Editor-in-Chief, Deputy Editor 2017-2019





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Original Article

Fifteen years single center experience in the management of progressive familial intrahepatic cholestasis of infancy

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Recent advances in genetics and in physiopathology of bile com-position and excretion have clarified the understanding of pro-gressive familial intrahepatic cholestasis (PFIC). The aim of the present study is to review the experience of our center in terms of diagnosis, management and outcome of 49 pedi-atric PFIC patients, belonging to the three classical subtypes described. We analyse the clinical, biological, and histological patterns and review the response to the medical and surgical treatment and the global outcome. The only clinical difference between the different subtypes of PFIC patients was the intensity of pruritus. Serum gamma-glu-tamyltransferase (GGT) and liver histology allowed to differenti-ate PFIC III from PFIC I and II patients. High levels of biliary bile acids in 2 low-GGT patients was asso-ciated with favourable outcome. Response to ursodesoxycholic acid (UDCA) varies from patient to patient and was not associat-ed to a particular subtype of PFIC. In five patients of this cohort, external biliary diversion was performed without improvement. Transplantation is indicated whenever medical treatment fails to restore normal social life, growth and well being of the child and it is associated with excellent survival (> 90%) [Product Details...]

Non-endoscopic diagnosis of multifocal atrophic gastritis ; efficacy of serum gastrin-17, pepsinogens and Helicobacter pylori antibodies

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Background/Aims : Infection with H.pylori is an important risk factor for the development of gastric cancer and glandular atrophy is an intermediate stage in gastric carcinogenesis. While screening the patients with atrophic gastritis by endoscopy is unrealistic, a concept of serological gastric biopsy based on measurement of gastric secretory proteins and peptides should be further validated. We sought to determine if the laboratory panel composed of serum PGI and protein stimulated gastrin-17 might select patients with MAG, and what is diagnostic significance of H.pylori serology in population of high prevalence of H.pylori infection. Material and methods : 55 consecutive patients of both sexes (M/F 25/30 ; range of age 55 -81 years) were referred for gastro-scopy with antrum and corpus mucosal biopsies. Patients with histological signs of glandular atrophy at any site of the stomach were considered to have multifocal atrophic gastritis. A first blood sample was collected for measurement of basal gastrin-17, pepsinogens and H.pylori IgG-antibodies, and second was taken 20 minutes after use of protein-rich drink to measure stimulated gastrin-17. Results : Signs of mucosal atrophy were found in 19 patients, while 29 patients showed non-atrophic gastritis and seven H.pylori-negative patients had no histological pathology. Low serum level of stimulated gastrin-17 (< 5 pmol/l) and/or pepsino-gen I (< 50 g/l), were found in 16 of 19 patients (84,2%) with and in 7 of 36 patients (19,4%) without atrophy in the histological study. Combining of H.pylori serology with serum levels of secre-tory peptides had no significant effect on diagnostic sensitivity of the test panel. Conclusion : The test panel composed of pepsinogen I and pro-tein stimulated gastrin-17 may be used as the serological gastric biopsy detecting multifocal atrophic gastritis. The diagnostic sen-sitivity of this test panel is not increased by knowledge of H.pylori status. [Product Details...]

Role of chronic atrophic gastritis of the body-fundus and achlorydria in the development of epithelial dysplasia and gastric carcinoma

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Background/ Aim : Chronic atrophic gastritis of the body-fun-dus with hypo-achlorydria has been long since considered the pre-cursor of gastric cancer (GC). A study has been made about the histological pattern of the body-fundic mucosa (oxyntic area) in course of preneoplastic lesions (epithelial dysplasia), associated or progressed to gastric cancer, in order to evaluate the real association with chronic atrophic gastritis and, therefore, with a reduced acid secretion. Methodology : The study of the histological condition of the body-fundic mucosa and of the acid secretion has been effected in 120 cases of epithelial dysplasia (ED) from January 1990 to November 1997. The casuistry is composed of 70 cases of low grade dysplasia (LGD) and 50 cases of high grade dysplasia (HGD). Gastric biopsy specimens were studied for dyspepsia : for each patient, at least 8 specimens were obtained from the lesion area and in surrounding areas. Besides, at least 4 biopsies have been performed in the opposite gastric region. ED diagnosis was effect-ed according to well defined criteria. The histological study of gas-tric mucosa in gastritis was effected or revised in accordance with the updated Sydney sistem (Houston). Stimulated acid secretion was expressed as Maximal Acid Output (MAO), which is the amount of HCl produced in one hour, following stimulation with pentagastrin (6 microng/kg). The clinical outcome subdivision of ED was made using the cri-teria of Rugge et al. (12). Results : HGD significantly associates with GC in comparison with LGD. The histological evaluation of the oxyntic area shows severe chronic atrophic gastritis (SCAG) in a low percentage of cases (15/120 : 12.5%) : LGD 9/70 : 12.85% ; HGD 6/50 : 12%. Complete achlorydria has been noted in 5 cases of LGD and in 1 case of HGD only. In case of GC (43 subjects) SCAG has been evidenced in 10 cases and complete achlorydria in 5 cases. Conclusions : From the data of the present experience emerges that the presence of SCAG of the oxyntic area in course of ED or early GC is limited to a low percentage of cases. Such concepts induce to modify some indications related to the endoscopic surveillance and, in accordance with the American Society of Gastrointestinal Endoscopy we are stating that there are no sufficient data to support subsequent endoscopic surveillance for the subjects with atrophic gastritis. [Product Details...]

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