Widespread cardiovascular autonomic dysfunction in primary amyloidosis: does spontaneous hyperventilation have a compensatory role against postural hypotension?

Objective: To investigate the possible causes of abnormal blood pressure control in light chain related (primary, AL) amyloidosis.

Design: Cardiovascular, autonomic, and respiratory response to passive tilting were investigated in 51 patients with primary amyloidosis (mean (SEM) age 56 (2) years) and in 20 age matched controls. Spontaneous fluctuations in RR interval, respiration, end tidal carbon dioxide, blood pressure, and skin microcirculation were recorded during supine rest and with tilting.

Holter monitoring in AL amyloidosis: prognostic implications.

The heart is involved in more than one third of patients with primary (AL) amyloidosis at diagnosis and it is by far the most common cause of death. Rhythm and conduction abnormalities generally represent the terminal event. The aims of this study were to determine the spectrum of Holter abnormalities found in AL amyloidosis and to assess their prognostic significance, particularly in relation to sudden death.

A modified high-dose dexamethasone regimen for primary systemic (AL) amyloidosis.

High-dose dexamethasone (HD-Dex) has been reported to benefit AL amyloidosis patients with varying response rates. Our preliminary experience with the usual HD-Dex schedule indicated that the induction phase was rather toxic in AL patients. We therefore adopted a milder schedule consisting of dexamethasone 40 mg on d 1-4 q21 d for up to eight cycles.

Clotting alterations in primary systemic amyloidosis.

Background And Objective: The bleeding manifestations frequently observed in patients with immunoglobulin light chain amyloidosis (AL) have been attributed to different pathogenetic factors: amyloid deposits in several organs and systems leading to failures of these latter, the affinity of amyloid for some clotting factors, and the presence of plasma components interfering with fibrin formation could all induce alterations of clotting tests. This investigation was aimed at defining the prevalence of clotting abnormalities and their clinical manifestations in patients with AL.

Design And Methods: Thirty-six consecutive patients with biopsy proven amyloidosis and documented monoclonal gammapathy were enrolled within one year.

Abnormalities in thrombin-antithrombin pathway in AL amyloidosis.

Various pathogenic factors have been proposed to explain the abnormal hemostasis observed in AL amyloidosis. Since imbalance between clotting factors and inhibitors could play a pathogenic role in both hemorrhagic and thrombotic manifestations, we investigated the thrombin-antithrombin pathway in 35 patients with AL amyloidosis. Ten patients suffered from bleeding while 3 patients experienced deep venous thrombosis.

The new apolipoprotein A-I variant leu(174) --> Ser causes hereditary cardiac amyloidosis, and the amyloid fibrils are constituted by the 93-residue N-terminal polypeptide.

We identified a novel missense mutation in the apolipoprotein A-I gene, T2069C Leu(174) --> Ser, in a patient affected by familial systemic nonneuropathic amyloidosis. The amyloid deposits mostly affected the heart of the proband, who underwent transplantation for end-stage congestive heart failure. Amyloid fibrils of myocardial and periumbilical fat samples immunoreacted exclusively with anti-ApoA-I antibodies.

The degrees of plasma cell clonality and marrow infiltration adversely influence the prognosis of AL amyloidosis patients.

Background And Objective: Primary amyloidosis is a lethal form of plasma cell (PC) dyscrasia characterized by deposits of monoclonal immunoglobulin light chains that cause organ dysfunction. In contrast to multiple myeloma, the amyloid clone is typically indolent and of small size, and marrow PC clonality is not always apparent. This is generally investigated by analyzing the light chain isotype ratio in bone marrow PC.

Clonality and specificity of cryoglobulins associated with HCV: pathophysiological implications.

Background/aims: Hepatitis C virus (HCV) infection plays a central role in the pathogenesis of mixed cryoglobulinemia through molecular mechanisms which remain to be elucidated. The aim of this study was to investigate the role of antibody responses to HCV in the pathogenesis of cryoglobulinemia through characterization of the anti-HCV specificity and immunochemical characteristics of the immunoglobulins involved in cryoprecipitation.

Methods: Sera from 50 consecutive patients with chronic HCV infection (RNA positive) were screened for the presence of cryoglobulins.

Immunochemical characterization of the cryoglobulins: pathophysiologic implications.

Immunochemical study of the cryoglobulins has produced a classification of three main types which present clinical correlates. With the current standard immunofixation technique the vast majority of cryoglobulins are presently classified as type II. High-resolution immunoelectrophoretic techniques (immunoblotting and two-dimensional polyacrylamide gel electrophoresis) have helped to identify new sub-types whose clinical relevance is as yet undetermined.

[Disturbances of equilibrium in the aged].

The occurrence during the last year of vestibular disorders, faints and drop attacks was investigated in 150 patients consecutively admitted to a geriatric hospital. The clinical features of these episodes were recorded by means of a proper questionnaire. True vertigo and/or feeling of unstable equilibrium were referred by 54,6% of the patients inquired into, whereas episodes interpretable as faints and drop attacks showed by far lower prevalences, respectively 13,3% and 6%.