Creutzfeldt-Jakob disease presenting as refractory nonconvulsive status epilepticus

A 70-year-old woman was admitted to the intensive care unit with refractory nonconvulsive status epilepticus. Extensive evaluation including neuroimaging and cerebrospinal fluid examination was unrevealing. Brain biopsy revealed spongiosis, and prion disease was confirmed by immunostaining, providing the diagnosis of Creutzfeldt-Jakob disease.     

Treatment of radioulnar synostosis by radical excision and interposition of a radial forearm adipofascial flap

A patient had radical excision of type II diaphyseal radioulnar synostosis and interposition of a radial forearm adipofascial flap. Neither adjuvant nonsteroidal anti-inflammatory medications nor radiation therapy were used. Three years after surgery the patient showed 90 degrees of pronation and 90 degrees of supination without any evidence of recurrence.     

Admission lactate level and the APACHE II score are the most useful predictors of prognosis following torso trauma

BACKGROUND: Markers of dysoxic metabolism and scoring systems for triage have been widely used in critically injured patients. However, so far, no model is sufficiently reliable to predict the outcome in trauma victims. The purposes of the present study, therefore, were to determine whether a correlation exits between the main trauma scoring systems and the markers of dysoxic metabolism. Moreover, to assess if any of the admission parameters can be used to indicate outcome. METHODS: Sixty-four patients were included in this study. Admission data, including arterial lactate level, base deficit (BD), pH, revised trauma score (RTS), injury severity score (ISS), shock index (SI), and Acute Physiology and Chronic Health Evaluation (APACHE II), were collected and analysed by logistic regression analysis. Degree of association between continuous variables were calculated by either Pearson's or Spearman's correlation coefficient, where applicable. The dependence of lactate on two or more other variables was evaluated by multiple linear regression analysis. RESULTS: Logistic regression analysis showed that the fatal outcome following major torso trauma was principally associated with the APACHE II score and lactate. The specificity and the sensitivity of this logistic regression model was 94.6 and 79.2%, respectively. According to standardised linear regression coefficients, BD was the best single predictor of lactate, and APACHE II added a small amount of predictive power. The proportion of total variation in lactate level explained by base deficit, APACHE II and age is R2=85.2%. CONCLUSION: APACHE II score and the arterial lactate level are the most important determinants of clinical outcome in critically injured patients. A correlation exits between lactate and APACHE II and between lactate and base deficit.     

Colonic interposition vs. gastric pull-up after total esophagectomy

Gastric pull-up is the most frequent reconstruction after esophagectomy. In this report we aimed to compare gastric pull-up with colonic interposition in terms of graft function and patient satisfaction. Of 62 patients undergoing esophagectomy, reconstruction was performed by colonic interposition in 11 and gastric pull-up in 51 (without pyloric drainage in 44 and with pyloric drainage in 7). All esophagectomies were performed transhiatally. Patient follow-up ranged from 6 to 132 months (median 14 months). Follow-up examinations were performed 1, 9, 15, and 24 months postoperatively. The following factors were evaluated: time to the start of oral liquid and solid nutrients without vomiting, frequency of regurgitation, presence of pillow staining (night regurgitation), postprandial fullness, and degree of satisfaction during and after eating compared between groups undergoing colonic interposition and gastric pull-up with or without pyloric drainage. Among patients undergoing gastric pull-up, regurgitation was observed in 22% to 27% during follow-up. None of the patients with colonic interposition had reflux or regurgitation. Twenty-five percent of patients with gastric pull-up without drainage and 66% of patients with gastric pull-up plus drainage had reflux esophagitis at 15 months. No esophagitis was observed in patients with colonic interposition during the same period. Overall satisfaction was superior in patients undergoing colonic interposition followed by gastric pull-up with no drainage. Colonic interposition after esophageal resection is a viable option. Our study suggests that function of the replacement is better in this group of patients.     

The CEO and the future of technology. Panel discussion

CEOs play a pivotal role in driving the selection and implementation of clinical and information technology within their organizations. Lack of standardization is viewed as the biggest obstacle to clinical transformation.     

Ethical issues in non-heart-beating donation

A shortage of organ donors and the large number of patients desperately waiting for kidney transplant have led to the search for new sources of transplantable organs. The waiting list has grown at an alarming rate resulting in increased waiting times and deaths. The introduction of non heart beating (NHB) donation programmes generates a lot of ethical issues. How should death of a patient be defined in the case of NHB donation? Is there a strict separation of responsibilities of the medical teams in the different phases of the procedure (patient treatment and actual donation)? How should consent be obtained? Is sufficient respect and care given to the patient and his family? How is the viability of the organs assessed and how should the organs be allocated? We believe that it is very important to debate these issues and to try to outline an ethical framework for NHB donation that can enjoy the widest possible community support.     

Molecular pathology shows p16 methylation in nonadenomatous pituitaries from patients with Cushing's disease.

PURPOSE: The majority of cases of Cushing's disease are due to the presence of a corticotroph microadenoma. Less frequently no adenoma is found and histology shows either corticotroph hyperplasia, or apparently normal pituitary. In this study we have used molecular pathology to determine whether the tissue labeled histologically as "normal" is indeed abnormal. EXPERIMENTAL DESIGN: Tissue from 31 corticotroph adenomas and 16 nonadenomatous pituitaries were subject to methylation-sensitive PCR to determine the methylation status of the p16 gene CpG island. The proportion of methylated versus unmethylated CpG island was determined using combined bisulphite restriction analysis. Methylation status was correlated with immunohistochemical detection of p16. RESULTS: Seventeen of 31 adenomas (54.8%), 4 of 6 cases of corticotroph hyperplasia, and 7 of 10 apparently normal pituitaries showed p16 methylation. Ten of 14 (71%; P = 0.01) adenomas and 2 of 3 cases of corticotroph hyperplasia, which were methylated, failed to express p16 protein. However, only 2 of 7 apparently normal pituitaries that were methylated failed to express p16 protein. Quantitative analysis of methylation using combined bisulphite restriction analysis showed only unmethylated CpG islands in postmortem normal pituitaries; however, in adenomas 80-90% of the cells within a specimen were methylated. The reverse was true for corticotroph hyperplasia and apparently normal pituitaries where only 10-20% of the cells were methylated. Thus, the decreased proportion of cells that were methylated, particularly in those cases of apparently normal pituitary, is the most likely explanation for the lack of association between this change and loss of cognate protein in these cases. CONCLUSIONS: To our knowledge this is the first report that describes an intrinsic molecular change, namely methylation of the p16 gene CpG island, common to all three histological patterns associated with Cushing's disease. Thus, the use of molecular pathology reveals abnormalities undetected by routine pathological investigation. In cases of "apparently" normal pituitaries it is not possible to determine whether the change is associated with adenoma cells "scattered" throughout the gland, albeit few in number, or with the ancestor-clonal origin of these tumor cells.