[Chronic liver disease associated with cystic fibrosis: energy expenditure at rest, risk factors, and impact on the course of the disease]. / Hepatopatía crónica asociada a fibrosis quística: gasto energético en reposo, factores de riesgo y repercusión en la evolución de la enfermedad.

ABSTRACT

INTRODUCTION AND GOALS Cystic fibrosis is the most frequent recessive disease. There are various hepato-biliary alterations, of which the most important is the development of biliary obstruction and periportal fibrosis. The goal is to assess the influence of liver disease on the nutritional status, the progress of the disease and the associated risk factors. SCOPE Nutrition Unit of the Gastroenterology Department at La Paz Children's Hospital. MATERIAL AND METHODS: Prospective longitudinal study with 53 patients assessed on three occasions over 3 years at inclusion, after 1 year and after 3 years. Only 37 of them were followed-up for 3 years. A total of 111 measurements were taken, including analysis of body composition, energy expenditure, intake and energy losses as well as nitrogen balance. Simultaneously, respiratory function tests were performed and the presence of repeated acute respiratory problems was evaluated. RESULTS: 37 patients, 19 females and 18 males (mean age 13.04 years +/- 3.28). Twelve (32.43%) were diagnosed as having liver disease (mean age 12.16% +/- 3.86 SD, 11 males, 1 female), of whom 1 presented meconial ileum, 5 were homozygotic, 5 heterozygotic and the other two presented other mutations. Those with liver disease presented anthropometric parameters that were better than or similar to the patients without liver involvement (p NS). Mean Waterlow index in liver disease sufferers 93.62% +/- 7.87 SD; without liver disease 93.06% +/- 10.97 SD (p NS). Mean of LVEF in liver disease sufferers 88.81 +/- 27.32 SD; without liver disease 75.21 +/- 27.92 SD (p < 0.05). Mean FVC in liver disease sufferers 95.38 +/- 22.92 SD; without liver disease 83 +/- 23.7 SD (p < 0.05). Mean energy expenditure at rest/lean body mass in liver disease sufferers 38.81 kcal +/- 7.26 SD; without liver disease 42.36 kcal +/- f 6.91 SD (p < 0.05). CONCLUSIONS: The prevalence of chronic liver disease in patients with cystic fibrosis increases with age and is more frequent in males. Liver disease sufferers present a better evolution in pulmonary function and present less energy expenditure.