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Liver transplantation is currently an accepted first-line treatment for patients with end-stage acute or chronic liver disease, but postoperative complications may limit the long-term success of transplantation. The most common and most clinically significant complications are arterial and venous thrombosis and stenosis, biliary disorders, fluid collections, neoplasms, and graft rejection. Early diagnosis is crucial to the successful management of all these complications, and imaging plays an important role in the diagnosis of all but graft rejection. A multimodality approach including ultrasonography and cross-sectional imaging studies often is most effective for diagnosis. Each imaging modality has specific strengths and weaknesses, and the diagnostic usefulness of a modality depends mainly on the patient's characteristics, the clinical purpose of the imaging evaluation, and the expertise of imaging professionals.

Objective: Only few large families with multiple endocrine neoplasia type 1 (MEN1) have been documented. Here, we aimed to investigate the clinical features of a seven-generation Brazilian pedigree, which included 715 at-risk family members. Design: Genealogical and geographic analysis was used to identify the MEN1 pedigree. Clinical and genetic approach was applied to characterize the phenotypic and genotypic features of the family members. Results: Our genetic data indicated that a founding mutation in the MEN1 gene has occurred in this extended Brazilian family. Fifty family members were diagnosed with MEN1. Very high frequencies of functioning and non-functioning MEN1-related tumors were documented and the prevalence of prolactinoma (29.6%) was similar to that previously described in prolactinoma-variant Burin (32%). In addition, bone mineral density analysis revealed severe osteoporosis (T, K2.87G0.32) of compact bone (distal radius) in hyperparathyroidism (HPT)/MEN1 patients, while marked bone mineral loss in the lumbar spine (T, K1.95G0.39), with most cancellous bone, and femoral neck (mixed composition; T, K1.48G0.27) were also present. Conclusions: In this study, we described clinically and genetically the fifth largest MEN1 family in the literature. Our data confirm previous findings suggesting that prevalence of MEN1-related tumors in large families may differ from reports combining cumulative data of small families. Furthermore, we were able to evaluate the bone status in HPT/MEN1 cases, a subject that has been incompletely approached in the literature. We discussed the bone loss pattern found in our MEN1 patients comparing with that of patients with sporadic primary HPT.European Journal of Endocrinology 159 259-274

Background:Therapeutic options for patients with advanced hepatocellular carcinoma (HCC) are limited. There is emerging evidence that the growth of cancer cells may be altered by very low levels of electromagnetic fields modulated at specific frequencies.Methods:A single-group, open-label, phase I/II study was performed to assess the safety and effectiveness of the intrabuccal administration of very low levels of electromagnetic fields amplitude modulated at HCC-specific frequencies in 41 patients with advanced HCC and limited therapeutic options. Three-daily 60-min outpatient treatments were administered until disease progression or death. Imaging studies were performed every 8 weeks. The primary efficacy end point was progression-free survival ⩾6 months. Secondary efficacy end points were progression-free survival and overall survival.Results:Treatment was well tolerated and there were no NCI grade 2, 3 or 4 toxicities. In all, 14 patients (34.1%) had stable disease for more than 6 months. Median progression-free survival was 4.4 months (95% CI 2.1–5.3) and median overall survival was 6.7 months (95% CI 3.0–10.2). There were three partial and one near complete responses.Conclusion:Treatment with intrabuccally administered amplitude-modulated electromagnetic fields is safe, well tolerated, and shows evidence of antitumour effects in patients with advanced HCC.

METHODS:The clinical diagnosis of MEN1 was made in accordance with the Consensus on multiple endocrine neoplasias. Mutation analysis of the entire MEN1 tumor suppressor gene and genetic screening of at-risk family members were performed by direct sequencing. To analyze the implementation of genetic diagnosis, the studied patients were separated into 3 groups: MEN1 index cases (group I), clinically diagnosed MEN1 cases (group II), and genetically diagnosed MEN1 cases (group III). RESULTS: In total, 154 individuals were clinically and genetically studied. We identified 12 different MEN1 mutations. Fifty-two MEN1 cases were identified: 13 in group I, 28 in group II, and 11 in group III. The mean age in group III (27.0 years) was significantly lower than in groups I (39.5 years) and II (42.4 years; P = 0.03 and P = 0.01, respectively). Patients in groups I and II mostly presented 2 or 3 MEN1-related tumors, while 81.8% of those in group III presented 1 or no MEN1-related tumor. Additionally, in group III, 45.4% of cases were asymptomatic, and no metastasis or death was verified. Surveillance for MEN1 mutations allowed the exclusion of 102 noncarriers, including a case of MEN1 phenocopy. CONCLUSION: Our data supports the benefits of clinical and genetic screening for multiple endocrine neoplasia type 1 in the management of this syndrome.

The carcinoid tumor is a relatively rare type of endocrine tumor, which arises mainly in the gastrointestinal tract. Primary gallbladder and biliary duct system carcinoids comprise less than 1% of all carcinoid tumors arising from any tissue or organ in the body. We describe a case of carcinoid tumor of the gallbladder in a 39-year-old man. There have been only 32 cases described in the literature.

CA 19-9 may be a useful adjunct to response evaluation is this setting. In addition to CA 19-9 responses, prechemotherapy levels of this marker seem to have strong prognostic significance.

Groove pancreatitis in an uncommon type of focal chronic pancreatitis. Its appearance may resemble pancreatic adenocarcinoma. Although distinction between these two entities remains challenging, knowledge of MRI findings is important in suggesting the correct diagnosis and programming therapeutic decisions.