Cystic Fibrosis of the Pancreas and Its Relation to Celiac Disease

Abstract

The pathology and pathologic physiology of celiac disease remain obscure in spite of the many attempts that have been made to understand them. It has become clearer in recent years that celiac disease is a clinical picture "characterized by arrest of growth, a distended abdomen, and attacks of diarrhoea with large, pale, foul-smelling stools" 1 rather than a disease entity and that the underlying pathologic condition may differ in different cases.2 A tradition exists that pancreatic steatorrhea can be readily differentiated from idiopathic steatorrhea by the low percentage of split fat in the stools associated with the former and the normal percentage characterizing the latter. A careful survey of the literature, however, reveals few cases of either disease in which careful clinical observations have been followed by adequate postmortem examination. The present study was initiated because of the findings in case 44 (XX), in which a patient with celiac disease who had a high percentage of split fat in the stools and who had responded favorably to treatment for celiac disease was found at autopsy to have cystic fibrosis of the pancreas. This demonstrated that the recognized criteria of differentiation between the steatorrhea due to pancreatic insufficiency and that due to other causes are unreliable.To establish more reliable criteria it is necessary to study cases in which normal acinar tissue has been proved by postmortem examination to be absent or inadequate and to compare them with cases of celiac disease associated with a normal pancreas. The plan of the present investigation has been to collect the cases in which pancreatic insuffi¬ ciency has been proved by microscopic examination of the pancreas, to

‘Double or quits’: perceptions and management of organ transplantation by adults with cystic fibrosis

“…CF is most commonly found in Caucasian populations; a lower occurrence of 1:3500 live births has been described amongst people from Mediterranean countries (Bossi, Battistini, & Braggion, 1999). In the early 1930s 70% of people with CF died before their first birthday (Anderson, 1938); however by 1993 the median survival age was reported to be 27.6 years (FitzSimmons, 1993). Further still, children born in 1990 have been predicted to live for 40 years on average (Elborn, Shale, & Britton, 1991).…”

Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis

“…orothy Hansine Andersen was the first person to comprehensively describe and document cystic fibrosis (CF) in the 1930s [1]. In subsequent decades, clinical practice evolved in a stepwise fashion by delivering improved symptom-modifying treatments that improved patient prognosis and quality of life.…”

Adult cystic fibrosis care in the 21st century

“…In 1938, 70% of babies died within the first year of life [7]. In contrast, in the last decade, the majority of CF patients survive into adulthood.…”

“…Since 1938 [30] liver disease has been a recognised complication of CF. In the United States 10.8% of patients with CF develop hepatic disease and 15% progress to liver failure needing liver transplantation [10].…”