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NDLR : Un groupe de néphrologues issus du Club des jeunes Néphrologues sélectionne chaque mois à tour de rôle un article de la littérature internationale en rapport avec la dialyse à domicile, Dialyse Péritonéale ou Hémodialyse à Domicile, et en propose un résumé en français et son analyse. Ces résumés sont librement téléchargeables sur le site du RDPLF à l’adresse :https://www.rdplf.org/biblio.html. Depuis décembre 2018 ceux sélectionnés au cours du trimestre par ces néphrologues sont publiés sous leur nom dans la présente rubrique du Bulletin de la Dialyse à Domicile.

Introduction (NDLR) (only available in French) Each month, a group of nephrologists from the Young Nephrologists Club (https://www.cjnephro.com/)takes turns selecting an article from the international literature related to home dialysis, Peritoneal Dialysis or Home Hemodialysis, and offers a summary in French and its analysis. These summaries are also freely downloadable from the RDPLF website at: https: //www.rdplf.org/biblio.html. Since December 2018 those selected during the quarter by these nephrologists have been published under their name in this section of the Bulletin de la Dialysis a Domicile.

INTRODUCTION: The health crisis linked to the COVID-19 epidemic has required lockdown measures in France and changes in practices in dialysis centers. The objective was to assess the depressive and anxiety symptoms during lockdown in hemodialysis patients and their caregivers. METHODS: We sent, during lockdown period, between April and May 2020, self-questionnaires to voluntary subjects (patients and caregivers), treated by hemodialysis or who worked in hemodialysis in one of the 14 participating centers in France. We analyzed their perception of dialysis sessions (beneficial or worrying), their stress level (VAS rated from 0 to 10), their anxiety and depressive symptoms (Hospital anxiety and depression scale). Factors associated with stress, anxiety and depression were analyzed with multiple linear regression models. RESULTS: 669 patients and 325 caregivers agreed to participate. 70 % of participants found it beneficial to come to dialysis during confinement. The proportions of subjects with a stress level ≥ 6 linked to the epidemic, confinement, fear of contracting COVID-19 and fear of infecting a loved one were respectively 23.9%, 26.2%, 33.4% and 42%. 39.2% presented with certain (13.7%) or doubtful (19.2%) anxious symptoms. 21.2% presented a certain (7.9%) or doubtful (13.3%) depressive symptomatology. Age, gender, history of psychological disorders and perception of dialysis sessions were associated with levels of stress, anxiety and depression. CONCLUSION: During the lockdown period, in France, the majority of hemodialysis patients and caregivers found it beneficial to come to dialysis. One in three subjects had anxiety symptoms and one in five subjects had depressive symptoms.; INTRODUCTION: La crise sanitaire liée à l’épidémie de COVID-19 a nécessité des mesures de confinement en France et des changements de pratiques dans les centres de dialyse. L’objectif était d’évaluer les symptômes d’anxiété, de dépression et de stress pendant le confinement chez les patients hémodialysés et leurs soignants. MÉTHODES: Nous avons adressé, pendant le confinement, entre avril et mai 2020, des autoquestionnaires à des sujets volontaires qui étaient traités par hémodialyse (patients) ou qui travaillaient en hémodialyse (soignants) dans l’un des 14 centres de France participants. Nous avons analysé leur perception des séances de dialyse (bénéfique ou inquiétante), leur niveau de stress (échelle visuelle analogique cotée de 0 à 10), et leur symptomatologie anxieuse et dépressive (échelle Hospital anxiety and depression scale). Les facteurs associés au stress, à l’anxiété et à la dépression ont été analysés avec des modèles de régression linéaire multiple. RÉSULTATS: Parmi les 669 patients et 325 soignants ayant répondu, 70 % trouvaient bénéfique de venir en dialyse pendant le confinement. Les proportions de sujets présentant un niveau de stress ≥ 6 lié à l’épidémie, au confinement, à la peur de contracter la COVID-19 et à la peur de contaminer un proche étaient respectivement de 23,9, 26,2, 33,4 et 42 %. De plus, 39,2 % présentaient une symptomatologie anxieuse certaine (13,7 %) ou douteuse (19,2 %), et 21,2 % présentaient une symptomatologie dépressive certaine (7,9 %) ou douteuse (13,3 %). L’âge, le sexe, les antécédents de troubles psychologiques et la perception des séances de dialyse étaient les facteurs principaux associés aux niveaux de stress, d’anxiété et de dépression. CONCLUSION: Pendant le confinement, en France, la majorité des patients et des soignants en hémodialyse ont trouvé bénéfique de venir en séance de dialyse. Un sujet sur trois présentait une symptomatologie anxieuse et un sujet sur cinq présentait une symptomatologie dépressive.

