Unusual Paraneoplastic Syndrome Accompanies Neuroendocrine Tumours of the Pancreas

Neuroendocrine tumours comprise a small percentage of pancreatic neoplasia (10%) (1). Diagnosis of neuroendocrine tumours is difficult, especially if the tumours are small and nonfunctional. CT scans, MRI, and nuclear scans are sufficiently sensitive assessment tools for tumours with diameters of at least 2 cm; otherwise, the sensitivity and specificity of these techniques is less than 50% (2). Myasthenia gravis (MG) is a heterogeneous neuromuscular junction disorder that is primarily caused when antibodies form against the acetylcholine receptors (Ab-AchR). MG can develop in conjunction with neoplasia, making MG a paraneoplastic disease. In those cases, MG is most commonly associated with thymomas and less frequently associated with extrathymic malignancies. The mechanism underlying this paraneoplastic syndrome has been hypothesized to involve an autoimmune response against the tumour cells (3). No published reports have linked malignant pancreatic diseases with MG. Here, we report the case of a young woman, negative for Ab-AchR, with a neuroendocrine tumour in the pancreatic head, who experienced a complete resolution of her MG-like syndrome after surgical enucleation of the tumour

Methylprednisolone-induced toxic hepatitis after intravenous pulsed therapy for multiple sclerosis relapses

High-dose, intravenous methylprednisolone (MP) is the only recommended first-line treatment for multiple sclerosis relapses. However, there are increasing reports on liver toxicity induced by this treatment regimen. We report of 4 multiple sclerosis patients with no history of viral/metabolic liver disorders or alcohol/hepatotoxic drug intake, who developed hypertransaminasaemia following intravenous MP. In 2 of the patients, liver biopsy showed periportal fibrosis, piecemeal necrosis, and inflammatory cell infiltrates. A rechallenge test confirmed a causal association in 1 case. MP-induced liver toxicity may be more frequent than commonly thought and it is important to report this adverse reaction, which is potentially lethal, and to raise awareness on the potential hepatotoxicity of corticosteroid pulses

Multimodality Imaging in Cardiomyopathies with Hypertrophic Phenotypes

Multimodality imaging is a comprehensive strategy to investigate left ventricular hypertrophy (LVH), providing morphologic, functional, and often clinical information to clinicians. Hypertrophic cardiomyopathy (HCM) is defined by an increased LV wall thickness not only explainable by abnormal loading conditions. In the context of HCM, multimodality imaging, by different imaging techniques, such as echocardiography, cardiac magnetic resonance, cardiac computer tomography, and cardiac nuclear imaging, provides essential information for diagnosis, sudden cardiac death stratification, and management. Furthermore, it is essential to uncover the specific cause of HCM, such as Fabry disease and cardiac amyloidosis, which can benefit of specific treatments. This review aims to elucidate the current role of multimodality imaging in adult patients with HCM

A longitudinal study on BIO14.6 hamsters with dilated cardiomyopathy: micro-echocardiographic evaluation

<p>Abstract</p> <p>Background</p> <p>In recent years, several new technologies for small-animal imaging have been developed. In particular, the use of ultrasound in animal imaging has focused on the investigation of accessible biological structures such as the heart, of which it provides a morphological and functional assessment. The purpose of this study was to investigate the role of micro-ultrasonography (μ-US) in a longitudinal study on BIO14.6 cardiomyopathic hamsters treated with gene therapy.</p> <p>Methods</p> <p>Thirty hamsters were divided into three groups (n = 10): Group I, untreated BIO 14.6 hamsters; Group II, BIO 14.6 hamsters treated with gene therapy; Group III, untreated wild type (WT) hamsters. All hamsters underwent serial μ-US sessions and were sacrificed at predetermined time points.</p> <p>Results</p> <p>μ-US revealed: in Group I, progressive dilation of the left ventricle with a change in heart morphology from an elliptical to a more spherical shape, altered configuration of the mitral valve and subvalvular apparatus, and severe reduction in ejection fraction; in Group II, mild decrease in contractile function and ejection fraction; in Group III, normal cardiac chamber morphology and function. There was a negative correlation between the percentage of fibrosis observed at histology and the ejection fraction obtained on μ-echocardiography (Spearman r: -0.839; p < 0.001).</p> <p>Conclusions</p> <p>Although histological examination remains indispensable for a conclusive diagnosis, high-frequency μ-echocardiography, thanks to the high spatial and contrast resolution, can be considered sufficient for monitoring therapeutic efficacy and/or the progression of dilated cardiomyopathy, providing an alternative tool for repeatable and noninvasive evaluation.</p

Diagnostic performance of FibroTest, SteatoTest and ActiTest in patients with NAFLD using the SAF score as histological reference

