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.     

Dexamethasone decreases the pro- to anti-inflammatory cytokine ratio during cardiac surgery

Background. Cytokines regulate inflammation associated withcardiopulmonary bypass (CPB). Pro-inflammatory cytokines maycause myocardial dysfunction and haemodynamic instability afterCPB, but the release of anti-inflammatory cytokines is potentiallyprotective. We studied the effects of dexamethasone on pro-and anti-inflammatory cytokine responses during coronary arterybypass grafting surgery. Methods. Seventeen patients were studied: nine patients receiveddexamethasone 100 mg before induction of anaesthesia (group1) and eight patients acted as controls (group 2). Plasma levelsof tumour necrosis factor (TNF)-     

儿童哮喘非侵入性气道炎症监测的研究进展

尽管目前对哮喘的发病机制已有较深入的了解,但是许多研究显示,在世界的许多地方,哮喘并未能得到理想控制[1].虽然气道炎症是哮喘发病的最重要原因之一,哮喘治疗的根本是抗炎,但常规的临床评估并未涉及准确的气道炎症评价.气道炎症的严重度与特应性和气道高反应性(AHR)密切相关.     

Histidinaemia: a benign metabolic disorder

Histidinaemia is a relatively common inherited metabolic disorder with an incidence similar to phenylketonuria. This paper reports the long term outcome of patients diagnosed by newborn screening in the north west of England. Between 1966 and 1990, 108 infants were diagnosed as having histidinaemia by a regional neonatal screening programme (incidence 1:11,083). A further five children were detected following diagnosis in a sibling. Of the 113, nine were lost to follow up. Infants diagnosed before 1981 (n = 47) were placed on a low histidine diet (225 mg/kg/d) for an average period of 21 months (SD 4.5). All patients were reviewed regularly, Griffiths developmental quotients (DQ) were assessed at 2 and 4 years, and WISC-R intelligence quotients (IQ) at 8, 12, and 18 years. IQ data were converted to standard deviation scores (IQ SDS) to account for increasing IQ norms with time. Neither DQ nor IQ correlated with plasma histidine at diagnosis or with the mean plasma histidine throughout life. Growth was normal in all patients. There was no apparent benefit from a low histidine diet in early childhood. In contrast to other studies, there was no excess of clinical symptoms. On the basis of these findings, histidinaemia is a benign metabolic disorder that does not require treatment.     

Massive Cystic Hepatomegaly in a Female Patient with Polycystic Kidney Disease Treated by Combined Hepatic and Renal Transplantation

A patient with cystic kidney disease of adult onset and severecystic hepatomegaly is presented. The patient was severely disabledsolely by her abdominal bulk. Simultaneous liver and renal transplantationwas undertaken successfully.     

Nephrology nurse practitioners in a collaborative care model

The use of nurse practitioners in the primary care setting is increasingly common. However, little information is available on their use in specialty care areas. This is especially true in nephrology, in which end-stage renal disease (ESRD) lends itself well to this type of practice. We describe our experience with a university-based collaborative care model for ESRD with nurse practitioners in nephrology. Our experience shows that nephrology nurse practitioners have a significant impact on extending the quality and quantity of patient care provided by the nephrologist. They facilitate a holistic patient-friendly approach, and, as an integral part of the collaborative model, there is an associated significant decrease in patient mortality relative to standard comparators. Nurse practitioners provide a large amount of care for ESRD patients in a relatively independent fashion but under the supervision and with the collaborative interaction of nephrologists. Patient satisfaction, approval, respect, and trust for the nurse practitioners are exceptional. The use of nephrology nurse practitioners provides the potential for augmenting patient care, satisfaction, and access to care. It provides an avenue for potential cost reduction in nephrology while maintaining quality of care. It further provides a partial solution to the anticipated shortage of nephrologists in the twenty-first century.     

The complementary value of Doppler-ultrasonography and ocular pneumoplethysmography according to Gee (OPG-Gee) in carotid obstructions

The results of the combination of CW-Doppler ultrasonography and OPG-Gee are described in 68 patients. The Doppler examination had a sensitivity of 92% and a specificity of 89%, the percentages for the OPG-Gee were 69 en 79. The combination of the Doppler examination with the OPG-Gee improves our positive predictive value from 83 to 94%, the negative predictive value from 95 to 98% in concordant results. OPG-Gee fails especially in low-graded (50-74%) stenoses. Since there is no correlation between the degree of the stenosis and the pathophysiological changes of the cerebral circulation, OPG-Gee is of complementary value for the evaluation of the hemodynamic significance of a stenosis, established with the Doppler method.     

The protective effect of intranasal immunization with inactivated influenza virus vaccine

Protection against influenza after killed influenza spray vaccine was assessed in nine double blind, placebocontrolled clinical trials and two field studies, involving a total number of 10,000 subjects.