Secrets, puzzles and mysteries of macroamylasemia

Physiological features of amylase synthesis and excretion are considered in the article, presence of other sources of amylase synthesis different from pancreas and salivary glands is emphasized. Definitions of hyperenzymemia and macroamylasemia (MAE) are given. MAE is a state characterized by presence of circulating complexes of normal serum amylase with protein or carbohydrates in blood. There are 3 types of MAE: first — classical (constant hyperamylasemia, decreased amylase level in urine, high blood concentration of macroamylase complexes); second — hyperamylasemia with slightly decreased amylase activity in urine, macroamylase/normal amylase ratio is less than in the first type; third — normal blood and urine amylase activity, low macroamylase/normal amylase ratio. Pathogenesis is explained by connection of blood amylase and acute phase protein in different inflammatory, infectious diseases, malabsorption. MAE clinical manifestations could be absent, sometimes abdominal pain is possible. Hyperamylasemia and reduced urine amylase activity are typical. MAE diagnostics means determination of macroamylase complexes in blood (chromatography, calculation of the clearance ratio of amylase and creatinine). The article presents clinical cases describing extra-pancreatic MAE in women with malignant ovarian lesions. The question of expediency of thorough diagnostic examination in asymptomatic MAE is raised, which may turn out to be a symptom of cancer. The lack of specific treatment for MAE is emphasized.

Risk Factors of Acute Pancreatitis in Oral Double Balloon Enteroscopy

Double balloon enteroscopy (DBE) was introduced 15 years ago. The complications of diagnostic DBE are rare, acute pancreatitis is most redoubtable one (incidence about 0.3%). Hyperamylasemia after DBE seems to be a rather common condition respectively. The most probable cause seems to be a mechanical straining of the pancreas. We tried to identify patients in a higher risk of acute pancreatitis after DBE. We investigated several laboratory markers before and after DBE (serum cathepsin B, lactoferrin, E-selectin, SPINK 1, procalcitonin, S100 proteins, alfa-1-antitrypsin, hs-CRP, malondialdehyde, serum and urine amylase and serum lipase). Serum amylase and lipase rose significantly with the maximum 4 hours after DBE. Serum cathepsin and procalcitonin decreased significantly 4 hours after DBE compared to healthy controls and patients values before DBE. Either serum amylase or lipase 4 hours after DBE did not correlate with any markers before DBE. There was a trend for an association between the number of push-and-pull cycles and procalcitonin and urine amylase 4 hours after DBE; between procalcitonin and alfa-1-antitrypsin, cathepsin and hs-CRP; and between E-selectin and malondialdehyde 4 hours after DBE. We found no laboratory markers determinative in advance those patients in a higher risk of acute pancreatitis after DBE.

Sex-related Differences in Effect of Ethanol Administration and Folic Acid Supplementation on Pancreatic Amylase in Rats

The present study was designed to determine whether folic acid supplement is sufficient to reverse the negative effects of ethanol consumption on amylase activity during gestation, lactation, and growth. Moreover, this study investigated the sex-related differences in amylase content in the pancreatic tissue, serum, and urine. The animals were randomized into three groups: Control group (CG) received water and a basic rat diet during pregnancy, lactation, and growth; Ethanol-rats (EG) were fed an ethanol diet during pregnancy, the suckling period, and growth until death; and Ethanol + folic acid group (E + FG) were handled the same way as those of EG, except they received a folic acid supplement from reproduction until the end of experimental period. Our results showed that ethanol consumption decreased the pancreatic amylase level in offspring rats at 2 months postpartum. Folic acid supplementation did not alter pancreatic amylase activities. In offspring males, ethanol administration decreased serum amylase activity at 2 months postpartum. Folic acid supplementation in males resulted in higher serum amylase levels than those corresponding to the ethanol-fed group. In females, no significant differences between groups in serum amylase levels were found. Ethanol consumption decreased urinary amylase excretion (at 30 days and 2 months postpartum), but the folic acid-supplemented group showed a more pronounced decrease in urine amylase activity than in the ethanol-fed group. At 30 days postpartum, no sex difference in urinary amylase was identified. However, in general, males showed higher values for urine amylase than females at 2 months postpartum. A folic acid-supplemented diet exerts an advantageous effect on amylase in serum in offspring males at 2 months postpartum of mothers fed ethanol during gestation and lactation periods, because amylase renal absorption is increased. In offspring females, amylase renal absorption is also increased, but we did not observed an advantageous effect on amylase in serum. It may be that sexual differentiation in females at 2 months postpartum exerts a definitive effect on amylase in serum. We found a sex-related difference in amylase activities; therefore, we suggest that in future all results of the exocrine pancreas function, in male and female animals, be analyzed separately.

