Prospective Study of Fatty Acid Supplementation Over 3 Years in Patients with Cystic Fibrosis

Kusoffsky, E.; Strandvik, Birgitta; Troell, S.

doi:10.1097/00005176-198302030-00007

Cited by 33 publications

(10 citation statements)

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“…Corn oil, saMower oil, and linoleic acid monoglyceride have been used in long-term linoleic acid supplementation trials in CF patients. Results were variable but most showed minimal (7,35) or no (36) improvement in EFA status; however, caloric intakes, when reported, were below the RDA for control subjects (7). Improvements of plasma linoleic acid was observed in CF patients when dietary supplementation was directed at increasing calories as well as linoleic acid (9,37).…”

Section: Discussionmentioning

confidence: 99%

Supplemental Calories Improve Essential Fatty Acid Deficiency in Cystic Fibrosis Patients

et al. 1988

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ABSTRACT. Fatty acid composition of plasma lipids was analyzed in malnourished cystic fibrosis patients undergoing 6 months of nutritional rehabilitation. There were three males and five females (mean age 15.1 yr); five patients had pancreatic insufficiency. Nutritional rehabilitation in seven of eight patients was accomplished by nocturnal nasogastric infusion of a high-carbohydrate semisynthetic diet, in addition to daily meals. One patient received highenergy food supplements as snacks in addition to regular meals. All patients were moderately to severely malnourished on entry to the study and showed significant improvement over the 6 months in (3 f SE) energy intake (96 + 8.0 to 126 f 11% recommended daily allowance) and body composition (80 f 4 to 90 f 4% ideal body weight). Daily intakes of linoleic acid were not significantly different before or during nutritional rehabilitation either as an absolute amount (383 f 45 to 557 f 124 mg/kg/day) or as a percentage of total calories (4.50 f 0.40 to 4.73 f 0.14%). In comparison to the controls, the relative percentage of plasma cholesterol ester fatty acids of the CF patients on entry into the study showed a marked decrease of linoleic acid (52.7 f 1.0 versus 42.3 f 2.7%) with elevated palmitoleic (2.34 f 0.2 versus 5.64 2 0.7%) and oleic (18.7 f 1.0 versus 25.2 2 1.4%) acids; a pattern consistent with essential fatty acid deficiency. However, this pattern is not truly characteristic of a pure linoleic acid deficiency as the metabolites of linoleic acid were not decreased. After nutritional rehabilitation the linoleic acid concentration reached control values in the phospholipid and cholesterol ester plasma lipids in six of eight and five of eight patients, respectively. These findings indicate a low caloric intake is an important factor in determining the essential fatty acid status of cystic fibrosis patients and recovery of both the body composition and essential fatty acid deficiency can be accomplished by increasing the caloric intake to 150% of recommended daily allowances. (Pediatr Res 24: 353-356,1988) Abbreviations EFA, essential fatty acid CF, cystic fibrosis RDA, recommended daily allowance Many investigators have described a high incidence of biochemical EFA deficiency in CF patients (1-5). These patients

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Section: Discussionmentioning

confidence: 99%

Supplemental Calories Improve Essential Fatty Acid Deficiency in Cystic Fibrosis Patients

et al. 1988

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“…This might be owing to the intensive substitution with Intralipid during that period, when approximately half of the patients received Intralipid regularly for 3 years. 15 There seemed to be a predominance of patients that were women up to 20 years of age (Table 2). At older ages there was no sex difference.…”

Section: Characterization Of the Study Population With Regard To Livermentioning

confidence: 99%

“…15 All patients were investigated annually for inflammatory status and lung function tests including forced vital capacity (FVC), forced expiratory volume in one second (FEV 1.0 ), chest radiograph, serum levels of retinol and ␣-tocopherol, liver function tests (LFTs), and the fatty acid pattern of serum phospholipids. LFTs included serum activities of alanine transaminase (ALT), aspartate transaminase (AST), and ␥-glutamyltransferase (␥GT) (with an upper reference level of Ͻ0.8, Ͻ0.8, and Ͻ0.5 µkata/l, respectively).…”

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confidence: 99%

Natural history of liver disease in cystic fibrosis

1999

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The median age of the population with cystic fibrosis (CF) has increased worldwide, which has led to the suggestion that the prevalence of liver disease would increase. The aim of this study was to evaluate the natural history of CF‐associated liver disease over a 15‐year period in a well‐controlled population of patients with CF. During the years 1976 through 1993, 124 patients were followed up by yearly liver function tests (LFTs). Fifteen patients were followed up with liver biopsies throughout the whole study period. More than 50% of the patients had pathological LFTs in infancy, later being normalized. Approximately 25% of children 4 years of age or older had biochemical markers of liver disease during the study period. In about 10% of the patients, cirrhosis or advanced fibrosis was confirmed at biopsy and 4% of patients had cirrhosis with clinical liver disease. Severe liver disease developed mainly during prepuberty and puberty. Of the 15 patients prospectively followed up with liver biopsies, only 3 had progressive fibrosis. No specific risk factor was identified, but deficiency of essential fatty acids was found more often in patients with marked steatosis (P < .05). No patient developed clinical liver disease in adulthood and the histological changes in the liver biopsies were usually not progressive. Liver disease was no more frequent at the end of the study period although the median age of the patient population had increased. Modern treatment might positively influence liver disease because it seemed less common, less progressive, and less serious than previously reported.

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“…24 A short-term benefit has been observed after intense supplementation with intravenous fatty acids. 169 However, parenteral therapy does not seem to offer substantial clinical benefit, and addition of routine intravenous therapy may add undue psychological stress to treated patients. 169,170 Orally administered omega-3 fatty acids in the form of fish oil may mitigate lung damage in patients with cystic fibrosis.…”

Section: ␤-Carotenementioning

confidence: 99%

Antiinflammatory Therapies for Cystic Fibrosis: Past, Present, and Future

Prescott

Johnson

2005

Pharmacotherapy

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Inflammation is a major component of the vicious cycle characterizing cystic fibrosis pulmonary disease. If untreated, this inflammatory process irreversibly damages the airways, leading to bronchiectasis and ultimately respiratory failure. Antiinflammatory drugs for cystic fibrosis lung disease appear to have beneficial effects on disease parameters. These agents include oral corticosteroids and ibuprofen, as well as azithromycin, which, in addition to its antimicrobial effects, also possesses antiinflammatory properties. Inhaled corticosteroids, colchicine, methotrexate, montelukast, pentoxifylline, nutritional supplements, and protease replacement have not had a significant impact on the disease. Therapy with oral corticosteroids, ibuprofen, and fish oil is limited by adverse effects. Azithromycin appears to be safe and effective, and is thus the most promising antiinflammatory therapy available for patients with cystic fibrosis. Pharmacologic therapy with antiinflammatory agents should be started early in the disease course, before extensive irreversible lung damage has occurred.

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Prospective Study of Fatty Acid Supplementation Over 3 Years in Patients with Cystic Fibrosis

Cited by 33 publications

References 0 publications

Supplemental Calories Improve Essential Fatty Acid Deficiency in Cystic Fibrosis Patients

Supplemental Calories Improve Essential Fatty Acid Deficiency in Cystic Fibrosis Patients

Natural history of liver disease in cystic fibrosis

Antiinflammatory Therapies for Cystic Fibrosis: Past, Present, and Future

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