CF Article

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Warning for CF Patients

Dec. 30, 2002 (Ivanhoe Newswire) -- A new study recommends physicians

monitor Cystic Fibrosis patients more closely for signs of liver

disease. Italian researchers report liver disease is a frequent and

early complication of CF, particularly in males. However, they say it

does not necessarily act any differently in CF patients than in those

without CF.

Patients with CF are now enjoying longer life expectancy than in the

past. Doctors point out, though, that liver disease is now emerging as a

significant medical problem. Liver disease is the third leading cause of

death in CF patients. The authors studied 177 CF patients without liver

disease and followed them for 14 years.

During that time, 48 patients developed liver disease, with cirrhosis

already present in five. Patients who were at high risk were males under

age 10 who had a history of meconium ileus (an obstruction of the

intestine) or who tested positive for a specific genotype. In fact, CF

patients with a history of meconium ileus were five-times as likely to

develop liver disease as those without it and were diagnosed earlier in

life. They also found that while the clinical advancement of the disease

follows the same course in CF patients as it does in others, in some

members of this group the advancement was rapid and quickly led to the

need for a transplant.

The researchers say the good news in all of this is liver disease did

not appear to exacerbate CF symptoms. They write, " We observed no

differences in the occurrence rates of other clinically relevant end

points such as respiratory failure, need of oxygen therapy, or number of

hospitalizations during the last years of follow-up between patients

with or without liver disease. " The say it is important for doctors to

screen for the disease as an early complication.

SOURCE: Hepatology, 2002;36:1374-1382

Becki

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