@article{Holaman:2026:10.1016/j.jcf.2026.09.014,
author = {Holaman, J and Sills, D and Saumtally, H and Johnson, C and Recto, A and Marsh, R and Prayle, A and Gathercole, K and Monaghan, T and Marciani, L and Spiller, R and Barr, HL and Downey, DG and van, der Gast C and Peckham, D and Stewart, I and Smyth, A},
doi = {10.1016/j.jcf.2026.09.014},
journal = {J Cyst Fibros},
title = {Distinct gastrointestinal symptom phenotypes in adults with cystic fibrosis are linked to complications and medication use: the GRAMPUS-CF study.},
url = {http://dx.doi.org/10.1016/j.jcf.2026.09.014},
year = {2026}
}

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TY  - JOUR
AB  - BACKGROUND: Gastrointestinal symptoms remain common in adults with cystic fibrosis (CF) despite cystic fibrosis transmembrane conductance regulator modulator use, suggesting persistent and heterogeneous gut dysfunction. This prospective observational study tests the hypothesis that distinct gut symptom phenotypes in CF can be observed and linked to underlying mechanisms. METHODS: Adults from three UK CF centres completed the Gastrointestinal Symptom Rating Scale, Patient Assessment of Constipation Symptoms and a bowel-habit questionnaire. Latent class analysis using an ordinal logistic model was applied to 36 symptom indicators. Associations between phenotypes and demographic, clinical, and treatment variables were examined using generalised linear models. RESULTS: Three hundred participants completed questionnaires (54% male; median 31 years). We identified four symptom phenotypes: mild; moderate-constipation predominant; moderate-diarrhoea predominant and severe. The severe phenotype was associated with gastroesophageal reflux (RRR 2.86; 95%CI: 1.30-6.31; p = 0.009), distal intestinal obstruction syndrome (RRR 2.46; 95%CI: 1.04-5.81; p = 0.04), proton pump inhibitor (RRR 3.29; 95%CI 1.39-7.74; p = 0.007), and laxative use (RRR 6.13; 95%CI 2.54-14.84; p < 0.001). CF-related liver disease was associated with both moderate-constipation and diarrhoea phenotypes, respectively (RRR 2.08; 95%CI 1.13-3.81; p = 0.018; RRR 2.11; 95%CI 1.03-4.29; p = 0.04). There was a lower likelihood of long-term oral antibiotic use in the moderate-constipation phenotype (RRR 0.53; 95%CI 0.3-0.92; p = 0.025) and moderate-diarrhoea phenotype (RRR 0.46; 95%CI 0.24-0.91; p = 0.025). CONCLUSIONS: Four distinct symptom phenotypes were identified, independent of demographics and pancreatic status, but associated with specific complications and medication profiles. These phenotypes provide a framework for mechanistic studies within the GRAMPUS-CF cohort and precision management of CF-related g
AU  - Holaman,J
AU  - Sills,D
AU  - Saumtally,H
AU  - Johnson,C
AU  - Recto,A
AU  - Marsh,R
AU  - Prayle,A
AU  - Gathercole,K
AU  - Monaghan,T
AU  - Marciani,L
AU  - Spiller,R
AU  - Barr,HL
AU  - Downey,DG
AU  - van,der Gast C
AU  - Peckham,D
AU  - Stewart,I
AU  - Smyth,A
DO  - 10.1016/j.jcf.2026.09.014
PY  - 2026///
TI  - Distinct gastrointestinal symptom phenotypes in adults with cystic fibrosis are linked to complications and medication use: the GRAMPUS-CF study.
T2  - J Cyst Fibros
UR  - http://dx.doi.org/10.1016/j.jcf.2026.09.014
UR  - https://www.ncbi.nlm.nih.gov/pubmed/42888123
ER  - 

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