Cystic Fibrosis.
Bone Density
Child
Cystic Fibrosis
/ complications
Cystic Fibrosis Transmembrane Conductance Regulator
/ genetics
Female
Humans
Infant
Infant, Newborn
Liver Diseases
/ etiology
Lung
/ diagnostic imaging
Lung Transplantation
Male
Respiratory Tract Infections
/ etiology
Trypsinogen
/ blood
Vitamins
/ therapeutic use
Journal
Pediatrics in review
ISSN: 1526-3347
Titre abrégé: Pediatr Rev
Pays: United States
ID NLM: 8103046
Informations de publication
Date de publication:
Feb 2021
Feb 2021
Historique:
entrez:
2
2
2021
pubmed:
3
2
2021
medline:
9
9
2021
Statut:
ppublish
Résumé
Cystic fibrosis (CF) is one of the most commonly diagnosed genetic disorders. Clinical characteristics include progressive obstructive lung disease, sinusitis, exocrine pancreatic insufficiency leading to malabsorption and malnutrition, liver and pancreatic dysfunction, and male infertility. Although CF is a life-shortening disease, survival has continued to improve to a median age of 46.2 years due to earlier diagnosis through routine newborn screening, promulgation of evidence-based guidelines to optimize nutritional and pulmonary health, and the development of CF-specific interdisciplinary care centers. Future improvements in health and quality of life for individuals with CF are likely with the recent development of mutation-specific modulator therapies. In this review, we will cover the current understanding of the disease manifestations, diagnosis, and management as well as common complications seen in individuals with CF.
Identifiants
pubmed: 33526571
pii: 42/2/55
doi: 10.1542/pir.2019-0212
pmc: PMC8972143
mid: NIHMS1784541
doi:
Substances chimiques
CFTR protein, human
0
Vitamins
0
Cystic Fibrosis Transmembrane Conductance Regulator
126880-72-6
Trypsinogen
9002-08-8
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
55-67Subventions
Organisme : NHLBI NIH HHS
ID : T32 HL072748
Pays : United States
Informations de copyright
© American Academy of Pediatrics, 2021. All rights reserved.
Déclaration de conflit d'intérêts
AUTHOR DISCLOSUREDrs Dickinson and Collaco have disclosed no financial disclosures relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of commercial products/devices.
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