Pancreatic Enzyme Insufficiency Research Articles

Deficiency of lipolytic pancreatic enzymes in an adult man.

A case of severe steatorrhoea late after a Polya type subtotal gastrectomy in an adult man is presented, The cause of the steatorrhoea was found to be a selective deficiency of lipolytic pancreatic en

Pancreatic exocrine enzyme deficiency associated with asphyxiating thoracic dystrophy.

To analyze B lymphocyte central tolerance in a polyclonal immune system, mice were engineered to express a superantigen reactive to IgM of allotype b (IgM^b). <i>IgM^b/b</i> mice carrying superantigen were severely B cell lymphopenic, but small numbers of B cells matured. Their sera contained low levels of IgG and occasionally high levels of IgA. In bone marrow, immature B cells were normal in number, but internalized IgM and had a unique gene expression profile, compared with those expressing high levels of surface IgM, including elevated recombinase activator gene expression. A comparable B cell population was defined in wild-type bone marrows, with an abundance suggesting that at steady state ∼20% of normal developing B cells are constantly encountering autoantigens in situ. In superantigen-expressing mice, as well as in mice carrying the 3H9 anti-DNA IgH transgene, or 3H9 H along with mutation in the murine κ-deleting element RS, IgM internalization was correlated with CD19 downmodulation. CD19^low bone marrow cells from 3H9;RS^−/− mice were enriched in L chains that promote DNA binding. Our results suggest that central tolerance and attendant L chain receptor editing affect a large fraction of normal developing B cells. <i>IgH^a/b</i> mice carrying the superantigen had a ∼50% loss in follicular B cell numbers, suggesting that escape from central tolerance by receptor editing from one <i>IgH</i> allele to another was not a major mechanism. IgM^b superantigen hosts reconstituted with experimental bone marrow were demonstrated to be useful in revealing pathways involved in central tolerance.

Stool trypsin and chymotrypsin. Value in the diagnosis of pancreatic insufficiency in cystic fibrosis.

THE DIAGNOSIS of cystic fibrosis is classically based on a clinical picture of recurrent respiratory involvement in the form of cough, wheezing, and respiratory distress, and gastrointestinal signs of frequent, bulky, foul stools and hyperphagia. Confirmation of the diagnosis has depended on the demonstration of the elevation of sweat-sodium chloride and deficiency of pancreatic enzymes. In the past decade the ease and reliability of the sweat test has led to its use and substitution for the duodenal intubation for pancreatic enzymes. While some have completely stopped the appraisal of pancreatic insufficiency, others have utilized indirect techniques for assessment of digestive defect, eg, lipoidol excretion, stool trypsin by film test, radioactive fat absorption and excretion, and various absorption tests. With increasing experience in the differential diagnosis of cystic fibrosis, it has become apparent that there are a number of instances where the sweat test for sodium or chloride falls within the

Evaluation of 200 D(plus)-xylose blood level time curves as an index of intestinal absorption.

Two hundred consecutived (+) -xylose blood level time curves performed on 180 individuals using a previously described method were evaluated. It was found that the test is useful to indicate malabsorption states in idiopathic nontropical sprue, scleroderma, Whipple's disease, and diabetic diarrhea with steatorrhea, as well as in mesenteric lymphatic obstruction. It also shows low values in cases of multiple diverticula of the small intestine and cholecystoduodenostomy. It does not consistently demonstrate the malabsorption caused by regional enteritis, resection of the distal small intestine, and pancreatic enzyme deficiency. Complete pyloric obstruction interferes with the test.

Observations on secondary malabsorption syndromes of intestinal origin. Regional enteritis, lymphoma, jejunal diverticulosis, gastrojejunocolic fistula.

Article1 September 1961Observations on Secondary Malabsorption Syndromes of Intestinal OriginRegional Enteritis, Lymphoma, Jejunal Diverticulosis, Gastrojejunocolic FistulaHAROLD H. SCUDAMORE, PH.D., M.D., F.A.C.P.HAROLD H. SCUDAMORE, PH.D., M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-55-3-433 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptMalabsorption syndromes may occur in any of the diseases or conditions which impair absorption from the small intestine. It may be impaired by faulty digestion within the small intestine, by conditions that interfere directly with the process of absorption, or by abnormalities of the stomach.A classification based on the site and mechanism of the malabsorptive defect is given in Table 1 (1, 2). The origin of the defect may be from the small intestine or its mesentery, from gastric abnormalities, from pancreatic disease leading to a pancreatic enzyme insufficiency, or from hepatobiliary conditions causing a lack of bile salts....

The occurrence of intestinal atresia in newborns with meconium ileus. The pathogenesis of an acquired anomaly.

