Serum Pancreatic Isoamylase Research Articles

Can Serum Pancreatic Amylase and Lipase Levels Be Used as Diagnostic Markers to Distinguish Between Patients With Mucinous Cystic Lesions of the Pancreas, Chronic Pancreatitis, and Pancreatic Ductal Adenocarcinoma?

This study aimed to assess the presence of pancreatic hyperenzymemia in patients with pancreatic cystic lesions as compared to other chronic diseases of the pancreas. Ninety-one patients were studied: 32 had mucinous cystic lesions, 35 had chronic pancreatitis (CP), and 24 had pancreatic ductal adenocarcinoma (PDAC). Surgery was carried out in 10 of the 32 patients with mucinous cystic lesion (7 of them had severe dysplasia), in 5 patients with CP, and in 9 patients with PDAC. Abnormally high serum pancreatic isoamylase activity was present in 11 (34.4%) patients with mucinous cystic lesions, in 14 (40.0%) patients with CP, and none in patients with PDAC (P = 0.002); whereas serum lipase activity was abnormally high in 8 (25.0%) patients with mucinous cystic lesion, in 17 (48.6%) patients with CP, and in 3 (12.5%) patients with PDAC (P = 0.009). In 7 patients with mucinous cystic lesions and histologically confirmed severe dysplasia, abnormally high levels of both serum pancreatic amylase and lipase were present in 3 (42.9%) patients. High serum concentrations of pancreatic amylase and lipase were found in no more than half of the patients with mucinous cystic lesions. High levels of pancreatic enzymes were not associated with a greater risk of malignancy.

The North American Shwachman-Diamond Syndrome Registry: Genetically Undefined Shwachman-Diamond Syndrome

Shwachman-Diamond syndrome (SDS) is an inherited marrow failure syndrome associated with exocrine pancreatic dysfunction and an increased risk of myelodysplasia and leukemia. The majority of individuals with SDS carry biallelic SBDS gene mutations, however a subset of patients remain genetically undefined. The objective of this study was to compare the clinical characteristics of patients with and without SBDS mutations. To address these questions, we conducted a retrospective study of patients enrolled on the North American Shwachman-Diamond Syndrome Registry (SDSR). Clinical data from the SDSR were available for 55 individuals with biallelic SBDS mutations and 16 individuals who fulfilled clinical diagnostic criteria for SDS but lacked biallelic mutations in the SBDS gene. Study subject ages for SBDS mutation positive and negative cohorts span 2-52.4 and 2.8-21.4 years with median ages of 12.4 and 10.9 years respectively. Cytopenias were present for both SBDS mutation positive and negative cohorts, with neutropenia the most common event in 94% and 81% respectively. Bone marrow hypocellularity was reported in 91% of those with SBDS mutations and 69% of those without. Marrow dysplasia was reported in 65% of those with SBDS mutations and none of those without. Clonal abnormalities were present in 44% and 25% of those with and without SBDS mutations with median age of initial appearance at 9 years (0.8-45.1) and 7 years (1.2-14) respectively. Abnormalities included del7q and del20q in both groups as well as iso7q, trisomy 8 and others in the SBDS mutation positive group. Clonal abnormalities were all transient in the SBDS mutation negative cohort. One SBDS mutation positive individual developed AML. None of the SBDS mutation negative individuals developed malignancy or progressed to require HSCT thus far. Pancreatic dysfunction determined by low serum trypsinogen or pancreatic isoamylase was similar in both cohorts 79% vs 80%. However, only 27% (15/55) of SBDS mutation positive individuals reported requiring enzyme therapy with 33% (18/55) documenting failure to thrive, in contrast to 75% (12/16) of SBDS mutation negative individuals with 73% (11/15) having failure to thrive. A broad spectrum of congenital anomalies were reported in 55% and 56% of SBDS mutation positive and negative individuals respectively, with skeletal anomalies being the most common in both groups. Medical comorbidities commonly reported in both groups included eczema and endocrinopathies. Elevated liver transaminases were seen in 27% of SBDS mutation positive individuals but this was not seen in the SBDS mutation negative cohort.Conclusion: Patients with genetically undefined (SBDS mutation negative) SDS share clinical characteristics with SBDS mutation positive patients; however, the risk of leukemia in the genetically undefined patients remains unclear due to low patient numbers with short follow-up. Further studies of this young cohort are required to inform medical management and to advance our understanding of genetic etiology, mechanism, disease pathophysiology and treatment of these marrow failure disorders. DisclosuresDale:Amgen: Consultancy, Honoraria, Research Funding, Speakers Bureau.

