Paediatric trauma with hyperamylasemia.

In this case report, we describe a paediatric case of hyperamylasemia following parotid trauma. A 12-year-old boy was hit by a motorcycle, sustaining only superficial lacerations to the face overlying the parotid. A hyperamylasemia was noted, and further characterised by a fractionated isoamylase test, as being predominantly of the salivary type. Serum lipase levels were low. Based on these investigations, pancreatic injury was judged unlikely with the minor parotid trauma being the probable source of the hyperamylasemia. The patient was spared further unnecessary investigations and managed conservatively.

A 5-year experience of benign pancreatic hyperenzymemia.

OBJECTIVE: Benign pancreatic hyperenzymemia is characterized by a long-term increase of serum pancreatic enzymes in otherwise healthy subjects. This study was designed to determine (a) whether all pancreatic enzymes are elevated, (b) the extent of each enzyme increase, (c) the relative frequency of the familial form, and (d) the relative frequencies of pancreatic and salivary hyperamylasemia and macroamylasemia. METHODS: Two hundred seven asymptomatic subjects with benign pancreatic hyperenzymemia were studied during the 5-year period. Serum amylase, isoamylase, and lipase levels were assessed by immunoenzymatic assays. RESULTS: Most (n = 183; 88.4%) patients had benign pancreatic hyperenzymemia; 155 (74.9%) patients had an abnormal increase of all 3 enzymes, 15 (7.2%) patients of only lipase, and 13 (6.3%) patients of only amylase and pancreatic isoamylase. Lipase levels were the highest (1.1-21 times above upper limit). Of the 183 subjects, 72 were members of 35 different families, 15 (7.2%) had increased salivary amylase, and 9 (4.3%) had macroamylasemia. Wide day-to-day fluctuations of pancreatic enzymes, including falls within the reference ranges, were recorded. CONCLUSIONS: All enzymes were increased in benign pancreatic hyperenzymemia, with lipase showing the highest elevation. Doctors should reassure patients about the benign nature of this condition and limit repeating useless examinations.

[Pancreatic hyperenzymemia: new advances in the field of clinical-diagnostic approach, with particular attention about Gullo's syndrome]. / Iperenzimemia pancreatica: recenti acquisizioni in tema di approccio clinico-diagnostico, con particolare attenzione alla sindrome di Gullo.

An increase in serum levels of pancreatic enzymes is a well-known manifestation of pancreatic disease, especially of inflammatory or neoplastic nature, even if several extrapancreatic diseases can equally cause that increase. In addition to this pathological type of hyperenzymemia, different "non-pathological" forms have also been identified, including macroamylasemia, salivary, and mixed salivary and pancreatic hyperamylasemia, in all of which only amylase elevations are seen. Nevertheless, in 1996 a new syndrome characterized by an abnormal, chronic, benign increase in levels of serum amylase, pancreatic isoamylase, lipase and trypsin, asymptomatic and usually discovered incidentally, was described for the first time by Lucio Gullo et al. Hyperamylasemia/hyperlipasemia's observation is nowadays on the increase because general practitioners tend to include more frequently amylase and lipase in routine blood tests and, moreover, because the constant evaluation of this biochemical alteration in the Emergency Unit: for this reason, this syndrome was clearly identified only recently. Therefore, it's characterized by serum elevation of all pancreatic enzymes in the absence of underlying diseases; it occurs in either sporadic or familial form and it persists over time with considerable fluctuation in serum enzyme concentrations, including frequent normalizations. Proper diagnosis of this form of hyperenzymemia is important because it reassures the subjects having this anomaly that the syndrome is benign, and because it can prevent multiple and expensive diagnostic tests or useless hospitalizations or therapies.

Pancreatic injury during AAA repair: a comparison between EVAR and open repair.

