ABSTRACT
OBJECTIVE: To investigate routine ultrasonography (US) as an early marker and to identify risk factors for the development of cirrhosis and portal hypertension (PHT) in cystic fibrosis (CF). STUDY DESIGN: A cohort of 106 children with CF aged 5.9+/-2.3 years were followed for 10.4+/-0.2 years in a CF clinic. RESULTS: At enrollment, the US was normal, but biochemical and/or clinical disease was present in 10%. By the end of the study, 19 had developed US changes, eight with evidence of PHT. At the time of the initial US change, only 36.4% of those had, at the end of the study, either a heterogeneous or a nodular parenchyma, and only 50% of those with PHT had biochemical and/or clinical disease. Of the 30 patients treated with ursodeoxycholic acid for biochemical and/or clinical disease with (n=15) and without (n=15) associated US changes, PHT developed in six of the former and two of the latter. Univariate analysis and logistic regression showed that children with more severe disease in terms of forced expiratory volume in one second were at somewhat greater risk (P<.06) of PHT developing. CONCLUSION: US was an early marker of liver disease and more severe CF disease, a predictor of progressive liver disease. A controlled trial should be done to assess isolated US-detected disease as an indication for UDCA.
Subject(s)
Cystic Fibrosis/complications , Cystic Fibrosis/diagnostic imaging , Hypertension, Portal/diagnostic imaging , Hypertension, Portal/etiology , Liver Cirrhosis/diagnostic imaging , Liver Cirrhosis/etiology , Population Surveillance , Ultrasonography , Adolescent , Child , Child, Preschool , Cohort Studies , Female , Follow-Up Studies , Humans , Male , Predictive Value of Tests , Risk Factors , Severity of Illness Index , Time FactorsABSTRACT
OBJECTIVES: To compare the absorption of a lysophosphatidylcholine, monoglyceride, and fatty acid matrix (organized lipid matrix, OLM) with that of a triacylglycerol (TG)-based fat meal in patients with cystic fibrosis (CF). STUDY DESIGN: Five adolescents with CF and 3 control patients were given fat meals supplemented with retinyl palmitate of either OLM or TG at a 2-week interval. In a clinical trial, 73 patients with CF were randomly assigned to nutritional supplements containing either OLM or TG for a 1-year double-blind trial followed by a 6-month observation period. RESULTS: The peak increases and areas under the curve for TG and retinyl palmitate after the fat meal were 10-fold higher after OLM than after the TG fat load and did not differ from values obtained in control patients. OLM led to better clinical outcomes in terms of energy intake from the diet, weight-for-age Z score, essential fatty acid status, vitamin E, and retinol binding protein. Height-for-age Z score and FEV(1) only reached statistical significance at the end of the 6-month observation period. CONCLUSIONS: These results suggest that OLM is a readily absorbable source of fat and energy in CF and is an effective nutritional supplement.