Variable Clinical Presentation of Shwachman-Diamond Syndrome: Update From the North-American Shwachman-Diamond Syndrome Registry.

AbstractAbstract 2367 Background:Shwachman-Diamond syndrome (SDS) is an autosomal recessively inherited marrow failure syndrome associated with exocrine pancreatic dysfunction and an increased leukemia risk. SDS is caused by biallelic mutations in the SBDS gene. Timely diagnosis prior to the development of life-threatening complications is essential for optimal medical management and outcomes. SDS hematologic complications such as severe marrow failure or leukemia are treated with a hematopoietic stem cell transplant. SDS patients require reduced intensity conditioning regimens to avoid undue regimen-related toxicities, thus the recognition of the underlying diagnosis of SDS is critical. The common constellation of clinical characteristics reported for SDS includes neutropenia, steatorrhea, and failure to thrive. Hypothesis:With the advent of genetic testing, diagnosis is now possible for patients with non-classical presentations of the inherited marrow failure syndromes. The aim of this study was to investigate the range of clinical presentations for SDS with the long-term goal of improving diagnosis. Methods:This study was a retrospective review of medical records obtained by the North American Shwachman-Diamond Syndrome Registry (SDSR) which was founded in 2008. The SDS Registry works in partnership with the Severe Chronic Neutropenia International Registry. Results:Genetic reports of biallelic SBDS mutations confirming the diagnosis of SDS were available for 31 patients. This SDS study cohort included 19 male and 12 female patients. Median patient age was 10 years with a range of 2 – 49 years. Radiologic reports were available for 23 patients. Pancreatic lipomatosis was noted in 22/23 patients. One patient had an atrophic pancreas without lipomatosis on CT scan at age 6.9 years. Two patients initially had normal early pancreatic studies by CT or ultrasound at ages 1.3 years and 2.6 years, but subsequently were found to have pancreatic lipomatosis by the same imaging modalities later in life. Fecal elastase has not been previously evaluated as a screen for SDS. 14/17 patients (82%) had low fecal elastase levels while 3/17 patients had normal fecal elastase levels. Serum trypsinogen or pancreatic isoamylase were low in all 18 patients tested. 26/31 patients (84%) presented with failure to thrive. 17/31 (54%) patients presented with diarrhea or steatorrhea. 17/31 patients (55%) had congenital anomalies. Neutropenia was the most common hematologic abnormality at presentation (77%). Strikingly, 2 patients presented with isolated thrombocytopenia, 3 patients presented with severe anemia requiring transfusion support, and 5 patients presented without any cytopenias. One patient presented with the initial diagnosis of MDS with a del 20q clone. One patient presented with short stature and a sibling who died from AML. Interestingly, one patient had very short telomeres (<1st percentile for age) across 3 lymphocyte subsets as well as in total lymphocytes, a pattern typically associated with dyskeratosis congenita. Conclusion:Data from the North-American Shwachman Diamond Syndrome Registry reveals an unexpected range of clinical presentation for this rare disorder that expands beyond that which is currently classically recognized. The diagnosis of SDS should be considered even in the absence of neutropenia or diarrhea. In our cohort, clues to the underlying diagnosis of SDS included other cytopenias, congenital anomalies, family history, and the del20q clonal marrow abnormality. Reliance on classical clinical descriptions of SDS would miss or delay diagnosis of a significant subset of SDS patients. Disclosures:Dale:AMGEN: Consultancy.