Obstruction of the intestine shortly after birth by plugs of inspissated, tenacious meconium—generally known as meconium ileus—has been recognized as a complication of pancreatic disease since the observations of Landsteiner 1 in 1905. Following the work of Kornblith and Otani, 2 indicating an associated congenital stenosis of the main pancreatic duct, and the observations of Dodd, 3 suggesting a deficiency of pancreatic enzymes as the cause of meconium ileus, Andersen's 4 studies of pancreatic achylia clearly indicated that meconium ileus was a complication of cystic fibrosis of the pancreas. The work of Farber, 5 which established fibrocystic disease as a generalized process, confirmed Andersen's view, and he also demonstrated that the physical abnormality of the meconium was related to defective production of pancreatic enzymes. He postulated that children who survived meconium ileus in the newborn period would later develop the usual clinical manifestations of fibrocystic disease. Later work has indicated

Pathogenetic Concepts of the Malabsorption Syndrome

The malabsorption syndrome discussed in this paper is characterized by defective absorption of nutrients from the upper small in. testine and the passage of an excessive amount of long chain fat in the stools, which may also be bulky, pale, fluid, and offensive, in the absence of previous surgery or obvious causative pathology. Other features of the syndrome may include lassitude, anorexia, loss of weight, anemia, ulcerated tongue, or buccal mucous membrane, bone changes, or stunted growth. In presenting the pathogenetic concepts of the malabsorption syndrome, consideration will first be given to the possible causes and significance of the changes in the stools, which may be common to all forms of the syndrome. Secondly, three conditions associated with the malabsorption syndrome will be discussed, in each of which the absorptive defect is due to a different cause. These are pancreatic enzyme deficiency, gluten-induced enteropathy, and sprue as seen in Hong Kong. Thirdly, the etiology and possible significance of the other features of the syndrome will be considered.

Effect of Dosage Schedule on the Efficacy of Substitution Therapy in Pancreatic Insufficiency

Summary The effect of pancreatin dosage schedules upon pancreatic steatorrhea was studied in 11 patients with pancreatic enzyme deficiency. Eight gm. of Viokase per day was more effective in the reduction of fecal fat when it was administered in 12 doses of 0.66 gm. hourly than when given in three doses of 2.7 gm. with each meal. In 3 cases the fecal fat returned to normal levels during the hourly regimen of Viokase administration. Excretion of fecal nitrogen, measured in 4 of the 11 patients, was also reduced to a greater degree by hourly administration of Viokase than when it was given in three doses. These data demonstrate the importance of the continuous presence of pancreatin in the gastrointestinal tract during the period of digestion in the treatment of pancreatic steatorrhea and azotorrhea.

Spruce vs. pancreatogenous steatorrhea; an evaluation of the small bowel study as an aid in differentiation.

The clinical and laboratory differentiation of the various causes of steatorrhea is often difficult. Recently interest has been aroused in the possibility of distinguishing sprue from pancreatogenous steatorrhea by roentgen examination. Numerous reports on this subject have appeared in the past two decades. Snell in 1939 (1) stated that it was not possible to make a distinction between these two conditions. Weber and Kirklin (2) are equally firm in their conclusion that the small bowel pattern of sprue cannot be distinguished from that of pancreatogenous steatorrhea. This might be said to represent the majority opinion today. Bjerkelund and Husebye (1950), on the contrary, believe that there are characteristics in the small bowel pattern that permit this differentiation (3). Weigen, Pendergrass, Ravdin, and Machella, in two experimental studies on dogs in 1952 (4) and 1953 (5), reinforced the affirmative side of the argument. These workers in the first of their studies extirpated the pancreas, and in the second ligated the pancreatic duct. They demonstrated that the two procedures did not alter the small bowel pattern in the dog. Golden (6), in 1941 wrote: “As far as can be determined now, the presence or absence of pancreatic enzymes makes no difference in the small intestinal pattern.” The published experience of the last two decades reveals two leading causes for steatorrhea. These are sprue and pancreatic intestinal enzyme deficiency. Three basic classifications of steatorrhea are generally accepted: a. Idiopathic, as seen in sprue and severe deficiency states. b. Pancreatogenous, resulting from a deficiency of pancreatic intestinal enzyme. Intrinsic inflammatory or neoplastic disease, duct obstruction, and extirpative surgery are the chief causes. c. Symptomatic, the result of obstruction of small bowel lacteals by neoplastic or inflammatory processes. This is a heterogeneous group, including amyloid disease, lymphoma, Whipple's disease, regional enteritis, and others. Reference to these classifications is found in two symposia by Ricketts, Maimon and Knowlton (7), and Durant and Zibold (8). Bjerkelund and Husebye (3), and Pearson (9) have also contributed. The present report will be concerned only with the idiopathic and pancreatogenous types, and will pertain only to the small bowel pattern of adults. Zwerling and Nelson (10) have demonstrated the inconstancy of this pattern in the normal pediatric group. Material This report is based upon small bowel studies in 19 cases of sprue, compared with those in 10 cases of pancreatogenous steatorrhea. These cases were seen on the Medical and Surgical Services of Duke Hospital during the last twenty-three years.