Omega-3 fatty acids (from fish oils) for cystic fibrosis.

BACKGROUND: Epidemiological (population studies) and other studies suggest that a diet rich in omega-3 essential fatty acids (derived from fish oil) may have beneficial anti-inflammatory effects for chronic conditions such as cystic fibrosis. OBJECTIVES: To determine whether there is evidence that omega-3 polyunsaturated fatty acid supplementation reduces morbidity and mortality. To identify any adverse events associated with omega-3 polyunsaturated fatty acid supplementation. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group specialised trials register, which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Authors and persons interested in the question were contacted. Date of the most recent search of the Group's specialised register: May 2002. SELECTION CRITERIA: Randomised controlled trials in patients with cystic fibrosis in which omega-3 fatty acid supplements were compared with a placebo oil. DATA COLLECTION AND ANALYSIS: Two reviewers independently selected the trials to be included in the review and assessed the methodological quality of the trials using two approaches: Cochrane assessment of allocation concealment and Jadad quality assessment score. Using data acquisition forms, two reviewers independently extracted data. Missing data has been requested. MAIN RESULTS: The initial literature search identified six trials. Two trials, involving 31 participants satisfied our inclusion criteria and were included in the review. Both compared omega-3 fatty acids to olive oil controls for a six week treatment period. One study (19 participants) showed an improvement in FEV1, FVC, Shwachman score and reduction in sputum volume in the fish oil group at the end of this short treatment period. REVIEWER'S CONCLUSIONS: The review of trials found that regular omega-3 supplements may provide some benefits for people with cystic fibrosis with relatively few adverse effects, although the evidence is insufficient to draw firm conclusions. There is insufficient evidence to recommend routine use of supplements of omega-3 fatty acids in people with cystic fibrosis. The most notable feature highlighted by this review was the lack of data for many of the outcomes likely to be meaningful to people with or making treatment decisions about CF. A large, long-term, multi-centre, randomised controlled study is needed in order to determine if there is a significant therapeutic effect and to assess the influence of disease severity, dosage and duration of treatment. Future researchers should note the need for additional pancreatic enzymes.

Disproportionate head growth retardation in cystic fibrosis.

Monthly increments of length, weight gain, and head circumference were recorded from birth to 4 years of age in 52 children with cystic fibrosis. At birth the children showed stunting. Standard deviation (SD) scores at birth for height, weight, and head circumference were -1.24, -0.72, and -1.82 respectively. Over the first four years, length and weight SD scores showed a consistent improvement. However, there was very limited improvement in head circumference, which stabilised 1 SD below the mean from 1.5 years to 4 years. Our data suggest that head growth lags behind gain in height and weight in children with cystic fibrosis despite good nutritional management in early infancy. The data may also support the expression of cystic fibrosis transmembrane conductance regulator in choroid plexus and ependyma.

Familial hypercholesterolaemia: pilot study to identify children at risk.

AIMS: To evaluate a more effective method of identifying children with familial hypercholesterolaemia by screening a population at high risk. METHODS: Domiciliary measurement of random cholesterol concentration was made in 200 children who were first or second degree relatives of subjects with premature onset coronary artery disease. Measurements were taken by a health visitor using a portable analyser. RESULTS: Twelve new cases of familial hypercholesterolaemia were identified during the first nine months of the study. Random cholesterol concentrations were within the normal range (< 5.2 mmol/l) in 70.5% of samples tested. Forty two (21%) of patients tested had a borderline cholesterol (5.2-5.9 mmol/l) but 50% of these fell within the normal range when fasting capillary samples were analysed. Children with significant hypercholesterolaemia on random testing (concentrations of > 5.9 mmol/l) (8.5%) also had fasting venous blood assayed for high density lipoprotein (HDL) cholesterol and tri-glyceride in the laboratory. Results indicated that 6.5% of patients screened were at high risk of cardiovascular disease (ratio of total: HDL cholesterol of > 4.5), and 1% had a moderately increased risk (ratio 3.5-4.5). CONCLUSIONS: Children with familial hypercholesterolaemia can be identified from a selected "high risk" population by measuring random capillary cholesterol concentration.