OBJECTIVES: Enzymatic pancreatic injury (EPI) in abdominal aortic aneurysm (AAA) treatment has been scarcely studied in the literature. Aim of this work was to compare perioperative EPI in AAA patients treated by endovascular repair (EVAR) or open repair (OR). METHODS: Forty AAA patients consecutively treated with either EVAR (GI, 20 pts) or OR (GII, 20 pts) were prospectively evaluated in terms of epidemiology, comorbidities and technical details. Serum levels of amylase, lipase and pancreatic isoamylase were assessed before treatment (T0), before aortic clamping/endograft deployment (T1), 1, 2, and 6 hours after aortic declamping/endograft deployment (T2, T3, T4) and 24, 48, and 72 hours after the procedure (T5, T6, T7). GI and GII were compared by Mann Whitney test with significance set at p < 0.05. RESULTS: GI patients were significantly older and with higher frequency of preoperative renal insufficiency than GII ones (p = 0.001 and 0.047 respectively). Other characteristics were not significantly different. Pancreatic enzymes values at T0 were within normal parameters in all patients. Total serum amylase was significantly greater at T4 (p = 0.003), T5 (p = 0.010), T6 (p = 0.003), T7 (p = 0.011) and isoamylase at T3 (p = 0.052), T4 (p = 0.037), T5 (p = 0.016) and T6 (p = 0.014) in GII compared with GI. Amylase and isoamylase peak occurred 24 hours after the procedure. Lipase was significantly different in the two groups only in T4 (p = 0.028). No acute pancreatitis occurred in the whole study group. CONCLUSIONS: EVAR significantly reduces EPI compared with OR in the AAA treatment.

Evidence of a generalized defect of acinar cell function in Shwachman-Diamond syndrome.

BACKGROUND: : 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. PATIENTS AND METHODS: : 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. RESULTS: : 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). CONCLUSIONS: : 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.

Magnetic resonance cholangiopancreatography in asymptomatic pancreatic hyperenzymemia.

OBJECTIVES: To determine the type and frequency of pancreatic lesions detected by magnetic resonance cholangiopancreatography (MRCP) in subjects with asymptomatic pancreatic hyperenzymemia and to assess for a possible relationship between these lesions and the hyperenzymemia. METHODS: From January 2005 to May 2008, 63 subjects with asymptomatic pancreatic hyperenzymemia were studied by MRCP. In addition, amylase, pancreatic isoamylase, and lipase were determined for 5 consecutive days. RESULTS: In most subjects (n = 57, 90.5%), MRCP showed a normal pancreas. In the remaining 6 subjects (9.5%), the following alterations were found: pancreas divisum in 2, small intrapancreatic cyst in 2, anatomic variant of the Wirsung in 1, and mild dilatation of 3 secondary ducts in 1. In these 6 subjects, hyperenzymemia was highly variable from day to day, with frequent normalizations, as was also true for the 30 subjects with no MRCP alterations in whom diurnal enzyme determinations were made. CONCLUSIONS: Most of the subjects with asymptomatic pancreatic hyperenzymemia did not have pancreatic lesions detectable by MRCP. In the few subjects in whom a lesion was found, the great variability and the frequent transient normalization of serum enzyme levels tend to exclude a relation between the lesion and the hyperenzymemia.

SPINK1 and PRSS1 mutations in benign pancreatic hyperenzymemia.

UNLABELLED: The aim of this study was to determine whether mutations in SPINK1/PRSS1 genes are associated with benign pancreatic hyperenzymemia (BPH). METHODS: Sixty-eight subjects with BPH (including 13 familial cases) were studied. In all, we sequenced germline DNA for all the exons and intro-exon boundaries of PRSS1 and SPINK1. RESULTS: Nine (13.2%) of the 68 subjects harbored PRSS1 or SPINK1 mutations. As to PRSS1, no hereditary pancreatitis-associated variant was detected, whereas previously undescribed mutations (p.Ala148Val and c.40+1G>A) were respectively found in 2 subjects (2.9%). SPINK1 mutations were detected in 7 subjects (10.3%). Five of them exhibited known mutations (3 p.Asn34Ser, 1 p.Pro55Ser, and 1 c.88-23A>T), whereas 2 had a newly found variant (p.Arg67Gly and c.*32C>T, respectively). Only 2 familial BPH, belonging to 2 different families, were found to carry a mutation (1 with p.Ala148Val for PRSS1 and 1 with p.Asn34Ser for SPINK1). CONCLUSIONS: No known mutations of PRSS1 have been found in BPH, whereas the frequency of known SPINK1 variants is similar to that reported in the general population. No segregation of PRSS1/SPINK1 variants occurs in BPH families. Benign pancreatic hyperenzymemia cannot be explained by mutations in genes whose variants are known to be associated with pancreatitis or by mutations in other PRSS1/SPINK1 genes.

