Impact of residual pulmonary obstruction on the long-term outcome of patients with pulmonary embolism.

The impact of residual pulmonary obstruction on the outcome of patients with pulmonary embolism is uncertain.We recruited 647 consecutive symptomatic patients with a first episode of pulmonary embolism, with or without concomitant deep venous thrombosis. They received conventional anticoagulation, were assessed for residual pulmonary obstruction through perfusion lung scanning after 6 months and then were followed up for up to 3 years. Recurrent venous thromboembolism and chronic thromboembolic pulmonary hypertension were assessed according to widely accepted criteria.Residual pulmonary obstruction was detected in 324 patients (50.1%, 95% CI 46.2-54.0%). Patients with residual pulmonary obstruction were more likely to be older and to have an unprovoked episode. After a 3-year follow-up, recurrent venous thromboembolism and/or chronic thromboembolic pulmonary hypertension developed in 34 out of the 324 patients (10.5%) with residual pulmonary obstruction and in 15 out of the 323 patients (4.6%) without residual pulmonary obstruction, leading to an adjusted hazard ratio of 2.26 (95% CI 1.23-4.16).Residual pulmonary obstruction, as detected with perfusion lung scanning at 6 months after a first episode of pulmonary embolism, is an independent predictor of recurrent venous thromboembolism and/or chronic thromboembolic pulmonary hypertension.

[The appropriateness of diagnostic angiography in cardiology]. / L'appropriatezza in diagnostica angiografica cardiologica.

Coronary angiography is the most frequently performed invasive diagnostic test in the western world, but regional differences are common and have been outlined in both observational and randomized studies. Appropriateness evaluation is hence fundamental, as the use of invasive cardiac procedures is strongly associated with the population-based availability of catheterization facilities. A procedure is judged appropriate if the expected health benefit exceeds the possible negative consequences by a sufficiently wide margin; it is necessary when not performing it could result in harm for the patient. In the first period (1980-1995) researchers seemed to try to find an explanation for geographic variations regarding overuse: the appropriateness remained at the same level through time (75%), while a trend towards a reduction in the number of inappropriate procedures (< or = 20%) and an increase in that of the uncertain ones was evident. The different opinions of the expert panels constituted the major cause of variability. The factors mainly affecting the appropriateness were advanced age, angina class, intensity of medical therapy, exercise test results and income. Canadians and Europeans seemed to request a higher standard of scientific evidence as compared to US doctors; surgeons tended to give higher scores than cardiologists and internists. Inappropriate indications were similar in high- and low-use hospitals. More recently, important data emerged on the lower than necessary use of this procedure and this was more evident in hospitals without on-site catheterization facilities and in patients without fee-for-service insurance. Patient selection was suboptimal and coronary angiography was more frequently performed in low-risk populations. This phenomenon is of concern, because the lower than necessary use of indicated procedures can bear on the patients' outcome. In fact, an inverse relationship between mortality and coronary angiography use has been observed, especially in patients in whom it has been judged necessary. No lower than necessary use of differences in appropriateness have been found in females, but this is possible in ethnic minorities. If the appropriateness is to be improved, specific actions have to be directed to increase the know-how of doctors, patients, and administrators, to promote research in the fields where knowledge is still missing and to implement simplified guidelines and appropriateness criteria, in order to favor a more extensive use. It is mandatory to assess the necessity of coronary angiography procedures and to grant access to those patients who meet the necessity criteria.

Panencephalopathic Creutzfeldt-Jakob disease with distinct pattern of prion protein deposition in a patient with D178N mutation and homozygosity for valine at codon 129 of the prion protein Gene.

Prion diseases include sporadic, acquired and genetic forms linked to mutations of the prion protein (PrP) gene (PRNP). In subjects carrying the D178N PRNP mutation, distinct phenotypes can be observed, depending on the methionine/valine codon 129 polymorphism. We present here a 53-year-old woman with D178N mutation in the PRNP gene and homozygosity for valine at codon 129. The disease started at age 47 with memory deficits, progressive cognitive impairment and ataxia. The clinical picture slowly worsened to a state of akinetic mutism in about 2 years and the disease course was 6 years. The neuropathologic examination demonstrated severe diffuse cerebral atrophy with neuronal loss, spongiosis and marked myelin loss and tissue rarefaction in the hemispheric white matter, configuring panencephalopathic Creutzfeldt-Jakob disease. PrP deposition was present in the cerebral cortex, basal ganglia and cerebellum with diffuse synaptic-type pattern of immunoreactivity and clusters of countless, small PrP deposits, particularly evident in the lower cortical layers, in the striatum and in the molecular layer of the cerebellum. Western blot analysis showed the presence of type 1 PrP(Sc) (Parchi classification). These findings underline the clear-cut distinction between the neuropathological features of Creutzfeldt-Jakob disease associated with D178N PRNP mutation and those of fatal familial insomnia.