Fecal Chymotrypsin Research Articles

Randomized controlled study of digestive enzyme activity following trophic feeding

A randomized, controlled, prospective study of 80 preterm infants of birthweight less than 1750 g requiring ventilatory support was performed. While ventilatory support was required group TF (39 infants) received trophic feeding (0.5–1 ml h−1) along with parenteral nutrition, whereas group C (41 infants) received parenteral nutrition alone. When ventilatory support was no longer required milk feeds were started in group C and then increased in both groups until full milk feeds were established. The ratio of lactase to sucrase activity (L:S ratio) was measured in aspirated proximal intestinal fluid on 3 occasions: immediately after ventilatory support was withdrawn (TO), 7 days later (T7) and 14 days later (T14). On the same 3 occasions faecal chymotrypsin activity was measured. The mean difference (95% confidence interval) L:S ratio was significantly higher in group TF at both T0 and T14, 1.8 (0.03, 3.57) and 0.78 (0.2,1.35) U l−1, respectively. There was no significant difference in faecal chymotrypsin concentration. Conclusion: Trophic feeding alters relative intestinal disaccharidase activity, probably by inducing lactase production, but has no effect on pancreatic chymotrypsin activity.

Prospective study of pancreatic b-cell and exocrine function following duct decompression in tropical calcific pancreatitis.

Tropical calcific pancreatitis (TCP) is a chronic, nonalcoholic pancreatitis, which is limited to developing countries. In this condition, surgical decompression of the pancreatic duct consistently leads to relief of abdominal pain. However, no data are available on the effect of such intervention on pancreatic function. The aim of the present study was to prospectively evaluate b-cell and exocrine function following ductal drainage in patients with TCP. We studied 14 consecutive TCP patients who underwent ductal decompression for abdominal pain (longitudinal pancreaticojejunostomyin 12 patients, endoscopic sphincterotomy and ductal stenting in 2 subjects). Six patients who refused similar intervention served as controls. Patients were evaluated prospectively (median follow-up 13 months) for pain score, fasting and oral glucose stimulated plasma C-peptide, serum trypsin, and fecal chymotrypsin. After intervention, 1 patient died 2 months after surgery, and 2 others were lost in follow-up. The pain score improved significantly following duct decompression (median 8.0 vs. 0, p < 0.01), while in the control group there was no change in pain score (7.0 vs. 7.0). There was no change in b-cell function after intervention (fasting plasma C-peptide [mean +/- SEM] 0.41 +/- 0.08 vs. 0.42 +/- 0.05 nmol/l; peak plasma C-peptide 2.24 +/- 0.20 vs. 2.32 +/- 0.24 nmol/l). Fecal chymotrypsin was diminished in all patients prior to intervention (1.9 +/- 0.7 U/g), and did not normalize after ductal drainage in any subject. Serum trypsin levels were variable, being elevated in 29% and diminished in 47% of subjects. All 4 subjects with elevated baseline trypsin levels had a sharp fall after intervention (1020 vs. 175 ng/ml). However, serum trypsin did not normalize after ductal drainage in any patient with a diminished baseline value. In conclusion, patients with TCP have significant reduction in abdominal pain after decompression of the main pancreatic duct. However, there is no significant change in b-cell function. A fall in elevated serum trypsin suggests that there may be relief of subclinical inflammation after intervention; however, there is no improvement in exocrine function after a follow-up of 1 year.

Fecal elastase-1 as a test for pancreatic function: a review.

Pancreatic elastase-1 is a specific human protease synthetized by the acinar cells. Immunoreactive elastase-1 cannot be detected in either porcine or bovine pancreatic enzyme preparations. It is very stable and, in contrast to fecal chymotrypsin, elastase is unaffected by exogenous pancreatic enzyme treatment, and correlates well with exocrine pancreatic function tests. The measurement of this proteolytic enzyme in stool by means of an enzyme-linked immunosorbent assay (ELISA) is a sensitive, specific, and relatively inexpensive non-invasive test. It is an accurate function test for patients with chronic pancreatitis confirmed by endoscopic retrograde cholangiopancreatography and computerized axial tomography. It shows higher sensitivity and specificity for exocrine pancreatic insufficiency than fecal chymotrypsin determination and is comparable to oral pancreatic function tests such as the pancreolauryl test.

