X-linked Inhibitor of Apoptosis Complicated by Granulomatous Lymphocytic Interstitial Lung Disease (GLILD) and Granulomatous Hepatitis.

The X-linked inhibitor of apoptosis (XIAP) deficiency is a primary immunodeficiency characterized by Epstein-Barr virus (EBV)-driven hemophagocytic lymphohistiocytosis (HLH), splenomegaly, and colitis. Here, we present, for the first time, granulomatous hepatitis and granulomatous and lymphocytic interstitial lung disease (GLILD) as manifestations of XIAP deficiency. We report successful treatment of GLILD in XIAP deficiency with rituximab and azathioprine and discuss the role of XIAP deficiency in immune dysregulation.

Assisted suicide a 20th century problem, Palliative care a 21st century solution.

Assisted suicide and euthanasia are two forms of what is being called 'assisted dying', and they are touted by proponents as "progressive" and "compassionate". In fact, they are, on the contrary, relics from the last century: today, in the 21st century, we have moved beyond such archaic solutions - we now have, instead, proper evidence-based palliative care. It is this that should be demanded for all. This article will dispel the myths around dying that are often cited. It will also explore the oft-overlooked tragedies generated by assisted suicide, in the hope you, the reader, can be better informed about this retrogressive practice.

Assisted suicide and capital punishment: a mirror image?

OBJECTIVES: To outline the jurisprudential position in UK law regarding capital punishment, the death penalty and contrast this with proposed legislation for assisted dying and euthanasia (AD/E). METHODS: A historical medico-legal jurisprudential research approach, focusing on investigating the case law which resulted in the eventual cessation of capital punishment and contrasting this with the arguments used in current proposed legislation for AD/E. RESULTS: As a society, we are confronted with a similar choice in AD/E as we did in the 1960s with capital punishment, where it has demonstrated, despite a full judiciary process with a jury, that incorrect decisions have been made, resulting in death. In the context of two doctors making irrevocable decisions with prognosis uncertain, diagnostic errors and autonomy being relational-influenced by how others behave towards us-even campaigners for AD/E admit errors are inevitable. Some will have lethal drugs under a wrong diagnosis, an incorrect timespan or coercive pressures; is this cost acceptable? CONCLUSION: If the abolition of capital punishment is hard won, with the risk set that no one incorrectly should die, is this the standard that should be set for lethal drugs today? Is the right to choose so important that others' lives lost are an acceptable cost to pay? If we do accept a radical individual autonomy ethos, what proportion of incorrect deaths is acceptable?

Community palliative medicine out-of-hours needs and the 7-day week: a service evaluation.

BACKGROUND: Evidence regarding out-of-hours (OOH) community palliative care provision is required to inform the need for a 7-day work. AIM: This paper seeks to provide evidence for this discussion by defining general practitioners' (GPs) OOH workload and patients' demographics, symptomology and interventions. By quantifying the challenges faced, we can understand current practice and focus on what provision is required. DESIGN: Using Shropshire Doctors Co-operative's recorded data, the authors have collated a representative picture of the OOH GP palliative care practice over a year from 161 OOH GP-patient interactions. SETTING: Primary care. RESULTS: Palliative care makes up 11.4% of the total OOH GP home visits (HV). Overall 56% of OOH GP HVs are for patients who are expected to die within 48 hours, with 80% of the symptoms being agitation, secretions and pain. Overall 5.7% of OOH GP palliative HVs resulted in hospital admission; however, this decreased to 0.6% adjusting for the last 48-hour prognosis. CONCLUSION: OOH Shropshire GPs deal with a wide variety of scenarios in a heterogeneous population. The greatest demand is from 17:00 to 00:00 (65% of the total shift) on weekdays, and from 09:00 to 00:00 on weekends (82% of the shift). These data begin to quantify the role being performed by OOH GPs, have implications for service provision and support 7-day work.