Comparison of Effectiveness of Pancreatic Enzyme Preparations in Cystic Fibrosis

Mischler, Elaine H.; Parrell, Sara W; Farrell, Philip M.; Odell, Gerard B.

doi:10.1001/archpedi.1982.03970480026006

Cited by 32 publications

(23 citation statements)

References 8 publications

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“…Oral supplementation with Microlipid did increase significantly both plasma % 18:2 and RBC % 18:2. It should be recognized that the supplement was taken with pancreatic enzyme replacement in the form of enteric coated microspheres which we have unequivocally shown to be more effective in promoting absorption than the enzyme powder preparations used previously (20). The use of this type of enzyme plus compliance in taking Microlipid may explain why normalization of plasma and 18:2 levels was readily achieved in this study at a dose of approximately 0.45 g/kg/day of linoleate.…”

Section: Discussionmentioning

confidence: 69%

Correction of Linoleic Acid Deficiency in Cystic Fibrosis

et al. 1986

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ABSTRACT. To identify evidence of essential fatty acid deficiency, we screened 64 patients with cystic fibrosis by analyzing total lipid extracts from plasma. Forty-three had an abnormal linoleate (18:2) level (less than 26%). Thirteen deficient patients (aged 10-24 yr) ingested for 1 yr 7% of their total calories as linoleate derived from a daily supplement of Microlipid. Five deficient patients (aged 10-37 yr) served as controls. Plasma and erythrocyte fatty acid composition were monitored by gas chromatography of total lipid extracts seven times during the twelve month period. Prostaglandins E2 and FZa and their 15 keto 13, 14 dihydrometabolite, 6-keto F,,, and thromboxane B2 were measured by radioimmunoassay. Sweat tests, oxygen saturation, growth indices, clinical severity scores, compliance, and possible side effects from taking Microlipid were followed. Results showed that oral supplementation with Microlipid can significantly increase plasma and erythrocytes %18:2. One compliant patient died during the study and had normal tissue 18:2 levels. Nine of 13 patients gained more weight while taking Microlipid than in the previous year. No significant changes in sweat electrolytes, clinical scores, or oxygen saturation were found during the study year. Prostaglandin metabolites prostaglandin E2 showed an upward trend in supplemented patients, compared to controls. Prostaglandin FZa remained unchanged over 1 yr but showed a trend significantly downward over the final 6 months in supplemented patients. We conclude that linoleate deficiency can be corrected with daily Microlipid supplements and that correction may alter prostaglandin metabolism (Pediatr Res 20: 36-41, 1986) Abbreviations CF, cystic fibrosis 18:2, linoleate PGM, 13,14-dihydro-15-PGFza PG, prostaglandin EFA, essential fatty acid RBC, red blood cells Several workers (1 -3) have demonstrated markedly decreased concentration of essential fatty acid (EFA) in plasma from CF patients. The major changes include a decreased level of the predominant EFA, linoleate (1 8:2), and increased palmitoleate

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Section: Discussionmentioning

confidence: 69%

Correction of Linoleic Acid Deficiency in Cystic Fibrosis

et al. 1986

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“…Mischler and colleagues reported that enteric-coated pancrelipase improved both CFA (84.1% on treatment vs. 45.7% placebo; p b 0.005) and CNA (83.3% on treatment vs. 64.3% placebo; p b 0.01) [40]. A study of supplemental pancreatic enzyme therapy by Konstan et al compared two doses of pancrelipase (12,000 and 20,000 lipase units per capsule) to placebo [41].…”

Section: Discussionmentioning

confidence: 99%

EUR-1008 pancreatic enzyme replacement is safe and effective in patients with cystic fibrosis and pancreatic insufficiency

Wooldridge

Heubi

Amaro-Galvez

et al. 2009

Journal of Cystic Fibrosis

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“…Nitrogen absorption is usually adequately controlled on enzymes. 66,67 Carbohydrate malabsorption is minimal. 17 In specialist CF centers where fat absorption is not monitored regularly, even by semiquantitative methods, it is likely that a considerable number of patients will be taking unnecessarily high doses of enzymes.…”

Section: Control Of Malabsorption Achieved With Present-day Enzymesmentioning

confidence: 99%

Diagnosis and Treatment of Intestinal Malabsorption in Cystic Fibrosis

Littlewood

Wolfe

Conway

2005

Pediatric Pulmonology

102

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Summary. Intestinal malabsorption is severe and of early onset in virtually all people who have cystic fibrosis. The main cause is deficiency of pancreatic enzymes. Bicarbonate deficiency, abnormal bile salts, mucosal transport problems, motility differences, and anatomical structural changes are other contributory factors. Effective treatment should allow a normal to high-fat diet to be taken, control symptoms, correct malabsorption, and achieve a normal nutritional state and growth. Appropriate pancreatic enzyme replacement therapy will achieve normal or near-normal absorption in most people with cystic fibrosis. Early identification and treatment of intestinal malabsorption is critical to achieving optimal nutritional status. The occurrence of fibrosing colonopathy in a few patients on very high doses of those enzymes which have the copolymer Eudragit L30 D55 in their covering resulted in guidelines in the UK to avoid doses equivalent to more than 10,000 IU lipase per kg per day, and also to avoid preparations containing this copolymer in children and adolescents. For patients not responding to 10,000 IU lipase per kg per day review of adherence to treatment, change of enzyme preparation, variation in time of administration, and reduction in gastric acid may improve absorption. The importance of early investigation to exclude other gastrointestinal disorders as a cause of the patient's symptoms, rather than merely increasing the dose of enzymes, is stressed. With modern pancreatic enzymes in doses up to or only slightly in excess of 10,000 IU lipase per kg per day, adequate control of gastrointestinal symptoms and absorption can be achieved, and a normal nutritional state and growth rate maintained in most people with cystic fibrosis.

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Comparison of Effectiveness of Pancreatic Enzyme Preparations in Cystic Fibrosis

Cited by 32 publications

References 8 publications

Correction of Linoleic Acid Deficiency in Cystic Fibrosis

Correction of Linoleic Acid Deficiency in Cystic Fibrosis

EUR-1008 pancreatic enzyme replacement is safe and effective in patients with cystic fibrosis and pancreatic insufficiency

Diagnosis and Treatment of Intestinal Malabsorption in Cystic Fibrosis

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