995 (1.110–3.442 95% CI). Only 7/46 patients, all with severe haemophilia, had received a prophylactic regimen before the ICH, none with mild. Inhibitors were present in 10.9% of patients. In adult PWHs 17/31 suffered from hypertension; 85.7% of the mild subjects and 29.4% of the moderate/severe ones (p < 0.05). ICH was spontaneous in the 69.6% with lower rate in children (46.7%). Surgery was required in 21/46 patients for cerebral hematoma evacuation. Treatment with coagulation factor concentrates for at least three weeks was needed in 76.7% of cases. ICH was fatal in 30.4% of the cases. Of the survivors, 50.0% became permanently disabled. Only one-third of adult patients received long term prophylaxis after the acute treatment. Conclusion: The results from our Registry confirm the still high incidence of ICH in infants <2 years and in adults, particularly in mild PWHs presenting hypertension and its unfavorable outcomes. The majority of PWHs were treated on-demand before ICH occurred, suggesting the important role of prophylaxis in preventing such life-threatening bleeding. MDPI 2022-04-01 /pmc/articles/PMC8999820/ /pubmed/35407576 http://dx.doi.org/10.3390/jcm11071969 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Zanon, Ezio Pasca, Samantha Demartis, Francesco Tagliaferri, Annarita Santoro, Cristina Cantori, Isabella Molinari, Angelo Claudio Biasoli, Chiara Coppola, Antonio Luciani, Matteo Sottilotta, Gianluca Ricca, Irene Pollio, Berardino Borchiellini, Alessandra Tosetto, Alberto Peyvandi, Flora Frigo, Anna Chiara Simioni, Paolo Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry |
title | Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry |
title_full | Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry |
title_fullStr | Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry |
title_full_unstemmed | Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry |
title_short | Intracranial Haemorrhage in Haemophilia Patients Is Still an Open Issue: The Final Results of the Italian EMO.REC Registry |
title_sort | intracranial haemorrhage in haemophilia patients is still an open issue: the final results of the italian emo.rec registry |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8999820/ https://www.ncbi.nlm.nih.gov/pubmed/35407576 http://dx.doi.org/10.3390/jcm11071969 |
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