J Pediatr Gastroenterol Nutr 1989 Jul;9(1):17-20

Meconium ileus and its equivalent as a risk factor for the development o
cirrhosis: an autopsy study in cystic fibrosis

Maurage C, Lenaerts C, Weber A, Brochu P, Yousef I, Roy CC
Department of Pediatrics, Hopital Ste-Justine, Montreal, Quebec, Canada.

Abstract
Although dehydrated obstructing mucus is thought to account for the obstructive
pathology involving the lungs, the pancreas, the reproductive system, and the
intestinal tract, its relationship with CF-associated liver disease remains
largely hypothetical and little is known about possible risk factors. Complete
clinical and autopsy records were available in 38 of 73 deaths occurring over a
10-year period. The liver was normal in only five cases, and they were all
infants. Steatosis was the only lesion present in 9, hypoxic liver disease was
documented in 8, and biliary cirrhosis in 16 (focal in 10 and multilobular in
6). There was no relationship between the presence of cirrhosis, gallbladder
abnormalities, age at death, and clinical status recorded during the year
precoding their demise. Mucus plugs characterized by amorphous eosinophilic
material within proliferated bile ductules were present in 75% of children with
focal or multilobular biliary cirrhosis as opposed to 14% in those without (p =
0.015). A history of meconium ileus or its equivalent was recorded more
frequently (p = 0.038) in those with cirrhosis. Finally, biliary cirrhosis was
invariably present when there was a history of meconium ileus or its equivalent
and when mucus plugs were noted. These findings suggest that patients with
intestinal obstruction are at greater risk for the development of cirrhosis and
that strategies should be developed to increase the detergent capacity of bile
and its flow in order to decrease the viscosity of mucus in the biliary tree