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  1. Article ; Online: Sodium-not harmful?

    Deschênes, Georges

    Pediatric nephrology (Berlin, Germany)

    2020  Volume 35, Issue 9, Page(s) 1771–1776

    Abstract: Background: The temporality between the mandated reduction of salt in processed food and the decrease of death from stroke and ischemic heart disease, the association of hypertension, and cardiovascular disease led many public health organizations to ... ...

    Abstract Background: The temporality between the mandated reduction of salt in processed food and the decrease of death from stroke and ischemic heart disease, the association of hypertension, and cardiovascular disease led many public health organizations to recommend reducing dietary sodium to a maximum of 2300 mg per day. It turns out that some nuances can be brought about to this universally shared belief.
    Methods & results: Indeed, consideration of health outcomes instead of only blood pressure as a surrogate marker of cardiovascular disease and prognosis gave contradictory results whereas low sodium intake is associated to an excess of death and cardiovascular events.
    Conclusions: Accordingly, sodium intake should be adapted to individual risk factors, and evidence is still clearly lacking to support indiscriminate recommendations in healthy people. By contrast, a restricted sodium diet is certainly useful in patients with chronic kidney disease exposed to salt retention, and by reciprocity, low sodium diet must be absolutely avoided in all patients presenting renal or extra renal sodium wasting where sodium depletion is a life-threatening condition.
    Language English
    Publishing date 2020-05-21
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-019-04356-x
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: How to improve response to rituximab treatment in children with steroid-dependent nephrotic syndrome: answer to Drs. Fujinaga and Nishino.

    Hogan, Julien / Deschenes, Georges

    Pediatric nephrology (Berlin, Germany)

    2018  Volume 34, Issue 2, Page(s) 361–362

    MeSH term(s) B-Lymphocytes ; Child ; Humans ; Nephrotic Syndrome ; Recurrence ; Rituximab
    Chemical Substances Rituximab (4F4X42SYQ6)
    Language English
    Publishing date 2018-11-07
    Publishing country Germany
    Document type Letter ; Comment
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-018-4133-x
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Obinutuzumab in Frequently Relapsing and Steroid-Dependent Nephrotic Syndrome in Children.

    Dossier, Claire / Bonneric, Stéphanie / Baudouin, Véronique / Kwon, Thérésa / Prim, Benjamin / Cambier, Alexandra / Couderc, Anne / Moreau, Christelle / Deschenes, Georges / Hogan, Julien

    Clinical journal of the American Society of Nephrology : CJASN

    2023  Volume 18, Issue 12, Page(s) 1555–1562

    Abstract: Background: B-cell depletion with rituximab induces sustained remission in children with steroid-dependent or frequently relapsing nephrotic syndrome. However, most patients relapse after B-cell recovery, and some patients do not achieve B-cell ... ...

