Cystic Fibrosis of the Pancreas

Abstract

HomeRadiologyVol. 46, No. 4 PreviousNext EditorialCystic Fibrosis of the PancreasPublished Online:Apr 1 1946https://doi.org/10.1148/46.4.412MoreSectionsPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In AbstractWith the increasing knowledge of certain disease processes and the accumulation of data by various investigators, many conditions that were previously discovered only at the postmortem table are now being diagnosed in the living with relative frequency. Among these is cystic fibrosis of the pancreas. While this has been accepted as a clinical entity, actually it is in most instances a part of a generalized process consisting in fibrosis and insufficiency of the pancreas, glandular involvement, and chronic lung changes. These last, demonstrable roentgenographically, may establish the diagnosis.The work of Andersen (1) and of Black-fan and May (3) in 1938 did much to familiarize the medical profession with cystic disease of the pancreas as a disease entity. Their reports were the forerunners of a fairly numerous series by other workers who have added further details to our knowledge of the condition and the widespread changes associated with it.The etiology has not yet been satisfactorily established. Some have regarded the pancreatic changes as of congenital origin, but this theory has not met with general acceptance. A familial tendency has, however, been recorded by a number of observers. The disease is essentially one of early infancy, the average age of onset in one series of 35 cases (3) being two months, with death at eight months. Some patients survive longer, in which event a diagnosis of “celiac disease” is usually made.When death ensues shortly after birth, there is usually intestinal obstruction by thick mucilaginous meconium—so-called meconium ileus. If the child survives beyond this period, symptoms of chronic respiratory disease may so dominate the picture that the underlying condition may be missed entirely. In children living to six months or longer, the celiac syndrome-emaciation, abdominal distention, large fatty stools—becomes more obvious. At the same time the pulmonary changes persist and may be the immediate cause of death.Attwood and Sargent (2) have given an excellent description of the associated pulmonary lesions in cystic fibrosis of the pancreas. These consist, essentially, in a purulent bronchitis with bronchiectatic abscess formation and surrounding pneumonitis. The roentgen picture is one of infiltration throughout both lungs, decreasing toward the periphery, the greatest density occurring over the hilar areas. These changes suggest a chronic or subacute process rather than an acute one. Neuhauser (4), in a paper published in this issue of Radiology, recognizes two stages of pulmonary involvement: a pre-infection stage, which is characterized by emphysema and atelectasis secondary to obstructing phenomena, and a second stage of superimposed infection with the changes described above. Neuhauser gives, also, an excellent description of the roentgen features of meconium ileus associated with pancreatic insufficiency.Article HistoryPublished in print: Apr 1946 FiguresReferencesRelatedDetailsRecommended Articles RSNA Education Exhibits RSNA Case Collection Vol. 46, No. 4 Metrics Altmetric Score PDF download