Non invasive evaluation of endothelial function in patients with Anderson-Fabry disease

Giuseppe Pistone, Maria Rita Bongiorno, Salvatore Novo, Egle Corrado, Novo, Danilo Puccio, Giuseppe Coppola, Ida Maria Muratori, Mario Arico'

Risultato della ricerca: Article

12 Citazioni (Scopus)

Abstract

AIM: Fabry's disease is an X-linked recessive abnormality of glycosphingolipid metabolism. Increased levels of endothelial prothrombotic factors have recently been demonstrated in Fabry's disease, whereas endothelial function has not been studied using high resolution ultrasound.METHODS: We enrolled 6 patients (4 male, 2 female; mean age, 37 years) and 12 sex matched control subjects (mean age, 37 years). Patients' exclusion criteria included a prior history of cardiac disease, diabetes and treated or untreated hypertension. Patients underwent: anamnesis, physical examination, EKG, 2-dimensional echocardiography with tissue Doppler, measurement of body weight and height, blood pressure. Biochemistry variables were also considered: fasting blood sugar, total cholesterol, HDL-C, LDL-C, triglycerides, fibrinogen, C reactive protein and homocysteine. Using high resolution ultrasound, we assessed the brachial vasodilator response to reactive hyperemia (endothelium-dependent) and sublingual nitroglycerin (NTG) (endothelium-independent). Flow-mediated dilatation (FMD) was expressed as percentage change in post-stimulus diameter in comparison with the baseline.RESULTS: In baseline condition, there was no significant difference between patients and controls in the brachial artery diameter (3.5+/-0.55 vs 3.1+/-0.4). After reactive hyperemia, the FMD change was significantly higher in controls than in patients (16.5+/-6.3% vs 9.3+/-6.2%, P<0.05). After NTG, endothelium-independent vasodilation did not show a significant difference between cases and controls (15+/-7.7% vs 13.8+/-7.1%).CONCLUSIONS: Our study demonstrated the presence of endothelial dysfunction in patients with Fabry's disease in comparison to controls. We hypothesized that endothelial dysfunction may contribute to the pathogenesis of ischemic events in patients with Fabry's disease.
Lingua originaleEnglish
pagine (da-a)295-299
Numero di pagine5
RivistaINTERNATIONAL ANGIOLOGY
Volume24
Stato di pubblicazionePublished - 2005

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Fabry Disease
Endothelium
Hyperemia
Nitroglycerin
Dilatation
Glycosphingolipids
Body Height
Brachial Artery
Homocysteine
Vasodilator Agents
Vasodilation
Biochemistry
C-Reactive Protein
Fibrinogen
HDL Cholesterol
Physical Examination
Echocardiography
Blood Glucose
Heart Diseases
Fasting

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine

Cita questo

Non invasive evaluation of endothelial function in patients with Anderson-Fabry disease. / Pistone, Giuseppe; Bongiorno, Maria Rita; Novo, Salvatore; Corrado, Egle; Novo; Puccio, Danilo; Coppola, Giuseppe; Muratori, Ida Maria; Arico', Mario.

In: INTERNATIONAL ANGIOLOGY, Vol. 24, 2005, pag. 295-299.

Risultato della ricerca: Article

Pistone, Giuseppe ; Bongiorno, Maria Rita ; Novo, Salvatore ; Corrado, Egle ; Novo ; Puccio, Danilo ; Coppola, Giuseppe ; Muratori, Ida Maria ; Arico', Mario. / Non invasive evaluation of endothelial function in patients with Anderson-Fabry disease. In: INTERNATIONAL ANGIOLOGY. 2005 ; Vol. 24. pagg. 295-299.
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abstract = "AIM: Fabry's disease is an X-linked recessive abnormality of glycosphingolipid metabolism. Increased levels of endothelial prothrombotic factors have recently been demonstrated in Fabry's disease, whereas endothelial function has not been studied using high resolution ultrasound.METHODS: We enrolled 6 patients (4 male, 2 female; mean age, 37 years) and 12 sex matched control subjects (mean age, 37 years). Patients' exclusion criteria included a prior history of cardiac disease, diabetes and treated or untreated hypertension. Patients underwent: anamnesis, physical examination, EKG, 2-dimensional echocardiography with tissue Doppler, measurement of body weight and height, blood pressure. Biochemistry variables were also considered: fasting blood sugar, total cholesterol, HDL-C, LDL-C, triglycerides, fibrinogen, C reactive protein and homocysteine. Using high resolution ultrasound, we assessed the brachial vasodilator response to reactive hyperemia (endothelium-dependent) and sublingual nitroglycerin (NTG) (endothelium-independent). Flow-mediated dilatation (FMD) was expressed as percentage change in post-stimulus diameter in comparison with the baseline.RESULTS: In baseline condition, there was no significant difference between patients and controls in the brachial artery diameter (3.5+/-0.55 vs 3.1+/-0.4). After reactive hyperemia, the FMD change was significantly higher in controls than in patients (16.5+/-6.3{\%} vs 9.3+/-6.2{\%}, P<0.05). After NTG, endothelium-independent vasodilation did not show a significant difference between cases and controls (15+/-7.7{\%} vs 13.8+/-7.1{\%}).CONCLUSIONS: Our study demonstrated the presence of endothelial dysfunction in patients with Fabry's disease in comparison to controls. We hypothesized that endothelial dysfunction may contribute to the pathogenesis of ischemic events in patients with Fabry's disease.",
author = "Giuseppe Pistone and Bongiorno, {Maria Rita} and Salvatore Novo and Egle Corrado and Novo and Danilo Puccio and Giuseppe Coppola and Muratori, {Ida Maria} and Mario Arico'",
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T1 - Non invasive evaluation of endothelial function in patients with Anderson-Fabry disease

