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Background Chronic kidney disease (CKD) after lung transplantation (LT) is underestimated. The aim of the present study was to measure the loss of glomerular filtration rate (GFR) 1 year after LT and to identify the risk factors for developing Stage ≥3 CKD. Methods LT patients in the University Hospital of Lyon had a pre- and post-transplantation measurement of their GFR (mGFR), and GFR was also estimated using the Chronic Kidney Disease Epidemiology Collaboration equation. Results During the study period, 111 patients were lung transplant candidates, of which 91 had a pre-transplantation mGFR, and 29 had a mGFR at 1 year after LT. Six patients underwent maintenance haemodialysis after transplantation. Mean mGFR was 106 mL/min/1.73 m2 before LT and 58 mL/min/1.73 m2 1 year after LT (P Conclusion The present study underlines the value of mGFR in the pre-LT stage and found major renal function loss after LT, and consequently two-thirds of patients have Stage ≥3 CKD at 1 year. All patients with a pre-LT mGFR

Renal positron emission tomography (PET) functional imaging allows non-invasive and dynamic measurements of functional and metabolic parameters. [In 10 healthy subjects, dynamic PET acquisitions were performed after [Renal time activity curves of [In healthy subjects, over a wide range of renal perfusion, direct estimates of renal oxygen consumption as well as tissue perfusion can be obtained by PET with a single tracer [

International audience; Objective Morbid obesity is associated with a higher independent risk of chronic kidney disease (CKD). Estimated glomerular filtration rate (eGFR) has been evaluated in a limited number of study participants with severe obesity. Methods A total of 706 measured GFR (mGFR) results from 598 participants with obesity (BMI \textgreater= 35 kg/m(2)) were retrospectively collected. The performance of the Modification of Diet in Renal Disease (MDRD) equation, Chronic Kidney Disease-Epidemiology (CKD-EPI) equation, and deindexed eGFR were compared with mGFR from the gold standard technique (inuline or iohexol), adjusted (mGFRr) or nonadjusted (mGFR) to body surface area. Absolute bias, precision, and accuracy were calculated. Results Mean mGFRr (58 +/- 31 mL/min/1.73 m(2)) was significantly different from CKD-EPI and MDRD (P \textless 0.001). Mean mGFR (nonindexed) (70 +/- 40 mL/min) was significantly higher than mGFRr (P \textless 0.001). eGFR showed important biases and low accuracies for CKD-EPI and MDRD (10.7 +/- 10.7 and 12.2 +/- 13.7 mL/min/1.73 m(2); 78% vs. 75% respectively). Deindexation worsened bias and accuracy 30% (percentage of GFR estimates within 30% of mGFRr or mGFR) between eGFR and mGFR. Conclusions eGFR overestimates mGFR and is associated with important biases and inaccuracies in patients with severe obesity, and deindexing eGFR worsens the overestimation. These findings may have important implications in examining kidney function in patients with obesity.

International audience; BACKGROUND: In atherosclerotic renal artery disease, the benefit of revascularization is controversial. A clinical decision-making process based on a multidisciplinary meeting was formalized in the Lyon university hospital. OBJECTIVES: To investigate whether this decisional process ensured a clinical benefit to patients assigned to renal revascularization. METHODS: Single-centre retrospective cohort study, including patients diagnosed from April 2013 to February 2015 with an atherosclerotic renal artery disease with a peak systolic velocity \textgreater180cm/s. For each patient, the decision taken in multidisciplinary meeting (medical treatment or revacularization) was compared to the one guided by international guidelines. Blood pressure values, number of antihypertensive medications, presence of an uncontrolled or resistant hypertension, and glomerular filtration rate at one-year follow-up were compared to baseline values. Safety data were collected. RESULTS: Forty-nine patients were included: 26 (53%) were assigned to a medical treatment and 23 (47%) to a renal revascularization. Therapeutic decision was in accordance with the 2013 American Health Association guidelines and with the 2017 European Society of Cardiology guidelines for 78% and 22% of patients who underwent revascularization, respectively. Patients assigned to revascularization presented a significant decrease in systolic blood pressure (-23+/-34mmHg, p = 0.007), diastolic blood pressure (-12+/-18mmHg, p = 0.007), number of antihypertensive medications (-1.00+/-1.03, p = 0.001), and number of uncontrolled or resistant hypertension (p = 0.022 and 0.031) at one-year follow-up. Those parameters were not modified among patients assigned to medical treatment alone. There was no grade 3 adverse event. CONCLUSION: Based on a multidisciplinary selection of revascularization indications, patients on whom a renal revascularization was performed exhibited a significant improvement of blood pressure control parameters with no severe adverse events.

