Spinal muscular atrophy (SMA) is a severe neuromuscular disorder due to a defect in the survival motor neuron 1 (SMN1) gene. Its incidence is approximately 1 in 11,000 live births. In 2007, an International Conference on the Standard of Care for SMA published a consensus statement on SMA standard of care that has been widely used throughout the world. Here we report a two-part update of the topics covered in the previous recommendations. In part 1 we present the methods used to achieve these recommendations, and an update on diagnosis, rehabilitation, orthopedic and spinal management; and nutritional, swallowing and gastrointestinal management. Pulmonary management, acute care, other organ involvement, ethical issues, medications, and the impact of new treatments for SMA are discussed in part 2.

This is the second half of a two-part document updating the standard of care recommendations for spinal muscular atrophy published in 2007. This part includes updated recommendations on pulmonary management and acute care issues, and topics that have emerged in the last few years such as other organ involvement in the severe forms of spinal muscular atrophy and the role of medications. Ethical issues and the choice of palliative versus supportive care are also addressed. These recommendations are becoming increasingly relevant given recent clinical trials and the prospect that commercially available therapies will likely change the survival and natural history of this disease.

ObjectiveThe authors determined if more radical surgery with extended lymphadenectomy improves the results of gastrectomy in patients with adenocarcinoma of the gastric antrum. Summary Background DataThe overall survival in patients with gastric cancer is disappointing. Improved survival has been reported by Japanese authors. Whether this is because of a higher number of early gastric cancers in the Japanese series, different biologic behavior in Asians, or the adoption of radical surgery with lymphadenectomy remains unclear. MethodsR1 subtotal gastrectomy with omentectomy and R3 total gastrectomy (omentectomy, splenectomy, distal pancreatectomy, lymphatic clearance of the celiac axis, and skeletonization of vessels in the porta hepatis) were evaluated in a prospective, randomized comparison. ResultsFifty-five patients were randomized-25 to the R1 group and 30 to the R3 group. The two groups were comparable for age, sex, tumor size, TNM stage, and length of follow-up. The R3 group had a longer operating time (140 vs. 260 min; p < 0.05), a greater transfusion requirement (0 vs. 2 units, p < 0.05) and a longer hospital stay (8 vs. 16 days; p < 0.05) (medians; Mann-Whitney U test). The only postoperative death was in the R3 group and was caused by intra-abdominal sepsis. Fourteen patients in the R3 group developed left subphrenic abscesses. There were no major complications in the R1 group. Overall survival was significantly better in the R1 group (median survival estimated by Kaplan-Meier method, 1511 vs. 922 days, p < 0.05, log-rank test). ConclusionsR3 total gastrectomy can be performed with a low mortality, but it has a high morbidity because of intra-abdominal sepsis. The data do not support the routine use of R3 total gastrectomy for treatment of patients with antral cancer. 176

There is a growing international agreement on the need to provide greater access to research data and bio-specimen collections to optimize their long-term value and exploit their potential for health discovery and validation. This is especially evident for rare disease research. Currently, the rising value of data and bio-specimen collections does not correspond with an equal increase in data/sample-sharing and data/sample access. Contradictory legal and ethical frameworks across national borders are obstacles to effective sharing: more specifically, the absence of an integrated model proves to be a major logistical obstruction. The Charter intends to amend the obstacle by providing both the ethical foundations on which data sharing should be based, as well as a general Material and Data Transfer Agreement (MTA/DTA). This Charter is the result of a careful negotiation of different stakeholders' interest and is built on earlier consensus documents and position statements, which provided the general international legal framework. Further to this, the Charter provides tools that may help accelerate sharing. The Charter has been formulated to serve as an enabling tool for effective and transparent data and bio-specimen sharing and the general MTA/DTA constitutes a mechanism to ensure uniformity of access across projects and countries, and may be regarded as a consistent basic agreement for addressing data and material sharing globally. The Charter is forward looking in terms of emerging issues from the perspective of a multi-stakeholder group, and where possible, provides strategies that may address these issues.

Hiatus hernia, Mesh repair, Laparoscopy, Randomized controlled trial. Trial registration -This trial is registered with the Australia and New Zealand ClinicalTrials Registry ACTRN12605000725662 Determine whether absorbable or non-absorbable mesh in repair of large hiatus hernias reduces the risk of recurrence, compared to suture repair. Summary Background Data:Repair of large hiatus hernia is associated with radiological recurrence rates of up to 30%, and to improve outcomes mesh repair has been recommended. Previous trials have shown less short term recurrence with mesh, but adverse outcomes limit mesh use. Methods:Multicentre prospective double blind randomized controlled trial of 3 methods of repair; sutures vs. absorbable mesh vs. non-absorbable mesh. Primary outcome -hernia recurrence assessed by barium meal X-ray and endoscopy at 6 months. Secondary outcomes -clinical symptom scores at 1, 3, 6 and 12 months. Results:126 patients enrolled -43 sutures, 41 absorbable mesh and 42 non-absorbable mesh. 96.0%were followed to 12 months, with objective follow-up data in 92.9%. A recurrent hernia (any size) was identified in 23.1% following suture repair, 30.8% -absorbable mesh, and 12.8%