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Introduction Le risque d’insuffisance renale terminale au cours des vascularites a ANCA est evalue par le score de Brix, selon les pourcentages de glomerules normaux, de fibrose interstitielle et la fonction renale au diagnostic. L’arterite des petites arteres renales, n’est pas prise en compte. L’objectif de cette etude est de decrire les caracteristiques clinico-histologiques et l’impact pronostique de l’arterite des petites arteres renales, au diagnostic d’une vascularite a ANCA. Description Entre 2000 et 2019, nous avons analyse les biopsies renales adressees par 5 centres, au diagnostic d’une vascularite a ANCA. L’atteinte des petites arteres renales etait definie par la presence d’une necrose fibrinoide de la paroi arterielle et/ou une inflammation de la media. Methodes La survie renale etait analysee selon la methode de Kaplan–Meier. Les variables predictives de la survie renale par modele de Cox et la discrimination pronostique par les index de concordance. Resultats Deux cent cinquante et un patients ont ete inclus dont 13,5 % avec une atteinte vasculaire (ANCA_V+) ( Fig. 1 A–B) et 86,5 % sans atteinte vasculaire (ANCA_V−). Au diagnostic, les ANCA_V+ etaient plus âges (72 ans vs 63 ans, p = 0,005) et presentaient un taux de CRP plus eleve (176 mg/L vs 64 mg/L, p Fig. 1 C). En analyse multivariee, le risque d’insuffisance renale terminale etait significativement augmente en cas d’atteinte vasculaire (HR = 3,10, IC95 % = 1,65–5,81, p = 0,001). L’ajout du statut vasculaire (V+/V−) ameliorait la discrimination pronostique du score de Brix, chez les patients a risque faible ou modere selon le score de Brix ( Fig. 1 D–F). Conclusion Les vascularites a ANCA avec arterite des petites arteres renales au diagnostic, sont une forme particuliere de vascularite a ANCA, au pronostic renal pejoratif.

International audience; Background: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab.Study design: Case series of C3 glomerulopathy.Setting & participants: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Québec, Canada.Outcomes: Global or partial clinical renal response.Measurements: Evolution of serum creatinine and proteinuria values.Results: 26 patients (13 children/adolescents) were included. 22 (85%) patients had received steroids, plasma exchange, or immunosuppressive therapy before eculizumab, and 3 of them had rapid progression of their kidney disease despite treatment. At the initiation of eculizumab therapy, 11 (42%) patients had chronic kidney disease, 7 (27%) had rapidly progressive disease, and 3 (12%) required dialysis. After eculizumab treatment (median duration, 14 months), 6 (23%) patients had a global clinical response; 6 (23%), a partial clinical response; and 14 (54%), no response. Compared with those who had a partial clinical or no response, patients who had a global clinical response had lower estimated glomerular filtration rates, a more rapidly progressive course, and more extracapillary proliferation on kidney biopsy. Age, extent of renal fibrosis, frequency of nephrotic syndrome, low serum C3 and C3 nephritic factor and elevated soluble C5b-9 concentrations, or complement gene variants did not differ between responders and nonresponders.Limitations: Retrospective design without a control group, relatively small number of cases, inclusion of pediatric and adult cases.Conclusions: Eculizumab appears to be a potential treatment for patients with crescentic rapidly progressive C3 glomerulopathy. Its benefit in patients with non-rapidly progressing forms seems to be limited.