Both fecal elastase-1 and fecal chymotrypsin concentrations are decreased i vegetarians

Both fecal elastase-1 and fecal chymotrypsin concentrations are decreased i vegetarians

Both fecal elastase-1 and fecal chymotrypsin concentrations are decreased i vegetarians

Both fecal elastase-1 and fecal chymotrypsin concentrations are decreased i vegetarians

Diagnostic accuracy of fecal elastase 1 assay in patients with pancreatic maldigestion or intestinal malabsorption: a collaborative study of the Italian Society of Pediatric Gastroenterology and Hepatology.

Several reports have indicated that fecal elastase-1 (EL-1) determination is a new, sensitive, and specific noninvasive pancreatic function test; however, very few patients with malabsorption due to small intestine diseases have been included in the previous studies. The aim of the study was to compare the diagnostic accuracy of fecal EL-1 and fecal chymotrypsin (FCT) in distinguishing between pancreatic maldigestion and intestinal malabsorption. Three groups of subjects were studied: group A included 49 patients with known cystic fibrosis (25 males, median age 5 years); group B included 43 subjects with various small intestine diseases (17 males, median age 6 years); and group C included 45 children without any history of gastrointestinal disease (22 males, median age 5 years). In all patients, stools were collected for 72 h on a standard diet and fecal EL-1, FCT, and steatocrit tests were performed. Both EL-1 and FCT were below normal limits in all CF patients with pancreatic maldigestion not treated with pancreatic enzyme (100% sensitivity for both assays); El-1, but not FCT, was also below normal in all the CF patients with pancreatic maldigestion treated with pancreatic extracts. Both EL-1 and FCT values in the CF group were significantly lower than in subjects with various small intestinal diseases and in children without any history of gastrointestinal disease (P < 0.0001). FCT, but not EL-1, values showed an inverse statistically significant correlation with steatocrit values in the whole CF group (P < 0.001); FCT was below normal in three of four CF patients with steatorrhea on pancreatic enzyme therapy. Both EL-1 and FCT had 100% specificity when calculated in children without any history of gastrointestinal disease; in contrast, specificity was 86% for EL-1 and 76% for FCT if we considered the control group with small intestinal diseases: low EL-1 was observed in two cases of intestinal giardiasis, two cases of short bowel syndrome, one case of celiac disease, and one case of intestinal pseudobstruction; FCT was abnormal in four cases of intestinal giardiasis, three cases of celiac disease, one case of short bowel syndrome, one case of Crohn's disease, and one case of intestinal pseudobstruction. Diagnostic accuracy was 92% for fecal EL-1 and 82% for FCT. Steatocrit values were over the normal limit in 11 patients with small intestine diseases; in 7/11 of these patients at least one of the pancreatic test results was below the normal limit. In conclusions, in patients with CF, fecal EL-1 determination is not more sensitive than FCT in identifying pancreatic maldigestion; however, fecal EL-1 assay is more specific than FCT determination in distinguishing pancreatic maldigestion from intestinal malabsorption.

Fecal Elastase-1 Determination: ?Gold Standard? of Indirect Pancreatic Function Tests?

Background: Tubeless pancreatic function tests measuring the content of elastase-1 and the activity of chymotrypsin in stool are used with different cut-off levels and with varying success in diagnosing functional impairment of the pancreas. The aim of our study was to re-evaluate the sensitivity and specificity of elastase-1 and chymotrypsin in stool in the assessment of exocrine pancreatic insufficiency. Methods: In 127 patients displaying clinical signs of malassimilation, the secretin-caerulein test ('gold standard'), fecal fat analysis, fecal chymotrypsin activity and fecal elastase-1 concentration were performed. Exocrine pancreatic insufficiency was graded, according to the results of the secretincaerulein test, into mild, moderate and severe. Chymotrypsin and elastase-1 in stool were estimated using two commercially available test kits. Fecal elastase-1 concentration of 200 and 100 µg/g stool and chymotrypsin activity of 6 and 3 U/g stool were used separately as cut-off levels for calculation. Results: 1) In 65 patients, a normal pancreatic function was found using the secretin-caerulein test. In 62 patients, an exocrine pancreatic insufficiency was found and classified into severe (n = 25), moderate (n = 14) and mild (n = 23). 2) The correlation between fecal elastase-1 and chymotrypsin with duodenal enzyme outputs of amylase, lipase, trypsin, chymotrypsin and elastase-1 ranged between 33% and 55% and 25% and 38%, respectively. 3) Using a cut-off of 200 µg elastase-1/g, stool sensitivities of fecal elastase-1 and fecal chymotrypsin (cut-off: 6 U/g) were 100% and 76%, respectively (P < 0.0001 and P < 0.001 respectively) in severe exocrine pancreatic insufficiency, 89% and 47% respectively (P < 0.001; P = 0.34, respectively) in moderate and 65% for both in mild pancreatic insufficiency. Specificities of elastase-1 and chymotrypsin in stool were 55% and 47%, respectively. 4) Elastase-1 based diagnostic provided a positive predictive value of 50% using a 'cut-off' 200 µg/g stool in a representative group of consecutively recruited patients with gastroenterological disorders. Conclusion: Determination of fecal elastase-1 is highly sensitive in the diagnosis of severe and moderate exocrine pancreatic insufficiency and is of significantly higher sensitivity than fecal chymotrypsin estimation. Specificity for both stool tests is low. Correlation between elastase-1 and chymotrypsin in stool and duodenal enzyme outputs is moderate. Neither test is suitable for screening, as they provide a pathologic result in roughly half of 'non-pancreas' patients.

