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  1. Article ; Online: Levamisole in childhood idiopathic nephrotic syndrome: new promises, and advocacy for global access.

    Chan, Eugene Yu-Hin / Boyer, Olivia

    Kidney international

    2024  Volume 105, Issue 5, Page(s) 932–934

    Abstract: In the current issue of Kidney International, Sinha et al. present data from an open-label, noninferior, randomized controlled trial comparing 12-months of alternate-day prednisolone, given daily during infection, versus levamisole, in children with ... ...

    Abstract In the current issue of Kidney International, Sinha et al. present data from an open-label, noninferior, randomized controlled trial comparing 12-months of alternate-day prednisolone, given daily during infection, versus levamisole, in children with frequently relapsing or steroid-dependent nephrotic syndrome. This study suggests that both of these strategies are efficacious and safe. Results of this study should redefine the role of levamisole in future guidelines, and a call for global availability of levamisole should be advocated.
    MeSH term(s) Child ; Humans ; Levamisole/adverse effects ; Nephrotic Syndrome/drug therapy ; Prednisolone ; Glucocorticoids ; Recurrence
    Chemical Substances Levamisole (2880D3468G) ; Prednisolone (9PHQ9Y1OLM) ; Glucocorticoids
    Language English
    Publishing date 2024-02-22
    Publishing country United States
    Document type Journal Article
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1016/j.kint.2024.01.030
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Is ABO Incompatible Living Donor Kidney Transplantation in Children a Better Option than the Use of Optimal Grafts From Deceased Donors? A Plea for Better Prioritization of Deceased Kidney Grafts for Children.

    Boyer, Olivia / Pape, Lars

    Transplant international : official journal of the European Society for Organ Transplantation

    2023  Volume 36, Page(s) 11911

    MeSH term(s) Humans ; Child ; Kidney Transplantation ; Living Donors ; Kidney ; Blood Group Incompatibility ; ABO Blood-Group System ; Graft Survival ; Graft Rejection
    Chemical Substances ABO Blood-Group System
    Language English
    Publishing date 2023-09-18
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 639435-8
    ISSN 1432-2277 ; 0934-0874
    ISSN (online) 1432-2277
    ISSN 0934-0874
    DOI 10.3389/ti.2023.11911
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  3. Article ; Online: Le mot des coordinateurs : La néphrologie pédiatrique.

    Dorval, Guillaume / Boyer, Olivia

    Medecine sciences : M/S

    2023  Volume 39, Issue 3, Page(s) 205

    Title translation Pediatric nephrology.
    MeSH term(s) Child ; Humans ; Nephrology ; Surveys and Questionnaires ; Attitude of Health Personnel
    Language French
    Publishing date 2023-03-21
    Publishing country France
    Document type Journal Article
    ZDB-ID 632733-3
    ISSN 1958-5381 ; 0767-0974
    ISSN (online) 1958-5381
    ISSN 0767-0974
    DOI 10.1051/medsci/2023026
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  4. Article ; Online: Multipopulation genome-wide association meta-analysis in pediatric steroid-sensitive nephrotic syndrome.

    Boyer, Olivia / Dorval, Guillaume

    Kidney international

    2023  Volume 105, Issue 1, Page(s) 14–17

    MeSH term(s) Humans ; Child ; Nephrotic Syndrome/diagnosis ; Nephrotic Syndrome/drug therapy ; Nephrotic Syndrome/genetics ; Genome-Wide Association Study ; Genetic Predisposition to Disease
    Language English
    Publishing date 2023-09-13
    Publishing country United States
    Document type Meta-Analysis ; Journal Article
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1016/j.kint.2023.08.022
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  5. Article ; Online: Hemolytic-Uremic Syndrome in Children.

    Boyer, Olivia / Niaudet, Patrick

    Pediatric clinics of North America

    2022  Volume 69, Issue 6, Page(s) 1181–1197

    Abstract: Hemolytic uremic syndrome is characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. Most cases are caused by Shiga-toxin-producing bacteria, especially Escherichia coli. Transmission occurs through ... ...

    Abstract Hemolytic uremic syndrome is characterized by a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. Most cases are caused by Shiga-toxin-producing bacteria, especially Escherichia coli. Transmission occurs through ground beef and unpasteurized milk. STEC-HUS is the main cause of acute renal failure in children. Management remains supportive. Immediate outcome is most often. Atypical HUS represents about 5% of cases, has a relapsing course with more than half of the patients progressing to end-stage kidney failure. Most cases are due to variants in complement regulators of the alternative pathway. Complement inhibitors, such as eculizumab, have considerably improved the prognosis.
    MeSH term(s) Cattle ; Animals ; Humans ; Child ; Hemolytic-Uremic Syndrome/diagnosis ; Hemolytic-Uremic Syndrome/therapy ; Kidney Failure, Chronic ; Thrombocytopenia
    Language English
    Publishing date 2022-10-29
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 215711-1
    ISSN 1557-8240 ; 0031-3955
    ISSN (online) 1557-8240
    ISSN 0031-3955
    DOI 10.1016/j.pcl.2022.07.006
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  6. Article ; Online: Is withdrawal of nocturnal hyperhydration possible in children with primary hyperoxaluria treated with RNAi?

