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The aim of our study was to assess the metabolic consequences of short-term administration of growth hormone in children after gut resection and influence on polyamine production in red blood cells (RBC). Twelve children aged 4-60 months were studied. All children remained on parenteral nutrition and 11 also received oral feeding. Total non-protein energy intake was 429 +/- 86 kJ/kg body weight (BW)/day. Recombinant growth hormone (GH) was administered subcutaneously at a dose of 0.3 IU/kg BW/day for 10 days. Resting energy expenditure (REE; kJ/kg BW/day) was: 316.07 +/- 54.08 before and 346.04 +/- 54.40 during GH administration (P < 0.02), but daily weight gain before GH administration was significantly lower than during treatment. A significant increase of polyamine concentrations was observed in the RBC (spermidine: 30.1 +/- 15.1 and 43.8 +/- 24.9 nmol/ml packed RBC, P < 0.003; spermine: 15.6 +/- 5.1 and 19.6 +/- 10.6 nmol/ml packed RBC, P < 0.02) and in jejunal mucosa (spermidine: 172.10 +/- 142.35 nmol/g tissue and 193.92 +/- 108.15 nmol/g tissue). The authors concluded that increased polyamine concentrations in the RBC and jejunal mucosa reflect a cellular response to GH administration. The anabolic effect of GH results in higher weight gain, although increased REE may indicate increased energy requirements during GH treatment.

This study was designed to assess resting energy expenditure (REE) and nutritional status in children with hepatic and prehepatic portal hypertension in comparison with healthy controls. Twenty-five patients with portal hypertension (PHT) and a history of variceal bleeding were compared with 14 healthy volunteers selected after matching for age and sex. PHT patients were allocated to one of two groups: 11 children with liver cirrhosis and/or chronic hepatitis, aged 14.0 +/- 3.3 y (means +/- SD) or 14 children with extrahepatic portal vein obstruction, aged 12.3 +/- 2.8 y. The control group consisted of 14 healthy children, aged 14.0 +/- 1.8 y. REE (indirect calorimetry) assessed after an overnight fast was significantly higher in PHT patients than in controls when related to body mass (143.7 +/- 29.5 and 116.1 +/- 5.9 kJ/kg, respectively; p < 0.004), lean body mass (168.0 +/- 28.9 and 146.4 +/- 14.1 kJ/kg, respectively; p < 0.02), and body surface area (7480 +/- 736 and 6590 +/- 567 kJ/1.73 m2, respectively; p < 0.001). The ratios of measured REE to basal energy expenditure calculated from standard equations (Schofield equations) indicated higher REE in PHT patients (102.24 +/- 6.90% and 93.54 +/- 4.47%, respectively; p < 0.001). Fat was the predominant source of energy in both PHT patients and controls; the percentage of nonprotein energy derived from carbohydrate oxidation was equaled: 36.04 +/- 18.84% and 37.15 +/- 15.71%, respectively. Analysis of percentage of undernutrition in PHT patients and controls revealed significant differences (44% and 21%, respectively; p < 0.001). Children with PHT are susceptible to malnutrition and have elevated REE compared with healthy controls. Fat is the principal basal state oxidative substrate for patients with PHT and healthy children.

Objective To assess the clinical efficacy and the impact of infliximab (IFX) induction therapy on mucosal healing in Polish children with Crohn's disease (CD). Methods A total of 66 children (29 boys and 37 girls) aged 14.06 ± 3.59 years with CD diagnosed at the mean age of 8.4 ± 7.3 years were included in the study. Patients received IFX (5 mg/kg) in three repeated infusions at 0, 2, and 6 weeks. The clinical activity of the disease was assessed using the Pediatric Crohn's Disease Activity Index (PCDAI) and the endoscopic activity was scored using the Simple Endoscopic Score for Crohn's disease at baseline and at week 10. Results Twenty-two (33%) of the studied patients reached clinical remission (PCDAI ≤ 10), 26 (39%) showed a clinical response (PCDAI between 15 and 30), and 18 (28%) did not respond to the therapy. When comparing data at baseline and at week 10, significant decreases were observed in the median PCDAI, C-reactive protein, and platelet count. In addition, a significant increase in BMI was noted. A significant decrease in the Simple Endoscopic Score for CD was observed between the initial and the control colonoscopies. Fifteen out of 66 patients (22.7%) had score 0 in the control endoscopy at week 10. No adverse events leading to therapy termination were observed. Conclusion Biological therapy with IFX enables mucosal healing in pediatric patients with CD. Induction therapy with infliximab was found to be clinically effective in 72% of Polish pediatric patients with CD and induced a remission in 33% of them. Induction therapy with infliximab helps to increase BMI.

