Liver transplantation for erythropoietic protoporphyria liver disease.

In erythropoietic protoporphyria (EPP), there is excessive production of protoporphyrin, primarily in the bone marrow, resulting in increased biliary excretion of this heme precursor. Some patients will develop progressive liver disease that may ultimately require liver transplantation. However, excessive production of protoporphyrin by the bone marrow continues after transplantation, which may cause recurrent disease in the allograft. This study was performed to define post-transplant survival, the risk of recurrent disease, and specific management issues in patients transplanted for EPP liver disease. The patients studied consisted of twelve males and eight females, with an average age of 31 (range, 13-56) years at the time of transplantation. The estimated maximum MELD score prior to transplant was 21 (range, 15-29). Unique complications in the perioperative period were light induced tissue damage in four patients and neuropathy in six, requiring prolonged mechanical ventilation in four. Patient and graft survival rates were 85% at 1 year, 69% at 5 years, and 47% at 10 years. Recurrent EPP liver disease occurred in 11 of 17 patients (65%) who survived more than 2 months. Three patients were retransplanted at 1.8, 12.6, and 14.5 years after the initial transplant for recurrent EPP liver disease. In conclusion, the 5-year patient survival rate in patients transplanted for EPP liver disease is good, but the recurrence of EPP liver disease appears to diminish long term graft and patient survival.     

Role of Alcohol-induced Hypothermia in Mediating the Teratogenic Effects of Alcohol in C57BL/6 J Mice

The purpose of this study was to determine the role of alcohol-induced maternal hypothermia in the teratogenic actions of alcohol. C57BL/6J mice were administered an acute dose of alcohol (5.8 g/kg orally) or isocaloric sucrose on day 10 of gestation. One half of each group was placed for 6 hr in an incubator set at 32 degrees C and the other half was housed in the incubator at room temperature (22 degrees C). As expected, acute prenatal alcohol exposure at this time of gestation was associated with decreased birth weight and an increase in limb and kidney malformations. The significant alcohol x environmental temperature interaction on these dependent variables indicated that the teratogenic insult was not attenuated, but was in fact even greater for the 32 degrees C/alcohol group. An absence of a main effect of environmental temperature indicated that the 32 degrees C environment, per se, was not teratogenic. Thus, maternal hypothermia is probably not an etiological factor in animal models of fetal alcohol syndrome. Moreover, antagonism of alcohol-induced maternal hypothermia exacerbates the teratogenic actions of alcohol observed at room temperature.     

Psychiatric rehabilitation in a French-speaking setting: current practices, future challenges]

This paper aims to assess current interventions in psychiatric rehabilitation in the French-speaking world and to discuss future developments. We review examples of policies and practices in Quebec and Europe and discuss the role and involvement of professionals; namely, the psychiatrists and the nursing staff. We also present different rehabilitation strategies and techniques used in the French-speaking world, such as case management, social-skills training, cognitive therapies for psychotic symptoms, family interventions, and return-to-work interventions. In conclusion, we invite psychiatrists to play a more active role in rehabilitation. We recommend the creation of small, specialized units closely linked to the needs of clients, and we propose to integrate social and medical interventions, rather than opposing them.     

Methodological issues in music interventions in oncology settings: A systematic literature review

This paper is part of a systematic literature review and presents methodological issues within studies, investigating therapeutic applications of music in cancer patients. This review focuses on published international research from the USA, Canada, Australia and European countries using four electronic databases: PSYCHINFO, WEB OF SCIENCE, MEDLINE and SCIENCEDIRECT. Thirty-two papers were identified and each paper was coded in terms of sample type, basic research question, research design and methodological details, results and implications for future research. This paper addresses questions related to study design and methodology. It explores and discusses the relative advantages and disadvantages of various approaches.     

Percutaneous transluminal coronary angioplasty in a patient with paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired chronic hemolytic anemia associated with an unusual susceptibility to hemolytic crisis, infection, and venous thrombosis which would be aggravated by a number of factors including surgery. We report a case of PNH undergoing percutaneous transluminal coronary angioplasty and discuss the corresponding perioperative management.