Urine trypsinogen-2 as marker of acute pancreatitis

We examined the clinical utility of urine trypsinogen-2 as a marker of acute pancreatitis (AP). Fifty-nine patients with AP, 42 with acute abdominal diseases of extrapancreatic origin, and 63 without evidence of acute abdominal disease were studied. Urine trypsinogen-2 was determined by a time-resolved immunofluorometric assay. As reference methods we used serum trypsinogen-2, urine amylase, and serum amylase. The diagnostic accuracy of the markers was evaluated by receiver-operating characteristic (ROC) analysis. At admission, urine trypsinogen-2 differentiated patients with AP from controls with high accuracy. The area under the ROC curve (AUC) was 0.978, which was equal to that of serum trypsinogen-2 (0.998) and serum amylase (0.969) and significantly larger than that of urine amylase. For differentiation between severe and mild AP, urine trypsinogen-2 (0.730) was equal to serum trypsinogen-2 (0.721), and clearly better than amylase in serum and urine. These results suggest that determination of urine trypsinogen-2 is a useful test to detect AP and to evaluate disease severity.

Telangiectasia, ataxia, hypermobility syndrome, hypertrophic cardiomyopathy and diabetes - a new syndrome?

The proportion of people with genetic syndromes accompanied by diabetes mellitus or impaired tolerance to carbohydrates is less than 1% among all patients with diabetes mellitus. Currently, more than 70 such syndromes are described, in the clinical manifestation of which impaired tolerance to carbohydrates or diabetes mellitus is important. Examples include ataxia - telangiectasia, myotonic dystrophy, generalized or partial lipodystrophy. In the available literature, we have not seen any observations of the combination of diabetes mellitus with telangiectasias, ataxia, hypermobility of the joints, hyper-stretch of the skin, hypertrophic cardiomyopathy. We give an observation. Patient A., 15 years old, was admitted to the children's department of the Institute of Diabetes of the Endocrinology Research Centre of the Russian Academy of Medical Sciences with complaints of sharp weakness in the legs, inability to move independently, bleeding gums, nosebleeds, thirst, polyuria. Mother 39 years old, father 43 years old, sister 12 years old, all are healthy. A patient from the 3rd pregnancy, which proceeded with the threat of interruption throughout pregnancy. Two previous pregnancies in the patient's mother ended in miscarriages. Childbirth at the 8th month in the buttock presentation, with placental abruption. Body weight at birth 1800 g, body length 44 cm. The patient was born in asphyxia, with hemorrhages in the skin of the face. At the age of 2 years, 3 months after ARVI, the child developed shortness of breath, liver enlargement up to 6 cm from under the edge of the costal arch along the midclavicular line, and an increase in systolic blood pressure to 130 mm Hg. Art. At 3 years 4 months old, the diagnosis was established: idiopathic hypertrophic subaortic stenosis. From this age, the patient was periodically disturbed by severe abdominal pain, accompanied by nausea and vomiting, which were regarded as an exacerbation of chronic pancreatitis. The last attack at 15 years (urine amylase within normal limits). At 6 years of age, due to frequent nosebleeds observed from the age of 5, as well as telangiectasia of the skin vessels, he was diagnosed with OslerWeberRendu syndrome disease. In connection with the persisting hepatomegaly, a glucose tolerance test was performed at the age of 15 to exclude glycogenosis. A violation of tolerance to carbohydrates was revealed. After 1 month, symptoms of diabetes appeared. After another 1 month in a precomatous state, the patient was hospitalized in the regional children's hospital at the place of residence. At discharge, the daily dose of insulin was 44 units. 3 months after the manifestation of diabetes mellitus appeared weakness in the legs, which progressed rapidly. After 5 months, the patient was hospitalized at the Endocrinology Research Centre of RAMS.