    Abstract Background: B-cell depletion with rituximab induces sustained remission in children with steroid-dependent or frequently relapsing nephrotic syndrome. However, most patients relapse after B-cell recovery, and some patients do not achieve B-cell depletion. Obinutuzumab is a second-generation anti-CD20 antibody designed to overcome such situations in B-cell malignancies and was recently reported to be safe and effective in other autoimmune diseases affecting the kidneys.
    Methods: We retrospectively report 41 children with steroid-dependent or frequently relapsing nephrotic syndrome treated with a single low-dose infusion of obinutuzumab at Robert-Debre Hospital between April 2018 and December 2020. Participants were treated because of rituximab resistance or relapse after rituximab and received a single infusion of 300 mg/1.73 m 2 obinutuzumab with cessation of oral immunosuppressors within 2 months.
    Results: B-cell depletion was achieved in all participants and lasted a median of 8.3 months (interquartile range, 6.4-11.1), a duration exceeding that for last rituximab treatment. At 12 and 24 months, 92% (38/41) and 68% (28/41) of patients, respectively, were in sustained remission. Mild infusion reactions occurred in five participants (12%) and neutropenia in nine (21%). No significant decrease in IgG level was reported during treatment, and whereas IgM levels decreased in 34 patients (83%), they were normal at last follow-up in 32 (78%).
    Conclusions: These results identified low-dose obinituzumab as a promising treatment option in children with steroid-dependent or frequently relapsing nephrotic syndrome, including those resistant to rituximab. The tolerance profile of obinutuzumab was similar to that of rituximab, but hemogram and immunoglobulin levels should be monitored.
    MeSH term(s) Child ; Humans ; Rituximab/adverse effects ; Nephrotic Syndrome/drug therapy ; Retrospective Studies ; Steroids/therapeutic use ; Recurrence ; Immunosuppressive Agents/adverse effects ; Treatment Outcome
    Chemical Substances Rituximab (4F4X42SYQ6) ; obinutuzumab (O43472U9X8) ; Steroids ; Immunosuppressive Agents
    Language English
    Publishing date 2023-09-06
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2226665-3
    ISSN 1555-905X ; 1555-9041
    ISSN (online) 1555-905X
    ISSN 1555-9041
    DOI 10.2215/CJN.0000000000000288
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  4. Article: The Struggling Odyssey of Infantile Primary Hyperoxaluria.

    Guillaume, Adrien / Chiodini, Benedetta / Adams, Brigitte / Dahan, Karin / Deschênes, Georges / Ismaili, Khalid

    Frontiers in pediatrics

    2021  Volume 9, Page(s) 615183

    Abstract: Introduction: ...

    Abstract Introduction:
    Language English
    Publishing date 2021-04-20
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2021.615183
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  5. Article ; Online: Treating the idiopathic nephrotic syndrome: are steroids the answer?

    Deschênes, Georges / Dossier, Claire / Hogan, Julien

    Pediatric nephrology (Berlin, Germany)

    2018  Volume 34, Issue 5, Page(s) 777–785

    Abstract: The use of steroids in idiopathic nephrotic syndrome is the major discovery of the twentieth century in the field of pediatric nephrology. At onset of the twenty-first century, steroids remain the first line of treatment at first flare. All the protocols ...

    Abstract The use of steroids in idiopathic nephrotic syndrome is the major discovery of the twentieth century in the field of pediatric nephrology. At onset of the twenty-first century, steroids remain the first line of treatment at first flare. All the protocols to treat the first flare are similar by a common sequence including a first phase of daily prednisolone/prednisone at a dose of 60 mg/m
    MeSH term(s) Child ; Dose-Response Relationship, Drug ; Drug Administration Schedule ; Drug Therapy, Combination/methods ; Glucocorticoids/administration & dosage ; Humans ; Immunosuppressive Agents/administration & dosage ; Nephrotic Syndrome/drug therapy ; Nephrotic Syndrome/immunology ; Prednisolone/administration & dosage ; Prednisone/administration & dosage ; Recurrence ; Remission Induction/methods ; Secondary Prevention/methods ; Treatment Outcome
    Chemical Substances Glucocorticoids ; Immunosuppressive Agents ; Prednisolone (9PHQ9Y1OLM) ; Prednisone (VB0R961HZT)
    Language English
    Publishing date 2018-06-04
    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/s00467-018-3963-x
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: Asymptomatic bacteriuria in pediatric kidney transplant recipients: to treat or not to treat? A retrospective study.

    Bonnéric, Stéphanie / Maisin, Anne / Kwon, Theresa / Deschênes, Georges / Niel, Olivier

    Pediatric nephrology (Berlin, Germany)

    2019  Volume 34, Issue 6, Page(s) 1141–1145

    Abstract: Background: Urinary tract infections (UTI) are common infectious complications in kidney transplant recipients (KTR); asymptomatic bacteriuria (AB) is also frequent. It is unclear whether treatment of AB reduces subsequent UTI in KTR; no guideline is ... ...

