[Chronic liver disease associated with cystic fibrosis: energy expenditure at rest, risk factors, and impact on the course of the disease].
OBJECTIVE
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.


METHODS
Nutrition Unit of the Gastroenterology Department at La Paz Children's Hospital.


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.
Tabernero da Veiga
S
S
Unidad de Nutrición Infantil, Hospital Infantil Universitario La Paz, UAM, Madrid. ya-su@airtel.net
González Lama
Y
Y
Lama More
R
R
Martínez Carrasco
M C
MC
Antelo Landeria
M C
MC
Jara Vega
P
P
D000293:Adolescent; D000328:Adult; D002648:Child; D002908:Chronic Disease; D003550:Cystic Fibrosis / Q000150:complications / Q000378:metabolism*; D004734:Energy Metabolism; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008107:Liver Diseases / Q000150:complications / Q000378:metabolism*; D008137:Longitudinal Studies; D008297:Male; D011446:Prospective Studies; D012146:Rest; D012307:Risk Factors