International audience; Rationale: Renal positron emission tomography (PET) functional imaging allows non-invasive and dynamic measurements of functional and metabolic parameters. [O-15]H2O is used as a perfusion tracer, and [C-11]acetate as an oxidative metabolism in this purpose, requiring two injections to assess those fundamental parameters. Yet, in cardiac physiology study, the high first-pass myocardial extraction fraction of [C-11]acetate allowed to use its influx rate as a blood flow marker too. Since [C-11]acetate has been characterized by a 20-25% single pass renal extraction in dogs, it could be used as a potential tracer for renal perfusion. The aim of this study was to determine whether [C-11]acetate influx rate can be used as quantitative in vivo marker of kidney perfusion in human. Methods: In 10 healthy subjects, dynamic PET acquisitions were performed after [O-15]H2O and [C-11]acetate injections spaced by a 15-minute interval. As previously validated, with compartmental modeling of kinetics, renal perfusion and oxidative metabolism were estimated respectively with influx rate of [O-15]H2O and efflux rate of [C-11]acetate. Additionally, influx rate of [C-11]acetate was regressed to influx rate of [O-15]H2O. Results: Renal time activity curves of [C-11]-acetate was best fitted with a mono compartmental model compared to a bi-compartmental model (p \textless 0.0001). [C-11]acetate influx rate was significantly correlated with perfusion quantified with [O-15]H2O (r(2) = 0.37, p \textless 0.001) at baseline. This regression allowed the computation of a renal [C-11]acetate extraction fraction (EF), and further the computation of renal blood flow from its influx rate. Conclusion: In healthy subjects, over a wide range of renal perfusion, direct estimates of renal oxygen consumption as well as tissue perfusion can be obtained by PET with a single tracer [C-11]acetate. This approach needs to be validated in CKD patients, and would be of great interest to design clinical protocol aiming at evaluating ischemic nephropathies candidate to revascularization. (C) 2019 Elsevier Inc. All rights reserved.

International audience; Pregnancy in women with end-stage renal disease is rare. Multiple pregnancies carry a high risk of complications even in healthy individuals. We report the case of a 36-year-old woman who had four pregnancies while she was on dialysis, including one twin pregnancy. The last pregnancy occurred while in the 14th year of hemodialysis. At 8 weeks of gestation (WG), ultrasonography diagnosed a dichorionic diamniotic twin pregnancy. The frequency of dialysis was increased from 3 to 6 times a week and each session lasted 4 h. At 22 WG, polyhydramnios was diagnosed. At 25 WG, the patient presented respiratory distress and was transferred to intensive care where continuous hemodialysis, non-invasive ventilation, antibiotic and tocolysis were initiated. Because of tocolysis failure, a cesarean section was performed and she delivered male twins. The two newborns weighed 790 and 870 g, respectively. To our knowledge, this is the first report of four pregnancies in hemodialysis including one twin pregnancy. The incidence of pregnancy and a better outcome in patients on hemodialysis has increased in recent years but a tight coordination between nephrologists and obstetricians is essential.

Since a couple of years, a new paradigm of care has taken place where the patient plays a major role in the choice of his treatment. Patient information in a context where the doctor-patient relationship has drastically changed and where knowledge is continuously growing, is a challenge for the nephrologist. For the patient who decides to undergo home dialysis, numerous questions arise during the decision-making process. How should treatment alternatives be discussed with the patient? What are the current data available on the different methods? As comfort, quality of life and level of autonomy often drive the choice of care management, home dialysis can be promoted more with appropriate assistance from both the nephrologist and the healthcare team. Cet article fait partie du numéro supplément Innovations en Néphrologie réalisé avec le soutien institutionnel de Vifor Fresenius Medical Care Renal Pharma.

Introduction Au cours d’une seance d’hemodialyse (HD), il est considere que seulement 25 a 40 % du phosphate inorganique (Pi) epure provient du compartiment extra-cellulaire. Des donnees suggerent que les 60 a 75 % restants pourraient provenir du compartiment intracellulaire. En utilisant la spectroscopie par resonance magnetique du phosphore 31 (31P-MRS), une augmentation de la concentration intracellulaire en Pi durant l’HD chez le porc a ete observee dans un modele d’insuffisance renale aigue. Ce resultat conforte l’hypothese d’une origine intracellulaire du phosphate epure, sans qu’une origine osseuse n’ait pu completement etre ecartee. Le but de notre etude est de verifier si le Pi intracellulaire augmente au cours d’une seance d’hemodialyse chez les patients insuffisants renaux chroniques terminaux (IRCT) en hemodialyse chronique depuis plus de 6 mois. Patients/Materiels et methodes Onze patients IRCT ont ete inclus dans cette etude monocentrique, prospective, interventionnelle ou les concentrations intracellulaires en Pi et en ATP ont ete mesurees par 31P-MRS pendant une seance d’HD standard de 4 heures. Le bilan phosphocalcique, la PTH (debut et fin de dialyse) et la balance calcique ont egalement ete dose. Observation/Resultats Pendant l’HD, la phosphatemie chute rapidement au cours de la premiere heure (−41 %, p Discussion Au vu des resultats de la balance calcique, il apparait peu probable que le compartiment osseux participe a une liberation de phosphate dans le plasma durant l’HD. Conclusion Cette etude confirme pour la premiere fois une origine intracellulaire du phosphate epure pendant l’HD chez des patients IRCT.