Asian chronic pancreatitis: The common and the unique

Chronic pancreatitis (CP) is a disabling condition worldwide that presents as pain, maldigestion, and diabetes. It is usually perceived as alcohol related, or classified as idiopathic otherwise. However, this is true only for Western countries. Asian CP comprises not only alcohol-related CP but also tropical pancreatitis (TP). Tropical pancreatitis is a unique entity commonly found in South Asia. It shares similar presentations with other CPs for pain and maldigestion. However, its diabetes is more ketosis resistant. Non-surgical approach like endoscopic therapy plus extracoporeal shock wave lithotripsy provides better outcome for TP than other CPs, because of its less strictured pancreatic duct that is more amenable to endoscopic stone clearance. Diagnosis of CP in Asia mainly relies on image studies such as ultrasonography ± computed tomography (CT) scan, and non-invasive tests on fecal chymotrypsin, serum trypsin, and serum pancreatic isoamylase. Endoscopic ultrasonography and intestinal tubing tests are used mainly in some research centers. Autoimmune pancreatitis (AIP) is another CP originated from Asia in large series and subsequently being recognized in the West. AIP patients seldom present with maldigestion until the disease progresses to a very late stage and this occurs in less than one third of patients. In contrast, AIP is usually presented as pseudotumor of the pancreatic head, causing obstructive jaundice. Immunoglobulin G4 level is typically elevated in AIP and can be used as a marker for responsiveness. Without a need of surgery, steroids are the standard treatment. Those who relapse and are resistant to steroids should be placed on long-term immunosuppressive agents.

Evidence of a Generalized Defect of Acinar Cell Function in Shwachman‐Diamond Syndrome

: Because the acinar cells of the exocrine pancreas in patients with Shwachman-Diamond syndrome (SDS) are severely depleted, we hypothesized that a similar deficiency may be present in acinar cells of the parotid gland. : We determined serum pancreatic isoamylase and parotid amylase activities in 16 patients with SDS, 13 healthy controls, and 13 disease controls (cystic fibrosis or fibrosing pancreatitis). Parotid amylase and electrolyte concentrations were measured in stimulated parotid gland secretions. Starch digestion was assessed by breath hydrogen testing in patients with SDS (with and without enzyme supplements) and healthy controls. : Serum pancreatic and parotid isoamylase values were lower in the patients with SDS than in the healthy controls (P < 0.0001 and P = 0.0002, respectively). Serum pancreatic isoamylase, but not parotid isoamylase, was significantly lower in the disease controls than in the healthy controls (P < 0.0001 and P = 0.17, respectively). Secreted parotid gland amylase concentration (units per milligram of protein) in patients with SDS was lower than that in the healthy controls (P = 0.04), whereas the disease controls were comparable to the healthy subjects (P = 0.09). Secreted parotid chloride concentration was inversely correlated with amylase concentration in the patients with SDS (P = 0.01), but no correlation was seen in the healthy controls or disease controls. When patients with SDS ingested starch without enzyme supplementation, their breath hydrogen excretion was significantly higher than that in the healthy controls (P = 0.009). Following starch ingestion with enzymes, breath hydrogen in the patients with SDS was lower (P < 0.05) than with no enzyme treatment, and no different from controls (P = 0.37). : Mutations in the SBDS gene cause a generalized functional abnormality of exocrine acinar cells.

A Study of Pancreatic Function among Subjects over Ninety Years of Age

Background: Among the various studies of pancreatic function in the elderly published so far, none have dealt with subjects over 90 years of age. The aim of this study was to examine pancreatic function in healthy individuals over 90 years old. Methods: Sixty-eight healthy noninstitutionalized elderly persons, aged 91–104 years, with a mean age of 95 years, and 63 younger controls were studied. Pancreatic function was studied by determining fecal elastase 1 concentration. In addition to this test, we also measured serum amylase, pancreatic isoamylase and lipase in 53 of the 68 elderly subjects. Results: All but 1 of the 68 elderly subjects had normal elastase 1 values; the one who did not had a value slightly below normal. No significant difference with controls was found. Serum pancreatic enzymes were normal in almost all of the 53 elderly studied; 3 had a mild elevation only of amylase and 1 had a persistent elevation of amylase, pancreatic isoamylase and lipase. Conclusions: In subjects over 90 years of age, exocrine pancreatic function continues to be normal; if an impairment occurs, it is mild and not significant for digestion of food. In addition, serum pancreatic enzymes remain within normal limits in the vast majority of cases.

