Showing papers by "Fabrizio Salvi published in 2005"

Non-invasive evaluation of the myocardial substrate of cardiac amyloidosis by gadolinium cardiac magnetic resonance

Abstract: Objective: To investigate the prevalence and distribution of gadolinium (Gd) enhancement at cardiac magnetic resonance (CMR) imaging in patients with cardiac amyloidosis (CA) and to look for associations with clinical, morphological, and functional features. Patients and design: 21 patients with definitely diagnosed CA (nine with immunoglobulin light chain amyloidosis and 12 transthyretin related) underwent Gd-CMR. Results: Gd enhancement was detected in 16 of 21 (76%) patients. Sixty six of 357 (18%) segments were enhanced, more often at the mid ventricular level. Transmural extension of enhancement within each patient significantly correlated with left ventricular (LV) end systolic volume ( r = 0.58). The number of enhanced segments correlated with LV end diastolic volume ( r = 0.76), end systolic volume ( r = 0.6), and left atrial size ( r = 0.56). Segments with > 50% extensive transmural enhancement more often were severely hypokinetic or akinetic (p = 0.001). Patients with > 2 enhanced segments had significantly lower 12 lead QRS voltage and Sokolow-Lyon index. No relation was apparent with any other clinical, morphological, functional, or histological characteristics. Conclusion: Gd enhancement is common but not universally present in CA, probably due to expansion of infiltrated interstitium. The segmental and transmural distribution of the enhancement is highly variable, and mid-ventricular regions are more often involved. Enhancement appears to be associated with impaired segmental and global contractility and a larger atrium.

Adult Alexander's disease without leukoencephalopathy.

Abstract: poison control center consultation. Tamoxifen infrequently causes reversible neurotoxicity, but only at much higher doses ( 160mg/m daily). Recent investigations into the use of artemisinin compounds in cancer treatment have not been substantiated in clinical trials. Because of pervasive reports of animal brainstem toxicity, and the gradual emergence of similar patient cases, it becomes imperative to ascertain the safety of prolonged courses of artemisinin for cancer prophylaxis.

Early onset aggressive hereditary amyloidosis: report of an Italian family with TTR Arg47 mutation.

Abstract: Arg47 is a rare transthyretin-related (TTR) amyloidosis variant that is characterised by polyneuropathy and autonomic failure. We describe an Italian family with this mutation whose members (two women and their father) showed a rapid progression of the peripheral nervous system involvement and died within 5 years of clinical onset. Patients with Arg47 or other aggressive TTR amyloidoses should be considered high priority patients for orthotopic liver transplantation.