Chronic kidney disease is associated with an increased cardiovascular risk, and altered biological properties of high-density lipoproteins (HDL) may play a role in these events. This study aimed to describe the HDL proteome from non-diabetic hemodialysis patients and identify potential pathways affected by the dysregulated expression of HDL proteins. HDL were sampled from nine non-diabetic hemodialysis (HD) and eight control patients. Samples were analyzed using a nano-RSLC coupled with a Q-Orbitrap. Data were processed by database searching using SequestHT against a human Swissprot database and quantified with a label-free quantification approach. Proteins that were in at least five of the eight control and six of the nine HD patients were analyzed. Analysis was based on pairwise ratios and the ANOVA hypothesis test. Among 522 potential proteins, 326 proteins were identified to be in the HDL proteome from HD and control patients, among which 10 were significantly upregulated and nine downregulated in HD patients compared to the control patients (p &lt, 0.05). Up and downregulated proteins were involved in lipid metabolism, hemostasis, wound healing, oxidative stress, and apoptosis pathways. This difference in composition could partly explain HDL dysfunction in the chronic kidney disease (CKD) population and participate in the higher cardiovascular risk observed in this population.

OBJECTIVE Chronic kidney disease is associated with higher morbidity and mortality in patients with diabetes. A low-protein diet is recommended to slow diabetic nephropathy progression because each protein load leads to renal hemodynamic variations. The aim of our study was to evaluate whether the advanced glycation end products (AGE) content of a protein load is responsible for the protein-induced renal hemodynamic variations in humans. RESEARCH DESIGN AND METHODS Ten healthy subjects were assigned to a high-protein (1 g/kg) low-AGE (3,000 kU AGE) versus high-AGE (30,000 kU AGE) meal. Renal perfusion, oxygen consumption, and oxygen content were measured before and 120 min after each meal. RESULTS Renal perfusion (3.2 ± 0.5 vs. 3.8 ± 0.4 mL/min/g; P = 0.0002) and oxygen consumption (0.3 ± 0.04 vs. 0.4 ± 0.08 min−1; P = 0.005) increased significantly after the high-AGE meal compared with the low-AGE meal. CONCLUSIONS Our results suggest that the AGE content of a protein load is responsible for renal hemodynamic modifications. Therefore, prevention of diabetic nephropathy progression could aim predominantly at reducing food AGE content.

We report a case of a patient who had the mitochondrial cytopathy complex of neuropathy, ataxia, and retinitis pigmentosa (NARP) syndrome diagnosed at age 11 years with a biopsy-proven kidney involvement that progressed to end-stage renal disease at age 21 years. Mutations of mitochondrial DNA (mtDNA) are maternally inherited and lead to mitochondrial cytopathies with predominant neurologic manifestations: psychomotor retardation, epilepsy, ataxia, neuropathy, and myopathy. Given the ubiquitous nature of mitochondria, cellular dysfunction can also appear in tissues with high metabolic turnover; thus, there can be cardiac, digestive, ophthalmologic, and kidney complications. Mutations in the MT-ATP6 gene of mtDNA have been shown to cause NARP syndrome without renal involvement. We report a patient who had NARP syndrome diagnosed at age 11 years in whom glomerular proteinuria was present very early after diagnosis. Although neurologic manifestations were stable over time, he developed worsening proteinuria and kidney function. He started dialysis therapy at age 21 years. Kidney biopsy confirmed the mitochondrial cytopathy histologically, with abnormal mitochondria seen on electron microscopy. The MT-ATP6 gene mutation was detected in the kidney biopsy specimen.

