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Effects of modulator therapies on endocrine complications in adults with cystic fibrosis: a narrative review.
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- Author(s): Kumar S;Kumar S;Kumar S; Soldatos G; Soldatos G; Soldatos G; Teede HJ; Teede HJ; Teede HJ; Pallin M; Pallin M
- Source:
The Medical journal of Australia [Med J Aust] 2023 Nov 20; Vol. 219 (10), pp. 496-502. Date of Electronic Publication: 2023 Oct 15.- Publication Type:
Journal Article; Review; Research Support, Non-U.S. Gov't- Language:
English - Source:
- Additional Information
- Source: Publisher: Australasian Medical Publishing Co Country of Publication: Australia NLM ID: 0400714 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1326-5377 (Electronic) Linking ISSN: 0025729X NLM ISO Abbreviation: Med J Aust Subsets: MEDLINE
- Publication Information: Publication:
: Pyrmont, NSW : Australasian Medical Publishing Co.
Original Publication: Sydney : Australasian Medical Pub. Co. - Subject Terms: Cystic Fibrosis*/complications ; Cystic Fibrosis*/drug therapy ; Cystic Fibrosis*/genetics ; Diabetes Mellitus, Type 2*/drug therapy; Pregnancy ; Female ; Adult ; Humans ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics ; Cystic Fibrosis Transmembrane Conductance Regulator/therapeutic use ; Mutation ; Quality of Life
- Abstract: Cystic fibrosis is a monogenic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, which transports chloride ions in secretory organs. Modulator therapies are small molecules that correct CFTR dysfunction and can lead to a wide range of benefits for both pulmonary and extrapulmonary complications of cystic fibrosis. With advancements in airway, antimicrobial and nutritional therapies and now introduction of modulator therapies, most people living with cystic fibrosis in Australia are now adults. For adults with cystic fibrosis, endocrine manifestations such as cystic fibrosis-related diabetes, metabolic bone disease, and reproductive health are becoming increasingly important, and emerging evidence on the endocrine effects of CFTR modulator therapies is promising and is shifting paradigms in our understanding and management of these conditions. The management of cystic fibrosis-related diabetes will likely need to pivot for high responders to modulator therapy with dietary adaptions and potential use of medications traditionally reserved for adults with type 2 diabetes, but evidence to support changing clinical care needs is currently lacking. Increased attention to diabetes-related complications screening will also be required. Increased exercise capacity due to improved lung function, nutrition and potentially direct modulator effect may have a positive impact on cystic fibrosis-related bone disease, but supporting evidence to date is limited. Fertility can improve in women with cystic fibrosis taking modulator therapy. This has important implications for pregnancy and lactation, but evidence is lacking to guide pre-conception and antenatal management. Provision of multidisciplinary clinical care remains ever-important to ensure the emergence of endocrine and metabolic complications are optimised in adults with cystic fibrosis.
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- Contributed Indexing: Keywords: Cystic fibrosis; Diabetes mellitus, type 2; Endocrinology; Metabolic diseases; Reproduction
- Accession Number: 126880-72-6 (Cystic Fibrosis Transmembrane Conductance Regulator)
- Publication Date: Date Created: 20231015 Date Completed: 20231122 Latest Revision: 20231124
- Publication Date: 20231215
- Accession Number: 10.5694/mja2.52119
- Accession Number: 37839059
- Source:
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