Hepatosplenic T-cell lymphoma, immunosuppressive agents and biologicals: what are the risks?

We present three cases of the rare hepatosplenic T-cell lymphoma (HSTCL); two patients suffering from Crohn disease who developed HSTCL on azathioprine without exposure to biologicals, and a third patient who had psoriasis treated using etanercept, cyclosporine and methotrexate. The evidence for an association between HSTCL and immunosuppressive drugs and biologicals is reviewed. We argue for improved pharmacovigilance processes to help determine the benefit to risk ratios for the use of these and other new agents.

Gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS): a new autosomal dominant syndrome.

OBJECTIVE: The purpose of this study was the clinical and pathological characterisation of a new autosomal dominant gastric polyposis syndrome, gastric adenocarcinoma and proximal polyposis of the stomach (GAPPS). METHODS: Case series were examined, documenting GAPPS in three families from Australia, the USA and Canada. The affected families were identified through referral to centralised clinical genetics centres. RESULTS: The report identifies the clinical and pathological features of this syndrome, including the predominant dysplastic fundic gland polyp histology, the exclusive involvement of the gastric body and fundus, the apparent inverse association with current Helicobacter pylori infection and the autosomal dominant mode of inheritance. CONCLUSIONS: GAPPS is a unique gastric polyposis syndrome with a significant risk of gastric adenocarcinoma. It is characterised by the autosomal dominant transmission of fundic gland polyposis, including areas of dysplasia or intestinal-type gastric adenocarcinoma, restricted to the proximal stomach, and with no evidence of colorectal or duodenal polyposis or other heritable gastrointestinal cancer syndromes.

Advances in the management of inflammatory bowel disease.

Our understanding of inflammatory bowel diseases (IBD) is constantly evolving, and many new treatment options have emerged recently. This review critically examines the evidence for these new developments and aims to provide an overview for medical professionals involved in the care of patients with IBD. Proposed changes in the use of aminosalicylates, immunosuppressants and biological agents are described, and the evidence for several promising novel agents is reviewed.

Capsule endoscopy versus magnetic resonance enterography for the detection of small bowel polyps in Peutz-Jeghers syndrome.

Our study aimed to assess the diagnostic utility of magnetic resonance enterography (MRE) compared to capsule endoscopy (CE) for the detection of small bowel polyps in patients with Peutz-Jeghers syndrome (PJS); with findings verified by balloon enteroscopy (BE). Adult patients were prospectively recruited across two tertiary centres and underwent MRE followed by CE, with a subsequent BE performed in patients with significant (≥10 mm) polyps. The primary endpoint was the total number of significant (≥10 mm) small bowel polyps detected. The number of patients with at least one significant polyp, correlation with BE findings, and patients' preferences were secondary endpoints. A total of 20 patients (7 male; mean age 34.9 years) underwent both investigations. The number of polyps ≥10 mm detected by CE was greater than by MRE (47 vs 14 polyps, P = 0.02). The number of patients with at least one significant polyp identified by CE was 11 (55 %) compared with 7 (35 %) identified by MRE (P = 0.25). Subsequent BE in 12 patients identified a total of 26 significant polyps in 8 patients. The positive predictive value of finding a polyp at BE was higher for MRE (100 %) compared to CE (60 %). Overall patient preferences identified CE as the preferred modality. This prospective study demonstrated that CE identifies significantly more small bowel polyps compared with MRE in patients with PJS. Correlation between the two techniques and subsequent BE however was relatively poor.

Endothelin-1 plasma levels are not elevated in patients with obstructive sleep apnoea.

Endothelin-1 (ET-1), a potent vasoconstrictor, is released mainly by vascular endothelial cells under the influence of hypoxia and other stimuli. ET-1 is related to endothelial dysfunction, as well as arterial and pulmonary hypertension, all of which are thought to be associated with obstructive sleep apnoea (OSA). This study evaluated venous plasma concentrations of ET-1 and noradrenaline and 24-h systemic blood pressure in 29 patients with OSA (age=56.9+/-1.6 yrs; body mass index=29.5+/-0.7 kg x m2 (mean+/-SEM)). Blood samples were taken in the morning, evening and during sleep. In the same way, the patients were assessed during a night of continuous positive airway pressure (CPAP) and after 13.9+/-1.4 months while still on CPAP. ET-1 levels were compared to those of control subjects, who were selected from in- and outpatients and were matched to patients for age, sex, presence of arterial hypertension and coronary artery disease. ET-1 plasma levels were not elevated in the patients compared to the controls (41.6+/-2.2 and 44.9+/-1.3 pg x mL(-1), respectively, p=0.20). The ET-1 concentration did not change significantly, neither during sleep nor in the first night on CPAP therapy, nor under long-term treatment with CPAP. ET-1 neither correlated to the severity of OSA nor to that of systemic hypertension. The results suggest that endothelin-1 does not play a crucial role in the pathophysiology of obstructive sleep apnoea.