Evaluation of subclinical exocrine pancreatic involvement in primitive Sjogren's syndrome (pSS) by mixed triglyceride breath test and fecal chymotrypsin and its relation with Schirmer's test

Evaluation of subclinical exocrine pancreatic involvement in primitive Sjogren's syndrome (pSS) by mixed triglyceride breath test and fecal chymotrypsin and its relation with Schirmer's test

METABOLIC CONSEQUENCES OF ORTHOTOPIC PANCREATICODUODENAL TRANSPLANTATION WITH PRESERVATION OF NEAR NORMAL PHYSIOLOGY1

Several case reports suggested the use of pancreaticoduodenal allotransplantation alone or in combination with multivisceral transplants to treat exocrine and endocrine deficiency after pancreatectomy for chronic pancreatitis, upper abdominal malignancies, and cystic fibrosis. Our objective was to establish the metabolic consequences of this technique. Inbred rats, which either underwent pancreaticoduodenectomy before receiving an orthotopic duodenopancreas transplant (Tx, n= 18) or laparotomy (sham, n=18), were subjected 3 months postoperatively to oral and "isoglycemic" i.v. glucose tolerance tests with arterial blood sampling (n=12) or oral glucose tolerance test with additional portal blood sampling (n=6). Fecal fat and chymotrypsin were evaluated in the 11th postoperative week as indicators of pancreatic exocrine function in eight animals of each group. The incremental arterial plasma glucose integrated over a 90-min period was similar after oral and i.v. glucose in the respective groups, but was significantly lower in Tx versus sham rats after oral glucose. Incremental portal glucose was also lower after oral glucose, while hepatic glucose extraction remained unchanged. The incremental response of arterial glucose-dependent insulinotropic peptide, and of arterial and portal insulin, was comparable in Tx and sham rats; also in both groups the arterial response was significantly greater with oral versus i.v. glucose, and the incretin effect for insulin was intact after transplantation. Fecal fat and chymotrypsin levels did not differ between the two groups. 1) In the Tx rat lower incremental plasma glucose after oral glucose intake likely results from decreased intestinal glucose uptake; 2) preservation of a normal entero-insular axis of insulin together with the absence of intestinal malabsorption of lipids suggest that orthotopic transplantation of a duodeno-pancreas preserved endocrine and exocrine pancreatic function and therefore qualifies as treatment modality for the above named indications.