AU - Pistone, Giuseppe

AU - Bongiorno, Maria Rita

AU - Novo, Salvatore

AU - Corrado, Egle

AU - Novo, null

AU - Puccio, Danilo

AU - Coppola, Giuseppe

AU - Muratori, Ida Maria

AU - Arico', Mario

PY - 2005

Y1 - 2005

N2 - AIM: Fabry's disease is an X-linked recessive abnormality of glycosphingolipid metabolism. Increased levels of endothelial prothrombotic factors have recently been demonstrated in Fabry's disease, whereas endothelial function has not been studied using high resolution ultrasound.METHODS: We enrolled 6 patients (4 male, 2 female; mean age, 37 years) and 12 sex matched control subjects (mean age, 37 years). Patients' exclusion criteria included a prior history of cardiac disease, diabetes and treated or untreated hypertension. Patients underwent: anamnesis, physical examination, EKG, 2-dimensional echocardiography with tissue Doppler, measurement of body weight and height, blood pressure. Biochemistry variables were also considered: fasting blood sugar, total cholesterol, HDL-C, LDL-C, triglycerides, fibrinogen, C reactive protein and homocysteine. Using high resolution ultrasound, we assessed the brachial vasodilator response to reactive hyperemia (endothelium-dependent) and sublingual nitroglycerin (NTG) (endothelium-independent). Flow-mediated dilatation (FMD) was expressed as percentage change in post-stimulus diameter in comparison with the baseline.RESULTS: In baseline condition, there was no significant difference between patients and controls in the brachial artery diameter (3.5+/-0.55 vs 3.1+/-0.4). After reactive hyperemia, the FMD change was significantly higher in controls than in patients (16.5+/-6.3% vs 9.3+/-6.2%, P<0.05). After NTG, endothelium-independent vasodilation did not show a significant difference between cases and controls (15+/-7.7% vs 13.8+/-7.1%).CONCLUSIONS: Our study demonstrated the presence of endothelial dysfunction in patients with Fabry's disease in comparison to controls. We hypothesized that endothelial dysfunction may contribute to the pathogenesis of ischemic events in patients with Fabry's disease.

AB - AIM: Fabry's disease is an X-linked recessive abnormality of glycosphingolipid metabolism. Increased levels of endothelial prothrombotic factors have recently been demonstrated in Fabry's disease, whereas endothelial function has not been studied using high resolution ultrasound.METHODS: We enrolled 6 patients (4 male, 2 female; mean age, 37 years) and 12 sex matched control subjects (mean age, 37 years). Patients' exclusion criteria included a prior history of cardiac disease, diabetes and treated or untreated hypertension. Patients underwent: anamnesis, physical examination, EKG, 2-dimensional echocardiography with tissue Doppler, measurement of body weight and height, blood pressure. Biochemistry variables were also considered: fasting blood sugar, total cholesterol, HDL-C, LDL-C, triglycerides, fibrinogen, C reactive protein and homocysteine. Using high resolution ultrasound, we assessed the brachial vasodilator response to reactive hyperemia (endothelium-dependent) and sublingual nitroglycerin (NTG) (endothelium-independent). Flow-mediated dilatation (FMD) was expressed as percentage change in post-stimulus diameter in comparison with the baseline.RESULTS: In baseline condition, there was no significant difference between patients and controls in the brachial artery diameter (3.5+/-0.55 vs 3.1+/-0.4). After reactive hyperemia, the FMD change was significantly higher in controls than in patients (16.5+/-6.3% vs 9.3+/-6.2%, P<0.05). After NTG, endothelium-independent vasodilation did not show a significant difference between cases and controls (15+/-7.7% vs 13.8+/-7.1%).CONCLUSIONS: Our study demonstrated the presence of endothelial dysfunction in patients with Fabry's disease in comparison to controls. We hypothesized that endothelial dysfunction may contribute to the pathogenesis of ischemic events in patients with Fabry's disease.

UR - http://hdl.handle.net/10447/15100

M3 - Article

VL - 24

SP - 295

EP - 299

JO - INTERNATIONAL ANGIOLOGY

JF - INTERNATIONAL ANGIOLOGY

SN - 0392-9590

ER -