Day-to-Day Variations of Serum Pancreatic Enzymes in Benign Pancreatic Hyperenzymemia

Benign pancreatic hyperenzymemia is a newly identified syndrome that is characterized by a chronic increase of serum pancreatic enzymes in the absence of pancreatic disease. When checked at intervals of months or years, the enzyme levels show considerable variation from one test to another, and enzyme normalization is sometimes seen. The purpose of this study was to determine whether these variations can occur on a day-to-day basis. Forty-two subjects with benign pancreatic hyperenzymemia, 28 men and 14 women, mean age 50 years, range 16-68 years, were studied. In each subject serum amylase, pancreatic isoamylase, and lipase levels were determined once daily on each of 5 consecutive days; in 15 of the 42 subjects studied, serum trypsin level was also measured. In 8 (19%) of the 42 subjects, the serum enzyme levels were abnormally elevated for all 5 days of the study but showed wide fluctuations. In 33 (78.6%) subjects, the hyperenzymemia showed considerable day-to-day variations that included normalizations. In the remaining subject (2.4%), all enzymes were normal. In most of the subjects with hyperenzymemia, 37 of 41, all enzyme levels were elevated, with lipase, and trypsin when performed, showing the highest values. Of the remaining 4 subjects, in 3 only amylase and pancreatic isoamylase were increased and in 1 only lipase. The results show that there are marked day-to-day variations, with frequent normalizations, of serum enzyme levels in subjects with benign pancreatic hyperenzymemia. The reason for these wide day-to-day fluctuations is not known.

Benign pancreatic hyperenzymemia in children

One of us recently described chronic pancreatic hyperenzymemia in the absence of pancreatic or other disease in adult subjects. The aim of the present study was to describe this form of pancreatic hyperenzymemia in children. The study involved 15 children with this condition seen from September 1996 to December 2004. After initial evaluation, each child was seen by us once annually until the end of the study. Each check-up included serum amylase (normal values 20-220 IU/L), pancreatic isoamylase (17-115 IU/L) and lipase (24-270 IU/L) determinations as well as abdominal ultrasound. In six children, fecal elastase was also determined. At the initial assessment, 13 of the 15 children had abnormal serum increases in all three enzymes, one showed an increase only in lipase, and in the remaining child all three enzymes were present in normal concentrations. These latter two children had had abnormally elevated levels of all enzymes at the examination performed before this study began. During follow-up, enzyme concentrations remained abnormally elevated, although with wide fluctuations and transient normalizations. Considering the highest values of enzymes found in the various examinations performed during this study, the increase over the upper normal limit was in the range of 1.5- to 5.2-fold for amylase, 2.2-to 8.0-fold for pancreatic isoamylase, and 2.0- to 15.4-fold for lipase. Fecal elastase was normal in the six children in whom it was measured. No child had evidence of pancreatic or other disease, either at initial evaluation or during follow-up. The results of this study describe benign pancreatic hyperenzymemia in children. Although it is a benign condition, awareness of it is important to avoid unnecessary concern and multiple diagnostic procedures.

Survival after coronary revascularization among patients with kidney disease

Factors liable to cause hyperamylasemia after hepatectomy were studied retrospectively in 140 patients with chronic liver disease.The Pringle maneuver was performed in 113 patients (Pringle group), the hemihepatic vascular occlusion technique in 21 (hemihepatic group), and no vascular occlusion in 6 (no-occlusion group).In the Pringle group, postoperative serum amylase levels were elevated significantly in comparison with the preoperative levels, but were not elevated in hemihepatic and no-occlusion groups. In the Pringle group, there were 4 patients whose postoperative serum amylase levels exceeded 3.5 times the upper limit of the normal range together with serum pancreatic isoamylase or lipase elevation or both. When compared with the other 109 patients, these 4 patients had a significantly longer vascular occlusion time (51 ± 3 minutes versus 94 ± 8 minutes, P <0.005). One of them developed pancreatitis and died from hepatic failure.Prolongation of portal congestion carries a potential risk of serum amylase elevation and pancreatitis after hepatectomy in patients with underlying liver disease.

Low serum amylase levels in drinking alcoholics.

