Cystic Fibrosis-related Liver Disease is Associated With Increased Disease Burden and Endocrine Comorbidities
Harveen Singh1, Michael J Coffey2, Chee Y Ooi1,2,3
1Department of Gastroenterology, Sydney Children's Hospital, Randwick.
Insights
Cystic fibrosis-related liver disease (CFLD) increases disease burden in cystic fibrosis (CF) patients. Severe CFLD is linked to more hospitalizations, nutritional support, bone disease, and CF-related diabetes.
Area of Science:
- Hepatology
- Pulmonology
- Endocrinology
Background:
- Cystic fibrosis-related liver disease (CFLD) is a significant cause of mortality in cystic fibrosis (CF).
- Understanding the comprehensive disease burden and comorbidities associated with severe CFLD is crucial for patient management.
Purpose of the Study:
- To compare the disease burden and nonrespiratory comorbidities between patients with severe CFLD and those without (noCFLD).
- To evaluate the impact of severe CFLD on hospitalization rates, nutritional support, and the prevalence of specific comorbidities.
Main Methods:
- A retrospective nationwide longitudinal review was conducted in Australia from 1998 to 2016.
- Severe CFLD patients were compared to matched noCFLD controls (1:1 ratio) based on age, genotype, pancreatic insufficiency, and treatment center.
Main Results:
- Patients with severe CFLD exhibited significantly lower FEV1% compared to noCFLD controls.
- Higher hospitalization rates for respiratory, gastrointestinal, and other indications were observed in the CFLD cohort.
- Increased use of nutritional supplementation (nasogastric and gastrostomy) and higher frequencies of osteopenia, osteoporosis, and CF-related diabetes were noted in CFLD patients.
Conclusions:
- Severe CFLD significantly increases the overall disease burden in CF patients.
- Patients with severe CFLD experience more frequent hospitalizations, require greater nutritional intervention, and have a higher risk of developing bone disease and diabetes.
Background:
Cystic fibrosis-related liver disease (CFLD) is the leading nonpulmonary cause of mortality in cystic fibrosis (CF). We evaluated and compared the burden of disease and nonrespiratory comorbidities of those with severe CFLD and those without (noCFLD).
Methods:
A retrospective nationwide (Australia) longitudinal review (from 1998 to 2016) of severe CFLD patients compared with noCFLD controls (matched 1 : 1 for age, genotype, pancreatic insufficiency, and center).
Results:
One hundred sixty-six patients with severe CFLD and 166 with noCFLD were identified. Forced expiratory volume in 1 second percentage of predicted (FEV1%) was significantly lower in CFLD than noCFLD across all ages (estimate [SE] -6.05% [2.12]; P = 0.004). Median (IQR) hospitalizations per patient per year were higher in CFLD than noCFLD for: respiratory indications (0.6 [0.2-1.3] vs 0.4 [0.1-0.9]; P = 0.002); gastrointestinal indications (0.09 [0-0.2] vs 0 [0-0.05]; P < 0.001); and other indications (0.05 [0-0.2] vs 0 [0-0.1]; P = 0.03). In the CFLD cohort, there was increased use of nasogastric (12.6% vs 5.4%; OR 2.51 [95% CI 1.06-6.46]; P = 0.03) and gastrostomy nutritional supplementation (22.9% vs 13.2%; OR 1.93 [95% CI 1.05-3.63]; P = 0.03). Additionally, the CFLD cohort had a higher frequency of bone diseases, osteopenia (26.5% vs 16.8%; OR 1.77 [95%CI 1.01-3.15]; P = 0.04) and osteoporosis (16.2% vs 8.4%; OR 2.1 [95% CI 1.01-4.52]; P = 0.04), as well as CF-related diabetes (38.5% vs 19.2%; OR 2.61 [95% CI 1.55-4.47]; P = 0.001).
Conclusions:
Patients with severe CFLD have greater disease burden, with higher number of hospitalizations (both respiratory and nonrespiratory indications), nutritional interventions, and are at higher risk of CF-related bone disease and diabetes.
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