Background: Pregnancy in hemodialysis (HD) women is a rare event and often associated with maternal and fetal complications. Scarcity of available data from large cohorts impedes fair medical counseling. Methods: This is a descriptive, retrospective, multi-centric study. Pregnant women on HD during the period from 1985 to 2015 in France were included. The primary outcome was a living infant discharged from hospital, while secondary outcomes included gestational age and birth weight. Results: We identified 100 pregnancies in 84 women on HD, from 41 centers. Chronic HD was initiated during pregnancy for 17.7% (14/79) of patients explaining a 19.8% prevalence of catheter (19/96) and a preserved residual diuresis for 50% of pregnancy (43/86). Seventy-six (89.4%) women performed daily dialysis during the third trimester (6 times per week). Our primary outcome was met for 78% of newborns with a mean gestational age of 33.2 ± 3.9 weeks and a mean birth weight of 1,719 ± 730 g. Conclusions: Our study is one of the largest series of ­pregnancies in HD patients. Despite recent progresses, these pregnancies remain at high risk, reinforcing the need for an early nephrologist-obstetrician skilled team co-management.

Background: Pulmonary arterial hypertension (PAH) may lead to right heart failure and subsequently alter glomerular filtration rates (GFR). Chronic kidney disease (CKD, GFR 2) may also adversely affect PAH prognosis. This study aimed to assess how right heart hemodynamics was associated with reduced estimated GFR (eGFR) and the association of CKD with survival in PAH patients. Methods: In a prospective PAH cohort (2003–2012), invasive hemodynamics and eGFR were collected at diagnosis (179 patients) and during follow-up (159 patients). The prevalence of CKD was assessed at PAH diagnosis. Variables, including hemodynamics, associated with reduced eGFR at diagnosis and during follow-up were tested in multivariate analysis. The association of CKD with survival was evaluated using a multivariate Cox regression model. Results: At diagnosis, mean age was 60.4 ± 16.5 years, mean pulmonary arterial pressure was 43 ± 12 mm Hg, and eGFR was 74.4 ± 26.4 mL/min/1.73 m2. CKD was observed in 52 incident patients (29%). Independent determinants of reduced eGFR at diagnosis were age, systemic hypertension, and decreased cardiac index. Independent determinants of reduced eGFR during follow-up were age, female gender, PAH etiology, systemic hypertension, decreased cardiac index, and increased right atrial pressure. Age ≥60 years, female gender, NYHA 4, and CKD at diagnosis were independently associated with decreased survival. The adjusted hazards ratio for death associated with CKD was 1.81 (95% confidence interval [1.01–3.25]). Conclusion: CKD is frequent at PAH diagnosis and is independently associated with increased mortality. Right heart failure may induce renal hypoperfusion and congestion, and is associated with eGFR decrease.

International audience; Expanded haemodialysis (HDx) has emerged as a promising solution to improve haemodialysis effectiveness. A medium cutoff membrane allows the removal of a wider range of uraemic toxins. However, little is known about the potential interesting applications of HDx therapy. Feedback from the first routine use of HDx therapy under real-life conditions in European facilities was excellent for priming and rinse back. There was no adverse event after 5191 HDx treatments. Patients suffering from itching, restless legs syndrome, persistent asthenia or malnourishment could benefit from HDx therapy. Moreover, we discuss here the promising applications in which HDx could be valuable (myeloma, rhabdomyolysis or cardiovascular diseases). This enthusiastic message is mitigated by reminding why and how prudence should be taken in the design of future HDx studies.

Introduction Les traitements immunosuppresseurs ont revolutionne la survie des patients atteints de maladie a anticorps anti-membrane basale glomerulaire (anti-MBG), neanmoins le pronostic renal reste sombre. Chez les patients avec atteinte renale isolee severe, le benefice d’un traitement intensif reste discutable, notamment en regard du risque infectieux. L’objectif de cette etude etait d’evaluer la survie globale et les complications infectieuses dans cette population. Methodes Il s’agit d’une etude multicentrique retrospective francaise portant sur 201 patients ayants presente une maladie a anti-MBG entre 1997 et 2017. Les criteres de jugement etaient la mortalite globale a 3 ans et les infections severes a 1 an. Resultats obtenus ou attendus La population a ete divisee en 2 groupes en fonction de l’âge (>ou A 1 an, 140 infections severes ont ete repertoriees chez 116 patients. Les facteurs independant associes au risque d’infections a 1 an etaient la presence d’ANCA (HR 1,66) et le taux de CRP au diagnostic (HR 1,02 tous les 10 points). La mediane de suivi etait de 73 mois et la survie estimee a 3 ans etait de 89 %. Apres ajustement sur l’âge, les facteurs associes au deces etaient le diabete (HR 3,15) et l’indice de Charlson modifie (HR 1,38), l’HTA (HR 2,62) et l’infection precoce (HR 3,13). Conclusion La maladie a anticorps anti-MBG est associee a une survie globale satisfaisante conditionnee essentiellement par l’âge, les comorbidites cardiovasculaires et l’infection. Le risque infectieux a 1 an reste important, notamment chez les patients ANCA positifs.