We have reported that the serum level of amylase, different from other pancreatic enzymes, increases temporarily after abstinence in alcoholics. To elucidate the mechanism of this phenomenon, pancreatic isoamylase, salivary isoamylase, and amylase in urine were measured together with total serum amylase. Total serum amylase, pancreatic isoamylase, and salivary isoamylase values were measured in 38 male patients admitted to the National Alcoholism Center, Kurihama Hospital, for alcoholism after abstinence. In an investigation of amylase secretion, amylase in urine was measured in some patients after abstinence. In the group with abnormally high total serum amylase on admission, levels were found to decrease after abstinence. In patients with pancreatic disorders in this group, abstinence leads to a decrease in total serum amylase, but in patients with no such disorders, total serum amylase increases temporarily due to increases in salivary isoamylase. In the group with normal total serum amylase on admission, levels increased sharply after abstinence, and both pancreatic isoamylase and salivary isoamylase contributed to the gains. In the group with low total serum amylase, a sharp increase of 2-fold or more was noted after abstinence, and a major contributor was pancreatic isoamylase. The ratio of urine amylase to total serum amylase gradually declined, indicating clearly that abstinence led to a decrease in the excretion of amylase in urine. In cases of heavy alcohol consumption, a decrease in the production or secretion of pancreatic isoamylase and salivary isoamylase while drinking could happen. It was thus suggested that the increase in serum amylase might be due to the fact that this situation is improved by abstinence, plus the fact that excretion of amylase in urine increases during alcohol consumption, and abstinence brings about a decline in such excretion. Measurement of total serum amylase is not appropriate for diagnosing pancreatitis in alcoholic patients or those who consume large quantities of alcohol.

Serum pancreatic enzymes define the pancreatic phenotype in patients with Shwachman-Diamond syndrome

Objective: To evaluate the role of serum enzymes for defining the pancreatic phenotype in Shwachman-Diamond syndrome (SDS), an inherited multisystem condition. Study design: Serum pancreatic trypsinogen and isoamylase were measured in 164 patients known or presumed to have SDS. The diagnosis was confirmed in 90 patients. Among 74 unconfirmed cases, 35 (“probable SDS”) had hematologic dysfunction but lacked documented pancreatic dysfunction, whereas 39 patients (“improbable SDS”) lacked both documented pancreatic and hematologic dysfunction. Classification and regression tree (CART) analysis was performed in 90 patients with SDS and 134 control patients to establish a rule for defining the pancreatic phenotype of SDS; the rule was then applied to the patients with unconfirmed diagnosis. Results: In the control patients, serum trypsinogen showed little variation with age, whereas serum isoamylase values rose from birth on, attaining adult values by 3 years. For patients with SDS, serum trypsinogen values were low in young patients and tended to increase with age, whereas serum isoamylase values remained low at all ages. The CART rule combined results from both enzymes and classified the pancreatic phenotype in all but one SDS patient, who was <3 years of age. Excluding patients <3 years of age, CART identified the pancreatic phenotype in 82% and 7% of the “probable SDS” and “improbable SDS” cases, respectively. Conclusions: Serum pancreatic enzymes are useful for determining the pancreatic phenotype and confirming the diagnosis of SDS. (J Pediatr 2002;141:259-65)

Evaluation of amylase and lipase in the diagnosis of acute pancreatitis.

The diagnosis of acute pancreatitis relies heavily on a raised amylase. In the present study patients were prospectively categorized, without knowledge of pancreatic enzyme levels, into acute pancreatitis (AP; n = 51), disease controls (n = 35), indeterminate as to pancreatitis (n = 189) or exclusions (non-pancreatitis diseases where amylase may be elevated; n = 53). Enzyme levels were analysed by receiver operator characteristics (ROC) curves, with specificity > 80%. Day 1 serum lipase gave the greatest diagnostic accuracy (area under ROC curve = 0.128; P = 0.041 vs serum amylase). At the calculated diagnostic threshold of 208 U/L, lipase gave a sensitivity of 67% and a specificity of 97%. Other diagnostic thresholds (day 1) were: serum total amylase, 176 U/L (ROC 0.104, sensitivity 45%, specificity 97%), urinary total amylase, 550 U/L (ROC 0.108, sensitivity 62%, specificity 97%) and serum pancreatic isoamylase, 41 U/L (ROC 0.107, sensitivity 63%, specificity 85%). At delayed diagnosis (3 days) no enzyme was superior to lipase. The combination of lipase and amylase did not increase diagnostic accuracy. Serum lipase is recommended for diagnosis of AP, both early and late in the disease. Although highly specific when elevated, all pancreatic enzymes have low sensitivity for diagnosis.

