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The Clinical Picture of Pancreatic Insufficiency CHARLES L The Clinical Picture of Pancreatic Insufficiency CHARLES L. BROWN, M.D.. Philadelphia THERE ARE TWO KNOWN FUNCTIONS of the pancreas, * Minor degrees of pancreatic insufficiency that of internal secretion of insulin, having to do may go unrecognized. There is a paucity of with the metabolism of carbohydrate, and that of symptoms and physical findings in mild and external secretion of enzymes, important in the proc- moderate degrees of insufficiency and in such ess of digestion. The insufficiency of insulin results circumstances laboratory methods are neces- in the classic disease of diabetes mellitus. The insuf- sary to determine the presence of insufficiency. ficiency of the external secretion may be more ob- The clinical picture when insufficiency is well scure in its clinical manifestations. Pancreatic in- established may be characterized by loss in sufficiency, with or without disturbance in carbo- weighf; vague indigestion; voluminous, light- hydrate metabolism, may be related to any of the colored, glistening stools in which fat globulets diseases of the pancreas which cause destruction or may be seen; changes in the concentration of impairment of function of the acinous glandular tis- pancreatic enzymes in the blood indicative of sue, such as chronic pancreatitis, tumor, hemor- lowered pancreatic function; diminished rhage, or stones. amounts of pancreatic enzymes in the duodenal Pancreatic insufficiency, theoretically, may arise juice, and the related poor digestion of fat and to some degree in other conditions in which the protein in the food. Lowered tolerance of car- nervous and/or humoral mechanisms of pancreatic bohydrate, as found in diabetes mellitus, may secretion are disturbed, but probably so-called func- or may not be present. The location and char- tional pancreatic insufficiency is of little or no clin- acter of the disease in the pancreas causing ical significance, since it would be expected to be so the insufficiency may or may not be apparent. mild, transient and masked by other symptoms as to be difficult of recognition. The external secretion of the pancreas contains dences of indigestion are immediate if complete three important enzymes: amylase, which acts on obstruction of the pancreatic duct occurs. Partial starch; lipase, which splits fats; and trypsinogen, obstruction of the pancreatic duct may interfere which, after activation to trypsin, acts on proteins. with the egress of the pancreatic juice only enough Insufficient amounts of these enzymes reaching the to make indigestion slower or intermittent in ap- intestinal tract produce the clinical features of the pearance. inadequate digestion of these three elementary food Minor degrees of pancreatic insufficiency may re- materials. Minor degrees of insfficiency go unrecog- main obscure or go unrecognized in chronic alco- nized. The digestion of fat and protein is not greatly holism, Laennec's cirrhosis of the liver, and gall- impaired until there is a pronounced decrease, prob- bladder disease. ably up to 75 per cent, in the lipase and trypsinogen Diseases which may have an associated pancreatic below hormal; and an even greater diminution of insufficiency are acute pancreatitis, carcinoma of the amylase is necessary before carbohydrate diges- pancreas, carcinoma of the ampulla of Vater, stone tion is measurably impaired. The appearance of in the ampulla or major ducts, and benign ulcer of gross evidence of the indigestion of these food ele- the duodenum involving the ampulla. ments, of which fat indigestion is the most easily Diseases in which pancreatic insufficiency most recognized, varies with the nature of the disease often occurs are cystic fibrosis of the pancreas and causing the insufficiency. In chronic pancreatitis the chronic relapsing pancreatitis. In chronic relapsing disease process, except for the acute episodes, is pancreatitis, the pancreatic insufficiency is usually a slowly progressive, and the changes resulting from late manifestation. the indigestion may not appear clinically for weeks, months or years after the onset of the disease. Evi- SYMPTOMS OF PANCREATIC INSUFFICIENCY From the Department of Medicine, Hahnemann Medical College The intent and purpose of this paper is primarily and Hospital, Philadelphia. concerned with the insufficiency of the external From the Symposium on Diseases of the Pancreas, presented before the Sections on G;eneral Medicine, General Surgery and Radiology at secretion of the pancreas; therefore, pancreatic dia- the 81st Annual Session of the California Medical Association, Los Angeles, April 27-30, 1952. betes mellitus, a form of pancreatic insufficiency of 172 CALIFORNIA MEDICINE the internal secretion, insulin, will not be included, vitamin deficiencies