Background Homeostatic turnover of the extracellular matrix conditions the structure and function of the healthy lung. In lung transplantation, long-term management remains limited by chronic lung allograft dysfunction, an umbrella term used for a heterogeneous entity ultimately associated with pathological airway and/or parenchyma remodeling. Objective This study assessed whether the local cross-talk between the pulmonary microbiota and host cells is a key determinant in the control of lower airway remodeling posttransplantation. Methods Microbiota DNA and host total RNA were isolated from 189 bronchoalveolar lavages obtained from 116 patients post lung transplantation. Expression of a set of 11 genes encoding either matrix components or factors involved in matrix synthesis or degradation (anabolic and catabolic remodeling, respectively) was quantified by real-time quantitative PCR. Microbiota composition was characterized using 16S ribosomal RNA gene sequencing and culture. Results We identified 4 host gene expression profiles, among which catabolic remodeling, associated with high expression of metallopeptidase-7, -9, and -12, diverged from anabolic remodeling linked to maximal thrombospondin and platelet-derived growth factor D expression. While catabolic remodeling aligned with a microbiota dominated by proinflammatory bacteria (eg, Staphylococcus, Pseudomonas, and Corynebacterium), anabolic remodeling was linked to typical members of the healthy steady state (eg, Prevotella, Streptococcus, and Veillonella). Mechanistic assays provided direct evidence that these bacteria can impact host macrophage-fibroblast activation and matrix deposition. Conclusions Host-microbes interplay potentially determines remodeling activities in the transplanted lung, highlighting new therapeutic opportunities to ultimately improve long-term lung transplant outcome., Graphical abstract

Introduction Des etudes anciennes ont suggere une association entre l’infection a plasmodium et la survenue de glomerulopathies decrites essentiellement sous la forme de GNMP. L’objectif de cette etude est de decrire le spectre des atteintes glomerulaires pouvant survenir au decours d’une crise de paludisme. Patients/materiels et methodes L’ensemble des CHU de nephrologie ont ete contactes afin de colliger retrospectivement les observations des patients ayant presente un syndrome glomerulaire au decours d’un acces palustre documente par une PBR. Resultats Quinze patients (7 hommes et 8 femmes, âge moyen 47 ans) ont ete identifies. Dans 87 % des cas l’espece identifiee etait le Plasmodium falciparum (parasitemie moyenne a 5 %). Au cours de la crise de paludisme, tous les patients presentaient une insuffisance renale qui etait consideree comme aigue dans 12 cas (7 ont eu recours a l’hemodialyse). Au moment de la PBR (delai moyen de 26 jours apres la crise de paludisme), 13 patients avaient un syndrome nephrotique (SN) avec une proteinurie moyenne de 900 mg/mmol et une albuminemie moyenne a 20,4 g/L. L’etude anatomopathologique renale a mis en evidence chez 6 patients une nephropathie liee au VIH, une forme collapsante d’HSF sans infection par le VIH dans 6 cas, des lesions d’HSF dans 2 cas et un SNLGM chez le dernier patient. Les patients VIH positifs avaient tous sauf un une charge virale elevee (valeur moyenne de 560 000 copies/mL). Tous les patients testes (2 HIVAN et 4 HSF collapsante) presentaient des variants du gene APOL1 (4 homozygotes G1/G1 et 2 heterozygotes composites G1/G2). Sept patients ont eu un traitement specifique par corticoides (6 HSF collapsantes 2 HSF et 1 HIVAN) permettant une remission partielle du SN dans 3 cas. Au terme du suivi moyen de 21 mois, 5 patients presentaient une IR terminale. Chez les 10 patients sans traitement de suppleance le DFG moyen etait de 48 mL/min/1,73 m2. Conclusion Apres une crise de paludisme, les atteintes glomerulaires les plus frequemment observees sont la revelation d’une nephropathie liee au VIH ou une HSF collapsante. La presence dans les 6 cas testes de variant pathogene du gene APOL1 nous laisse penser que sur un terrain genetiquement predispose, une infection par le paludisme peut provoquer des lesions podocytaires et/ou des cellules epitheliales parietales. Une etude de la localisation renale de la proteine APOL1 chez ces patients est en cours ainsi que la recherche du parasite dans le rein de ces patients.

Background Metabolic disorders, in particular weight gain, increase cardiovascular mortality risk and can cause serious problems after renal transplantation. Weight and body mass index are imprecise indicators of nutritional status. Accurate determination of the body composition of renal transplant patients is essential; therefore, a simple tool that allows appropriate patient monitoring is crucial. Design A new device, the Body Composition Monitor (BCM, Fresenius Medical Care, Bad Homburg, Germany), expresses body weight in terms of adipose tissue, lean tissue mass, and excess fluid. We compared the performance of this 3-compartment model with dual-energy X-ray absorptiometry (DEXA) as a reference method in determining body composition in a renal transplant population. Subjects Thirty-three clinically stable renal transplant patients were studied. Bland-Altman plots and Passing-Bablok regression were used to compare methods. Results Mean lean mass was 51.8 ± 12.3 kg with DEXA and 39.0 ± 9.9 kg with BCM. Despite the Passing-Bablok regression failing to find significant differences, the predictive value of BCM for DEXA was poor. Mean fat mass was 19.4 ± 9.7 kg with DEXA and 30.0 ± 16.0 kg with BCM. The slope of the regression line of BCM over DEXA significantly differed from 1. Conclusion We conclude that, in this population, these methods cannot be substituted for one another.