A Randomised, Double-Blind Study of High and Low Dose Oral Delayed-Release Mesalamine Does Not Demonstrate a Difference in Treating Mild to Moderately Active Ulcerative Colitis in Children.

The IMAgINE 1 study (NCT00409682) evaluated the safety and efficacy of adalimumab double-blind maintenance dosing regimens following open-label induction for pediatric patients with moderate to severe Crohn's disease (CD).We studied 192 patients with Pediatric Crohn's Disease Activity Index (PCDAI) scores30 for whom conventional treatment was unsuccessful. Patients received open-label induction therapy with subcutaneous adalimumab at weeks 0 and 2 (160 mg and 80 mg, or 80 mg and 40 mg, for body weight ≥40 kg or40 kg). At week 4, 188 patients were assigned to groups based on achievement of clinical response (defined as decrease in PCDAI ≥15 points from baseline; 155/188 [82.4%]) and prior exposure to infliximab (82/188 [43.6%]). Groups were given double-blind maintenance therapy with adalimumab at high (40 mg or 20 mg for body weight ≥40 kg or40 kg; n = 93) or low doses (20 mg or 10 mg for body weight ≥40 kg or40 kg; n = 95) every other week for 48 weeks. Clinical remission (PCDAI ≤10) at week 26 (the primary end point) was compared between groups using the Cochran-Mantel-Haenszel test, adjusting for strata, with nonresponder imputation. Adverse events were monitored to evaluate safety.A total of 152 of 188 patients (80.9%) completed all 26 weeks of the study. At week 26, 63 patients (33.5%) were in clinical remission, with no significant difference between high- and low-dose groups (36/93 [38.7%] vs 27/95 [28.4%]; P = .075). No new safety signals were detected.Adalimumab induced and maintained clinical remission of children with CD, with a safety profile comparable to that of adult patients with CD. More children who received high compared with low dose were in remission at week 26, but the difference between dose groups was not statistically significant.

Background: There is no consensus regarding the optimal enteral formula in patients with neonatal short bowel syndrome. The common practice in many centers is to give a semielemental diet. Methods: To test the hypothesis that hydrolyzed protein is not superior to standard formula in promoting growth and development of children with short bowel syndrome, 10 children aged 4.08 ± 2.45 months (mean ± SD) underwent a prospective, randomized, crossover, double-blind study lasting 60 days (with crossover on day 31). Two enteral formulas, which differed only with respect to the nitrogen form-hydrolyzed and nonhydrolyzed whey protein-were used. The endpoints of the study were nitrogen balance and intestinal permeability measured by the sugar absorption test (lactulose/mannitol excretion ratio). Results: Energy intake from enteral formula in patients fed hydrolyzed and nonhydrolyzed formula was the same and amounted to about 31% of total intake. The ratio of total energy intake (enteral and parenteral) to resting energy expenditure was 1.7 ± 0.5 and 1.5 ± 0.3 in patients fed hydrolyzed and non hydrolyzed formula respectively. Nitrogen balance was 0.28 ± 0.05 g/kg/d and 0.29 ± 0.05 g/kg/day, respectively. Lactulose/mannitol ratio before the study was 0.85 ± 0.85 and after hydrolyzed and nonhydrolyzed formula was 0.59% ± 0.51% and 0.69% ± 0.72%, respectively. Conclusion: Intestinal permeability, energy, and nitrogen balance in short bowel syndrome were not influenced in the short term by hydrolysis of the enteral nitrogen source. Infant formula-Intestinal permeability.