occur, and in particular the except as it may be related secondarily to severe and symptoms and physical findings of the deficiencies extensive destructive pancreatic disease. of vitamins A, B, D and K may be present, which The symptoms of pancreatic insufficiency per se will account for dryness of the skin, hyperkeratosis, are those of indigestion and malnutrition owing to glossitis, cheilosis, edema and neuritis, osteoporosis, the lack of proper digestive enzymes and inadequate and bleeding in the mucous membranes and skin. utilization of food materials. As was pointed out If the disease of the pancreas has been severe before, these inadequacies in slowly progressive dis- and extensive enough to involve enough islet cells, ease may be so minor, or so long in reaching clin- diabetes mellitus may be a part of the picture of ical proportions, as to remain unrecognized until pancreatic insufficiency. the disease causing the insufficiency is well advanced. Pancreatic insufficiency, in itself, probably does For these reasons the milder degrees of pancreatic not cause pain, and extensive and long standing pan- insufficiency may be without symptoms, and then creatic disease may not have associated pain. The accompanied by vague and indefinite symptoms of most severe and characteristic pain of pancreatic indigestion long before the gross evidences of the disease is associated with obstruction of the pan- nature of the insufficiency appear. creatic duct, or edema or hemorrhage into pancre- Acute pancreatic insufficiency accompanies com- atic tissue. However, the diseases of the pancreas plete, or nearly complete, obstruction of the pan- which lead to pancreatic insufficiency are frequently creatic duct, such as may occur with impaction by enough the cause of pain that the discussion of symp- stone, or compression by tumor or edematous in- toms would not be complete without some mention flammatory condition. The symptom of pain or of the location and character of pancreatic pain. painful discomfort owing to the obstruction so Indeed, the pain of pancreatic disease may lead to greatly overshadows the clinical evidences of the a diagnosis long before there is any clinical evi- insufficiency of enzymes that the clinical features of dence of pancreatic insufficiency. In the diagnosis insufficiency are unimportant except in the follow-up of chronic relapsing pancreatitis, one of the more observations, treatment and prognosis. common diseases with which pancreatic insufficiency The symptoms in chronic pancreatic insufficiency may be associated, the most important symptom is may be classified as general, digestive, nutritional, the recurrent episodes of upper abdominal pain." 2 and metabolic. This pain may be mild and last only a few hours, The general symptoms appear only in the more or it may be severe and prolonged. Usually it is profound and prolonged instances, and are manifest located in the epigastrium or more generally in the by weakness, fatigue, and gradual debilitation, prob- upper abdomen. At times it may be more on the ably related to the nutritional deficiency. right side of the upper abdomen and extend through The digestive symptoms vary in degree and char- or around to the corresponding level in the back, acter, but include sense of abdominal fullness, espe- which may simulate the pain of biliary colic. At cially in the upper abdomen, flatulence, anorexia, other times it may be on the left side in the upper nausea and minor abdominal cramping or discom- abdomen and extend to the corresponding level in fort. Vomiting may occur. The tendency is toward the back, and this location is considered more char- constipation. Diarrhea or loose stools appear some acteristic. At still other times it may be described time in the course of the symptoms, usually when as band-like in the upper abdomen and back. It may steatorrhea and creatorrhea are well advanced. shift in these different locations and, indeed, it may When the insufficiency is severe the stools are volum- extend into or radiate into the anterior chest and inous, fatty or greasy, rancid, and malodorous, char- of shoulder regions. It tends to be gradual in onset and acteristic of the indigestion fat. disappearance. The character of the pain may be The nutritional and metabolic symptoms are re- steady or cramping, cutting, aching, boring, or lated to the severity and duration of the insufficiency, burning. The recumbent position may aggravate the and they vary from being unrecognizable to the pain, so that the patient may prefer to sit in a profound symptoms of weight loss, liver functional up impairment, hypoproteinemia, hypocalcemia, vita- bent forward position. min deficiency, and the vague painful discomforts The great variation in location and reference of which may be associated with
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