Effects of a proton-pump inhibitor in cystic fibrosis

Area of Science:

• Pediatric Gastroenterology
• Cystic Fibrosis Research
• Gastrointestinal Pharmacology

Background:

• Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, notably the pancreas.
• Steatorrhoea (fat malabsorption) persists in many CF patients despite pancreatic enzyme replacement therapy (PERT).
• Low duodenal pH is hypothesized to impair pancreatic enzyme function, contributing to persistent steatorrhoea.

Purpose of the Study:

• To evaluate the efficacy of lansoprazole, a proton-pump inhibitor (PPI), in reducing steatorrhoea.
• To assess the impact of lansoprazole on growth parameters and nutritional status in pediatric CF patients.
• To determine if lansoprazole improves fat absorption and overall nutritional markers in CF patients with persistent steatorrhoea.

Main Methods:

• A cohort of 15 pediatric cystic fibrosis patients with persistent steatorrhoea on PERT was studied.
• Lansoprazole (15 mg/day) was administered for 3 months, with assessments before, during, and 3 months after treatment.
• Measurements included acid steatocrit (for steatorrhoea), anthropometry, dual-energy X-ray absorptiometry (DXA) for body composition, and nutritional status Z-scores.

Main Results:

• Acid steatocrit significantly decreased, indicating reduced fat malabsorption (p=0.02).
• Significant improvements in weight and height Z-scores were observed during lansoprazole treatment (p=0.02 and p=0.03, respectively).
• Improvements in skinfold thickness, fat mass, and bone mineral content were also significant (p<0.01). Nutritional status deteriorated after treatment cessation.

Conclusions:

• Lansoprazole demonstrates significant efficacy as an adjuvant therapy in managing steatorrhoea in cystic fibrosis patients.
• The addition of lansoprazole improves nutritional status, growth parameters, and body composition in CF children.
• These findings suggest that PPIs can be a valuable therapeutic option for CF-related fat malabsorption.