Publications by authors named "Manon Aurelle"

Article Synopsis
  • The study investigates mineral bone disorder in patients with chronic kidney disease after kidney transplantation, focusing on bone biomarkers and microarchitecture changes before and 6 months after the surgery.
  • A subgroup of patients aged 10 to 18 who underwent their first kidney transplant was compared to healthy controls, revealing initially higher bone densities but notable declines in trabecular microarchitecture at the radius six months post-transplant.
  • Despite some improvements in bone health, many patients persisted with metabolic issues like acidosis and elevated parathyroid hormone levels after the transplant, indicating ongoing bone metabolism concerns.
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Kidney transplantation from living donors is particularly under-developed in France in comparison with the US and most European countries. Among others, the lack of a proactive and evidence-based communication from French health providers is a potential cause that has been overlooked thus far. With this as a backdrop, the SFNDT Commission of transplantation has elaborated a 10 points-call for promoting living kidney transplantation in France in 2023 with the aims at (1) providing the entire nephrology community with a scientific rationale and (2) strenghtening the conviction of health providers, patients, and their relatives regarding the relevance of this modality of kidney transplantation.

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Article Synopsis
  • Primary Coenzyme Q10 (CoQ) deficiency is an ultra-rare genetic disorder that often results in nephrotic syndrome and is linked to mutations in specific genes.
  • A global study of 116 patients showed that oral CoQ supplementation can lead to a significant reduction in proteinuria (by 88% at 12 months) and better preservation of kidney function over time.
  • The findings suggest that all patients with primary CoQ deficiency should receive early and long-term CoQ supplementation to slow kidney disease progression and protect other organs from damage, with few mild side effects reported.
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Primary Coenzyme Q10 deficiency is a rare mitochondriopathy with a wide spectrum of organ involvement, including steroid-resistant nephrotic syndrome mainly associated with disease-causing variants in the genes COQ2, COQ6 or COQ8B. We performed a systematic literature review, PodoNet, mitoNET, and CCGKDD registries queries and an online survey, collecting comprehensive clinical and genetic data of 251 patients spanning 173 published (47 updated) and 78 new cases. Kidney disease was first diagnosed at median age 1.

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Background: The assessment of phosphate homeostasis in clinical practice relies not only on circulating phosphate levels but also on phosphate tubular reabsorption, ideally assessed using the tubular maximum phosphate reabsorption per glomerular filtration rate (TmP/GFR). TmP/GFR reference values were established before the onset of isotope-dilution mass spectrometry-standardized (IDMS) creatinine assays and thus need to be updated. Our objective is to provide reference values for TmP/GFR from childhood to adulthood, using the gold-standard of GFR assessment and IDMS-standardized creatinine values.

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Vitamin D deficiency is frequent in pediatric nephrology. The 2017 European guidelines recommend keeping 25OH vitamin D (25-D) levels within the 75-120 nmol/L range, ideally with daily supplementation. Intermittent supplementation with D3 has also been proposed.

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