Some 90% of patients with cystic fibrosis receive pancreatic enzyme supplements for management of the symptoms of exocrine pancreatic insufficiency.
PANCREATIC ENZYME SUPPLEMENTS
Some
90% of patients with cystic fibrosis receive pancreatic enzyme supplements for
management of the symptoms of exocrine pancreatic insufficiency (FitzSimmons,
1993). By reducing steatorrhea and faecal fat excretion, the supplements
improve the nutritional status of the patient. Pancreatic extracts have been
used for many years, but in 1994, five cases of stricture of the ascending
colon in chil-dren with cystic fibrosis who were receiving extracts were
published (Smyth et al., 1994).
Additional cases (Campbell, Forrest and Musgrove, 1994; McHugh, Thomson and
Tam, 1994; Oades et al., 1994; Freiman
and FitzSimmons, 1996; FitzSimmons et al.,
1997) suggested that the strictures appeared to be tempo-rally related to the
recent introduction of high-dose pancreatic supplements.
These
reports, and a further 35 cases of colonic stricture reported to the US Cystic
Fibrosis Founda-tion, prompted the Foundation to organise a Consen-sus
Conference to examine the use of pancreatic enzymes in patients with cystic
fibrosis (Borowitz et al., 1995). The
Conference used the term ‘fibros-ing colonopathy’ to describe ‘a condition
associated with ingestion of large quantities of pancreatic enzyme supplements’
and which leads to colonic strictures. It was considered that patients at
highest risk were those who were less than 12 years of age, have taken more
than 6000 lipase units per kilogram per meal for more than 6 months, have a
history of meconium ileus or distal intestinal obstruction, have had
intesti-nal surgery or have a diagnosis of inflammatory bowel disease.
Although
it was initially suspected that it was high-dose pancreatic supplements only
that were caus-ing fibrosing colonopathy, subsequently there were reports of
the condition in children with cystic fibrosis who were receiving low-dose
preparations (Jones et al., 1995;
Taylor and Steiner, 1995; Freiman and FitzSimmons, 1996; O’Keefe, 1996).
However, a dose-related risk for the development of fibros-ing colonopathy has
been suggested (Smythe et al., 1995;
Bakowski and Prescott, 1997; FitzSimmons et
al., 1997).
A
detailed review of early cases of fibrosing colonopathy and the chronology of
events following the introduction in the United Kingdom and the United States
of new forms of pancreatic supplements has been published (Bakowski and
Prescott, 1997). The authors suggest that the patterns of use of the pancre-atic
supplements and the development of fibrosing colonopathy are highly suggestive
of a dose-related causal role for preparations of which methacrylic acid
co-polymer is a constituent of the enteric coating. This confirmed an earlier
observation that methacrylic acid could be a key factor in the development of
fibrosing colonopathy (van Velzen, 1995).
More
recently, there have been anecdotal case reports of fibrosing colonopathy in
two adult patients receiving pancreatic enzyme supplements (Hausler et al., 1998; Bansi et al., 2000). The first was of
a 25-year-old woman who developed symptomatic fibrosing colonopathy several
months after begin-ning high-dose (17 000 lipase units per kilogram per day)
pancreatic enzyme therapy (Hausler et al.,
1998). The second involved a woman in her late 20s who had undergone
cholecystectomy for gallstone disease followed thereafter by endoscopic
management of common bile duct stones (Bansi et al., 2000). She later underwent a pylorus-preserving
pancreaticoduodenec-tomy and in the subsequent 7 years received large amounts
of pancreatic enzyme supplements. After developing a large bowel obstruction, a
right hemi-colectomy was undertaken and fibrosing colonopathy of the ascending
colon and caecum was confirmed by histology.
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