Introduction L’insuffisance renale chronique (IRC) est associee a une importante morbi-mortalite cardiovasculaire. L’alteration des proprietes biologiques des HDL joue un role dans ce sur-risque et des modifications post-traductionnelles (MPT) pourraient etre des mediateurs possibles de la dysfonction de ces lipoproteines. Methodes Caracteriser en spectrometrie de masse les MPT presentes sur les HDL de patients hemodialyses non diabetiques. Les HDL etaient isolees par ultracentrifugations sequentielles, a partir du plasma de 9 patients hemodialysees non diabetiques (HD) et de 9 patients en bilan pour un don de rein (CTL). Les HDL etaient analysees dans un nano-RSLC couple a un spectrometre de masse Q-Orbitrap. Les donnees etaient analysees avec le logiciel Proteome Discover 2.2 en regard de la base de donnee humaine Swissprot. Les proteines ont ete quantifiees grâce a une approche « label-free ». L’oxydation, l’acetylation, la carbonylation (adduits covalents de 4-hydroxy-nonenal ou 4-HNE), la glycation (carboxy-methyl-lysine), la carbamoylation, la guanydinylation, la chlorination, la nitration et la nitrosylation etaient specifiquement recherchees. Resultats obtenus ou attendus Parmi les 522 proteines identifiees dans les HDL, 73 proteines presentaient des MPT. Les principaux adduits retrouves sont la glycation (26 %), la guanidynilation (17 %), la carbamoylation (15 %), la nitration (14 %), la carbonylation par le 4-HNE (11 %), la nitrosilation (9 %) et la chlorination (8 %). Ces proteines sont majoritairement impliquees dans le metabolisme lipidique, la reponse inflammatoire, l’hemostase, la reparation tissulaire et le metabolisme musculaire. En normalisant au nombre de residus acide-amines par proteines, les apolipoproteines A2 et A1 sont les plus modifiees (28 % et 27 % respectivement) suivies par l’albumine (15 %), l’apolipoproteine CIII (9 %) et la proteine serum amyloid A4 (8 %). La plupart des proteines clees du metabolisme des HDL sont la cible de MPT ( Fig. 1 ). Conclusion Les HDL issues de patients HD presentent de nombreuses MPT sur des proteines qui sont impliquees dans les fonctions biologiques des HDL. Ces modifications pourraient expliquer la dysfonction observee des HDL dans l’IRC.

La nephropathie du purpura rhumatoide (ou vascularite a IgA) peut etre grevee d’un pronostic renal severe, particulierement chez l’adulte. Bien que plusieurs classifications histologiques a visee pronostique aient ete developpees, aucune ne fait l’objet d’un consensus. Compte tenu des similitudes physiopathologiques entre la nephropathie a IgA primitive et celle du purpura rhumatoide, certaines etudes ont suggere que la classification d’Oxford pourrait etre utilisee dans le purpura rhumatoide. Nous avons voulu verifier la validite de la classification d’Oxford chez l’adulte caucasien porteur d’une nephropathie du purpura rhumatoide et comparer son eventuel interet pronostique a celui d’autres classifications. Methodes Il s’agit d’une cohorte retrospective et multicentrique incluant des patients porteurs d’une nephropathie du purpura rhumatoide. Les biopsies renales ont ete reclassifiees selon la classification d’Oxford, et en parallele selon la classification ISKDC, la classification decrite par Pillebout et le score GOS. L’association de la classification d’Oxford avec le declin du DFG observee apres la premiere annee a ete analysee de maniere univariee et multivariee. Differents modeles de prediction du declin renal integrant la classification d’Oxford ou les autres classifications ont ete generes et compares entre eux. Resultats obtenus ou attendus 120 adultes porteurs d’une nephropathie du purpura rhumatoide, issus de 7 centres francais et suivis en mediane pendant 7 ans ont ete analyses. La pente moyenne du DFG etait de − 3,3 ml/min/an. Seules les lesions S et T de la classification d’Oxford etaient significativement et independamment des caracteristiques biocliniques initiales, associees au declin de la fonction renale. Parmi les autres classifications, seules celle de Pillebout et le score GOS etaient significativement associees au declin du DFG. Conclusion Nos resultats confirment que la classification d’Oxford pourrait avoir un interet pronostique chez l’adulte caucasien porteur d’une nephropathie du purpura rhumatoide. La performance predictive de la classification d’Oxford dans ce contexte reste cependant modeste (R2

