Somatostatin analogs in patients with Zollinger Ellison syndrome (ZES): an observational study.

PURPOSE: Zollinger Ellison syndrome (ZES) is a rare syndrome caused by gastrin hypersecretion from a gastrinoma. Gastrinoma treatment has two goals: the control of acid hypersecretion and the control of tumor growth. While therapy for the syndrome is univocally based on proton pump inhibitors, the one for disease control is still debated. We here aimed at evaluating the role of somatostatin analogs (SSAs) in the control of tumor progression in a series of ZES patients. METHODS: A retrospective analysis of a prospectively collected database of ZES patients, followed and managed from 1990 to 2019, was performed. The patients' clinical, pathological, treatment, and follow-up data were analyzed. Data regarding SSAs therapy start, dosage, duration, and side effects were collected. RESULTS: 33 patients with ZES were diagnosed. Fourteen patients (42%) had a grade 1 (G1) neuroendocrine neoplasm (NEN), five had G2 (15%), none had G3. Fifteen patients (45%) had metastatic disease. Overall, 12 (36%) underwent SSAs therapy. The median treatment duration was 36 months. Eight patients (67%) had a sustained response to SSAs, four (33%) showed an early progression, with a significant difference in terms of PFS between the patients with early and late progression (84 vs 2 months, p = 0.004). No differences in terms of OS and PFS were observed between the treated and non-treated patients, despite the proportion of metastatic patients was greater in the SSAs-treated group (75% vs 29% in the non-treated group, p = 0.01). CONCLUSION: Present data support the use of SSAs in ZES, considering that gastrinoma is mainly a well-differentiated low-grade tumor (G1 or G2), with a high expression of somatostatin receptors.

A follow-up study of patients with Zollinger-Ellison syndrome in the period 1966-2002: effects of surgical and medical treatments on long-term survival.

AIM: To evaluate the clinical history of a series of patients with Zollinger-Ellison syndrome (ZES) in the period 1966 to 2002, before and after the introduction of the current antisecretive H2 receptor antagonists and proton pump inhibitors into clinical practice. PATIENTS AND METHODS: The study involved 18 ZES patients (9 males; mean age, 43 years; range, 12-70 years), 8 with Type 1 multiple endocrine neoplasia (MEN-1), diagnosed on the basis of standard criteria. We considered the type, number and effectiveness of surgical interventions before and after appropriate treatment, the localization of the gastrinoma, the presence of associated diseases, the causes of death, and the duration of survival. RESULTS: Total gastrectomy (but not antrectomy and vagotomy) and full compliance to antisecretory treatment reduced the number of operations from 29 to 9. One patient was cured (5.5%), whereas relapsing gastrinomas occurred in 4 patients and associated diseases or complications in ten. Death was related to ZES in 5 patients and to other causes in 4. CONCLUSIONS: Curing gastrinoma or appropriately inhibiting gastric acid hypersecretion in ZES patients prevent death and favors long-term survival, regardless of gastrin levels and the size or number of tumors.

Gluten intolerance: gender- and age-related differences in symptoms.

OBJECTIVE: Gluten intolerance is a common, immunologically mediated disorder with a widely variable clinical presentation that affects genetically predisposed subjects. Women seem to be more frequently affected although data on sex differences are poor. In this study the prevalence of different clinical pictures according to sex and age is analysed in a large series of patients. MATERIAL AND METHODS: A total of 1436 patients with gluten intolerance were retrospectively considered, diagnosed from January 1975 to August 2001 based on compatible small-bowel biopsy and response to a gluten-free diet, plus immunofluorescent detection of granular IgA in papillary derma for dermatitis herpetiformis. The clinical picture at onset (classic, non-classic, silent) and age at diagnosis (< or = 2 years, > 2 and < or = 14 years, > 14 years) was recorded; 362 parents of coeliac probands undergoing a familial screening were also studied. The relations among sex, age class and symptoms were analysed using the chi2 test with Yates's correction. RESULTS: The overall female/male ratio was 2.3:1 but the inter-sex difference was significant only when the diagnosis was made in adulthood where a significant association between iron-deficiency anaemia as manifestation at onset in adult women (34% versus 7%) was found. Low weight, dyspepsia and hypertransaminasaemia were more common in adult men than women (20%, 14% and 7% versus 13%, 3% and 2%, respectively). Dermatitis herpetiformis was present more frequently in men (16% versus 9%). The prevalence of silent cases was 6% in men and 3% in women. Familial screening showed the same prevalence (9.3%) of current coeliac disease in fathers and mothers. CONCLUSIONS: Diagnosis of coeliac disease is more frequent in women but physicians' awareness of sex- and age-related differences in clinical presentation could improve diagnostic performances in men.

Possible protective effect of 5-aminosalicylic acid on Helicobacter pylori infection in patients with inflammatory bowel disease.

GOALS: To evaluate the prevalence of (Hp) infection in a group of inflammatory bowel disease (IBD) outpatients and the possible influence of treatment. BACKGROUND: The low prevalence of Hp infection in these patients is usually attributed to environmental factors; the role of drugs has not been fully investigated. STUDY: Seventy-two consecutive outpatients underwent a C13-urea breath test for Hp: 32 with Crohn's disease (13 men; mean age, 48 years; range, 20-72 years) and 40 with ulcerative colitis (25 men; mean age, 49 years; range, 25-71 years). Thirty-one patients were treated with sulfasalazine and 41 with 5-ASA. The control group consisted of 72 age- and sex-matched subjects. RESULTS: The prevalence of Hp infection was 47% in the IBD patients and 61% in the controls (p = 0.089; odds ratio = 0.55; 95% CI = 0.283-1.089) with a statistically significant increase for each year of age ( p= 0.044; odds ratio = 1.02; 95% CI = 1.001-1.052). Among the IBD patients, age and gender, the type, activity, duration, extent of the disease, or the calendar year of diagnosis, had no influence on Hp infection. was detected in 65% of the patients treated with sulfasalazine and in 34% treated with 5-ASA (p = 0.017). CONCLUSIONS: Although low, the prevalence of Hp infection in our patients was not significantly different from that in the controls. 5-ASA, and not sulfasalazine, may have a protective effect against Hp infection.