Pseudo-diastrophic dysplasia

A new case of pseudo-diastrophic dysplasia in a boy born to consanguineous parents is reported. The patient developed neurologic (hydrocephalus) and respiratory anomalies and died at the age of 11 months of an unknown cause. Features that distinguish this chondrodysplasia from diastrophic dysplasia include decreased height of vertebral bodies and anomalies of the pelvis with short iliac wings and a horizontal orientation of the internal spine of the acetabular roof.     

Cavernous angiomas of the spinal cord

Five cases of histologically verified cavernous angiomas of the spinal cord are reported. Acute lower-extremity sensory disturbance was the initial symptom in four patients, and one presented with weakness of the hand. Progressive neurological deficit occurred in all patients, but the clinical course and outcome were extremely variable. Myelography revealed an intramedullary lesion in two cases but was completely normal in three; magnetic resonance imaging was diagnostic in these patients. Subtotal removal was accomplished in two cases, and myelotomy and biopsy were carried out in three. Four of the cavernous angiomas were located in the cervicothoracic region, whereas one was found in the thoracolumbar cord. All of the patients exhibited characteristic gross and microscopic features as well as hemosiderin-laden macrophages indicating remote hemorrhage. The diagnostic, therapeutic, and prognostic implications of this rare condition are discussed.     

Intraperitoneal infection with Salmonella abortusovis is partially controlled by a gene closely linked with the Ity gene.

The aim of the present study was to determine whether the Ity gene, which controls the resistance to S. typhimurium infection in mice, also governs the resistance to S. abortusovis, a serotype specific for goat and sheep. During either i.v. or i.p. infection, BALB/c mice (Itys) were not able to control the growth of S. abortusovis and eventually died from infection. In contrast CBA (Ityr) or (C.CB)F1 (Ityr/s) mice were able to control the growth of these bacteria. Using congenic C.D2 Ityr mice, we found that the gene controlling resistance to S. abortusovis was tightly linked to the Ity gene on chromosome 1. Furthermore, in the spleen and the liver of backcross BALB/c x (CBA x BALB/c) mice, the S. abortusovis resistance phenotype cosegregated with the two alleles of the Len-1 gene, a gene tightly linked to the Ity gene. By contrast, in these backcross mice, the level of infection of the peritoneal cavity, the site of inoculation, did not correlated with the Len-1 phenotype of the animal. These results provide evidence that after i.p. inoculation the control of S. abortusovis growth in the spleen and the liver is controlled by the Ity gene, but also suggest that additional gene(s) regulate the number of bacteria at the site of inoculation.     

Percutaneous transluminal coronary angioplasty in patients with spasm superimposed on atherosclerotic narrowing

Of 552 patients undergoing percutaneous transluminal coronary angioplasty 102 had coronary artery spasm superimposed on atherosclerotic narrowing. Coronary angioplasty was successful in 97 (95%). The patients were discharged on a regimen of nifedipine (40-60 mg/day). Seventy six patients were symptom free 6-8 months after the procedure. Restenosis was detected in 35% of patients. Coronary artery spasm was provoked in 38 (44%) of the 87 patients who underwent an ergometrine maleate test. Twenty seven of the 34 patients with restenosis had a provocation test and coronary artery spasm was superimposed on restenosis in 22 (81.5%). Coronary angioplasty is feasible in patients with coronary artery spasm superimposed on atherosclerotic narrowing but the rate of restenosis is high and coronary artery spasm could have a role in the pathogenesis of restenosis.     

Early beneficial effect of streptokinase on left ventricular function in acute myocardial infarction

The effect of intravenous streptokinase therapy on the time course of functional recovery was investigated in a controlled study of 64 patients randomized within 3 hours after the onset of acute myocardial infarction (AMI). Contrast ventriculography was performed 1 to 4 days after AMI and repeated 5 weeks later. Wall motion was analyzed by the centerline method in the central infarct, peripheral infarct and noninfarct regions. In patients with ventriculographic data at the early catheterization, streptokinase-treated patients had less severe hypokinesia in the central infarct region than control patients (-2.9 +/- 0.9 [n = 29] vs -3.4 +/- 0.7 standard deviations below normal [n = 21], p less than 0.05). The benefit of streptokinase was more marked in the peripheral infarct region (-1.5 +/- 0.7 vs -2.1 +/- 0.6, p less than 0.001). As a result, the ejection fraction was slightly higher in treated versus control groups (46 +/- 10 vs 43 +/- 7%, respectively; difference not significant). At 5 weeks, function in the streptokinase and control groups had diverged further because of continued improvement in the streptokinase-treated patients. This study shows that streptokinase benefits left ventricular (LV) function by 1 to 4 days after AMI, earlier than previously reported. The benefit was not limited to the peripheral infarct region, where ischemia might have been less severe, but was also seen in the central infarct region. The implication is that thrombolytic therapy can improve LV function during the period of myocardial stunning, while myocardial function is still recovering.     

Assessment of olfactory and trigeminal function using chemosensory event-related potentials.