Intravenous injection of angiogenesis-inhibitor drugs is used widely to treat cancers. Associated renal complications primarily involve proteinuria and hypertension, and thrombotic microangiopathies also have been described. Intravitreal anti-vascular endothelial growth factor (VEGF) therapy currently is used by ophthalmologists to treat neovascularization in age-related macular degeneration. However, there is some evidence that intravitreal anti-VEGF injections may result in systemic absorption, with the potential for injury in organs that are reliant on VEGF, such as the kidney. We report the first case to our knowledge of a patient who developed an acute decrease in kidney function, nonimmune microangiopathic hemolytic anemia with schistocytes, and thrombocytopenia after 4 intravitreal injections of ranibizumab. Light microscopy of a kidney biopsy specimen showed segmental duplications of glomerular basement membranes with endothelial swelling and several recanalized arteriolar thrombi. Because of the increasing use of intravitreal anti-VEGF agents, ophthalmologists and nephrologists should be aware of the associated risk of kidney disease. Early detection is crucial so that intravitreal injections can be stopped before severe kidney disease occurs.

Introduction L’incidence de l’insuffisance renale chronique (IRC) en post-transplantation pulmonaire chez le patient ayant une mucoviscidose est tres elevee. Les facteurs de risque sont multiples, l’impact d’une hyperoxalurie et la presence de cristaux d’oxalate de calcium sur la survenue et severite de l’insuffisance renale chronique (IRC) n’a jamais ete evalue. Patients/Materiels et methodes Il s’agit d’une etude monocentrique, prospective, menee entre 2008 et 2014. Les patients inclus devaient avoir une mucoviscidose, etre transplante pulmonaire et beneficier d’une analyse biochimique des urines ainsi que d’une cristallurie. Les patients ayant une IRC pre transplantation etaient exclus. Les analyses etaient centralisees dans le laboratoire d’exploration fonctionnelles de l’hopital Tenon. Deux analyses ont ete realisees : une analyse descriptive s’interessant a la prevalence des cristaux et des facteurs de risque associes chez tous les malades ; une analyse pronostique evaluant l’impact de la presence de cristaux sur la fonction renale post transplantation chez les patients ayant eu une cristallurie preoperatoire. Le critere de jugement etait la survenue d’une IRC durant le suivi post-transplantation definie par 2 mesures consecutives de DFG Observation/Resultats Quatre-vingt-sept patients ont ete inclus dans l’analyse descriptive et 45 patients dans l’analyse pronostique. Des cristaux s’observaient chez 32/87 (36 %) et 16/45 (35 %) patients. Les variables associees a leur presence etaient une augmentation significative de l’oxalurie (430 [315–635] vs 290 [175–437] μmol/L, p = 0,005), de calciurie (3,2 [1,4–5,20] vs 1,8 [1,1–2,7] mmol/L, p = 0,006) et de phosphaturie (26,5 [19,1–43,9] vs 20,2 [10,1–29,2] mmol/L, p = 0,008). L’IRC survenait chez 18/45 (40 %) des patients. L’âge (p = 0,05) et une proteinurie preoperatoire plus elevee (p = 0,007) etaient les seuls facteurs de risque identifies. La presence de cristaux urinaire n’etait pas associee a la survenue d’une IRC (p = 0,9). Dix-neuf biopsies chez 16 patients ont ete realisees durant le suivi. Les anomalies observees etaient principalement des lesions d’arteriosclerose, de glomerulosclerose et des calcifications intra-tubulaire. L’analyse par spectro-infrarouge n’a pas mis en evidence de cristaux oxalate de calcium. Discussion/Conclusion L’IRC est une complication frequemment observee chez le patient atteint de mucoviscidose. Malgre une prevalence importante de cristaux urinaire, ces derniers ne sont pas associes a la presence de cristaux intra-tissulaire et leur impact sur la fonction renale a moyen terme semble non contributive.

