Ultrasonography of the pancreas, liver, and biliary system in cystic fibrosis

SUMMARY Abdominal ultrasound imaging was performed in 50 children. Thirty nine were proved to have cystic fibrosis while 11 had respiratory infections and thus acted as controls. The pancreas was abnormal in 75 % of cystic fibrosis patients aged under 5 years and in 95 % over 5 years. In cystic fibrosis patients over 5 years old who had malabsorption, the pancreas was abnormal in 100%. Abnormalities of the liver parenchyma were found in 23%, of the gallbladder in 24%, and splenomegaly in 8 % of the cystic fibrosis patients. Cystic fibrosis is the most common recessive in-herited disease in Caucasians with an incidence of approximately 1:2500, and one in 20 of the popula-tion are carriers. In large centres experienced in the management of cystic fibrosis, 80 % of patients now live beyond their 20th birthday, ' largely because of advances in the management of the respiratory complications. Increased survival has resulted in the growing importance of problems related to the dysfunction of the pancreas, liver, and biliary system. Detection of liver involvement in cystic fibrosis is notoriously difficult. Over inflation of the lungs makes radiological and clinical assessment of the liver and splenic size unreliable. Liver function tests do not become abnormal until relatively late in the disease and may not be seriously deranged even in the presence of oesophageal varices. Needle biopsy is of limited value in detecting the focal liver involvement.2 Ultrasound scanning can be used to detect and, by serial examinations, monitor the progress of liver changes and the signs of portal hypertension. Occasionally the diagnosis of cystic fibrosis is in doubt and the ultrasonic finding of a definitely abnormal pancreas can be helpful in supporting the diagnosis. Patients A total of 50 patients were scanned. Eleven were children with chest infections who acted as controls and 39 had typical cystic fibrosis with abnormal sweat electrolytes by the pilocarpine iontophoresis method. The age range was 1 to 21 years and 12 of the cystic fibrosis patients were under 5 year