UP-TO-DATE MANAGEMENT OF LUNG DISEASE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS

  • Marina Praprotnik MD, MSc, University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases
  • Ana Kotnik Pirš MD, MSc, University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases
  • Barbara Salobir MD, MSc, University Clinical Center Ljubljana, Internal Clinic, Department for pulmonary diseases and allergology
  • Majda Oštir University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases
  • Matjaž Turel MD, MSc, University Clinical Center Ljubljana, Internal Clinic, Department for pulmonary diseases and allergology
  • Uroš Krivec MD, MSc, University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases
Keywords: screening test, lung disease, inhalations, infection control, lung transplantation

Abstract

Cystic fibrosis (CF) is a multi-organ disease,  affecting mostly lungs and gastrointestinal tract. Data from patient registries show that the survival of patients with CF has progressively improved over the past several decades, as a result of advances in antibiotic treatment, supplementation of pancreatic enzymes, better nutrition and a holistic approach to treatment in CF centres.

The purpose of this review is to survey recent developments in the treatment of lung disease  in children and adolescents with CF.

We describe newborn screening for CF.

When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue.

Lung disease is the most common cause of morbidity and mortality in CF patients. Emerging new therapies are targeted at all points in the pathogenesis of lung disease, from drugs that treat infection and inflammation in the airways to gene transfer studies  and to drugs that augment airway surface liquid height. A number of antibacterial agents formulated for inhalation are at various stages of study and there are several anti-inflammatory candidate drugs in  clinical trials.  The most important development  in the recent years is  modulation of the abnormal protein that causes CF, the cystic fibrosis transmembrane regulator (CFTR), where drugs are targeted at specific defects in the transcription, processing or functioning.

When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue. The role of the CF nurse, who has responsibilities in educating and teaching clinical skills to patients and families, is described.

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Author Biographies

Marina Praprotnik, MD, MSc, University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases

MD, MSc, University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases

mag. Marina Praprotnik, dr. med., specialistka pediatrije

Ana Kotnik Pirš, MD, MSc, University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases

Univerzitetni klinični center Ljubljana, Pediatrična klinika, Služba za pljučne bolezni,

dr, med, spec. pediatrije

Barbara Salobir, MD, MSc, University Clinical Center Ljubljana, Internal Clinic, Department for pulmonary diseases and allergology
Univerzitetni klinični center Ljubljana, Interna klinika, Služba za pljučne bolezni in alergijo, doc., dr. med., spec. interne medicine
Majda Oštir, University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases
Univerzitetni klinični center Ljubljana, Pediatrična klinika, Služba za pljučne bolezni, DMS, glavna medicinska sestra.
Matjaž Turel, MD, MSc, University Clinical Center Ljubljana, Internal Clinic, Department for pulmonary diseases and allergology
Univerzitetni klinični center Ljubljana, Interna klinika, Klinični oddelek za pulmologijo in alergologijo, mag., dr. med., spec. interne medicine
Uroš Krivec, MD, MSc, University Clinical Center Ljubljana, University Children's Hospital, Department for pulmonary diseases

Univerzitetni klinični center Ljubljana, Pediatrična klinika, Služba za pljučne bolezni,

dr. med, spec. pediatrije

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Published
2015-04-07
How to Cite
1.
Praprotnik M, Kotnik Pirš A, Salobir B, Oštir M, Turel M, Krivec U. UP-TO-DATE MANAGEMENT OF LUNG DISEASE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS. ZdravVestn [Internet]. 7Apr.2015 [cited 22Nov.2019];84(2). Available from: https://vestnik.szd.si/index.php/ZdravVest/article/view/1219
Section
Review

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