    Abstract Background: Urinary tract infections (UTI) are common infectious complications in kidney transplant recipients (KTR); asymptomatic bacteriuria (AB) is also frequent. It is unclear whether treatment of AB reduces subsequent UTI in KTR; no guideline is available in pediatric KTR. In this retrospective study, we analyzed the incidence of AB in pediatric KTR and the impact of screening and treating AB on the onset of subsequent UTI.
    Methods: Thirty-seven pediatric patients were included. Inclusion criteria were the occurrence of one or more episodes of AB between 2 and 24 months post-renal transplantation. Primary outcome was the cumulative incidence of acute pyelonephritis (APN) or lower urinary tract infections (LUTI) occurring between 2 and 24 months post-renal transplantation.
    Results: Thirty-seven patients presented 171 AB episodes. One hundred sixty-four AB episodes were untreated (95.9%); among them, 150 episodes (91.5%) were not followed by a clinical infection. Ten episodes (6.1%) led to APN, and 4 (2.4%) to LUTI. There were 53 episodes of APN: 10 (18.9%) after untreated AB and 43 (81.1%) de novo. There were 11 episodes of LUTI: 4 (36.4%) after untreated AB and 7 (63.6%) de novo. Multi-drug resistant bacteria were present in 27% of the patients and in 20% of patients with pre-existing uropathy.
    Conclusions: Our results are not in favor of systematic treatment of AB in pediatric KTR. Notably, limitation of antibiotic treatment is an urgent and important health issue in this population, in order to reduce multi-drug resistant bacteria emergence.
    MeSH term(s) Anti-Bacterial Agents/therapeutic use ; Bacteriuria/drug therapy ; Bacteriuria/epidemiology ; Bacteriuria/etiology ; Child ; Female ; Humans ; Incidence ; Kidney Transplantation/adverse effects ; Male ; Postoperative Complications/drug therapy ; Postoperative Complications/epidemiology ; Pyelonephritis/epidemiology ; Retrospective Studies ; Transplant Recipients ; Urinary Tract Infections/epidemiology
    Chemical Substances Anti-Bacterial Agents
    Language English
    Publishing date 2019-02-28
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-019-04204-y
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  7. Article ; Online: Variability of diagnostic criteria and treatment of idiopathic nephrotic syndrome across European countries.

    Deschênes, Georges / Vivarelli, Marina / Peruzzi, Licia

    European journal of pediatrics

    2017  Volume 176, Issue 5, Page(s) 647–654

    Abstract: The aim of the surveys conducted by the Idiopathic Nephrotic Syndrome Working Group of the ESPN was to study the possible variability of treatment in Europe at different stages of the disease by means of questionnaires sent to members of the Working ... ...

    Abstract The aim of the surveys conducted by the Idiopathic Nephrotic Syndrome Working Group of the ESPN was to study the possible variability of treatment in Europe at different stages of the disease by means of questionnaires sent to members of the Working Group. Four surveys have been completed: treatment of the first flare, treatment of the first relapse and the issue of steroid dependency, use of rituximab, and the management of steroid-resistant patients. A uniform treatment of the first flare was applied in only three countries, and ten additional centers have adopted one of the three main protocols. Reported treatment of the first relapse was relatively uniform, whereas the use of additional immunosuppressants in steroid dependency was widely variable. Rituximab had already been used in hundreds of patients, although the formal evidence of efficiency in steroid dependency was relatively recent at the time of the survey. The definition of steroid resistance was variable in the European centers, but strikingly, the first-line treatment was uniform throughout the centers and included the combination of prednisone plus calcineurin antagonists.
    Conclusion: The variability in the approach of idiopathic nephrotic syndrome is unexpectedly large and affects treatment of the first flare, strategies in the case of steroid dependency, as well as the definitions of steroid resistance. What is Known: • Steroids and immunosuppressants are the universal treatment of idiopathic nephrotic syndrome. What is New: • The variability of treatments and strategy of treatment in European centers of pediatric nephrology.
    Language English
    Publishing date 2017-05
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 194196-3
    ISSN 1432-1076 ; 0340-6199 ; 0943-9676
    ISSN (online) 1432-1076
    ISSN 0340-6199 ; 0943-9676
    DOI 10.1007/s00431-017-2891-2
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  8. Article ; Online: Idiopathic nephrotic syndrome: the EBV hypothesis.