Median pancreatectomy for tumors of the neck and body of the pancreas

Background: When enucleation is too risky because of possible damage of the main pancreatic duct, benign tumors located in the neck or body of the pancreas are usually removed by a left (spleno)-pancreatectomy or by a pancreatoduodenectomy. But standard pancreatic resection results in an important loss of normal pancreatic parenchyma and may cause impairment of exocrine and endocrine function. The aim of this study was to evaluate early and longterm results of median pancreatectomy, a limited resection of the midportion of the pancreas, in selected patients with benign or borderline tumors of the pancreas. Study Design: Records of patients at Ospedale Busonera between November 1985 and September 1998 were reviewed. Ten patients with tumors of the neck or body of the pancreas underwent median pancreatectomy; the cephalic stump was sutured and the distal stump was anastomosed with a Roux-en-Y jejunal loop. Followup included clinical evaluation and routine laboratory tests: abdominal ultrasonography, exocrine and endocrine pancreatic function with fecal chymotrypsin, and an oral glucose tolerance test. Results: Pathologic examination showed: insulinoma (n = 3), mucinous cystadenoma (n = 3), nonfunctioning endocrine tumor (n = 1), papillary-cystic neoplasm (n = 1), serous cystadenoma (n = 1), and intraductal mucinous tumor (n = 1). Operative mortality and morbidity were 0% and 40%, respectively; pancreatic fistula occurred in three patients. At mean followup of 62.7 months, no recurrence was found and no patient had exocrine insufficiency or glucose metabolism impairment. Conclusions: Median pancreatectomy is a safe and effective alternative to major pancreatic resection in selected patients with benign or low-malignant lesions of the pancreas. This procedure carries a surgical risk similar to that of the standard operation, but avoids extensive pancreatic resection and pancreatic function impairment.

Proteases in the evaluation of pancreatic function and pancreatic disease

This paper reviews the role of pancreatic proteases (focusing upon trypsin, chymotrypsin and elastase) in the diagnosis and management of chronic pancreatic insufficiency (CPI), emphasizing advances over the last 5 years. Some important novel aspects of these enzymes in acute pancreatitis are also described, including their role in diagnosis and their interaction with cholecystokinin in the pathogenesis of the disease. The recent interest in these enzymes as agents promoting the spread of cancer in animals and human subjects is also described. A hierarchical approach has been taken to explore the advantages and limitations of tests in different source materials: serum, feces, duodenal aspirate, and non-invasive pancreatic function tests. The practical usefulness of fecal elastase-1 and of fecal chymotrypsin concentrations in diagnosis and management of CPI, respectively, is one of the major lessons to be learned from analysis of the recent literature, and forms the principal message of this review.

Impaired fecal elastase excretion in uremic pancreopathy.

To evaluate pancreatic exocrine function in uremia, 25 patients undergoing regular hemodialysis without clinical evidence of pancreatic disease and 25 healthy control subjects were studied by fecal elastase 1 and chymotrypsin. Abdominal ultrasonography and measurement of serum lipase, calcium, phosphate, and parathormone were also carried out. Fecal elastase was significantly lower (P < 0.001) in patients than in controls. Abnormally low values were found in 12/25 patients of whom six had values <100 microg/g. Fecal chymotrypsin was significantly lower (P < 0.05) in patients than in controls, with lower than normal values found in 10/25 patients. Fecal elastase was not related to the serum calcium, phosphate, or parathormone levels or to the period of dialysis. In patients serum lipase was normal or slightly elevated (<300 units/liter), and there was no evidence of pancreatic disease at ultrasound examination. The results lend further support to the existence of pancreatic function impairment in a significant number of patients with renal failure despite the absence of clinical and morphological evidence of pancreatic disease.

Orthotopic pancreas transplantation with portal venous drainage in rats. Surgical technique and metabolic effects(*).

Heterotopic pancreas transplantation in type I diabetic patients does not correct hyperglucagonemia, which is thought to be due to insufficiently suppressed glucagon release by the host pancreas. The diabetogenic effects of glucagon then have to be corrected by higher than normal insulin secretion from the transplant, with the attendant risk of earlier loss of islet cell function, and development of atherosclerosis. To establish whether this situation can be prevented, we investigated glucose homeostasis and blood lipids, as well as fecal fat and chymotrypsin as indicators for pancreatic exocrine function 14 weeks after orthotopic pancreas transplantation in inbred rats. The pancreas was resected before orthotopic transplantation of the donor pancreas with portal venous drainage (n=8). Laparotomized animals served as controls (n=8). Basal plasma glucagon, basal plasma insulin to glucagon molar ratio, and basal and integrated incremental responses of plasma glucose, insulin, and C-peptide after an oral glucose load (2 g/kg body weight) were similar in both groups. However, hepatic insulin clearance was slightly but significantly lower in the transplanted group (1.1+/- 0.1 vs 1.6+/-0.2; P<0.05). Basal plasma levels of free fatty acids, phospholipids, triglycerides, cholesterol, low-density lipoproteins, and high-density lipoproteins were unchanged after transplantation. Also unchanged were fecal fat and chymotrypsin levels, thus indicating preserved pancreatic exocrine function. We concluded that orthotopic pancreas transplantation with portal venous drainage achieves almost optimal metabolic control with respect to endocrine and exocrine pancreatic function as well as blood lipids. This technique could therefore be used to treat combined endocrine and exocrine insufficiency in chronic pancreatitis and thus enlarges the spectrum of indications for pancreas transplantation.

