Hepatic Cirrhosis Associated with Fibrocystic Disease of the Pancreas: Clinical and Pathological Reports of Five Patients

Abstract

The purpose of this paper is to describe five patients who developed an unusual type of hepatic cirrhosis in the course of fibrocystic disease of the pancreas, and to comment on the aetiology of the cirrhosis. These five patients were Examination revealed a pale, sick, wasted girl with flushed face, slightly cyanosed lips and clubbed fingers and toes. The chest was barrel shaped, the percussion note hyper-resonant, air entry poor, and fine rales were heard over the entire chest. The abdomen was protuberant, the superficial veins prominent, the liver 343 palpable three fingerbreadths below the right costal margin, its edge and surface feeling hard and nodular. The tip of the spleen was palpable. The patient's stools were large, pale, of the consistency of a thick porridge and malodorous. Analysis showed that there was 430h of fat by weight in the dried faeces and 670o of this quantity was split fat. The Mantoux test, performed with 0 1 ml. of 1 in 1,000 old tuberculin, and the Wassermann reaction were negative. The child coughed much yellow mucopus, from which Staphylococcus aureus was grown.Radiological examination revealed widespread and uniform mottling in the lung fields, diffuse emphysema, thick, heavy bronchovascular markings and enlarged, dense hilar shadows. The blood examination disclosed only a polymorphonuclear leucocytosis. Duodenal aspiration was not attempted as the child was too ill.The clinical course during the next four months until death on September 27, 1946, was slowly and steadily downhill. A persistent, distressing cough attended by expectoration of large quantities of mucopurulent sputum, an irregular temperature, cyanosis and emaciation were the prominent features. During most of this time the stools were bulky, pale, and foul smelling, and contained an excessive amount of fat. On several occasions the girl developed bronchopneumonia but only temporary relief followed the administration of penicillin; for a period of one month she had ascites which spontaneously cleared. An attack of severe bronchopneumonia terminated this distressing illness.

An approach for treating the hepatobiliary disease of cystic fibrosis by somatic gene transfer.

“…Evidence of hepatic dysfunction can be found in 20-50% of all CF patients, with up to 5% ofpatients developing cirrhosis (4)(5)(6)(7)(8)(9). The primary pathological defect in CF liver disease appears to reside in the biliary tract (10,11).…”

Aspects of bile acid metabolism in cystic fibrosis

“…Liver damage of varying degree is a recognized component of cystic fibrosis (CF) and histological evidence of this has been recorded at all ages from the neonatal period until adult life (Bodian, 1952;Webster and Williams, 1953;di Sant' Agnese and Blanc, 1956;Tyson, Schuster, and Shwachman, 1968). Characteristically these changes consist of hyperplasia and dilatation of biliary ductules, some of which contain plugs of bile-stained or eosinophilic material; there is also proliferation of fibroblasts and infiltration of chronic inflammatory cells into the portal tracts.…”

Cystic fibrosis

“…The most common finding is focal biliary cirrhosis [12,151], found in up to 25% of CF patients, while the more severe multilobulated biliary cirrhosis [38] occurs in 2-5%. The etiologies of these forms of cirrhosis are unknown.…”