    Dossier, Claire / Jamin, Agnès / Deschênes, Georges

    Pediatric research

    2016  Volume 81, Issue 1-2, Page(s) 233–239

    Abstract: Steroid sensitive nephrotic syndrome is marked by a massive proteinuria and loss of podocytes foot processes. The mechanism of the disease remains debated but recent publications suggest a primary role of Epstein-Barr Virus (EBV). EBV replication in the ... ...

    Abstract Steroid sensitive nephrotic syndrome is marked by a massive proteinuria and loss of podocytes foot processes. The mechanism of the disease remains debated but recent publications suggest a primary role of Epstein-Barr Virus (EBV). EBV replication in the peripheral blood is found in 50% of patients during the first flare of the disease. The genetic locus of steroid sensitive nephrotic syndrome was also identified as influencing antibodies directed against EBNA1. EBV is able to establish, latent benign infection in memory B cells that display phenotypes similar to antigen-selected memory B cells. Consistently, memory B cells reconstitution after rituximab infusion is a predictor of the relapse of proteinuria. We suggest that a specific anti-EBNA1 antibody internalized in the podocytes via the neonatal Fc receptor might cross-react with a major protein present in the same cell trafficking compartment. The diversion of this major podocyte protein in the urinary space and the subsequent depletion is supposed to result in podocyte damages with loss of foot processes and massive proteinuria. Immunosuppression of B cells and subsequent clearance of anti-EBNA1 antibodies would lead to a restoration of the normal level of the protein allowing recovery of proteinuria and of normal podocyte morphology.
    MeSH term(s) Adolescent ; B-Lymphocytes/cytology ; Child ; Child, Preschool ; Epstein-Barr Virus Nuclear Antigens/chemistry ; Herpesvirus 4, Human ; Humans ; Immunoglobulins/chemistry ; Immunologic Memory ; Infant ; Kidney Glomerulus/immunology ; Kidney Glomerulus/virology ; Models, Theoretical ; Nephrotic Syndrome/immunology ; Nephrotic Syndrome/virology ; Podocytes/cytology ; Proteinuria/virology ; Steroids/therapeutic use
    Chemical Substances Epstein-Barr Virus Nuclear Antigens ; Immunoglobulins ; Steroids ; EBV-encoded nuclear antigen 1 (O5GA75RST7)
    Language English
    Publishing date 2016-09-28
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 4411-8
    ISSN 1530-0447 ; 0031-3998
    ISSN (online) 1530-0447
    ISSN 0031-3998
    DOI 10.1038/pr.2016.200
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  9. Article ; Online: Steroid therapy in children with IgA nephropathy.

    Cambier, Alexandra / Boyer, Olivia / Deschenes, Georges / Gleeson, James / Couderc, Anne / Hogan, Julien / Robert, Thomas

    Pediatric nephrology (Berlin, Germany)

    2019  Volume 35, Issue 3, Page(s) 359–366

    Abstract: IgA nephropathy (IgAN) is one the most common primary glomerulonephritis in children and adolescents worldwide, with 20% of children developing end-stage kidney disease (ESKD) within 20 years of diagnosis. There is a need for treatment guidelines, ... ...

