Nutrition in adults with cystic fibrosis.
ABSTRACT Improved survival has been associated with better nutritional status in patients with cystic fibrosis (CF). In this study we examined the relationship between nutritional state and other measures of clinical severity in adult patients with CF, attending a regional centre. Eighty-one patients (median age 21 years) were studied. Patients with CF were significantly under weight, compared to healthy individuals but were of similar height. Measurements of lung function, FEV1 and FVC were significantly related to body mass index. Lung function was poorer in patients with chronic pseudomonal infection but body weight and body mass index were not significantly different compared to those without such infection. In 53 patients who were alive 4 years later, FEV1 had declined by -10.5 (2.1)% (P < 0.001) but there was no significant change in body weight 1.5 (6.5) kgs. In 23 patients who died or had lung transplantation the change from 1994 to the date of death or transplantation the FEV1 was reduced by -7.9 (11.2)% (P = 0.004) and body weight -2.8 (4.4) kgs (P < 0.01). In 12 patients who had supplemental enteral feeding, the median increase in body weight was 7|kgs over a period of 12 months. This study confirms that young adult patients with CF are significantly under weight and declining health is associated with significant weight loss. In patients with severe malnutrition significant improvement can be achieved by enteral feeding.
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ABSTRACT: The German cystic fibrosis (CF) quality assurance (CFQA) project is a patient registry for CF which was founded in 1995. Relevant clinical and laboratory data, respiratory function test results, complications, and CF treatments are entered into the database once a year for each patient. Using the database, a study was undertaken to elucidate the relationship between nutrition and lung function in a large patient cohort by cross sectional and longitudinal analysis. A cohort of 3298 patients above 2 years of age was analysed. Patients were grouped according to the presence or absence of malnutrition (wasting and/or stunting). Cross sectional and longitudinal analyses over 2 and 3 years including mixed model analyses were performed. The prevalence of abnormal weight for height (<90% predicted) increased with age from 19% in children aged <6 years to 38% in adults with CF. Patients with malnutrition had significantly lower mean values of vital capacity, arterial oxygen tension (PO(2)), and forced expiratory volume in 1 second (FEV(1)) and higher serum IgG (p<0.05). Pseudomonas aeruginosa infection was also associated with decreased pulmonary function. Malnourished adolescents aged 12-18 years experienced a serious decline in FEV(1) of about 20% predicted, whereas mean FEV(1) values remained stable at above 80% predicted in adolescents of normal weight. Longitudinal follow up showed that malnourished patients of all ages and those with P aeruginosa infection had significantly worse lung function than their normally nourished counterparts and a greater yearly loss of FEV(1) % predicted. During 1 year of observation adolescents who experienced a >5% predicted decrease in weight for height had a concomitant mean loss of FEV(1) of 16.5% predicted during that year, whereas patients who gained relative weight had a parallel increase in FEV(1) of 2.1% predicted. These data emphasise the close relationship between nutrition, lung function, and clinical course in CF. Normal body weight and absence of P aeruginosa infection was associated with better preservation of lung function.Thorax 07/2002; 57(7):596-601. · 8.38 Impact Factor
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ABSTRACT: Nutritional support is an integral part of the management of cystic fibrosis patients. It is arguably best provided by a qualified dietitian and nutritional care sister working in conjunction with the rest of the cystic fibrosis team. The patient's nutritional needs should be assessed, regularly reviewed, and nutritional treatment tailored to meet the changing clinical and psychosocial needs of the patient. Nutritional intervention is not without complications, and in particular attention to normal feeding behaviour and vigilance when instituting supplementary nutrition may prevent many feeding difficulties.Annual Review of Nutrition 02/1993; 13:111-36. · 9.16 Impact Factor
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ABSTRACT: A cystic fibrosis (CF) clinic for adults was established in 1977. We have reviewed the data on 164 patients who attended between 1977 and 1989. Twenty four patients had died, 11 being over 20 years after time of death. Of the 140 patients still alive, 61% were male and 53% were aged over 20 years. Only 55% were diagnosed by 1 year and 88% by 10 years. Almost all patients had respiratory symptoms and sputum culture yielded Pseudomonas species in 69%. Other respiratory problems included major haemoptysis and pneumothorax, each in 10%. We found a wide range of respiratory impairment among older patients. Among 3 patients aged over 23 years the mean (+/- S.D.) percent predicted FEV1 and FVC were 53.3% (+/- 18%) and 71.4% (+/- 20%) respectively. Mean weight in this group was 92.5% (+/- 14) of predicted. Malabsorption occurred in most patients and meconium ileus equivalent occurred in most patients and meconium ileus equivalent occurred in 34%. Other complications were clinical hepatomegaly (16%), diabetes mellitus (9%) and arthropathy (20%). Most patients were taking continuous antibiotics by mouth (89%) and by nebuliser (48%), beta-2 agonists by inhaler (57%) and oral steroids (29%). Almost all were taking multivitamins, pancreatic replacement therapy and multiple nutritional supplements. The number of CF "bed days" grew 12 fold since 1979 and the mean stay in hospital was double the hospital mean. The economic impact was such that over 1/4 of the annual hospital antibiotic budget was expended on CF patients.Irish medical journal 07/1991; 84(2):48-51. · 0.51 Impact Factor