HIV infection and the pancreas: risk factors and potential management guidelines.

One thousand and eighty-one evaluable HIV-infected patients were assessed for pancreatic abnormalities in a prospective case-control study including the whole follow-up period of each patient (minimum 12 months). The 435 patients (40.2%), who experienced at least one episode of confirmed pancreatic laboratory abnormality had a longer duration of seropositivity, exposure to protease inhibitors, a more frequent immunodeficiency, AIDS, chronic liver and/or biliary disease and hypertriglyceridaemia, while no relation was found with antiretroviral administration, and the duration of type of nucleoside analogues, when compared with the 646 controls. High and prolonged laboratory alterations eventually associated with signs of organ involvement occurred in 166 cases (38.2%), and were related to the administration of didanosine, stavudine, lamivudine, pentamidine, cotrimoxazole or antitubercular/antimycobacterial therapy, cytotoxic chemotherapy, illicit substance or alcohol abuse, opportunistic infections, chronic liver and/or biliary disease, a protease inhibitor-based highly active antiretroviral therapy (HAART) and hypertriglyceridaemia (usually associated with HAART administration). No difference was noticed between the 46 patients with clinical and/or imaging evidence of pancreatic involvement and the 120 asymptomatic subjects. Although recurrences of enzyme alterations involved 69.6% of patients, only in 30.1% of cases did a change of the underlying antiretroviral or antimicrobial therapy become necessary. An acute, uncomplicated pancreatitis occurred in nine of the 46 symptomatic subjects (19.6%). A two to four week gabexate and/or octreotide administration (performed in 79 cases of 166, 47.6%), achieved a significant laboratory, clinical and imaging cure or improvement in 82.3% of cases, with a better success rate of combined (gabexate mesilate plus octreotide) vs. single (gabexate mesilate or ocreotide) therapy. Reduced disease recurrences and a better tolerability of antiretroviral regimens, were also noticed.

Pancreatic cellular injury after cardiac surgery with cardiopulmonary bypass: frequency, time course and risk factors.

Although often clinically silent, pancreatic cellular injury (PCI) is relatively frequent after cardiac surgery with cardiopulmonary bypass; and its etiology and time course are largely unknown. We defined PCI as the simultaneous presence of abnormal values of pancreatic isoamylase and immunoreactive trypsin (IRT). The frequency and time evolution of PCI were assessed in this condition using assays for specific exocrine pancreatic enzymes. Correlations with inflammatory markers were searched for preoperative risk factors. One hundred ninety-three patients submitted to cardiac surgery were enrolled prospectively. Blood IRT, amylase, pancreatic isoamylase, lipase, and markers of inflammation (alpha1-protease inhibitor, alpha2-macroglobulin, myeloperoxidase) were measured preoperatively and postoperatively until day 8. The postoperative increase in plasma levels of pancreatic enzymes and urinary IRT was biphasic in all patients: early after surgery and later (from day 4 to 8 after surgery). One hundred thirty-three patients (69%) experienced PCI, with mean IRT, isoamylase, and alpha1-protease inhibitor values higher for each sample than that in patients without PCI. By multiple regression analysis, we found preoperative values of plasma IRT >or=40 ng/mL, amylase >or=42 IU/mL, and pancreatic isoamylase >or=20 IU/L associated with a higher incidence of postsurgery PCI (P < 0.005). In the PCI patients, a significant correlation was found between the 4 pancreatic enzymes and urinary IRT, total calcium, myeloperoxidase, alpha1-protease inhibitor, and alpha2-macroglobulin. These data support a high prevalence of postoperative PCI after cardiac surgery with cardiopulmonary bypass, typically biphasic and clinically silent, especially when pancreatic enzymes were elevated preoperatively.