Behaviour of serum pancreatic enzymes in chronic pancreatitis

Aim. To establish whether serum pancreatic enzyme determination is useful in the identification of patients with chronic pancreatitis and in revealing the presence of exocrine pancreatic insufficiency. Patients and methods. A total of 50 patients with chronic pancreatitis were included in the investigation: 19 were studied during a painful attack of the disease and 31 were observed during clinical remission of the disease and underwent a secretin-caerulein test; 21 of the 31 patients had severe pancreatic insufficiency. Thirty patients with non pancreatic digestive diseases were also studied. Serum amylase, pancreatic isoamylase, lipase, trypsinogen and elastase-1 were determined in all patients. Results. Serum levels of the 5 enzymes studied were significantly higher in patients with pancreatic pain than in those studied during a clinical remission of the disease, and in those with non-pancreatic digestive diseases. In patients with chronic pancreatitis studied during clinical remission of the disease serum levels of pancreatic isoamylase and trypsinogen were significantly lower than in those patients with non-pancreatic digestive diseases. Considering only low serum concentrations of the five enzymes studied in diagnosing chronic pancreatitis, trypsinogen showed a sensitivity of 28%, specificity of 100%, a predictive value of a positive test of 100% and a predictive value of a negative test of 96.4%. In the 21 patients with severe pancreatic insufficiency, abnormally low serum concentrations of trypsinogen were found in 12 patients (57%), of lipase and elastase-1 in 6 (29%), of pancreatic isoamylase in 5 (24%), and of amylase in 3 (14%). Conclusions. Serum pancreatic enzymes can not be considered a useful tool to identify patients with pancreatic insufficiency. However, of the five enzymes studied, serum trypsinogen appears to be a useful marker in the diagnostic work-up of chronic pancreatitis.

ISOLATED PANCREATIC AMYLASE DEFICIENCY-PROBABLE ERROR IN MOLECULAR BASIS

109 This study was to investigate further a 33 month-old Caucasian male found to have isolated amylase deficiency that appeared relevant to his failure-to-thrive. Secretin stimulated duodenal fluid collected at endoscopy at 20 months suggested isolated amylase deficiency (<3% of reference value), and normal pH, lipase ad proteases, that appeared to improve when repeated after catch-up weight gain on enzyme supplements to 3-10% of reference range. METHOD AND RESULTS: Serum pancreatic isoamylase activity measured by wheat germ enzyme inhibition was undetectable, but macroamylasemia, possibly suggesting disproportionate increase in salivary component was detected. Immuno-precipitation and Western blot analysis in the pancreatic fluid compared with positive and negative controls showed that the amylase activity was mainly from salivary isoenzyme and not pancreatic amylase at ages of 20 and 33 months, again suggesting compensatory salivary increase. Close homology to salivary isoform and multiple copies of genes made study of genomic DNA mutation difficult. We were able to isolate RNA from pancreatic fluid cellular debris to partly overcome these limitations. RT-PCR confirmed the presence of pancreatic amylase mRNA. cDNA sequences showed divergence in the amylase gene at 5′ end 927 bp coding region. Alignment with human pancreatic amylase revealed that most changes (7 out of 10 codon) were silent mutation, but Try67 to His, Cys118 to Ser and Thr178 to Val were significant. These three changes were located in the outer side of the core of activation domain of amylase. Combinations of these mutations however were documented in controls suggesting polymorphism. CONCLUSIONS: 1) apparent compensatory increase in salivary amylase was seen in duodenal fluid that can be misinterpreted as pancreatic isoform; 2) sequence divergence though probably from polymorphism could affect and lead to delay maturation; 3) method of isolation RNA from pancreatic fluid offers unique advantages in studying molecular basis of pancreatic enzyme deficiencies.

Influence of jejunal morphology changes on exocrine pancreatic function in celiac disease.