Objectifs L’hypercalciurie est une cause frequente de calculs renaux. Des mutations perte de fonction biallelique de cyp24a1, gene codant pour la vitamine d 24-hydroxylase, et identifiees dans des formes d’hypercalcemie idiopathie neonatale, ont ete egalement identifiees chez des patients avec lithiase renale et/ou nephrocalcinose. Decouverte d’hypercalciurie chez des patients lithiasiques avec mutation heterozygote de la cyp24a1, theoriquement non symptomatiques. Methodes Trois patients, (âge 32 ± 8 ans), ont consulte pour maladie lithiasique active. Les calculs etaient constitues de weddellite ou de brushite. Le bilan phosphocalcique montrait : calcemie normale a 2,34 ± 0,06 mmol/L, pth a 41 ± 13 ng/L (normes 15–65) ; 25-oh vit d a 76 ± 7 nmol/L et 1,25-(oh)2 vit d a 225 ± 98 pmol/L. La calciurie moyenne etait de 5,33 mmol/24 h, soit 0,09 ± 0,01 mmol/kg/j. La consommation sodee et proteique etait normale. Une charge calcique a ete realisee devant l’activite lithiasique importante et/ou l’hypercalciurie avec une absence d’hypercalciurie resorptive et une hypercalciurie absorptive (delta ca/creat a 0,73 n Resultats L’association d’une 1,25-(oh)2 vit d elevee, d’une hypercalcemie a h2 et h4 de la charge calcique avec une pth freinee a motive la recherche de mutation de la 24-hydroxylase. Il a ete identifie une mutation a l’etat heterozygote chez ces 3 patients (c.1124c > t/p.(pro375leu), c1226t > c/p.(leu409ser) et c.1348t > a/p.(phe450ile). Il serait interessant de doser la 24-25ohd2 chez ces patients ( Tableau 1 ). Conclusion Les mutations heterozygotes de cyp24a1 peuvent etre identifiees chez des patients lithiasiques hypercalciuriques avec une 1,25-(oh)2 vit d elevee. Une charge calcique avec hypercalciurie absorptive et freination majeure de la pth peut orienter la recherche d’une mutation de cyp24a1.

Resume Contexte Les troubles anxieux et du sommeil sont frequents chez les patients dialyses et justifient l’utilisation de benzodiazepines. Bien que la duree de traitement ne doive pas exceder 3 mois, beaucoup de patients dialyses utilisent ces molecules au long cours. Les risques d’une telle consommation sont bien connus. Les nephrologues doivent frequemment initier ou poursuivre des traitements par benzodiazepines chez les patients dialyses. Cette prescription est complexe et les etudes relatives aux facteurs associes a leur prise chez cette population particuliere sont pauvres. Objectifs Determiner la prevalence et les facteurs associes a une utilisation au long cours des benzodiazepines chez les patients dialyses, ainsi que la prevalence et les motivations des patients desirant entamer une demarche d’arret suite a l’apport d’une information sur les risques d’une telle consommation. Methodes L’etude a inclus 91 patients dialyses chroniques. Les donnees sont recueillies a partir du dossier medical informatise et au moyen d’entretiens avec les patients. Resultats Au total, 50,5 % des patients (âge moyen : 65,8 ans) consomment une benzodiazepine. La prevalence d’utilisation au long cours est de 78,3 %. Les patients utilisateurs chroniques sont (a) plus âges, (b) moins actifs, (c) plus frequemment atteints de diabete, (d) de depression, (e) plus frequemment en incapacite de marche, (f) moins souvent inscrits sur la liste d’attente de greffe renale, et (g) ont moins souvent beneficie d’une greffe renale prealable. Les doses de benzodiazepines des patients consommateurs au long cours etaient plus elevees que celles des consommateurs sur une courte periode. De plus, 60 % des patients consommateurs chroniques ayant beneficie de l’information desirent entreprendre une demarche d’arret. La constatation d’effets indesirables, l’impression d’inefficacite et la peur de la dependance constituent les motivations les plus souvent identifiees pour justifier le desir d’arret. Conclusions La prevalence de la consommation chronique de benzodiazepines chez les patients dialyses est elevee. L’apport d’une information concernant l’utilisation de ces molecules semble avoir un impact positif sur la demarche d’arret.