    Biebuyck, Nathalie / Destombes, Camille / Prakash, Richa / Boyer, Olivia

    Journal of nephrology

    2023  Volume 36, Issue 5, Page(s) 1473–1476

    Abstract: Primary hyperoxaluria type 1 is a rare genetic disorder caused by bi-allelic pathogenic variants in the AGXT gene leading to an overproduction of oxalate which accumulates in the kidneys in the form of calcium oxalate crystals. Thus, patients may present ...

    Abstract Primary hyperoxaluria type 1 is a rare genetic disorder caused by bi-allelic pathogenic variants in the AGXT gene leading to an overproduction of oxalate which accumulates in the kidneys in the form of calcium oxalate crystals. Thus, patients may present with recurrent nephrocalcinosis and lithiasis, with progressive impairment of the  renal function and eventually kidney failure.  There is no specific treatment besides liver-kidney transplantation, and pre-transplantation management by 24 h-hyperhydration, crystallisation inhibitors and high-dose pyridoxine has a high negative impact on quality of life, especially because of the discomfort due to nocturnal hyperhydration. Since 2020, lumasiran, an RNA-interfering therapy, has been approved for the treatment of primary hyperoxaluria type 1 in adults and children. However, to date, there are no recommendations regarding the discontinuation of other supportive measures during RNAi therapy. In this report, we present two patients with primary hyperoxaluria type 1 who were treated with lumasiran and stopped nocturnal hyperhydration with positive outcomes, i.e. normal urinary oxalate, absence of crystalluria, stable kidney function and improved well-being. These data suggest that discontinuing nocturnal hydration may be safe in children responding to lumasiran, and may have a positive impact on their quality of life. Additional data are needed to update treatment recommendations.
    MeSH term(s) Adult ; Humans ; Child ; Hyperoxaluria, Primary/genetics ; Hyperoxaluria, Primary/therapy ; Hyperoxaluria, Primary/urine ; RNA Interference ; Quality of Life ; Water Intoxication/genetics ; Oxalates
    Chemical Substances Oxalates
    Language English
    Publishing date 2023-05-20
    Publishing country Italy
    Document type Journal Article
    ZDB-ID 1093991-x
    ISSN 1724-6059 ; 1120-3625 ; 1121-8428
    ISSN (online) 1724-6059
    ISSN 1120-3625 ; 1121-8428
    DOI 10.1007/s40620-023-01611-1
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    Zs.A 3369: Show issues Location:
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  7. Article ; Online: Atteinte neurologique et syndrome néphrotique cortico-résistant - Des chevauchements troublants.

    Boyer, Olivia / Mollet, Géraldine / Dorval, Guillaume

    Medecine sciences : M/S

    2023  Volume 39, Issue 3, Page(s) 246–252

    Abstract: Genetic studies of hereditary steroid resistant nephrotic syndrome (SRNS) have identified more than 60 genes involved in the development of single-gene, isolated or syndromic forms of hereditary podocytoapthies. Sometimes, syndromic SRNS is associated ... ...

    Title translation Neurological disorders and hereditary podocytopathies: Some fascinating pathophysiological overlaps.
    Abstract Genetic studies of hereditary steroid resistant nephrotic syndrome (SRNS) have identified more than 60 genes involved in the development of single-gene, isolated or syndromic forms of hereditary podocytoapthies. Sometimes, syndromic SRNS is associated with neurological disorders. Over the past decades, various studies have established links between the podocyte, an epithelial glomerular cell involved in the renal filtration barrier, and neuronal cells, both morphologically (slit diaphragm and synapse) and functionally (signaling platforms). Variants of genes encoding proteins expressed in different compartments of the podocyte and neurons are responsible for phenotypes associating renal lesions with proteinuria to central and/or peripheral neurological disorders. In this review, we aim to focus on genetic syndromes associating proteinuria and neurological disease and to present the latest advances in the description of these neuro-renal disorders.
    MeSH term(s) Humans ; Nephrotic Syndrome/genetics ; Kidney Glomerulus/pathology ; Kidney/pathology ; Proteinuria ; Nervous System Diseases/genetics
    Language French
    Publishing date 2023-03-21
    Publishing country France
    Document type Review ; English Abstract ; Journal Article
    ZDB-ID 632733-3
    ISSN 1958-5381 ; 0767-0974
    ISSN (online) 1958-5381
    ISSN 0767-0974
    DOI 10.1051/medsci/2023029
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  8. Article ; Online: Childhood nephrotic syndrome.

    Vivarelli, Marina / Gibson, Keisha / Sinha, Aditi / Boyer, Olivia

    Lancet (London, England)

    2023  Volume 402, Issue 10404, Page(s) 809–824

    Abstract: Idiopathic nephrotic syndrome is the most common glomerular disease in children. Corticosteroids are the cornerstone of its treatment, and steroid response is the main prognostic factor. Most children respond to a cycle of oral steroids, and are defined ... ...

