8.4 Arterial Compliance and Carotid Atherosclerosis in Apolipoprotein A-I Amyloidosis (Leu75Pro)

Background: Hereditary amyloidosis are late-onset autosomal dominant disorders characterized by amyloid deposition in various tissues. Among them, Apolipoprotein A-I amyloidosis (Leu75Pro) is a rare autosomal dominant condition in which renal, hepatic, and testicular involvement has been demonstrate...

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Published inArtery research Vol. 3; no. 4; p. 160
Main Authors Paini, A., Muiesan, M. L., Salvetti, M., Aggiusti, C., Belotti, E., Agabiti Rosei, C., Gregorini, G., Cancarini, G., Agabiti Rosei, E.
Format Journal Article
LanguageEnglish
Published Dordrecht Springer Netherlands 01.12.2009
Springer Nature B.V
BMC
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Summary:Background: Hereditary amyloidosis are late-onset autosomal dominant disorders characterized by amyloid deposition in various tissues. Among them, Apolipoprotein A-I amyloidosis (Leu75Pro) is a rare autosomal dominant condition in which renal, hepatic, and testicular involvement has been demonstrated.No data are available about vascular alterations in this condition.Aim of the study was to evaluate arterial stiffness,assessed by pulse wave velocity (PWV)and carotid artery intima-media thickness (IMT),evaluated by ultrasound, in patients with Apolipoprotein A-I amyloidosis (APO AI). Patients: In 104 patients with APO AI(mean age 52 ± 16 years,56 F) and in 104 subjects matched for age, sex, body mass index (BMI) and blood pressure (BP), PWVand IMTwere measured. Results: By definition no differences for age, sex, BMI, BP, heart rate were observed. PWV was significantly higher in patients with APO AI than controls (11.5 ± 2.9 and 10.7 ± 2.3,p< 0.05), even after adjusting for cholesterol, creatinine, mean BP and heart rate measured during PWV assessment. In patients with APO AI the prevalence of increased arterial stiffness (defined as PWV> 12 m/sec) was significantly greater than in controls (31% vs 17%, p < 0.05). Mean common,bifurcation and internal carotid artery IMT were comparable in the two groups(0.87 ± 0.21 vs0.88 ± 0.17;1.23 ± 0.41 vs 1.25 ± 0.38;0.95 ± 0.33 vs 0.95 ± 0.28 respectively for APO AI vs controls,p = ns). Similar results were obtained for MeanMax IMT and TMax (1.02 ± 0.29 vs 1.03 ± 0.26 and 1.60 ± 0.69 vs 1.56 ± 0.58 p = ns). Conclusion: In patients with Apolipoprotein A-I amyloidosis (Leu75Pro)a significant increase in arterial stiffness is observed,on the contrary carotid artery IMT is comparable to that of matched control subjects.These results may add significant information to the clinical features of this rare genetic disorder.
ISSN:1872-9312
1876-4401
1876-4401
DOI:10.1016/j.artres.2009.10.176