Kidney stones and renal failure are more common in patients with CF compared to general population. Hyperoxaluria caused by fat malabsorption and the lack of intestinal oxalate degrading bacteria Oxalobacter formigenes is known in CF patients. Hyperoxaluria-induced crystal formation is associated with kidney stones and renal failure. However data concerning detection of crystals in CF patients are lacking in the literature. We conducted a prospective study in Pneumology Center, Foch Hospital, during 8 months. Forty cystic fibrosis adult patients were enrolled to have a sample spot urine test on the first morning micturition. Median age was 29 years-old (range 20−55). Sixteen patients were pulmonary transplanted. Thirty-eight (95%) and 24 (61%) patients had exocrine and endocrine pancreas insufficiency, respectively. Three patients had glomerulonephropathy (2 diabetic nephropathy and 1 amyloidosis). The median plasmatic creatinine was 69mmol/l (range 39–400mmol/l). Factors for crystals formation were researched. Thirty-five percent of patients (13/40) had hypercalciuria (>3.5mmol/l), 60% (24/40) had hypershy;oxaluria (>0.3mmol/l) and 72% (28/40) had hypocitraturia (

Solid organ transplantations (SOT) are performed successfully in selected HIV-infected patients. However, multiple and reciprocal drug–drug interactions are observed between antiretroviral (ARV) drugs and calcineurin inhibitors (CNIs) through CYP450 metabolization. Raltegravir (RAL), a novel HIV-1 integrase inhibitor, is not a substrate of CYP450 enzymes. We retrospectively reviewed the outcomes of 13 HIV-infected transplant patients treated by an RAL + two nucleosidic reverse transcriptase inhibitor (NRTI) regimen, in terms of tolerability, ARV efficacy (plasma viral load, CD4 cell count), drug interactions, RAL pharmacokinetics and transplant outcome. Thirteen patients with liver (n = 8) or kidney (n = 5) transplantation were included. RAL was initiated (400 mg BID) either at time of transplantation (n = 6), or after transplantation (n = 7). Median RAL trough concentration was 507 ng/mL (176–890), which is above the in vitro IC95 for wild type HIV-1 strains (15 ng/mL). Target trough levels of CNIs were promptly obtained with standard dosages of tacrolimus or cyclosporine. RAL tolerability was excellent. There was no episode of acute rejection. HIV infection remained controlled. After a median follow-up of 9 months (range: 6–14), all patients were alive with satisfactory graft function. The use of an RAL + two NRTI-based regimen is a good alternative in HIV-infected patients undergoing SOT.

Fabry disease (FD) is an X-linked genetic disease, resulting from the deficiency of alpha-galactosidase A, a lysosomal enzyme responsible for the cleavage of glycosphingolipids. In absence of enzyme replacement therapy (ERT), globotriaosylceramide (Gb3) accumulates in tissue, leading to progressive organ damage with severe renal, cardiac and central nervous system complications. We herein describe the first case of successful combined and simultaneous heart and kidney transplantation in a young male patient with FD complicated by end-stage renal disease and severe heart failure not responding to late-onset ERT. Combined heart and kidney transplantation can be recommended for Fabry patients with end-stage renal disease and overt hypertrophic cardiomyopathy, severe ischemic or valvular heart disease.

