20.500.12556/DiRROS-30579 Sodobno zdravljenje cistične fibroze Modern treatment of cystic fibrosis 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. 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. modulatorji CFTR CFTR modulators true false true Slovenski jezik Angleški jezik Neznano 2026-06-30 09:35:08 2026-06-30 09:35:28 2026-07-01 03:55:57 0000-00-00 00:00:00 2025 0 0 Besedilo v slov.; str. 85-91 Št. 2 Letn. 76 maj 2025 0000-00-00 Zaloznikova Objavljeno NiDoloceno 0000-00-00 0000-00-00 2025-05-01 616.2 0014-8229 271036931 https://www.sfd.si/wp-content/uploads/2025/05/salobir_fv.pdf 0 68a245bd-7456-11f1-9c8b-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=46528 salobir_fv.pdf salobir_fv.pdf 1 42997FACDE87B1EBCCFA8576385438C7 9ca9a2a73208c0142e5e14c8b2d1ff6d242b49cd104a57c4007fe55266a5bf1c 38ae3918-7456-11f1-9c8b-001a4af901a5 https://dirros.openscience.si/Dokument.php?lang=slv&id=46527 Slovensko farmacevtsko društvo Farmacevtski vestnik 0 0 0