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.     

The tracheal nonadrenergic noncholinergic inhibitory system during antigen challenge

This study was undertaken to test the hypothesis that malfunction of the nonadrenergic noncholinergic inhibitory system (NANCIS) induces hyperreactive airways. Antigen sensitized guinea pigs were divided into four groups: (1) antigen challenge (n = 6), (2) 2 min oxyhemoglobin (HbO2) + antigen challenge (n = 5), (3) 27 min HbO2 + antigen challenge (n = 4), and (4) 2 min HbO2 + transmural stimulation (TS) + antigen challenge (n = 6). These animals were sensitized with ovalbumin 10 days before the study. In addition, 12 normal control animals without antigen sensitization were used for comparison. Under artificial ventilation, the anesthetized-paralyzed animals were hourly injected with atropine (0.2 mg/kg) and propranolol (1 mg/kg). Cervical segment of the trachea was converted to a closed tracheal pouch filled with Krebs solution containing also atropine (1 microM) and propranolol (3.5 microM). A change in the pouch pressure (Pp) reflected NANCIS TS- or antigen (5 micrograms) challenge-induced relaxation and/or constriction. HbO2 was used to inhibit NANCIS transmitter. There was no significant difference between normal and sensitized animals in the NANCIS TS-induced relaxation. Antigen challenge resulted in biphasic alteration in Pp, an initial increase and then a decrease after about 7 min. HbO2 pretreatment alone did not potentiate antigen-induced increase in Pp. HbO2 + TS, however, significantly abolished the late relaxation phase after antigen challenge.     

Acute pancreatitis in the aged

Forty-seven patients aged more than 75 years with acute pancreatitis were studied. The most common cause of acute pancreatitis was biliary tract stones. The clinical presentation and severity of the disease as judged by the number of poor prognostic factors were not different from the group of patients aged less than 75 years. The mortality rate in the older group was thrice that of the younger group (21.3% versus 7.24%). The higher mortality rate was explained by a higher incidence of deaths related to complications of biliary stones and coincidental diseases. Significantly more aged patients with mild disease died, indicating the limitation of predictive ability of the scoring system in acute pancreatitis of the aged.     

In vitro synthesis of antimycobacterial antibodies with different specificities in various tissues of leprosy patients

For the detection of the synthesis in vitro of anti-Mycobacterium leprae antibodies in various tissues of leprosy patients, biopsy specimens of skin lesions, nasal mucosa, larynx, lymph nodes, and bone marrow were cultured in a medium containing 14C-labeled lysine and isoleucine. The culture fluids were analyzed by crossed immunoelectrophoresis with intermediate gel and autoradiography. The results show that synthesis of anti-M. leprae antibodies occurs at the investigated sites of leprosy patients and that the specificities of the synthesized antibodies differ between sites in individual patients. It is conceivable that these antibodies play a role in the local defense against M. leprae.     

Influence of age on the mortality from acute pancreatitis

The influence of age on the mortality rate of 268 patients with acute pancreatitis was studied. The hospital mortality rate for patients aged below 50 years was 5.9 per cent. The figure increased to 21.3 per cent in patients aged over 75; the high mortality was accounted for by a higher incidence of deaths related to concomitant medical or surgical diseases in the same hospital admission rather than to complications resulting directly from the pathological process of acute pancreatitis. When only deaths due to complications of acute pancreatitis were analysed, the mortality rate was not significantly different between the young and elderly groups. Moreover, the complication rate and the proportion of patients having severe disease (judged by the number of prognostic signs) were not higher in the elderly. Thus acute pancreatitis was intrinsically not more serious were it not for the presence of concomitant diseases with advanced age.     

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.     

隆鼻硅胶假体雕刻中的四个角度

目的探讨隆鼻硅胶假体雕刻中的四个角度与隆鼻并发症的关系。方法总结了420例硅胶假体隆鼻术患者,分析并发症与假体雕刻的关系,提出假体雕刻要与鼻部解剖形态相吻合的四个角度。结果术后1周,发现3例患者假体鼻根部向一侧轻度偏斜,2例6个月后鼻根部假体固定不良。1例术后主诉头痛,取出假体后症状消失。其余未发生并发症。结论假体雕刻与鼻部解剖形态的四个角度相吻合,可以减少并发症的发生,提高美容效果。