Concurrent exocrine pancreatic dysfunction may be one of the factors implicated in malabsorption in untreated celiac disease, as shown by studies on bicarbonate and pancreatic enzyme secretion. The purpose of this study was to evaluate exocrine pancreatic function in relation to jejunal morphology in celiac disease. Thirty-six patients fulfilling the ESPGHAN criteria for celiac disease, aged 3 to 18 years and 36 control subjects matched for age and sex were investigated. The design of the study included measurement of serum pancreatic isoamylase by a chromogenic method after selective inhibition of sialic isoamylase in the untreated phase in patients consuming a gluten-containing diet and after gluten elimination for a period of 1 year; fecal human elastase activity determined by enzyme-linked immunosorbent assay in patients consuming a gluten-free diet and again after gluten challenge for 6 months; correlation of serum pancreatic isoamylase and fecal elastase to the jejunal morphology, classified by criteria described by Marsch; the enzymes in the control group; and ultrasonography of the pancreas in both groups. Enzyme values obtained from celiac disease patients with normal mucosa were significantly higher than those obtained from patients with villous atrophy (p < 0.001) and comparable to those obtained from the control group. Serum pancreatic isoamylase activity increased to normal after gluten elimination, and human elastase activity decreased to values below 200 microg/g of stool after gluten challenge. Enzyme activity was related inversely to the degree of intestinal damage. The echogenicity of the pancreas was normal, regardless of enzyme activity or gut morphology. Exocrine pancreatic function is abnormal in celiac disease when mucosal atrophy is present. Exocrine pancreatic function parameters are associated with the changes of intestinal mucosal morphology in three consecutive phases of the disease.

Elevated pancreatic enzymes in inflammatory bowel disease are associated with extensive disease

OBJECTIVE: Our aim was to perform a cross-sectional study to estimate the prevalence of elevated pancreatic enzymes in patients with inflammatory bowel disease and to correlate the enzyme activities with clinical, endoscopic, and histological findings. METHODS: Two hundred thirty-seven patients diagnosed with inflammatory bowel disease (IBD), including a subgroup with known hepatobiliary disease, were studied cross-sectionally. Serum and urinary pancreatic enzymes were prospectively sampled and compared to endoscopic and histological findings obtained previously. RESULTS: Hyperamylasemia was found in 11% and hyperlipasemia in 7% of the total study group. The corresponding prevalences in patients with Crohn’s disease were 17% and 9%, those in ulcerative colitis 9% and 7%, and those in indeterminate colitis 10% and 5%, respectively. High levels of serum amylase and pancreatic isoamylase were associated with extensive colonic disease ( p < 0.005) and high histological activity ( p < 0.05). Amylase, but not lipase, was significantly elevated in patients with primary sclerosing cholangitis. Smokers showed higher urinary amylase levels than non- and ex-smokers. The use of medication had no influence on the enzyme levels. CONCLUSIONS: Pancreatic enzymes are elevated in a significant proportion of patients with IBD, and the enzyme increase is associated with a more extensive and active disease, and in some cases with primary sclerosing cholangitis.

Elevated pancreatic enzymes in inflammatory bowel disease are associated with extensive disease.

Our aim was to perform a cross-sectional study to estimate the prevalence of elevated pancreatic enzymes in patients with inflammatory bowel disease and to correlate the enzyme activities with clinical, endoscopic, and histological findings. Two hundred thirty-seven patients diagnosed with inflammatory bowel disease (IBD), including a subgroup with known hepatobiliary disease, were studied crosssectionally. Serum and urinary pancreatic enzymes were prospectively sampled and compared to endoscopic and histological findings obtained previously. Hyperamylasemia was found in 11% and hyperlipasemia in 7% of the total study group. The corresponding prevalences in patients with Crohn's disease were 17% and 9%, those in ulcerative colitis 9% and 7%, and those in indeterminate colitis 10% and 5%, respectively. High levels of serum amylase and pancreatic isoamylase were associated with extensive colonic disease (p < 0.005) and high histological activity (p < 0.05). Amylase, but not lipase, was significantly elevated in patients with primary sclerosing cholangitis. Smokers showed higher urinary amylase levels than non- and ex-smokers. The use of medication had no influence on the enzyme levels. Pancreatic enzymes are elevated in a significant proportion of patients with IBD, and the enzyme increase is associated with a more extensive and active disease, and in some cases with primary sclerosing cholangitis.

Serum pancreatic enzymes in patients with coma due to head injury or acute stroke.

