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Title:Sodobno zdravljenje cistične fibroze
Authors:ID Salobir, Barbara (Author)
ID Kalan, Katja (Author)
ID Krivec, Uroš (Author)
Files:URL URL - Source URL, visit https://www.sfd.si/wp-content/uploads/2025/05/salobir_fv.pdf
 
.pdf PDF - Presentation file, download (328,98 KB)
MD5: 42997FACDE87B1EBCCFA8576385438C7
 
Language:Slovenian
Typology:1.02 - Review Article
Organization:Logo SFD - Slovenian Pharmaceutical Society
Abstract:Cistična fibroza je najpogostejša redka, dedna, avtosomno recesivna, monogenska bolezen belcev. Povzroča jo napaka v genu za regulator transmembranske prevodnosti pri cistični fibrozi. Prizadene vse organske sisteme z izločevalnimi membranami. Najbolj so prizadeta dihala in prebavila, kar se kaže predvsem s pogostimi okužbami dihal in malabsorpcijskim sindromom. Sodobno zdravljenje temelji na sedmih stebrih obravnave: 1) zagotavljanje ustrezne energijske in beljakovinske prehrane in nadomeščanje v maščobi topnih vitaminov A, D, E in K; 2) nadomeščanje encimov trebušne slinavke; 3) podpora mukociliarnemu čiščenju dihalnih poti; 4) preprečevanje in zdravljenje okužb dihal; 5) zdravljenje sladkorne bolezni in osteoporoze; 6) multidisciplinarna obravnava v specializiranih centrih za cistično fibrozo, ki vključuje tudi kliničnega farmacevta; 7) preprečevanje prenosa okužb. Skupaj s prihodom zdravil iz skupine modulatorjev regulatorja transmembranske prevodnosti pri cistični fibrozi nekoč usodna bolezen otrok postaja kronična bolezen odraslih. Vloga farmacevtov pa ob kompleksnosti zdravljenja postaja vedno pomembnejša.
Keywords:modulatorji CFTR
Publication status:Published
Publication version:Version of Record
Publication date:01.05.2025
Year of publishing:2025
Number of pages:str. 85-91
Numbering:Letn. 76, Št. 2
PID:20.500.12556/DiRROS-30579 New window
UDC:616.2
ISSN on article:0014-8229
COBISS.SI-ID:271036931 New window
Note:Besedilo v slov.;
Publication date in DiRROS:30.06.2026
Views:31
Downloads:20
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Record is a part of a journal

Title:Farmacevtski vestnik : strokovno glasilo slovenske farmacije
Shortened title:Farm. vestn.
Publisher:Slovensko farmacevtsko društvo
ISSN:0014-8229
COBISS.SI-ID:3105794 New window

Licences

License:CC BY-NC 4.0, Creative Commons Attribution-NonCommercial 4.0 International
Link:http://creativecommons.org/licenses/by-nc/4.0/
Description:A creative commons license that bans commercial use, but the users don’t have to license their derivative works on the same terms.

Secondary language

Language:English
Title:Modern treatment of cystic fibrosis
Abstract:Cystic fibrosis is the most common rare, inherited, autosomal recessive, monogenic disease of Caucasians. Caused by a defect in the gene enciding the cystic fibrosis transmembrane conductance regulator, it affects all organs with secretory membranes. The respiratory and gastrointestinal tracts are most affected, manifesting mainly as frequent respiratory infections and malabsorption. Seven pillars of modern managment are: 1) provision of adequate energy and protein nutrition, supplementation of fatsoluble vitamins A, D, E and K; 2) supplementation of pancreatic enzymes; 3) support of mucociliary clearance of the airways; 4) prevention and treatment of respiratory tract infections; 5) treatment of diabetes mellitus and osteoporosis; 6) multidisciplinary management in specialised centres for cystic fibrosis, with the participation of clinical pharmacists; 7) prevention of transmission of infections. Toghether with new drugs modulating cystic fibrosis transmembrane conductance regulator, a once fatal disease of children is becoming a chronic disease of adults. The role of pharmacists is becoming increasingly important given the complexity of treatment.
Keywords:CFTR modulators


Collection

This document is a part of these collections:
  1. Farmacevtski vestnik

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