GOALS: To give an overview on the theoretical and practical applications of chemosensory event-related potentials. METHODS: Chemosensory event-related potentials (ERPs) may be elicited by brief and precisely defined odorous stimuli. Based on the principles of air-dilution olfactometry, a stimulator was developed in the late 1970s, which allows stimulation of the olfactory neuroepithelium and the nasal mucosa with no concomitant mechanical stimulation. Chemosensory ERPs were obtained after stimulation of the olfactory nerve (olfactory ERPs) or the trigeminal nerve (somatosensory or trigeminal ERPs). The characteristics of the stimulator for chemosensory research as well as the variables influencing the responses are discussed in this paper. RESULTS: Implementation and normative data from our department are reported with different clinical examples from otorhinolaryngologic clinic. The bulk of the evoked response consists of a large negative component (often referred to as N1), which occurs between 320 and 450 ms after stimulus onset. This component is followed by a large positive component, often referred to as P2, occurring between 530 and 800 ms after stimulus onset. Absence of olfactory ERPs and presence (even with subtle changes) of somatosensory ERPs is a strong indicator of the presence of an olfactory dysfunction. CONCLUSIONS: This review examines and discusses the methods of chemosensory stimulation as well as the electrophysiological correlates elicited by such stimuli. The clinical applications of chemosensory ERPs in neurology and otorhinolaryngology are outlined.     

Anti-tumor immunotherapy via antigen delivery from a live attenuated genetically engineered Pseudomonas aeruginosa type III secretion system-based vector.

Immunotherapy requiring an efficient T lymphocyte response is initiated by antigen delivery to antigen-presenting cells. Several studies have assessed the efficiency of various antigen loading procedures, including microbial vectors. Here a live strain of Pseudomonas aeruginosa was engineered to translocate a recombinant antigenic protein into mammalian cells via the type III secretion system, a bacterial device translocating effector proteins into host cells. Optimization of the vector included virulence attenuation and determination of the N-terminal sequence allowing translocation of fused antigens into cells. In vitro delivery of an ovalbumin fragment by the bacterial vector into dendritic cells induced the activation of ovalbumin-specific CD8(+) T lymphocytes. Mice injected with the ovalbumin-delivering vector developed ovalbumin-specific CD8(+) T lymphocytes and were resistant to a subsequent challenge with an ovalbumin-expressing melanoma. Moreover, in a curative assay, injection of the vaccine vector 5 and 12 days after tumor implantation led to a complete cure in five of six animals. These results highlight the utility of type III secretion system-based vectors for anti-tumor immunotherapy.     

Does Preoperative Coronary Angioplasty Improve Perioperative Cardiac Outcome?

Background: Percutaneous coronary intervention (PCI) is performed in patients with coronary artery disease who are undergoing major noncardiac procedures to reduce perioperative cardiac morbidity and mortality. However, the impact of this approach on postoperative outcome remains controversial.

Methods: The authors analyzed a cohort of 1,152 patients after abdominal aortic surgery in which 78 patients underwent PCI. A propensity score analysis was performed. Also, using a logistic regression model, the authors determined variables associated with a severe postoperative coronary event or a death in patients without PCI. Then, in patients with PCI, they compared the expected and observed outcome.

Results: Five variables (age > 75 yr, blood transfusion > 3 units, repeated surgery, preoperative hemodialysis, and previous cardiac failure) independently predicted (with 94% correctly classified) a severe postoperative coronary event, and five variables (age > 75 yr, repeated surgery, previously abnormal ST segment/T waves, previous hypertension, and previous cardiac failure) independently predicted (with 97% correctly classified) postoperative death. In the PCI group, the observed percentages of patients with a severe postoperative coronary event (9.0% [95% confidence interval, 4.4-17.4]) or death (5.1% [95% confidence interval, 2.0-12.5]) were not significantly different from the expected percentages (8.2 and 6.9%, respectively). When all patients were pooled together, the odds ratios of PCI were not significant. The propensity score analysis provided a similar conclusion.     

Return to work of professional drivers after cardiac rehabilitation]

This study analysed the possibilities of returning to work of professional drivers after a cardiac event and rehabilitation. The population comprised 94 consecutive patients, all men, average age 48.8 years (range 30 to 63 years) referred after coronary bypass surgery (N = 39), myocardial infarction (N = 38), angina (N = 4) or valve replacement surgery (N = 13). Advice on professional reinsertion was given after the rehabilitation program, authorization to drive being given in the absence of cardiac symptoms, residual myocardial ischaemia, severe left ventricular dysfunction and serious ventricular arrhythmias. After 35 months, 4 patients were lost to follow-up; of the 90 remaining patients, the frequency of return to work (maximal at the 9th month) was 65.6% with 84.7% obtaining a renewal of their driving licence. In this series, 81% of patients were asymptomatic, 2 died, 16.7% had further cardiovascular complications. The morbidity and mortality were significantly greater in the group who had to stop driving (N = 40) (32.5% vs 8%, p < 0.001). Non complications occurred during work in those who resumed driving. This study confirms the safety of allowing low risk professional drivers, identified during cardiac rehabilitation by simple, reliable clinical and paraclinical criteria, to return to work.