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Hemophilia Care in the Pediatric Age

Hemophilia is the most common of the severe bleeding disorders and if not properly managed since early infancy can lead to chronic disease and lifelong disabilities. However, it enjoys the most efficacious and safe treatment among the most prevalent monogenic disorders. Hemophilia should be consider...

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Autores principales: Bertamino, Marta, Riccardi, Francesca, Banov, Laura, Svahn, Johanna, Molinari, Angelo Claudio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5447945/
https://www.ncbi.nlm.nih.gov/pubmed/28534860
http://dx.doi.org/10.3390/jcm6050054
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author Bertamino, Marta
Riccardi, Francesca
Banov, Laura
Svahn, Johanna
Molinari, Angelo Claudio
author_facet Bertamino, Marta
Riccardi, Francesca
Banov, Laura
Svahn, Johanna
Molinari, Angelo Claudio
author_sort Bertamino, Marta
collection PubMed
description Hemophilia is the most common of the severe bleeding disorders and if not properly managed since early infancy can lead to chronic disease and lifelong disabilities. However, it enjoys the most efficacious and safe treatment among the most prevalent monogenic disorders. Hemophilia should be considered in the neonatal period in the case of unusual bleeding or in the case of positive family history. Later, hemophilia should be suspected mainly in males because of abnormal bruising/bleeding or unusual bleeding following invasive procedures—for example, tonsillectomy or circumcision. Prophylactic treatment that is started early with clotting-factor concentrates has been shown to prevent hemophilic arthropathy and is, therefore, the gold standard of care for hemophilia A and B in most countries with adequate resources. Central venous access catheters and arterovenous fistulas play an important role in the management of hemophilia children requiring repeated and/or urgent administration of coagulation factor concentrates. During childhood and adolescence, personalized treatment strategies that suit the patient and his lifestyle are essential to ensure optimal outcomes. Physical activity is important and can contribute to better coordination, endurance, flexibility and strength. The present article focuses also on questions frequently posed to pediatric hematologists like vaccinations, day-care/school access and dental care.
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spelling pubmed-54479452017-06-13 Hemophilia Care in the Pediatric Age Bertamino, Marta Riccardi, Francesca Banov, Laura Svahn, Johanna Molinari, Angelo Claudio J Clin Med Review Hemophilia is the most common of the severe bleeding disorders and if not properly managed since early infancy can lead to chronic disease and lifelong disabilities. However, it enjoys the most efficacious and safe treatment among the most prevalent monogenic disorders. Hemophilia should be considered in the neonatal period in the case of unusual bleeding or in the case of positive family history. Later, hemophilia should be suspected mainly in males because of abnormal bruising/bleeding or unusual bleeding following invasive procedures—for example, tonsillectomy or circumcision. Prophylactic treatment that is started early with clotting-factor concentrates has been shown to prevent hemophilic arthropathy and is, therefore, the gold standard of care for hemophilia A and B in most countries with adequate resources. Central venous access catheters and arterovenous fistulas play an important role in the management of hemophilia children requiring repeated and/or urgent administration of coagulation factor concentrates. During childhood and adolescence, personalized treatment strategies that suit the patient and his lifestyle are essential to ensure optimal outcomes. Physical activity is important and can contribute to better coordination, endurance, flexibility and strength. The present article focuses also on questions frequently posed to pediatric hematologists like vaccinations, day-care/school access and dental care. MDPI 2017-05-19 /pmc/articles/PMC5447945/ /pubmed/28534860 http://dx.doi.org/10.3390/jcm6050054 Text en © 2017 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Bertamino, Marta
Riccardi, Francesca
Banov, Laura
Svahn, Johanna
Molinari, Angelo Claudio
Hemophilia Care in the Pediatric Age
title Hemophilia Care in the Pediatric Age
title_full Hemophilia Care in the Pediatric Age
title_fullStr Hemophilia Care in the Pediatric Age
title_full_unstemmed Hemophilia Care in the Pediatric Age
title_short Hemophilia Care in the Pediatric Age
title_sort hemophilia care in the pediatric age
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5447945/
https://www.ncbi.nlm.nih.gov/pubmed/28534860
http://dx.doi.org/10.3390/jcm6050054
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