Increased aortic stiffness is a major factor responsible for the high cardiovascular mortality in patients with end-stage renal disease, but the impact of kidney transplantation on recipient aortic stiffness remains poorly defined. The use of expanded-criteria kidney donors is associated with decreased recipient survival compared with the use of standard-criteria donors, although the underlying mechanisms are incompletely understood. It was hypothesized that donor characteristics may affect recipient aortic stiffness, which may contribute to cardiovascular mortality in these patients. Aortic stiffness was evaluated by measurement of carotid-femoral pulse wave velocity in 74 cadaveric kidney recipients at 3 and 12 mo after transplantation. At 3 mo, aortic stiffness was associated exclusively with recipient-related factors: Age, gender, and mean BP. At 12 mo, age of the donor kidney emerged as an additional determinant. The change in aortic stiffness between 3 and 12 mo strongly correlated with donor age; stiffness improved in recipients of young kidneys (first tertile of donor age) and worsened in recipients of older kidneys (upper tertile of donor age). At 12 mo, the carotid-femoral pulse wave velocity was >1 m/s higher in recipients of the oldest kidneys than in the recipients of younger kidneys. The association between donor age and aortic stiffness was independent of recipient age, gender, mean BP, pretransplantation dialysis duration, conventional cardiovascular risk factors, medication, posttransplantation events, and GFR. These results demonstrate that the impact of kidney transplantation on recipient aortic stiffness is dependent on donor age and suggest that ongoing damage to large arteries might contribute to the mechanism underlying the association of old-donor kidneys and increased cardiovascular mortality.

Du fait de l’augmentation du nombre de greffes et de l’amelioration de la survie apres transplantation, les complications cancereuses sont devenues une preoccupation majeure en transplantation renale. Peu de donnees sur le cancer du poumon existent chez les patients greffes renaux. Pour cette etude retrospective multicentrique (Foch, Bichât et Necker) des cas de cancers broncho-pulmonaires survenus apres greffe renale entre 2003 et 2012 ont ete compares a des temoins non transplantes, recrutes sur le centre de Foch, apparies sur la tranche d’âge (30 ; 30–50 ; 50–65 ; > 65 ans), le genre et la date de diagnostic du cancer. L’incidence du cancer broncho-pulmonaire chez les greffes renaux a ete calcule sur les annees 2008–2012 a partir des files actives annualisees de greffes de Foch et de Necker. Trente patients et 60 temoins ont ete inclus. Il s’agit de la plus large etude dans cette population a ce jour. L’incidence du cancer bronchique est de 2,55/1000 patients-annee. Les cas avaient un âge median de 60 ans, 80 % etaient des hommes, tous fumeurs, ayant fume une mediane de 30PA. Quarante pour cent des cancers survenus chez des patients greffes renaux ont ete diagnostiques au cours d’examens de routine. Soixante-dix pour cent etaient des stades III ou IV, la survie globale apres diagnostic etait de 19 mois. Chez les temoins non greffes, 78 % avaient un stade III ou IV la survie globale etait de 17 mois ( p = 0,65). Le deces etait relie au cancer chez 87 % des greffes renaux et 98 % des temoins. Les patients greffes renaux fumeurs ont un risque eleve de developper un cancer bronchopulmonaire. Malgre un suivi medical regulier, le diagnostic est le plus souvent fait a un stade avance et le pronostic global est sombre.

After a brief review of the randomization tests for one-way (M)ANOVA, it is shown how the user has of a wide choice of parameters such as choice of dissimilarity, choice of index of quality. Finally a computer program is discussed and applications are presented.

This study is intended to determine whether the additional presence of personality disorder predicts poor hospital and posthospital recovery in psychiatric inpatients with Axis I disorders. Seventy-eight patients who were subsequently hospitalized had reliable DSM-III-R Axis I and personality disorder (PD) assessments. At hospital admission, at discharge, and at 1- and 2-year follow-up, measures of clinical functioning (BPRS, MADS, Health Sickness Scale), ratings of treatment completion, and working alliance were obtained. Thirty-five percent of patients met criteria for personality disorder. Personality disorder significantly predicted (at either the .01 or the .05 level) greater comorbidity at admission; poorer working alliance and treatment completion at discharge; poorer global outcome, symptomatic severity, autonomy, and social investments at 1-year follow-up; and poorer interpersonal relationships at 2 years. At 2 years, PD patients had obtained more therapy but these were less specific treatments. ...

Dans un cadre mathematique general, sont proposes des indices de proximite inter-classe connus en classification hierarchique. D'autres egalement interessants sont deduits. Une nouvelle formule de reactualisation, s'appliquant parfaitement au cadre mathematique propose, est developpee

From 5 cases of partial anomalous pulmonary venous drainage of the left lung into the innominate vein, it has been thought of interest to situate this type of venous anomaly among the partial anomalous pulmonary venous drainage classification. It is revealed in 2 different ways. In adults, this malformation sets the problem of the diagnosis of an abnormal chest Xray, without symptom. In children, this vascular abnormality is discovered during the exploration of a congenital cardiopathy which causes the main symptoms. Embryological data are reviewed and statistics of frequency which lead to show that this type of partial anomalous venous drainage is often misunderstood, particularly with regard to anomalous venous drainage of the right lung.

