Amyloidosis is a disorder caused by misfolding of autologous protein and its extracellular deposition as fibrils, resulting in vital organ dysfunction and eventually death. Pulmonary amyloidosis may be localised or part of systemic amyloidosis.Pulmonary interstitial amyloidosis is symptomatic only if the amyloid deposits severely affect gas exchange alveolar structure, thus resulting in serious respiratory impairment. Localised parenchymal involvement may be present as nodular amyloidosis or as amyloid deposits associated with localised lymphomas. Finally, tracheobronchial amyloidosis, which is usually not associated with evident clonal proliferation, may result in airway stenosis.Because the treatment options for amyloidosis are dependent on the fibril protein type, the workup of all new cases should include accurate determination of the amyloid protein. Most cases are asymptomatic and need only a careful follow-up. Diffuse alveolar-septal amyloidosis is treated according to the underlying systemic amyloidosis. Nodular pulmonary amyloidosis is usually localised, conservative excision is usually curative and the long-term prognosis is excellent. Tracheobronchial amyloidosis is usually treated with bronchoscopic interventions or external beam radiation therapy.
BackgroundIn the Veneto region (north-eastern Italy) an entomological surveillance system has been implemented since the introduction of the Asian tiger mosquito (Aedes albopictus) in 1991. During the routine monitoring activity in a tiger mosquito-free area, an unexpected mosquito was noticed, which clearly did not belong to the recorded Italian fauna.FindingsAt the end of May 2011, twelve larvae and pupae were collected in a small village in Belluno province (Veneto region) from a single manhole. Ten adults reared in the laboratory were morphologically and genetically identified as Aedes (Finlaya) koreicus (Edwards, 1917), a species native to Southeast Asia. The subsequent investigations carried out in the following months in the same village provided evidence that this species had become established locally. Entomological and epidemiological investigations are currently ongoing in the surrounding area, to verify the eventual extension of the species outside the village and to trace back the route of entry into Italy.ConclusionsThis is the first report in Italy of the introduction of the exotic mosquito Ae. koreicus. This species has been shown experimentally to be competent in the transmission of the Japanese encephalitis virus and of the dog heartworm Dirofilaria immitis and is considered a potential vector of other arboviruses. Thus, the establishment of this species may increase the current risk or pose new potential threats, for human and animal health. This finding considerably complicates the entomological monitoring of the Asian tiger mosquito Ae. albopictus in Italy and stresses the importance of implementing the entomological surveillance for the early detection of and the rapid response against invasive mosquito species.
The validated criteria of hematologic response in light-chain (AL) amyloidosis are based on the measurement of circulating free light chains (FLCs). Patients with a difference between involved and uninvolved FLC (dFLC) <50 mg/L cannot be assessed for response and are excluded from clinical trials. We compared the clinical characteristics and outcome of 203 newly diagnosed patients with dFLC <50 mg/L (low dFLC) with 866 patients with measurable dFLC (high dFLC) evaluated between 2004 and 2015. Heart involvement was significantly less common and less advanced in the low-dFLC group (43% vs 83% and Mayo stage III 45% vs 15%, both < .001), whereas renal involvement was more frequent (77% vs 63%, < .001) and more severe (renal stage III 26% vs 18%, = .001). Overall survival (OS) was significantly better in the low-dFLC group (median 117 vs 21 months, < .001), whereas no difference was seen in renal survival (RS). Within each Mayo stage, patients with low dFLC had a longer survival. In the low-dFLC group, complete response was associated with a significant advantage in OS (median not reached vs 117 months, = .005) and with a better RS. A reduction in dFLC after therapy of<10 mg/L was associated with a better OS and RS in patients with at least a dFLC >20 mg/L baseline. Nineteen percent of newly diagnosed patients with AL amyloidosis have low dFLC and had a better outcome. Hematologic response assessed with adapted criteria predicts OS and RS in these patients, who can thus be assessed for response and included in clinical trials with appropriate stratification.
5POCT specific to NOAC are currently unavailable, but influences of NOAC on established coagulation POCT have been reported. [6][7][8][9][10] Data based on artificially spiked blood samples from healthy volunteers suggest a high correlation for the Background and Purpose-Specific coagulation assays for non-vitamin K antagonist oral anticoagulants (NOAC) are relatively slow and often lack availability. Although specific point-of-care tests (POCT) are currently not available, NOAC are known to affect established coagulation POCT. This study aimed at determining the diagnostic accuracy of the CoaguChek POCT to rule out relevant concentrations of rivaroxaban, apixaban, and dabigatran in real-life patients. Methods-We consecutively enrolled 60 ischemic stroke patients newly started on NOAC treatment and obtained blood samples at 6 prespecified time points. Samples were tested using the CoaguChek POCT, laboratory-based coagulation assays (prothrombin time and activated partial thromboplastin time, anti-Xa test and Hemoclot), and liquid chromatography-tandem mass spectrometry for direct determination of NOAC concentrations. Results-Three hundred fifty-six blood samples were collected. The CoaguChek POCT strongly correlated (r=0.82 P<0.001) with rivaroxaban concentrations but did not accurately detect dabigatran or apixaban. If used to estimate the presence of low rivaroxaban concentrations, POCT was superior to predictions based on normal prothrombin time and activated partial thromboplastin time values even if sensitive reagents were used. POCT-results ≤1.0 predicted rivaroxaban concentrations <32 and <100 ng/mL with a specificity of 90% and 96%, respectively. Conclusions-If anti-Xa test is not available, we propose the use of the
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