Gliomatosis cerebri: molecular pathology and clinical course

Gliomatosis cerebri is a rare, diffusely growing neuroepithelial tumor characterized by extensive brain infiltration involving more than two cerebral lobes. Among 13 patients with gliomatosis cerebri (median age, 46 years), biopsies showed features of diffuse astrocytoma (n = 4), oligoastrocytoma (n = 1), anaplastic astrocytoma (n = 5), anaplastic oligoastrocytoma (n = 1), or glioblastoma (n = 2). Molecular genetic investigation showed TP53 mutations in three of seven tumors and both PTEN mutation and epidermal growth factor receptor overexpression in one tumor. Amplification of CDK4 or MDM2 or homozygous deletion of CDKN2A was not detected. Three of 10 patients receiving radiotherapy showed a partial response (one patient) or had stable disease (two patients) lasting for more than 1 year. Four of six patients treated with procarbazine, carmustine, vincristine chemotherapy demonstrated partial remission (one patient), minor response (two patients), or stable disease (one patient). Median survival time from diagnosis was 14 months (range, 4-91+ months). Infratentorial involvement was associated with shorter survival. We conclude that (1) the molecular genetic alterations in gliomatosis cerebri resemble those in diffuse astrocytomas; (2) the prognosis of gliomatosis cerebri is variable but for at least 50% of patients as poor as for glioblastoma; and (3) some patients respond to radiotherapy and/or procarbazine, carmustine, vincristine chemotherapy.     

A new case of short-chain acyl-CoA dehydrogenase deficiency with isolated ethylmalonic aciduria

A 28-month-old Turkish girl presented with recurrent bronchopneumonia and severe muscular hypotonia. Urinary excretion of ethylmalonic acid was persistently elevated, methylsuccinate appearing only in stress situations. Studies in cultured fibroblasts showed a deficiency of short-chain acyl-CoA dehydrogenase.     

Interaction of different strains of Entamoeba histolytica with target cells: characterization of electrophysiological and morphological features

Two strains of Entamoeba histolytica with pathogenic zymodemes (SFL3, HK9), one strain with non-pathogenic zymodeme ("Bru") and one non-pathogenic Entamoeba sp. strain ("cold strain"), were investigated with respect to their interaction with target cells. Three test systems were used: 1) direct microscopical observation and qualitative as well as quantitative evaluation of contact and binding events with MDCK cells as targets, 2) kinetics of cytotoxic activity as measured by means of chromium release from 51Cr-labelled K562 cells, and 3) electrophysiological observations with freshly prepared mouse liver cells. We observed that the non-pathogenic cold strain interacted only shortly with target cells (statistical events, interaction type "I"), but did not induce morphological changes, chromium release or depolarization of targets. Non-pathogenic and avirulent strain "Bru" showed, apart from type "I"-binding, the ability to establish tight (type "II") and long-lasting contact (type "III") with targets, but again without cytotoxic effects. The pathogenic but avirulent strain HK9 tightly interacted (type "II") and sometimes long-lasting with target cells, but morphological changes and chromium release were of a moderate degree during the first 20 min, and depolarization was only a rare event. In contrast, strain SFL3 produced tight and long-lasting contacts (type "III" binding), leading to cell death in 83% (type "IV" interaction) within 20 min, substantial chromium release within 10 min and rapid depolarization ("electric collapse") of target cells.     

Mortality and complications after stoma closure

HYPOTHESIS: This study was undertaken to evaluate factors contributing to hospital mortality and complications of stoma closure. DESIGN: Retrospective cohort study. SETTING: Department of Surgery of a 2500-bed university hospital. PATIENTS: Consecutive eligible patients who underwent stoma closure were selected from a local registry containing 30 219 patients. The medical records of 587 adult patients were reviewed according to a predefined extraction form. Patients with additional, unrelated surgical interventions or younger than 18 years were excluded. Follow-up was complete for all included patients. MAIN OUTCOME MEASURES: The primary outcome variable was 30-day mortality; the secondary outcome variable was presence of surgery-related complications within 30 days. RESULTS: We analyzed 533 patients with stoma closure between 1993 and 2001. The overall stoma closure-related mortality rate was 3% (15 patients); the overall stoma closure-related surgical complications rate was 20% (107 patients). Wound infections (9%) and anastomotic leakage (5%) were the most common surgical complications. Age was the only significant risk factor for survival (P = .02). Use of a soft silicone drain for intraperitoneal drainage (odds ratio, 1.62 [95% confidence interval, 1.07-2.45]; P = .03) was the only significant risk factor for complications. In patients with carcinoma as the primary disease (odds ratio, 0.61 [95% confidence interval, 0.40 to 0.93]; P = .02), we observed significantly fewer complications. CONCLUSIONS: We found considerable mortality and complications after stoma closure. Apart from age, we could not identify any predictor for mortality in patients with stoma closure. Randomized studies are needed to determine whether certain types of drains influence outcome.     

Cardiac transplantation complicated by acute thrombotic occlusion of the right coronary artery

We report the case of a 63-year-old male patient undergoing cardiac transplantation due to fourth time aortic valve endocarditis. The postoperative course was complicated by thrombotic occlusion of the right coronary artery (RCA) causing acute right ventricular myocardial infarction, which required extracorporeal membrane oxygenation. The RCA could be reopened by catheter-based intervention and the patient stabilized. In order to avoid further immobilization, a right ventricular assist device was implanted and an aortocoronary bypass to the RCA was performed. After that, the patient stabilized progressively, could be weaned from the assist device, and was discharged home 6 weeks after transplantation. On coronary angiography, which is routinely performed 4 to 6 weeks after transplantation, a fistula from the RCA to the right ventricle was detected which was treated conservatively. Five months after transplantation, the patient is in good clinical condition without signs of recurrent endocarditis. This case shows that intense interdisciplinary cooperation of cardiac specialists allows the successful management of very complex patients in serious clinical conditions.