Name: | Description: | Size: | Format: | |
---|---|---|---|---|
72.07 KB | Adobe PDF |
Authors
Advisor(s)
Abstract(s)
RESUMO
Introdução: Passaram mais de sete décadas desde a
primeira descrição da fibrose quística enquanto doença fatal.
Desde então, a assistência clínica destes doentes em Centros
Especializados, assim como os progressos científicos na investigação,
refletiram-se significativamente na melhoria da qualidade
de vida e da esperança média de vida e como resultado os doentes
atingem a idade adulta, o que inicialmente não era observado.
Paradoxalmente, este aumento na sobrevida fez surgir novas
complicações da doença bem como problemas decorrentes da
terapêutica prolongada. A doença óssea relacionada com a fibrose
quística surge assim como uma das complicações frequentes
decorrente da maior sobrevida. Desde a sua primeira descrição
em 1979 tem sido publicado um elevado número de artigos relativos
a esta complicação secundária da fibrose quística.
Objetivos: Neste artigo pretendemos rever a literatura relacionada
com as várias áreas de investigação em torno da doença
óssea associada à fibrose quística resumindo os aspetos
mais relevantes sobre esta problemática.
Desenvolvimento: Ao longo do artigo os autores salientam
os conhecimentos sobre a epidemiologia, fisiopatologia e clínica,
e descrevem as recomendações mais atuais relativas ao diagnóstico,
prevenção, terapêutica e vigilância da doença óssea relacionada
com a fibrose quística.
Conclusões: A etiologia multifatorial e a interdependência
e complexidade dos mecanismos patológicos subjacentes
salientam a importância do acompanhamento dos doentes com
doença óssea relacionada a fibrose quística em centros especializados
que possibilitem um seguimento pluri e interdisciplinar
integrado e diferenciado.
ABSTRACT Introduction: More than seven decades have passed since the first description of cystic fibrosis as a deadly childhood disease. Since then, progress has been made to extend the lives of these patients. As a consequence, children with cystic fibrosis are living into adulthood, which was once an unexpected outcome. Nevertheless, this increased survival brought new secondary complications as well as problems caused by the long-lasting medication. Cystic fibrosis-related bone disease is a recent but common complication in long-term survivors. Since its first description in 1979, a large number of papers have been published concerning the various areas of research around this secondary complication of cystic fibrosis. Aims: This article attempts to review the literature related to several research areas around the cystic fibrosis-related bone disease summarizing the most relevant aspects of this problem. Development: Throughout the article the authors emphasize knowledge about the epidemiology, pathophysiology and clinical aspects, and describe the most current recommendations for the diagnosis, prevention, treatment and monitoring of cystic fibrosis - related bone disease. Conclusions: Multifactorial aetiology and interdependence and complexity of underlying pathological mechanisms stress the need and importance of monitoring patients with cystic fibrosis-related bone disease in specialized centers that allow a multi - and interdisciplinary integrated and differentiated follow-up.
ABSTRACT Introduction: More than seven decades have passed since the first description of cystic fibrosis as a deadly childhood disease. Since then, progress has been made to extend the lives of these patients. As a consequence, children with cystic fibrosis are living into adulthood, which was once an unexpected outcome. Nevertheless, this increased survival brought new secondary complications as well as problems caused by the long-lasting medication. Cystic fibrosis-related bone disease is a recent but common complication in long-term survivors. Since its first description in 1979, a large number of papers have been published concerning the various areas of research around this secondary complication of cystic fibrosis. Aims: This article attempts to review the literature related to several research areas around the cystic fibrosis-related bone disease summarizing the most relevant aspects of this problem. Development: Throughout the article the authors emphasize knowledge about the epidemiology, pathophysiology and clinical aspects, and describe the most current recommendations for the diagnosis, prevention, treatment and monitoring of cystic fibrosis - related bone disease. Conclusions: Multifactorial aetiology and interdependence and complexity of underlying pathological mechanisms stress the need and importance of monitoring patients with cystic fibrosis-related bone disease in specialized centers that allow a multi - and interdisciplinary integrated and differentiated follow-up.
Description
Keywords
Criança doença óssea relacionada com a fibrose quística Children cystic fibrosis related bone disease
Citation
Nascer e Crescer 2013; 22(4): 227-233