    Abstract IgA nephropathy (IgAN) is one the most common primary glomerulonephritis in children and adolescents worldwide, with 20% of children developing end-stage kidney disease (ESKD) within 20 years of diagnosis. There is a need for treatment guidelines, especially for steroids in children with primary IgAN, since the STOP-IgA trial casts doubts on the use of steroids in adults with intermediate risk. Pediatricians are prone to prescribe steroids in addition to renin-angiotensin system blockade (RASB) when proteinuria is > 0.5 g/l, eGFR deteriorates < 70 ml/min/1.73 m
    MeSH term(s) Adolescent ; Age Factors ; Biopsy ; Child ; Consensus ; Glomerular Filtration Rate/immunology ; Glomerulonephritis, IGA/complications ; Glomerulonephritis, IGA/drug therapy ; Glomerulonephritis, IGA/immunology ; Glomerulonephritis, IGA/pathology ; Glucocorticoids/therapeutic use ; Humans ; Immunosuppression/adverse effects ; Immunosuppression/methods ; Immunosuppression/standards ; Immunosuppressive Agents/therapeutic use ; Kidney Failure, Chronic/epidemiology ; Kidney Failure, Chronic/immunology ; Kidney Failure, Chronic/pathology ; Kidney Failure, Chronic/prevention & control ; Kidney Glomerulus/immunology ; Kidney Glomerulus/pathology ; Kidney Glomerulus/physiopathology ; Practice Guidelines as Topic ; Treatment Outcome
    Chemical Substances Glucocorticoids ; Immunosuppressive Agents
    Language English
    Publishing date 2019-02-18
    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/s00467-018-4189-7
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  10. Article ; Online: Quality of life in children with severe forms of idiopathic nephrotic syndrome in stable remission-A cross-sectional study.

    Roussel, Aphaia / Delbet, Jean-Daniel / Micheland, Laurianne / Deschênes, Georges / Decramer, Stephane / Ulinski, Tim

    Acta paediatrica (Oslo, Norway : 1992)

    2019  Volume 108, Issue 12, Page(s) 2267–2273

    Abstract: Aim: Severe forms of idiopathic nephrotic syndrome (INS) require immunosuppressive therapy: oral treatment or intravenous therapy (rituximab, RTX). The main objective was to describe quality of life (QOL) in these specific patients.: Methods: Cross- ... ...

    Abstract Aim: Severe forms of idiopathic nephrotic syndrome (INS) require immunosuppressive therapy: oral treatment or intravenous therapy (rituximab, RTX). The main objective was to describe quality of life (QOL) in these specific patients.
    Methods: Cross-sectional, multicentre, observational study analysed QOL using a standardised questionnaire in children from 7 to 17 years, with a steroid-dependent or steroid-resistant INS in stable remission. The questionnaire consisted of 30 questions concerning physical and emotional well-being, self-esteem, family, friends, school and disease resulting in a global score of 0-100.
    Results: A total of 110 patients with a mean age of 11.6 years from three French paediatric nephrology centres were included. A total of 71 patients had oral immunosuppressive treatment, 27 had RTX, and 12 had both. 13.6% of patients had a steroid-resistant INS. The mean number of relapses was 5.8. Seventy-eight patients answered the questionnaire. The global score in the whole study population was 74.7; 72.6 in the RTX group, 76.2 in the oral drugs group, (P = 0.49). The results of sub-dimension 'school' were statistically lower in RTX group (61.6 ± 19.5) compared with oral drugs group (71.4 ± 16; P = 0.02).
    Conclusion: Global QOL score was high in 'difficult-to-treat' patients with INS in stable remission on oral immunosuppressive or RTX treatment.
    MeSH term(s) Adolescent ; Child ; Cross-Sectional Studies ; Female ; Humans ; Immunosuppressive Agents/therapeutic use ; Male ; Nephrotic Syndrome/drug therapy ; Nephrotic Syndrome/psychology ; Quality of Life
    Chemical Substances Immunosuppressive Agents
    Language English
    Publishing date 2019-07-15
    Publishing country Norway
    Document type Journal Article ; Multicenter Study ; Observational Study
    ZDB-ID 203487-6
    ISSN 1651-2227 ; 0365-1436 ; 0803-5253
    ISSN (online) 1651-2227
    ISSN 0365-1436 ; 0803-5253
    DOI 10.1111/apa.14912
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