Is protein‐deficient diabetes mellitus a pancreatitis?

Malnutrition-related diabetes mellitus is a distinct clinical entity subdivided into protein-deficient diabetes mellitus (PDDM) and fibrocalculus pancreatic diabetes (FCPD). Whereas FCPD has obvious pancreatitis manifested by pancreatic duct calculi, the evidence for involvement of the pancreas in PDDM is limited to the presence of ketosis-resistant hyperglycaemia. We studied 10 patients with PDDM biochemically and radiologically. Endoscopic retrograde cholangiopancreatography was performed to determine if they had any evidence of chronic pancreatitis. Their mean faecal chymotrypsin level was low (13.2+/-5.72 microg/g), as was their basal c-peptide value (0.35+/-0.15 mmol/L). Islet cell antibodies were not detected in any of these patients. Ultrasound examination revealed pancreatic atrophy. In two patients, however, the pancreas was bulky. The ERCP showed generalized thinning of the pancreatic duct, measuring 2.4+/-0.06mm in the head, 2.01+/-0.08 mm in the body and 1.02 +/- 0.03 mm in tail region; side branches were seen but they were too sparse and thin. The significance of these changes is not clear, but they may represent an ongoing pancreatic disease and may, indeed, be the earliest changes of chronic pancreatitis.

Assessment of Exocrine Pancreatic Dysfunction in Chronic Pancreatitis

The clinical diagnosis of chronic pancreatitis is usually based on imaging studies, pancreatic function tests, and the presence of characteristic clinical features. In Japan, diagnostic criteria for chronic pancreatitis were established in 1995. The secretin test (a duodenal intubation test) and the combination of noninvasive tests, N-benzoyl-L-tyrosyl-p-aminobenzoic acid (BT-PABA) and fecal chymotrypsin (FCT), have been recommended for evaluating exocrine pancreatic function in patients with chronic pancreatitis. In the present study, the diagnostic value of these two noninvasive tests was compared to the secretin test. Although noninvasive tests are less sensitive and specific for determining exocrine pancreatic dysfunction than the secretin test, greater reliability for diagnosing chronic pancreatitis can be obtained by performing the BT-PABA and FCT simultaneously. Assessment of exocrine pancreatic function is important not only to diagnose chronic pancreatitis but also to decide a treatment method with pancreatic enzyme preparation.

Fecal elastase 1 determination in chronic pancreatitis.

This study assessed the diagnostic accuracy of fecal elastase 1 in chronic pancreatitis. Fifty-three healthy subjects, 44 patients with chronic pancreatitis (22 severe, 13 moderate, and 9 mild), and 43 patients with nonpancreatic digestive disease were studied. Elastase 1 concentration was determined on a small sample of feces using a commercially available kit. Fecal chymotrypsin was also measured. With a cutoff level of 190 microg/g, all healthy controls except one (98.1%), and the majority of patients with nonpancreatic digestive diseases (40 of 43; 93.0%) had elastase values above this limit. Among the 44 patients with chronic pancreatitis, 34 (77.3%) had pathological values: all 22 (100%) with severe disease, 10 of 13 (76.9%) with moderate disease and 2 of 9 (22.2%) with mild disease. Chymotrypsin values were pathological in 25 of 44 (56.8%) patients with chronic pancreatitis: 17 of 22 (77.2%) with severe pancreatitis, 7 of 13 (53.8%) with moderate pancreatitis, and 1 of 9 (11.1%) with mild disease. The specificity was 95.8% for elastase 1 and 85.4% for chymotrypsin. The difference both in sensitivity and specificity of the two enzymes was statistically significant (P < 0.05). Fecal elastase 1 has a high sensitivity, superior to that of fecal chymotrypsin, in the diagnosis of chronic pancreatitis. For its simplicity and rapidity, it could represent the tubeless test of choice in chronic pancreatitis.