Background: Hereditary Pancreatitis (HP) is a rare inherited condition. We reviewed our experience over the last 20 years. Aims: The aim of our study was to evaluate the clinical course of HP in children. Patients & methods: 240 children with chronic pancreatitis, hospitalized since 1988 to 2013, were enrolled into the study. The medical records of these patients were reviewed for data on the presentation, diagnostic findings and endoscopic treatment. All children were screened for the PRSS1 gene mutations. Results: Hereditary pancreatitis was diagnosed in 31 patients (12.9%). PRSS1 gene mutations were found in 27 patients. We detected R122H/in 13, R122C/in 8, N29I/in 5 and E79K/in 1 patient. Family history was positive in 26 children with HP (84%). In 4 patients without mutations diagnosis was made when patients satisfied the requirements of the family history. In one patient we found SPINK1 mutation. In 2 children CFTR mutation was present. There was no difference in age of the disease onset between HP group and non-HP group (8 vs. 9.1 years; NS). In children with PRSS1 mutation ERCP had mean 2,5o Cambridge grade, vs. 1.6o, p

s / Pancreatology 12 (2012) 502–597 565 We present the case of a 54 year old female patient, who underwent surgery for a neuroendocrine tumor, with a large pancreatic tail mass, diagnosed by computer tomography. Laparoscopic distal resection of the pancreas was conducted. The perioperative period was complicated by an infection of the wound and suppuration. The patient was discharged on the 7th day after the operation. Postoperative histopathological examination revealed the intrapancreatic accessory spleen. Accessory spleen mimicking a pancreatic tumor is difficult to diagnose. Some papers compare examination methods, but usually they are complicated, expensive and unavailable to patients. Moreover, in most of cases, EUS-FNA is impossible due to splenic vessels. In such cases, minimal invasive procedures appear to offer a good alternative to classic operations.

s / Pancreatology Background: Previous reports that the human equilibrative nucleoside transporter (hENT1) may be predictive of improved survival following adjuvant gemcitabine treatment have largely been based on retrospective non-randomized studies or studies of chemoradiotherapy. Here the therapeutic predictability of hENT1 expression and similar markers in patients undergoing resection for pancreatic cancer is assessed using the samples collected from the adjuvant ESPAC1/3 trials. Since these trials are randomized trials, this makes the results more scientifically compelling. Methods: An extensive purification processes was applied to antibodies to hENT1, deoxycytidine kinase-1 (dck1) and concentrative nucleoside transporter-1 (CNT1). These were validated (GCLP) using siRNA knockdowns, ICC and Western blotting prior and then optimized for immunohistochemistry. Tissue Micro Arrays (TMAs) containing tumour from non-trial pancreatic cancer patients (n1⁄469) were used to determine objective scoring systems of marker expression and then applied in a blinded manner on the ESPAC TMAs (n1⁄4434). Association between the different markers was measured using correlation of staining scores and this related to treatment group and other variables. Cox regression analysis was used to relate markers to survival prognosis, both individually and collectively, also taking in to account TNM status, tumour grade, resection margin status, smoking, age, and gender as well as survival status. Results: 59% of tumour samples had some level of hENT-1 staining, but only 3.7% had membranous staining. The median (IQR) survival was 15.2 (6.3, 37.7) months for observation (n1⁄431), 22.2 (11.7, 42.7) months for 5FU (n1⁄4201) and 23.8 (13.4, 41.6) for gemcitabine (n1⁄4202). Analysis of the relationship of staining and survival will be completed by the end March 2012. Conclusions: Potential extensive use of hENT1 staining as a stratification variable in trials means that verification of data for hENT1 is urgently required. The data generated from this trial will be potentially of great clinical value resulting in wide applicability given the unbiased nature of the randomised nature of the samples and the overall quality of the study based on GCLP and REMARK standards.