294 High requirement of pancreatic enzyme dose

Abstract

Pancreatic enzyme replacement therapy is essential for maintain adequate nutrition in patients with cystic fibrosis (CF) and steatorrhea. The different treatment guidelines suggest dose of up to 10,000 lipase unit/kg/day, but in clinical practice this dose is not sufficient to correct steatorrhea for a group of patients. Objective: To assess the requirement of pancreatic enzymes (PE) in a population of CF patients and correlate with genetic mutation and others clinical parameters. Patients and Methods: Crossover study of patients at the CF clinic in the period Jun−Dec 2010. They are treated according to rules of CF patients. All patients with PE dose greater than 10,000U/kg/d received inhibitors of proton pump. If patients had steatorrhoea despite having reached the recommended dose of PE, we indicated increase and regulate the dose according to the fatness of the meal. Were assessed PE dose; height, weight and BMI z score (ZH, ZW and ZBMI), genetic mutation, level of A&E vitamins. Results: One hundred and five patients were assessed, 54 males, mean age 6.7 years, with mean ZH −0.5, mean ZBMI −0.26. Median PE dose was 9,400U/kg/d (range 2,000–48,000) and 50 patients received more than 10,000U/kg/d. 36% of the patients were DF508 homozygote. These had higher requirements of PE (16,600U/kg/d vs 9,300, P: 0.0015 T Test), had worse ZH (−0.93 vs −0.38, P: 0.02 T test) and ZW (−1.4 vs −0.6, P: 0.003). There were no significant differences in ZBMI, Van de Kamer and A & E vitamins. Nobody had fibrosing colonopathy. Conclusions: High requirement of PE dose was common in this population of patients with CF and this was correlated with severe genetic mutation. Complications were no found.