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.

Day-to-day variations of serum pancreatic enzymes in benign pancreatic hyperenzymemia.

BACKGROUND & AIMS: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Can pancreatic steatosis explain the finding of pancreatic hyperenzymemia in subjects with dyslipidemia?

OBJECTIVE: It has been proposed by some investigators that benign pancreatic hyperenzymemia could result from pancreatic steatosis that they believe would have been caused by dyslipidemia; their diagnosis of steatosis was based on the finding of a hyperechogenic pancreas at ultrasound. The aim of this study was to assess the validity of this proposed model. METHODS: The study group was composed of 18 subjects with benign pancreatic hyperenzymemia, 12 men and 6 women; mean age, 55 years; range, 38 to 68 years. All 18 had dyslipidemia and 9 had hyperechogenic pancreas at ultrasound. In addition, 6 subjects with benign pancreatic hyperenzymemia but who did not have dyslipidemia or hyperechogenic pancreas and 10 healthy subjects with none of these conditions were also studied as controls. In each of these subjects, magnetic resonance imaging of the pancreas was performed to assess the presence of pancreatic steatosis. RESULTS: Magnetic resonance imaging showed normal pancreas with no signs of fatty infiltration in all 18 subjects with dyslipidemia, including those with both dyslipidemia and hyperechogenic pancreas at ultrasound. A similar result was found in all control subjects. CONCLUSION: The finding of a completely normal pancreas at magnetic resonance imaging does not support the proposed model in which pancreatic hyperenzymemia in subjects with dyslipidemia is attributed to pancreatic steatosis.

Serum total amylase, pancreatic amylase and lipase activities in epileptic children treated with sodium valproate monotherapy.

To investigate by a prospective, self-controlled method, whether early treatment with sodium valproate (VPA) monotherapy has some effect on serum total amylase and particularly on its pancreatic isoenzyme and lipase activities in epileptic children. Serum total amylase, pancreatic amylase and lipase activities have been evaluated in 23 epileptic children, before and at 6 and 12 months of VPA monotherapy. All children remained without clinical symptoms of pancreatitis during the period of study. Serum pancreatic amylase activities were significantly decreased at 6 and 12 months of treatment with VPA, whereas serum total amylase and lipase activities did not show any significant changes at 6 or 12 months of treatment. Non-pancreatic isoenzyme activities of amylase were significantly higher at 6 and 12 months of treatment. Three patients (13%) had slightly elevated serum total amylase levels at 6 and 12 months of treatment. There was no significant correlation of serum pancreatic amylase levels or non-pancreatic isoenzyme levels of amylase with serum VPA levels at 6 and 12 months of treatment. Non-pancreatic amylase activities, probably derived from salivary glands, may be increased in children treated with VPA monotherapy. Measurement of serum pancreatic amylase and/or serum lipase activities is indicated in patients with increased serum total amylase levels but without clinical symptoms of pancreatitis and, furthermore, in patients with symptoms suggesting dysfunction of pancreas, in order to avoid unnecessary discontinuing of VPA.

Unexplained elevated serum pancreatic enzymes: a reason to suspect celiac disease.