Pregnancy in patients presenting end-stage renal disease is rare and there are currently no recommendations for the management of these patients. In hemodialysis patients, reduced fertility and medical reluctance limit the frequency of pregnancies. Although the prognosis has significantly improved, a significant risk for unfavorable maternal (pre-eclampsia, eclampsia) and fetal (pre-term birth, intrauterine growth restriction, still death) outcome still remains. Increasing dialysis dose with the initiation of daily dialysis sessions, early adaptation of medications to limit teratogenicity and management of chronic kidney disease complications (anemia, hypertension) are required. A tight coordination between nephrologists and obstetricians remains the central pillar of the care. In peritoneal dialysis, pregnancy is also possible with modification of the exchange protocol and reducing volumes.

International audience; BackgroundThere is experimental evidence that lethal ischemia-reperfusion injury (IRI) is largely due to mitochondrial permeability transition pore (mPTP) opening, which can be prevented by cyclosporine A (CsA). The aim of our study is to show that a higher dose of CsA (10 mg/kg) injected just before ischemia or a lower dose of CsA (3 mg/kg) injected further in advance of ischemia (1 h) protects the kidneys and improves mitochondrial function.MethodsAll mice underwent a right unilateral nephrectomy followed by 30 min clamping of the left renal artery. Mice in the control group did not receive any pharmacological treatment. Mice in the three groups treated by CsA were injected at different times and with different doses, namely 3 mg/kg 1 h or 10 min before ischemia or 10 mg/kg 10 min before ischemia. After 24 h of reperfusion, the plasma creatinine level were measured, the histological score was assessed and mitochondria were isolated to calculate the calcium retention capacity (CRC) and level of oxidative phosphorylation.ResultsMortality and renal function was significantly higher in the CsA 10 mg/kg-10 min and CsA 3mg/kg-1 h groups than in the CsA 3mg/kg-10 min group. Likewise, the CRC was significantly higher in the former two groups than in the latter, suggesting that the improved renal function was due to a longer delay in the opening of the mPTP. Oxidative phosphorylation levels were also higher 24 h after reperfusion in the protected groups.ConclusionsOur results suggest that the protection afforded by CsA is likely limited by its availability. The dose and timing of the injections are therefore crucial to ensure that the treatment is effective, but these findings may prove challenging to apply in practice.

Chronic kidney disease causes hyperphosphatemia, which is associated with increased cardiovascular risk and mortality. In patients with end-stage renal disease, haemodialysis allows the control of hyperphosphatemia. During a 4-h haemodialysis session, between 600 and 700mg of phosphate are extracted from the plasma, whereas the latter contains only 90mg of inorganic phosphate. The precise origin of phosphates remains unknown. The modelling of phosphorus transfers allows to predict the outcome after changes in dialysis prescription (duration, frequency) with simple two-compartment models and to describe the transfers between the different body compartments with more complex models. Work using

Background: There are only few cases of renal pathology induced by Lyme borreliosis in the literature, as this damage is rare and uncommon in humans. This patient is the first case of minimal change glomerular disease associated with chronic Lyme borreliosis. Case presentation: A 65-year-old Caucasian woman was admitted for an acute edematous syndrome related to a nephrotic syndrome. Clinical examination revealed violaceous skin lesions of the right calf and the gluteal region that occurred 2 years ago. Serological tests were positive for Lyme borreliosis and skin biopsy revealed lesions of chronic atrophic acrodermatitis. Renal biopsy showed minimal change glomerular disease. The skin lesions and the nephrotic syndrome resolved with a sequential treatment with first ceftriaxone and then corticosteroids. Conclusion: We report here the first case of minimal change disease associated with Lyme borreliosis. The pathogenesis of minimal change disease in the setting of Lyme disease is discussed but the association of Lyme and minimal change disease may imply a synergistic effect of phenotypic and bacterial factors. Regression of proteinuria after a sequential treatment with ceftriaxone and corticosteroids seems to strengthen this conceivable association.

International audience

Large middle molecules (LMM) are an important subclass of uremic toxins. Many of them have been linked with poor outcomes in hemodialysis (HD) patients. The onset of high-flux membranes and convective techniques allowed to dramatically improve their clearance but without a clear and undebatable reduction of mortality in HD patients. Despite the real effect on the removal of selected toxins, little is known about the influence of modern HD techniques on the global removal of uremic toxins. Mostly explained by a lack of knowledge and selective assays, LMM removal is not evaluated appropriately. The development of highly sensitive and widespread detection techniques such as mass spectrometry could increase our knowledge about the real state of their removal in HD. Nevertheless, these techniques remain cost effective and are difficult to handle. On the contrary, the improvement of LMM removal raises the question of a tolerable albumin removal. Indeed, increasing membrane permeability can significantly increase LMM removal accompanied by a higher albumin loss. However, in chronic kidney disease and particularly in HD patients, albumin can be modified, and it subsequently exerts detrimental effects. This could be avoided by the clearance of the modified forms in HD, but future efforts should be done to investigate the real impact of their removal.

