eISSN: 2300 - 8660
ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
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4/2017
vol. 92
 
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abstract:
Guidelines/recommendations

ReCOMmendations for management in cystic fibrosis for General PrActitionerS - COMPAS CF*

Dorota Sands
,
Katarzyna Walicka-Serzysko
,
Zbigniew Doniec
,
Agnieszka Mastalerz-Migas
,
Halina Batura-Gabryel
,
Władysław Pierzchała

PEDIATRIA POLSKA 92 (2017) 431-445
Online publish date: 2018/03/07
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In recent years, enormous progress has been made in the diagnosis and treatment of cystic fibrosis, which is no longer considered a fatal disease of childhood, rather it is referred to as a life shortening disease. The introduction of newborn screening for cystic fibrosis in Poland has made it possible to recognize the disease often before the onset of clinical symptoms. As a consequence of genetic screening development, new CFTR mutations previously undetected are now reported. However, we are yet to unravel how mutations will manifest, consequently it is necessary to isolate this new group of patients:CFSPID (Screened Positive Patients with Hypertrypsinogenemia and an inconclusive Diagnosis) for further observation.Cystic fibrosis patients require a comprehensive (which represents best practice),coordinated care delivered by a multidisciplinary team.Only such a model of care can markedly increase the mean survival of patients and improve their quality of life.A breakthrough in the treatment of cystic fibrosis is the introduction of new biological drugs that act causally inpatients with a specific genotype. In the future,this may lead to individualized treatment and will change the course of the disease.
keywords:

COMPAS CF, Cystic fibrosis, Exocrine pancreaticinsufficiency, Bronchopulmonaryexacerbation