"The U.S. Food and Drug Administration today approved Vimizim (elosulfase alfa), the first FDA-approved treatment for Mucopolysaccharidosis Type IVA (Morquio A syndrome). Morquio A syndrome is a rare, autosomal recessive lysosomal storage disease "...
The most frequently reported adverse reactions to products containing pancrelipase are gastrointestinal in nature. Less frequently, allergic-type reactions have also been observed. Extremely high doses of exogenous pancreatic enzymes have been associated with hyperuricosuria and hyperuricemia when the preparations given were pancrelipase in powdered or capsule form, or pancreatin in tablet form.
Colonic strictures have been reported in cystic fibrosis patients treated with both high- and lower-strength enzyme supplements.10 A causal relationship has not been established. The possibility of bowel stricture should be considered if symptoms suggestive of gastrointestinal obstruction occur. Since impaired fluid secretion may be a factor in the development of intestinal obstruction, care should be taken to maintain adequate hydration, particularly in warm weather.11
“Fibrosing colonopathy” is a term used to describe a condition seen in patients with CF who have taken high amounts of pancreatic enzyme supplements ( > 6,000 lipase U/kg/meal). At its most advanced, this condition leads to colonic strictures.
- In whom should one consider the diagnosis of fibrosing colonopathy?
ESPECIALLY if they have:
- taken > 6,000 lipase U/kg/meal
- age less than twelve years
- history of meconium ileus
- prior intestinal surgery
- history of recurrent DIOS
- “inflammatory bowel disease”12
Read the Ultrase (pancrelipase) Side Effects Center for a complete guide to possible side effects
No information provided.
10. Smyth RL, van Velzen D, et al. Strictures of ascending colon in cystic fibrosis and high-strength pancreatic enzymes. The Lancet. 1994; 343:85-86.
11. Lands L, Zinman R, et al. Pancreatic function testing in meconium disease in CF: two case reports. J Ped Gastroenterol and Nut. 1988; 7:276-279.
12. Cystic Fibrosis Foundation Conference on Pancreatic Enzyme Supplementation in the Context of Fibrosing Colonopathy; Washington, D.C., March 23-24, 1995.
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Last reviewed on RxList: 1/20/2009
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