Pediatric Nephrology

, Volume 34, Issue 2, pp 319–327 | Cite as

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

  • Alexandra Bruel
  • Justine Bacchetta
  • Tiphanie Ginhoux
  • Christelle Rodier-Bonifas
  • Anne-Laure Sellier-Leclerc
  • Bérengère Fromy
  • Pierre Cochat
  • Dominique Sigaudo-Roussel
  • Laurence DubourgEmail author
Original Article



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).


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.


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.


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.


Hyperoxaluria type I Microcirculation Cardiovascular risk Renal transplantation Endothelium 


Compliance with ethical standards

The Micro-Oxa study was approved by the local IRB (Comité de Protection des Personnes Lyon Sud-Est II, initial approval March 22, 2014, addendum approval April 29, 2015). Patients (PH1 and Cont-T) received oral and written information, and oral non-opposition consent of the patient (and parents for children) was collected.

Conflicts of interest

The authors declare no conflict of interest.


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Copyright information

© IPNA 2018

Authors and Affiliations

  • Alexandra Bruel
    • 1
    • 2
  • Justine Bacchetta
    • 1
    • 3
  • Tiphanie Ginhoux
    • 4
  • Christelle Rodier-Bonifas
    • 5
  • Anne-Laure Sellier-Leclerc
    • 1
  • Bérengère Fromy
    • 6
  • Pierre Cochat
    • 1
    • 3
    • 6
  • Dominique Sigaudo-Roussel
    • 6
  • Laurence Dubourg
    • 1
    • 3
    • 6
    • 7
    Email author
  1. 1.Centre de Référence des Maladies Rénales Rares, Service de Néphrologie et Rhumatologie PédiatriquesHospices Civils de LyonLyonFrance
  2. 2.Service de Pédiatrie, Hôpital Mère et EnfantsCentre hospitalo-universitaire de NantesNantesFrance
  3. 3.Université Claude Bernard Lyon 1LyonFrance
  4. 4.EPICIME-CIC 1407 de Lyon, Inserm, Service de Pharmacologie Clinique, CHU-LyonLyonFrance
  5. 5.Service d’ophtalmologie, Groupement Hospitalier Edouard HerriotHospices Civils de LyonLyonFrance
  6. 6.Laboratory of Tissue Biology and Therapeutic Engineering, UMR 5305 CNRSUniversity Claude Bernard Lyon 1VilleurbanneFrance
  7. 7.Néphrologie, Dialyse, Hypertension et Exploration Fonctionnelle Rénale, Groupement Hospitalier Edouard HerriotHospices Civils de LyonLyonFrance

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