This work presents an analysis instrument and the first results obtained while applying it to 280 depressive patients compared to 496 non-depressive patients who were followed during a 4 year period. The treatment follow-up permits us to present the chronological "history" of the patient's contacts with different services and to establish a classification. One of the interests, for the clinician, consists in specifying the best therapeutical approach possible. A more detailed study should allow us to relate certain patterns with diagnostic subcategories and to determine if the high proportion of short patterns corresponds to the "successful" treatments or to the dropouts.

Report of one case of a woman aged 29 years with a severe cyanotic congenital heart disease complicated by brain abscess. The diagnosis of left juxtaposition of the atria combined with dextroversion, a d-transposition of the large vessels and a vestricular septal defect was done pre-operatively. An attempt at a Rastelli's operation was followed by immediate death. The post-mortem examination confirmed the pre-operative diagnosis and demonstrated besides a double conus and a pulmonary atresia. The juxtaposition of the atria was underlined.

10 cases of anomalous position of the great vessels are reported. They include 8 cases of d-transpositions, 1 of 1-transposition and 1 anatomically corrected malposition. These cases have in common the combination of a ventricular septal defect with a pulmonary stenosis. The anatomical study (6 of 10 cases) made it possible to describe the type of the VSD (related to a defect of development and/or alinement of parietal on the septal band) and of the pulmonary pathway stenosis. Blalock's anastomosis has made it possible to obtain an improvement in 6 patients. 4 operations of complete cure were undertaken in d-transpositions: 2 Mustard's operations ending in death. 2 operations according to Rastelli's principles with one death and one good result. The case of anatomically corrected malposition died after an attempt at complete cure, in view of a left ventricular hypoplasia. The indication for the type of complete cure was discussed in relation with the anatomical data.

This is a preliminary study focusing on a 2 years follow-up of 78 patients addressed to psychiatric hospitalization and psychodynamically oriented crisis intervention. Relationship between psychopathological, clinical and interactional profile at first medical interview and clinical/social assessment at 1 and 2 years follow-up were studied, indicating different predictors for long-term treatment (DSM III R diagnosis and psychotic symptoms scores) and global improvement (age and sex) as well as poorer outcome for social functioning than for symptomatic measures. Comment is also provided on the observed high concordance between clinical predictions at crisis treatment completion and research staff assessments at 1 and 2 years follow-up. The evaluated crisis intervention model was found to provide a valuable setting to structure acute psychiatric episode treatment and long-term care, even though transition from clinical recovery to effective social-interpersonal reinvestments was observed to raise problems in the addressed psychiatric population.

The case of a 60-year-old patient with severe stenosis of both proximal pulmonary arteries resulting from Takayasu's arteritis is reported. The thoracic aorta and pulmonary artery were calcified. Patch enlargement of the pulmonary arteries was successfully performed. Operative biopsies confirmed the diagnosis of nonspecific arteritis consistent with Takayasu's disease.

Objective. Management of nonviral cryoglobulinemia vasculitis has yet to be defined. Rituximab has emerged as a novel and promising therapeutic alternative, but data are scarce. Our objective was to evaluate the safety and efficacy of rituximab in nonviral cryoglobulinemia vasculitis in off-trial real-life patients. Methods. Prospective data from the French AutoImmunity and Rituximab (AIR) registry, which includes data on patients with autoimmune disorders treated with rituximab in off-label conditions, were analyzed. Results. Twenty-three patients received treatment with rituximab for cryoglobulinemia vasculitis. Tolerance was marked by the occurrence of side effects in almost half of the patients, including severe infections in 6 (26%) of 23, with a rate of 14.1 per 100 patient-years. These infections occurred in a particular subset of patients ages >70 years, with essential type II mixed cryoglobulinemia and renal failure with a glomerular filtration rate of