BACKGROUND & AIMS: The frequency of elevated serum pancreatic enzymes in patients with celiac disease (CD) is unknown. The aim of this study was to evaluate the serum levels of pancreatic enzymes in CD patients. METHODS: Serum pancreatic isoamylase and lipase levels were assayed in 90 adult and 112 pediatric consecutive CD patients at diagnosis and after 12 months of gluten-free diet (GFD). Serum elastase and trypsin levels were assayed in a subgroup of adult CD patients. Pancreatic ultrasonography was also performed. RESULTS: Twenty-six adult (29%) and 29 pediatric (26%) CD patients exhibited elevated values of serum pancreatic amylase and/or lipase; trypsin was elevated in 69% and elastase in 19%. The frequency of elevated serum pancreatic enzymes observed was identical in the patients with "typical" and "atypical" CD symptoms and in the asymptomatic patients. Most of the elevated values were lower than 2-fold the threshold limits. Elevated pancreatic enzymes were not associated with alcohol consumption, drug use, presence of abdominal pain, or diabetes mellitus. Abdominal ultrasound scan showed no abnormal findings in the pancreatic region in any of the CD patients. After 12 months of GFD, pancreatic amylase was elevated in 3 cases and lipase in 2 cases; these patients had not strictly adhered to the GFD. CONCLUSIONS: We demonstrated a frequency of about 25% of elevated pancreatic enzymes values in CD patients, including subjects without gastrointestinal manifestations and apparently asymptomatic subjects. The finding of elevated serum amylase or lipase level, in the absence of signs of pancreatic disease, would appear to suggest a need to screen for celiac disease.

Mutations of the CFTR gene in idiopathic pancreatic hyperenzymemia.

OBJECTIVES: Idiopathic pancreatic hyperenzymemia is a new syndrome that is characterized by a chronic increase of serum pancreatic enzymes in the absence of pancreatic disease. The aim of this study was to assess whether mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene may have a role in the etiology of this hyperenzymemia. METHODS: Seventy subjects with idiopathic pancreatic hyperenzymemia, 44 men and 26 women (mean age, 48 years; range, 8-74 years), were studied. Thirteen of these 70 subjects had the familial form of the syndrome. The mutation analysis of the CFTR gene was carried out using diagnostic commercial kits for the simultaneous detection of 29 mutations and Tn polymorphism. RESULTS: Among the 70 subjects studied, 7 (10.0%) had CFTR gene mutations. None of these 7 subjects had the familial form of pancreatic hyperenzymemia. These mutations were DeltaF 508 in 1 subject, 2789 + 5 G > A in another subject, and T5 allele in the remaining 5. All these mutations were heterozygous, with the exception of 1 T5 allele that was homozygous in 1 subject. CONCLUSIONS: The frequencies of the mutations of the CFTR gene found in these subjects are similar to the carrier frequencies in the general Italian population. This finding does not support a role for CFTR gene mutations in the etiology of idiopathic pancreatic hyperenzymemia.

Potential diagnostic value of pancreatic isoamylases for pancreaticobiliary maljunction with mild biliary dilatation in patients and a porcine model.

PURPOSE: The aim of this study was to evaluate the diagnostic value of serum pancreatic isoamylases for pancreaticobiliary maljunction (PBM) with mild biliary dilatation. METHODS: Serum and bile from 8 children with PBM and mild biliary dilatation (6 to 11 mm in diameter) and 4 young pigs with an anastomosis constructed between an isolated pancreas-duodenal segment and the gallbladder were studied for pancreatic isoamylases. Using an electrophoretic technique, the assay of pancreatic isoamylases was expressed by peak appearance rate (PAR). Serum from 20 healthy children served as normal controls. RESULTS: In the serum of the patients, preoperatively there were 5 pancreatic peaks with PAR as follows: P1, 100%; P2, 100%; P3, 100%; P4, 100%; and P5, 66.7%. These abnormal pancreatic isoamylases disappeared 2 weeks after operative treatment. In normal controls, there were only P1 (PAR, 40%) and P2 (PAR, 100%). Mild cylindrical dilatation (6 to 8 mm in diameter) of the common bile duct developed in the porcine PBM model. There were P1 (PAR, 100%) and P2 (PAR, 100%) in the porcine serum preoperatively. Thirty days and 60 days after establishing the model, there appeared in the serum 6 pancreatic peaks with PAR as follows: P1, 100%; P2, 100%; P3, 75%; P4, 100%; P5, 100%, and P6; 75%. The bile patterns of pancreatic isoenzymes in the patients and pigs were similar to those in serum. CONCLUSIONS: Abnormal pancreatic isoamylases are characteristically present in the serum from both children and a porcine model of PBM and mild biliary dilatation. Assay for these abnormalities is promising to recognize this subset of patients in whom diagnosis remains a challenge.