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01-02-2019 | Original Article

Skin microvascular dysfunction as an early cardiovascular marker in primary hyperoxaluria type I

Authors: Alexandra Bruel, Justine Bacchetta, Tiphanie Ginhoux, Christelle Rodier-Bonifas, Anne-Laure Sellier-Leclerc, Bérengère Fromy, Pierre Cochat, Dominique Sigaudo-Roussel, Laurence Dubourg

Published in: Pediatric Nephrology | Issue 2/2019

Abstract

Background

Primary hyperoxaluria type 1 (PH1) is an orphan inborn error of oxalate metabolism leading to hyperoxaluria, progressive renal failure, oxalate deposition, and increased cardiovascular complications. As endothelial dysfunction and arterial stiffness are early markers of cardiovascular risk, we investigated early endothelial and vascular dysfunction in young PH1 patients either under conservative treatment (PH1-Cons) or after combined kidney liver transplantation (PH1-T) in comparison to healthy controls (Cont-H) and patients with a past of renal transplantation (Cont-T).

Methods

Skin microvascular function was non-invasively assessed by laser Doppler flowmetry before and after stimulation by current, thermal, or pharmacological (nitroprussiate (SNP) or acetylcholine (Ach)) stimuli in young PH1 patients and controls.

Results

Seven PH1-Cons (6 F, median age 18.2) and 6 PH1-T (2 F, median age 13.3) were compared to 96 Cont-H (51 F, median age 14.2) and 6 Cont-T (4 F, median age 14.5). The endothelium-independent vasodilatation (SNP) was severely decreased in PH1-T compared to Cont-H. Ach, current-induced vasodilatation (CIV), and thermal response was increased in PH1-Cons and Cont-T compared to controls.

Conclusions

PH1-T patients displayed severely decreased smooth muscle capacity to vasodilate. An exacerbated endothelial-dependent vasodilation suggests a role for silent inflammation in the early dysfunction of microcirculation observed in PH1-Cons and Cont-T.

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Title: Skin microvascular dysfunction as an early cardiovascular marker in primary hyperoxaluria type I
Authors: Alexandra Bruel
Justine Bacchetta
Tiphanie Ginhoux
Christelle Rodier-Bonifas
Anne-Laure Sellier-Leclerc
Bérengère Fromy
Pierre Cochat
Dominique Sigaudo-Roussel
Laurence Dubourg
Publication date: 01-02-2019
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Nephrology / Issue 2/2019
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-018-4081-5

At a glance: The STEP trials

Springer Medicine

Skin microvascular dysfunction as an early cardiovascular marker in primary hyperoxaluria type I

Abstract

Background

Methods

Results

Conclusions

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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