alpha-chymotrypsin and beta-Thalassemia

The aim of this study was to evaluate MR imaging changes of the pancreas in patients with transfusion-dependent beta-thalassemia major.. Twenty patients with transfusion-dependent beta-thalassemia major were examined using MR imaging at 0.5 T, with spin-echo T1-weighted, fast spin-echo T2-weighted, and gradient-echo T2*-weighted sequences. Image analysis was performed to assess pancreas-to-fat signal intensity ratios for all pulse sequences. Pancreatic exocrine and endocrine function and serum ferritin levels were assessed. Twenty healthy volunteers underwent MR imaging with the same three sequences and served as a control group.. The pancreas-to-fat signal intensity ratio was significantly decreased in 17 (85%) of the 20 patients on spin-echo T1-weighted images (p < .05), fast spin-echo T2-weighted images (p < .01), and gradient-echo T2*-weighted images (p < .01) when compared with the 20 volunteers in the control group. The pancreas-to-fat signal intensity ratio was significantly increased in three (15%) of the 20 patients on spin-echo T1-weighted images (p < .01) and fast spin-echo T2-weighted images (p < .05). In addition, in the 20 patients, we found a significant correlation between increased pancreas-to-fat signal intensity ratios and decreased serum trypsin levels (r = -.77, p < .01 for spin-echo T1-weighted sequences; r = -.75, p < .05 for fast spin-echo T2-weighted sequences; and r = -.74, p < .05 for gradient-echo T2*-weighted sequences). Likewise, for the 20 patients, we found a significant correlation between decreased pancreas-to-fat signal intensity ratios and increased serum ferritin levels for gradient-echo T2*-weighted images (r = -.65, p < .01). No correlation was found for the other clinical parameters evaluated.. MR imaging revealed signal intensity changes in the pancreas of patients with transfusion-dependent beta-thalassemia major. Patients with a major impairment of the exocrine pancreatic function had higher signal intensity of the pancreas because of fatty replacement of the parenchyma.

Topics: Adolescent; Adult; Amylases; beta-Thalassemia; Blood Transfusion; Chymotrypsin; Feces; Female; Ferritins; Humans; Lipase; Magnetic Resonance Imaging; Male; Pancreas; Pancreatic Elastase; Pancreatic Function Tests; Trypsin

This study, using indirect tests, demonstrated that exocrine pancreatic function is impaired in a proportion of patients with beta-thalassemia major (TM), though this impairment is generally mild or moderate.. Impaired structure and function of the exocrine pancreas has been reported in patients with Beta-thalassemia major.. In this study we measured fecal fats and serum and fecal pancreatic enzymes in 30 patients (13 M, 17 F) with TM, mean age 22.1 yr (range 14-39) and compared them with those of a matched group of healthy controls. Results were correlated with age, serum ferritin, blood transfusion, and various nutritional parameters. Enzymes assays included: serum pancreatic amylase (PA), lipase (L), trypsin (T), fecal chymotrypsin (FCT), and fecal elastase (FE).. No patient was positive for steatorrhea. Comparison of the mean values showed a significant difference only for FE (p < 0.002). Using only the fecal tests as a reference, we found that 12 patients had FE values below the cutoff limit; of these, five had values between 100 and 185 micrograms/g, three between 50 and 99 micrograms/g and four below 50 micrograms/g. Ten patients had FCT values below the cutoff limit; seven presented impairment in both tests and six of them had FE values below 100 micrograms/g (including four diabetics). No correlations were found between enzyme values and mean serum ferritin values or mean blood consumption over the previous 3 yr. No correlation was found between FE and FCT levels or between enzymes and age.

Topics: Adolescent; Adult; Amylases; beta-Thalassemia; Chymotrypsin; Exocrine Pancreatic Insufficiency; Fats; Feces; Female; Humans; Hydrolases; Lipase; Male; Pancreatic Elastase; Trypsin