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.