Introduction La crise sanitaire liée à l’épidémie de COVID-19 a nécessité des mesures de confinement en France et des changements de pratiques dans les centres de dialyse. L’objectif était d’évaluer les symptômes d’anxiété, de dépression et de stress pendant le confinement chez les patients hémodialysés et leurs soignants. Méthodes Nous avons adressé, pendant le confinement, entre avril et mai 2020, des autoquestionnaires à des sujets volontaires qui étaient traités par hémodialyse (patients) ou qui travaillaient en hémodialyse (soignants) dans l’un des 14 centres de France participants. Nous avons analysé leur perception des séances de dialyse (bénéfique ou inquiétante), leur niveau de stress (échelle visuelle analogique cotée de 0 à 10), et leur symptomatologie anxieuse et dépressive (échelle Hospital anxiety and depression scale). Les facteurs associés au stress, à l’anxiété et à la dépression ont été analysés avec des modèles de régression linéaire multiple. Résultats Parmi les 669 patients et 325 soignants ayant répondu, 70 % trouvaient bénéfique de venir en dialyse pendant le confinement. Les proportions de sujets présentant un niveau de stress ≥ 6 lié à l’épidémie, au confinement, à la peur de contracter la COVID-19 et à la peur de contaminer un proche étaient respectivement de 23,9, 26,2, 33,4 et 42 %. De plus, 39,2 % présentaient une symptomatologie anxieuse certaine (13,7 %) ou douteuse (19,2 %), et 21,2 % présentaient une symptomatologie dépressive certaine (7,9 %) ou douteuse (13,3 %). L’âge, le sexe, les antécédents de troubles psychologiques et la perception des séances de dialyse étaient les facteurs principaux associés aux niveaux de stress, d’anxiété et de dépression. Conclusion Pendant le confinement, en France, la majorité des patients et des soignants en hémodialyse ont trouvé bénéfique de venir en séance de dialyse. Un sujet sur trois présentait une symptomatologie anxieuse et un sujet sur cinq présentait une symptomatologie dépressive.

Alkali therapy is frequently used during chronic kidney disease and nephrolithiasis: nephrologists and urologists are the key operators. Very few is known about the underlying conditions of such a prescription: the aim of this study was to delineate those determinants. We conducted a prospective survey where French nephrologists and urologists were involved. Responders were without gender distinction and principally nephrologists. Prescription frequency was associated with gender (women), specialty (nephrologists), indications and perceived efficiency. Urologists prescribe more often during nephrolithiasis and nephrologists during chronic kidney disease. Urologists were more expert (by scoring on mineral-based alkaline waters compositions knowledge). By multivariate analysis, prescription frequency is associated with gender (women), indications and perceived efficiency by prescribers, which is itself influenced by feedback from patients. These results could have been influenced by a huge representation of nephrologists but foster physicians to go on listening to feedback from patients, due to a lack of clinical trials on the efficiency of mineral-based alkaline waters in such a field. Finally, physicians' education (especially young nephrologists) on mineral-based alkaline waters should be intensified.

BACKGROUND Vemurafenib is a BRAF inhibitor that has become the cornerstone of metastatic or inoperable melanoma therapy since its approval in 2011 in the United States and 2012 in Europe. This targeted therapy has shown impressive results in terms of increased progression-free and overall survival as compared to dacarbazine. The safety profile did not include any renal manifestations at that time. METHODS This report is the first case series of 8 patients who experienced significant to severe renal insufficiency under vemurafenib treatment. RESULTS This case series shows that vemurafenib may induce potentially severe acute renal failure, including renal sequelae and persistent kidney disease in some cases. CONCLUSIONS Further studies are needed to investigate the effects of vemurafenib on the kidneys. Meanwhile, renal function should be closely monitored in treated patients for early detection of any renal dysfunction occurrence. Cancer 2014;120:2158–2163. © 2014 American Cancer Society.