    Abstract Idiopathic nephrotic syndrome is the most common glomerular disease in children. Corticosteroids are the cornerstone of its treatment, and steroid response is the main prognostic factor. Most children respond to a cycle of oral steroids, and are defined as having steroid-sensitive nephrotic syndrome. Among the children who do not respond, defined as having steroid-resistant nephrotic syndrome, most respond to second-line immunosuppression, mainly with calcineurin inhibitors, and children in whom a response is not observed are described as multidrug resistant. The pathophysiology of nephrotic syndrome remains elusive. In cases of immune-mediated origin, dysregulation of immune cells and production of circulating factors that damage the glomerular filtration barrier have been described. Conversely, up to a third of cases of steroid-resistant nephrotic syndrome have a monogenic origin. Multidrug resistant nephrotic syndrome often leads to kidney failure and can cause relapse after kidney transplant. Although steroid-sensitive nephrotic syndrome does not affect renal function, most children with steroid-sensitive nephrotic syndrome have a relapsing course that requires repeated steroid cycles with significant side-effects. To minimise morbidity, some patients require steroid-sparing immunosuppressive agents, including levamisole, mycophenolate mofetil, calcineurin inhibitors, anti-CD20 monoclonal antibodies, and cyclophosphamide. Close monitoring and preventive measures are warranted at onset and during relapse to prevent acute complications (eg, hypovolaemia, acute kidney injury, infections, and thrombosis), whereas long-term management requires minimising treatment-related side-effects. A subset of patients have active disease into adulthood.
    MeSH term(s) Child ; Humans ; Nephrotic Syndrome/drug therapy ; Calcineurin Inhibitors/therapeutic use ; Cyclophosphamide ; Immunosuppressive Agents/therapeutic use ; Acute Kidney Injury ; Drug-Related Side Effects and Adverse Reactions
    Chemical Substances Calcineurin Inhibitors ; Cyclophosphamide (8N3DW7272P) ; Immunosuppressive Agents
    Language English
    Publishing date 2023-08-31
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 3306-6
    ISSN 1474-547X ; 0023-7507 ; 0140-6736
    ISSN (online) 1474-547X
    ISSN 0023-7507 ; 0140-6736
    DOI 10.1016/S0140-6736(23)01051-6
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  9. Article ; Online: The rationale for nephron-sparing surgery in unilateral non-syndromic Wilms tumour.

    Taghavi, Kiarash / Sarnacki, Sabine / Blanc, Thomas / Boyer, Olivia / Heloury, Yves

    Pediatric nephrology (Berlin, Germany)

    2023  Volume 39, Issue 4, Page(s) 1023–1032

    Abstract: The central question of nephron-sparing surgery in unilateral non-syndromic Wilms tumour sits at a crossroads between surgery, oncology, and nephrology. There has been a significant paradigm shift in paediatric oncology towards reducing toxicity and ... ...

    Abstract The central question of nephron-sparing surgery in unilateral non-syndromic Wilms tumour sits at a crossroads between surgery, oncology, and nephrology. There has been a significant paradigm shift in paediatric oncology towards reducing toxicity and addressing long-term treatment-related sequalae amongst childhood cancer survivors. After paediatric nephrectomy and 30-50 years of follow-up, 40% of patients will have chronic kidney disease, including 22% with hypertension and 23% with albuminuria. It is difficult to predict which patients will progress to develop hypertension, reduced glomerular filtration rate, albuminuria, and a higher cardiovascular risk. For these reasons, nephron-sparing surgery when it is technically feasible must be considered. To decrease the incidence of positive surgical margins (viable tumour present at a resection margin), incomplete lymph node sampling, and complications, these procedures should be performed at specialist and experienced reference centres. Based on the impacts of individual treatment pathways, survivors of childhood WT need to be followed through adulthood for early detection of chronic kidney disease, hypertension, and prevention of cardiovascular events.
    MeSH term(s) Humans ; Child ; Kidney Neoplasms/pathology ; Albuminuria ; Wilms Tumor/pathology ; Nephrectomy/adverse effects ; Nephrectomy/methods ; Hypertension/etiology ; Hypertension/surgery ; Renal Insufficiency, Chronic/surgery ; Nephrons/pathology ; Retrospective Studies
    Language English
    Publishing date 2023-08-21
    Publishing country Germany
    Document type Journal Article ; Review
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-023-06099-2
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  10. Article ; Online: ANCA-associated vasculitis in children.

    Bernardi, Silvia / Seugé, Laure / Boyer, Olivia

    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association

    2022  Volume 38, Issue 1, Page(s) 66–69

    MeSH term(s) Humans ; Child ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ; Antibodies, Antineutrophil Cytoplasmic ; Peroxidase
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic ; Peroxidase (EC 1.11.1.7)
    Language English
    Publishing date 2022-09-27
    Publishing country England
    Document type Journal Article
    ZDB-ID 90594-x
    ISSN 1460-2385 ; 0931-0509
    ISSN (online) 1460-2385
    ISSN 0931-0509
    DOI 10.1093/ndt/gfac265
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