Fibrosing colonopathy in cystic fibrosis: results of a case-control study

Smyth, Rosalind L; O'Hea, Una; Burrows, E.; Ashby, Deborah; Lewis, Peter; Velzen, D. van; Dodge, J A

doi:10.1016/s0140-6736(95)91860-4

Cited by 146 publications

(73 citation statements)

References 10 publications

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“…A UK case-controlled study showed both dose and preparations to be significant factors in the development of FC. 47 A similar US study found only the dose of lipase to be a factor. 48 The age of the patient, the clinician's view on the importance of the copolymer Eudragit L30 D55 (in the coating of some preparations) as a factor causing FC (see below), the dose of enzyme that is required to control malabsorption, the cost, and local availability will also influence which preparation is used.…”

Section: Choice Of Pancreatic Enzyme Preparationmentioning

confidence: 95%

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: Choice Of Pancreatic Enzyme Preparationmentioning

confidence: 95%

Diagnosis and Treatment of Intestinal Malabsorption in Cystic Fibrosis

Littlewood

Wolfe

Conway

2005

Pediatric Pulmonology

102

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“…Meanwhile, allergic reactions to the porcine proteins and some others side effects may occur: Pancreatin extracts are prone to form insoluble complexes with folic acid resulting in folate deficiency (Russell & al., 1980). One serious adverse effect has been reported by Smyth et al (1994Smyth et al ( , 1995 (Table 5). Fifty-three patients were enrolled, 40 of these patients completed the study.…”

Section: Safety Concerns Side Effect and Treatment Failurementioning

confidence: 99%

Emerging Approaches for the Treatment of Fat Malabsorption Due to Exocrine Pancreatic Insufficiency

Turki¹,

Kallel²

2012

New Advances in the Basic and Clinical Gastroenterology

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“…Kommentar 9 -1-15 und 9 -1-16 Im Wesentlichen von einer Arbeitsgruppe ist nach Gabe extrem hoher Dosen Pankreatin bei Kindern mit Mukoviszidose sehr selten (< 0,1‰) die Ausbildung einer fibrosierenden Kolonopathie mit Ileusgefahr beschrieben worden [362]. Eine Kausalität ist nicht belegt und gilt als wenig wahrscheinlich [363 -366].…”

Section: Leitlinie 1187unclassified