Multicentric carpo-tarsal osteolysis (MCTO) with or without nephropathy is a rare osteolysis disorder beginning in early childhood and involving mainly carpal and tarsal bones. Renal disease appears later in life in the majority of cases and evolves quickly to end stage renal failure. Autosomal dominant (AD) inheritance has been demonstrated, with a high frequency of sporadic cases. Recently, mutations in a highly conserved region of the MAFB gene (v-maf musculoaponeurotic fibrosarcoma oncogene ortholog B) have been identified in MCTO patients by exome sequencing. MafB, known as a regulator of various developmental processes, is essential for osteoclastogenesis and renal development. We report here the molecular screening of MAFB in eight MCTO patients from six families. We identified MAFB mutations in all, including three novel missense mutations clustering within the hot spot mutation region. Among the eight patients, six only presented renal disease. Our report confirms the genetic homogeneity of MCTO and provides data underlying the clinical variability of this disorder.

Summary Despite their utility, immunosuppressive treatments have numerous side effects, including infectious complications, malignancies and metabolic disorders, all of which contribute to long-term graft loss. In addition to the development of new pharmaceutical products with reduced toxicity and more comfortable modes of administration, tailoring immunosuppression according to the immune status of each patient would represent a significant breakthrough. Gene expression profiling has been shown to be a clinically relevant monitoring tool. In this paper, we have assessed the overall long-term kidney transplant outcome and attempted to identify operationally tolerant-like patients among recipients with stable clinical status at least 5 years post-transplantation. We thus measured a combination of noninvasive blood biomarkers of operational tolerance in a cohort of 144 stable patients and showed that only 3.5% exhibited a gene expression profile of operational tolerance, suggesting that such a profile can be detected under immunosuppressive therapy but that its frequency is low in kidney transplant recipients when compared with liver transplant recipients. We suggest that a rational approach to patient selection, based on a combination of clinical and biological characteristics, may help to provide a safer method for identification of patients potentially suitable for immunosuppressive drug weaning procedures.

Alkali therapy is frequently used during chronic kidney disease and nephrolithiasis: nephrologists and urologists are the key operators. Very few is known about the underlying conditions of such a prescription: the aim of this study was to delineate those determinants. We conducted a prospective survey where French nephrologists and urologists were involved. Responders were without gender distinction and principally nephrologists. Prescription frequency was associated with gender (women), specialty (nephrologists), indications and perceived efficiency. Urologists prescribe more often during nephrolithiasis and nephrologists during chronic kidney disease. Urologists were more expert (by scoring on mineral-based alkaline waters compositions knowledge). By multivariate analysis, prescription frequency is associated with gender (women), indications and perceived efficiency by prescribers, which is itself influenced by feedback from patients. These results could have been influenced by a huge representation of nephrologists but foster physicians to go on listening to feedback from patients, due to a lack of clinical trials on the efficiency of mineral-based alkaline waters in such a field. Finally, physicians' education (especially young nephrologists) on mineral-based alkaline waters should be intensified.

Vemurafenib is a BRAF inhibitor that has become the cornerstone of metastatic or inoperable melanoma therapy since its approval in 2011 in the United States and 2012 in Europe. This targeted therapy has shown impressive results in terms of increased progression-free and overall survival as compared to dacarbazine. The safety profile did not include any renal manifestations at that time.This report is the first case series of 8 patients who experienced significant to severe renal insufficiency under vemurafenib treatment.This case series shows that vemurafenib may induce potentially severe acute renal failure, including renal sequelae and persistent kidney disease in some cases.Further studies are needed to investigate the effects of vemurafenib on the kidneys. Meanwhile, renal function should be closely monitored in treated patients for early detection of any renal dysfunction occurrence. Cancer 2014;120:2158-2163. © 2014 American Cancer Society.