Serum amylase and lipase were measured in 32 patients with cerebral ischemia, 19 with spontaneous cerebral hemorrhage, 15 with head injury and intracranial bleeding, and 22 with head injury without intracranial bleeding; 20 healthy subjects were also studied as controls. Serum pancreatic isoamylase concentrations were assayed in hyperamylasemic sera. The overall incidence of hyperamylasemia was 14% (12 of 88 patients: 4 with cerebral ischemia, 4 with spontaneous cerebral hemorrhage, 1 with head injury and intracranial bleeding, and 3 with head injury without intracranial bleeding). In 4 of the 12 patients the hyperamylasemia was of pancreatic origin: 1 patient with cerebral ischemia, 1 patient with spontaneous cerebral hemorrhage, 1 patient with head injury and intracranial bleeding, and 1 patient with head injury without intracranial bleeding. The incidence of hyperlipasemia was 7% (6 of the 88 patients: 1 patient with cerebral ischemia, 2 with spontaneous cerebral hemorrhage, and 3 with head injury without intracranial bleeding). We conclude that hyperamylasemia is more frequent than hyperlipasemia in patients with an altered state of consciousness due to head injury or stroke and is usually of non-pancreatic origin. This knowledge may save these patients from invasive and costly examinations.

FIFTEEN YEAR PROSPECTIVE STUDY OF PANCREATITIS IN A POPULATION OF PATIENTS WITH CYSTIC FIBROSIS

In Cystic Fibrosis (CF) pancreatic damage begins in utero. Pancreatic insufficiency (PI) is usually present at birth. In 15% of patients, the progression of pancreatic disease does not occur or is retarded. These patients are pancreatic sufficient (PS) and are susceptible to acute recurrent pancreatitis. The aim of this study is to investigate the frequency, natural history and outcome of pancreatitis in a CF population. Pancreatic function was assessed in 96 CF patients at diagnosis by measurement of serum pancreatic isoamylase activity (Phadebas PIA test, PIA normal values 40- 120 u/L). Fifteen CF patients (16%) who had pancreatic sufficiency (PS) (PIA 60-250 u/L) were followed up for episodes of pancreatic pain, evaluated with serial measurements of serum amylase, serum PIA and ultrasound of the pancreas. At the final phase, exocrine pancreatic function was monitored by serial fecal human pancreatic elastase (ELISA, Schebo-Tech, E1 normal values>200μg/gr stool). Clinical status was assessed by Schwachman score. Six of 15 patients with PS (40%) manifested recurrent, multiple episodes of acute pancreatic pain, marked elevation of pancreatic enzymes (PIA up to 2400 u/L) and evidence of pancreatitis on ultrasound. The episodes decreased gradually and serum PIA was stabilized at 60 to 90u/L. Gradual decline of fecal elastase to less than 200 (144, 170, 180) μg/gr stool was demonstrated in three while the remaining 12 PS patients had fecal elastase ranging from 257 to 2068μg/gr stool. Schwachman score in PS patients remained high throughout the observation period. It is concluded that acute recurrent pancreatitis occured in 6% of a CF population, exclusively in PS patients (40%). A subgroup of PS patients (20%) among those with recurrent pancreatitis developed PI and the need for pancreatic enzyme supplementation. Monitoring of exocrine pancreatic function in PS patients, especially in those with burnt out recurrent pancreatitis, is recommended.

Serum amylase elevation following hepatic resection in patients with chronic liver disease

Factors liable to cause hyperamylasemia after hepatectomy were studied retrospectively in 140 patient with chronic liver disease. The pringle maneuver was performed in 113 patients (Pringle group), the hemihepatic vascular occlusion technique in 21 (hemihepatic group), and no vascular occlusion in 6 (no-occlusion group). In the Pringle group, postoperative serum amylase levels were elevated significantly in comparison with the preoperative levels, but were not elevated in hemihepatic and no-occlusions groups. In the Pringle group, there were 4 patients whose postoperative serum amylase levels exceeded 3.5 times the upper limit of the normal range together with serum pancreatic isoamylase or lipase elevation or both. When compared with the other 109 patients, these 4 patients had a significantly longer vascular occlusion time (51 +/-3 minutes versus 94 +/- 8 minutes P<0.005). One of them developed pancreatitis and died from hepatic failure. Prolongation of portal congestion carries a potential risk of serum amylase elevation and pancreatitis after hepatectomy in patients with underlying liver disease.