Pancreatic exocrine and endocrine function after pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy

AIMTo evaluate long term detailed pancreatic endocrine and exocrine function in children with persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI) after 85–95% pancreatectomy.METHODSSix children with PHHI between 0.9 and 12.7 years...

Prevalence and significance of steatorrhea in patients with active Graves’ disease

Objective: The aim of this study was to determine the prevalence of steatorrhea in patients with Graves’ disease and to assess its significance and correlation with changes in body mass index (BMI), coefficient of fat absorption (COFA), and pancreatic exocrine function in these patients. Methods: Daily dietary fat intake, 24 h fecal fat, COFA, fecal chymotrypsin activity (as an index of pancreatic exocrine function), and total T3, T4, and TSH levels were assessed in 28 patients with active Graves’ disease. In 24 patients, reassessment was done after attaining a euthyroid state with carbimazole therapy. Results: In the thyrotoxic state, 13 of 28 patients had steatorrhea, whereas 15 had normal (<6 g/day) fat excretion (11.4 ± 6.7 g vs 2.9 ± 0.8 g, p = 0.0007). Daily fat intake, basal BMI, and serum T3 and T4 levels were similar in the steatorrheic and nonsteatorrheic groups. The mean COFA of the steatorrheic group was significantly lower than that of nonsteatorrheic group (91.6% ± 4.8% vs 97.7% ± 0.9%, respectively; p = 0.0006). In the steatorrheic group, fat excretion and COFA normalized after attainment of euthyroidism (changes in fat excretion and COFA = 7.3% ± 6.3 g/day and 7.7% ± 5.4%, respectively). Fecal chymotrypsin levels were similar in the steatorrheic and nonsteatorrheic thyrotoxics and in 16 healthy control subjects. The levels did not show any significant changes following attainment of euthyroid status. Conclusion: Steatorrhea associated with a decrease in COFA can occur in a reversible manner in 46% of patients with Graves’ disease. However, steatorrhea in these patients is not linked with weight loss or with pancreatic exocrine dysfunction.

Prevalence and significance of steatorrhea in patients with active Graves' disease.

The aim of this study was to determine the prevalence of steatorrhea in patients with Graves' disease and to assess its significance and correlation with changes in body mass index (BMI), coefficient of fat absorption (COFA), and pancreatic exocrine function in these patients. Daily dietary fat intake, 24 h fecal fat, COFA, fecal chymotrypsin activity (as an index of pancreatic exocrine function), and total T3, T4, and TSH levels were assessed in 28 patients with active Graves' disease. In 24 patients, reassessment was done after attaining a euthyroid state with carbimazole therapy. In the thyrotoxic state, 13 of 28 patients had steatorrhea, whereas 15 had normal (<6 g/day) fat excretion (11.4 +/- 6.7 g vs 2.9 +/- 0.8 g, p = 0.0007). Daily fat intake, basal BMI, and serum T3 and T4 levels were similar in the steatorrheic and nonsteatorrheic groups. The mean COFA of the steatorrheic group was significantly lower than that of nonsteatorrheic group (91.6% +/- 4.8% vs 97.7% +/- 0.9%, respectively; p = 0.0006). In the steatorrheic group, fat excretion and COFA normalized after attainment of euthyroidism (changes in fat excretion and COFA = 7.3% +/- 6.3 g/day and 7.7% +/- 5.4%, respectively). Fecal chymotrypsin levels were similar in the steatorrheic and nonsteatorrheic thyrotoxics and in 16 healthy control subjects. The levels did not show any significant changes following attainment of euthyroid status. Steatorrhea associated with a decrease in COFA can occur in a reversible manner in 46% of patients with Graves' disease. However, steatorrhea in these patients is not linked with weight loss or with pancreatic exocrine dysfunction.

Recurrent Pseudomonas bronchopneumonia and other symptoms as in cystic fibrosis in a child with type I pseudohypoaldosteronism

We report a child with multiple target organ pseudohypoaldosteronism type 1 with frequent recurrent pulmonary infections caused by Pseudomonas aeruginosa and Pasteurella multocida and high levels of chloride in sweat, urine and nasal secretion. Repetitive faecal chymotrypsin samples have all shown pathological values in spite of no other sign of exocrine pancreas dysfunction. The similarities with cystic fibrosis and the importance of the salt content in bronchial fluid are discussed.