Sir, Renal sinus lipomatosis (RSL) is a rare disease, described by replacement of parenchyma by sinus and/or perirenal fat. We report here an exceptional case of RSL on kidney graft after corticosteroid treatment was stopped, leading to functional graft transplantectomy. A 74-year-old man received a first renal allograft in 2004. He was maintained on tacrolimus and mycophenolate mofetil with no rejection. Corticosteroid was stopped at the sixth month. The lowest serum creatinine level was 100 μmol/L. On February 2007, renal ultrasonography, performed for acute renal failure, showed dilatation of renal cavities. Contrast-enhanced computed tomography (CT) showed an enlarged kidney (14.3 cm) with an increased amount of fatty-like tissue in renal sinus and perirenal area (Figure 1). For 6 months, he experienced several obstructive renal failures and infections, leading to permanent nephrostomy. Biopsy of the sinus mass revealed an extensive fatty tissue with fibrosis. Finally, because of bilateral pulmonary embolism secondary to vena cava compression, persistent obstruction and continuous graft enlargement without conclusive histology, and despite good renal function, he underwent transplantectomy. Histological examination showed RSL (immunophenotypical labelling and biomolecular investigations negative for sarcoma) and atrophic chronic pyelonephritis graft. Fig. 1 Filling defects secondary to thrombosis in the right RV and VCI (white arrows) after contrast administration from vena cava superior. RSL is essentially described on native kidneys [1,2]. Possible risk factors for RSL include ageing, obesity and pathologic states that cause renal inflammation such as intense corticotherapy and/or early rejection for transplants. Often, chronic or repeat urinary tract infections have been found, and one hypothesis is that RSL may be secondary to periodic leakages of urine into the peripelvic tissues [2]. RSL can also be part of replacement of destroyed or atrophic renal tissue [3]. It has also been suggested that high-dose steroid treatment by itself, Cushing's syndrome or obesity may have contributed to the development of fibrolipomatosis [4]. Intriguingly, a very few cases of RSL have been reported in the renal transplant population [5]. This case is intriguing for several reasons: The time between transplantation and the first symptoms was quite long (3 years). The recipient of the contralateral kidney did not develop any RSL. The patient experienced his first urinary tract infection at the same time as the first acute obstructive renal failure. No history of earlier acute rejection episodes has been found. He did not have a long-term steroid treatment and was not obese. Finally, our case is also exceptional because it led to transplantectomy. Transplantectomy was decided in view of the rapidly increasing graft mass, responsible of vena cava compression and suspicion of malignity. In conclusion, our case underlines that diagnosis of RSL, although exceedingly rare, should be considered when imaging exams demonstrate a fat-containing renal sinus mass. In transplant recipients, immunosuppressive regimen and malignancy risk underline the importance to establish differential diagnosis between fibrolipomatosis and fibrosarcoma, renal tumour or post-transplant lymphoproliferative disorder. Conflict of interest statement. None declared.

Introduction L’administration de bicarbonate est utilisee dans le ralentissement de la perte de fonction renale, le traitement de certaines maladies lithiasiques renales et d’autres indications : nephrologues et urologues sont les premiers acteurs de leur prise en charge. Peu d’informations sont disponibles sur les determinants de la prescription des eaux bicarbonatees. L’objectif de cette etude est d’evaluer les habitudes de leurs prescriptions. Materiels et methodes Une enquete prospective a ete realisee aupres de nephrologues et urologues francais. Les donnees demographiques, d’exercice medical et des connaissances ont ete analysees. Resultats Les repondants (n = 249) etaient sans distinction de genre mais principalement des nephrologues (81 %). La majorite (54 %) indique prescrire le traitement pour de courtes periodes ( Discussion Malgre la courte periode d’administration (4 mois), la participation a ete importante. La representation importante de nephrologues ne modifie pas les resultats principaux qui devraient etre confirmes au cours de nouvelles enquetes nationales. Conclusion Cette premiere etude montre que les medecins specialistes prescrivent d’autant plus qu’ils croient a l’efficacite des eaux bicarbonatees. La perception de leur efficacite par les prescripteurs est influencee par les retours positifs des patients (mais pas le retour negatif), les incitant a renouveler cette prescription.