BACKGROUND: Blood tests of liver injury are less well validated in non‐alcoholic fatty liver disease (NAFLD) than in patients with chronic viral hepatitis. AIMS: To improve the validation of three blood tests used in NAFLD patients, FibroTest for fibrosis staging, SteatoTest for steatosis grading and ActiTest for inflammation activity grading. METHODS: We pre‐included new NAFLD patients with biopsy and blood tests from a single‐centre cohort (FibroFrance) and from the multicentre FLIP consortium. Contemporaneous biopsies were blindly assessed using the new steatosis, activity and fibrosis (SAF) score, which provides a reliable and reproducible diagnosis and grading/staging of the three elementary features of NAFLD (steatosis, inflammatory activity) and fibrosis with reduced interobserver variability. We used nonbinary‐ROC (NonBinAUROC) as the main endpoint to prevent spectrum effect and multiple testing. RESULTS: A total of 600 patients with reliable tests and biopsies were included. The mean NonBinAUROCs (95% CI) of tests were all significant (P < 0.0001): 0.878 (0.864–0.892) for FibroTest and fibrosis stages, 0.846 (0.830–0.862) for ActiTest and activity grades, and 0.822 (0.804–0.840) for SteatoTest and steatosis grades. FibroTest had a higher NonBinAUROC than BARD (0.836; 0.820–0.852; P = 0.0001), FIB4 (0.845; 0.829–0.861; P = 0.007) but not significantly different than the NAFLD score (0.866; 0.850–0.882; P = 0.26). FibroTest had a significant difference in median values between adjacent stage F2 and stage F1 contrarily to BARD, FIB4 and NAFLD scores (Bonferroni test P < 0.05). CONCLUSIONS: In patients with NAFLD, SteatoTest, ActiTest and FibroTest are non‐invasive tests that offer an alternative to biopsy, and they correlate with the simple grading/staging of the SAF scoring system across the three elementary features of NAFLD: steatosis, inflammatory activity and fibrosis

Development of a gelatin-based polyurethane vascular graft by spray, phase-inversion technology

The capacity of a composite vascular graft constituting polyurethane (PU) and gelatin to support cell growth was investigated using human mesenchymal stem cells (hMSCs). Gelatin-based polyurethane grafts were fabricated by co-spraying polyurethane and gelatin using a spray, phase-inversion technique. Graft microstructure was investigated by light and scanning electron microscopy. Uniaxial tensile tests were performed to assess the grafts' mechanical properties in longitudinal and circumferential directions. hMSCs obtained from bone marrow aspirate were seeded onto flat graft samples. After 24, 48, and 72 h of incubation, cell morphology was evaluated by Giemsa staining and cell viability was calculated by XTT assay. SEM analysis evidenced that PU samples display a microporous structure, whereas the gelatin-based PU samples show a fibrillar appearance. The presence of cross-linked gelatin produced a significant increase of ultimate tensile strength and ultimate elongation in circumferential directions compared to PU material. Qualitative analysis of hMSC adhesion onto the grafts revealed remarkable differences between gelatin-based PU and control graft. hMSCs grown onto gelatin-based PU graft form a monolayer that reached confluence at 72 h, whereas cells seeded onto the control graft were not able to undergo appropriate spreading. hMSCs grown onto gelatin-based PU graft showed significantly higher viability than cells seeded onto bare PU at all time points. In conclusion, a composite vascular graft was successfully manufactured by simultaneous co-spraying of a synthetic polymer and a protein to obtain a scaffold that combines the mechanical characteristics of polyurethanes with the favorable cell interaction features of gelatin

Linear endoscopic ultrasonography vs magnetic resonance imaging in ampullary tumors

AIM: To assess linear endoscopic ultrasound (L-EUS) and magnetic resonance imaging (MRI) in biliary tract dilation and suspect small ampullary tumor

Adenocarcinoid tumor of the extrahepatic biliary tract.

The term adenocarcinoid was first coined by Warkel et al in 1978 to describe a group of uncommon low-grade malignant appendiceal tumors with morphologic and histochemical evidence of both glandular (adenocarcinoma) and neuroendocrine (carcinoid) differentiation for which several terms have been used in the past. Although the appendix is the most frequent site of this tumor, similar neoplasms have been reported also in other sites, such as colon, gallbladder, Vater's ampulla, and stomach. The biologic and clinical behavior of adenocarcinoid is still unclear. Provided that it can metastasize, a recent meta-analysis on appendiceal adenocarcinoids showed that right hemicolectomy is not required when the tumor is completely excised and there is no cecal involvement. In this article, the clinicopathologic features of an adenocarcinoid tumor occurring in the extrahepatic biliary tract with infiltration of the common hepatic duct wall that, to the best of our knowledge, represents the first report in this site is described