Maintenance of growth in cystic fibrosis despite reduction in pancreatic enzyme supplementation

Lowdon, Jacqueline; Goodchild, Mary C.; Ryley, H C; Doull, Iolo

doi:10.1136/adc.78.4.377

Cited by 18 publications

(8 citation statements)

References 3 publications

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“…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. 58 Following the description of excessive PERT as a cause of FC, dose reduction without deterioration in intestinal absorption was reported, 63,68 supporting the impression that doses were previously unnecessarily high in some people.…”

Section: Control Of Malabsorption Achieved With Present-day Enzymesmentioning

confidence: 96%

“…94 Matching the enzyme dose to the fat content of a meal has enabled significant reduction in dose to be made without compromising nutrition or growth. 68 Consideration should be given to changing to another brand of enzyme that, in the same dose, may result in a quite dramatic improvement in symptoms and absorption. Presumably the many differences in pharmacological characteristics may be relevant to their differing performance in practice, including their quantitative and qualitative enzyme content, 95 dissolution characteristics, 60 and size of the particles.…”

Section: Failure To Control Gastrointestinal Symptomsmentioning

confidence: 99%

See 1 more Smart Citation

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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Section: Control Of Malabsorption Achieved With Present-day Enzymesmentioning

confidence: 96%

Section: Failure To Control Gastrointestinal Symptomsmentioning

confidence: 99%

Diagnosis and Treatment of Intestinal Malabsorption in Cystic Fibrosis

Littlewood

Wolfe

Conway

2005

Pediatric Pulmonology

102

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“…First observations were made in Liverpool in five young boys who had been switched to high-dose pancreatin several months before they developed colonic obstruction requiring surgery. It remains unclear whether the new policy in pancreatic enzyme dosage could be a reason for this (Borowitz et al 1995;Lowdon et al 1998;Stevens et al 1998). The disease is generally a rare event, occurring exclusively in children below 12 years of age.…”

Section: Large Bowel Manifestations Of Cf: Submucosal Wall Thickeningmentioning

confidence: 99%

Pancreatic Disease

Lankisch¹,

DiMagno²

1999

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“…Most investigators and clinicians still make use of 72-h faecal collections for determining faecal losses of fat, water, nitrogen and energy for clinical investigations [2,5] and for monitoring patients with pancreatic insufficiency. However, different attempts have been made in order to develop alternative rapid and simple techniques [1,7].…”

Section: Introductionmentioning

confidence: 99%