Background: Animal models are important tools needed to understand the mechanisms underlying the progression of renal disease and to implement new therapeutic approaches. A non-surgical model of chronic kidney disease (CKD) developed by chemical nephrectomy using an adenine-enriched diet has been shown to be a robust model to induce kidney failure in mice and rats. The purpose of this study was to implement an adenine diet to induce CKD in rabbits. Methods: Male New Zealand rabbits were fed for 4 weeks with a diet containing 0.75% (w/w) adenine, and renal function was assessed by measuring plasma urea and creatinine concentrations. The glomerular filtration rate (GFR) was measured using the plasmatic clearance of Iohexol. Kidney histology was performed with haematoxylin erythrosine saffron and Sirius red staining. Results: In contrast to what was observed in rodents, adenine diet failed to induce kidney failure in rabbits as is evident in the plasma concentrations of creatinine and urea and the direct measurement of GFR or histopathological studies. Conclusion: Adenine diet is not a surrogate of subtotal nephrectomy to induce kidney failure in rabbits. Several interspecies differences in metabolism and renal physiology could account for this observation.

Background: In 1998, a French survey showed that the referral of patients with chronic kidney disease to a nephrologist was delayed, resulting in many emergency initiations of dialysis. In 2009, the ORACLE study aimed to describe the renal course of dialysis patients from their first nephrology visit to their first dialysis session. Methods: The ORACLE study was a multicentre retrospective study of all patients who started chronic dialysis. Data were collected at the first nephrology visit and at the first dialysis session. Results: In total, 720 patients were included (69 centres). At the first nephrology visit, the mean Cockcroft-Gault (CG) indicator was 31.8 mL/min (22.7 in 1998) and 52.4% of patients (73% in 1998) had a CG

International audience; BACKGROUND AND OBJECTIVES: Adequate estimation of renal function in obese patients is essential for the classification of patients in CKD category as well as the dose adjustment of drugs. However, the body size descriptor for GFR indexation is still debatable, and formulas are not validated in patients with extreme variations of weight. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This study included 209 stages 1-5 CKD obese patients referred to the Department of Renal Function Study at the University Hospital in Lyon between 2010 and 2013 because of suspected renal dysfunction. GFR was estimated with the Chronic Kidney Disease and Epidemiology equation (CKD-EPI) and measured with a gold standard method (inulin or iohexol) not indexed (mGFR) or indexed to body surface area determined by the Dubois and Dubois formula with either real (mGFRr) or ideal (mGFRi) body weight. Mean bias (eGFR-mGFR), precision, and accuracy of mGFR were compared with the results obtained for nonobese participants (body mass index between 18.5 and 24.9) who had a GFR measurement during the same period of time. RESULTS: Mean mGFRr (51.6 +/- 24.2 ml/min per 1.73 m(2)) was significantly lower than mGFR, mGFRi, and eGFRCKD-EPI. eGFRCKD-EPI had less bias with mGFR (0.29; -1.7 to 2.3) and mGFRi (-1.62; -3.1 to 0.45) compared with mGFRr (8.7; 7 to 10). This result was confirmed with better accuracy for the whole cohort (78% for mGFR, 84% for mGFRi, and 72% for mGFRr) and participants with CKD stages 3-5. Moreover, the Bland Altman plot showed better agreement between mGFR and eGFRCKD-EPI. The bias between eGFRCKD-EPI and mGFRr was greater in obese than nonobese participants (8.7 versus 0.58, P\textless0.001). CONCLUSIONS: This study shows that, in obese CKD patients, the performance of eGFRCKD-EPI is good for GFR \textless/= 60 ml/min per 1.73 m(2). Indexation of mGFR with body surface area using ideal body weight gives less bias than mGFR scaled with body surface area using real body weight.

Introduction Le traitement par dialyse peritoneale (DP) reste minoritaire en France. Une des hypotheses expliquant cette situation est la formation, encore insuffisamment developpee, des nephrologues en dialyse peritoneale. L’objectif de ce travail est de realiser un etat des lieux de l’experience des internes en dialyse peritoneale, afin de mettre en lumiere les faiblesses, mais aussi les forces de leur formation. Connaitre l’aisance des internes en nephrologie en DP selon leur anciennete, leur experience et leur lieu de formation et determiner les facteurs influencant l’impression de bonne maitrise de la technique, permettra de souligner les pistes d’amelioration. Methodes En 2015, un questionnaire a ete envoye par mail a l’ensemble des internes de nephrologie des centres hospitaliers universitaires de Lyon, Saint-Etienne, Clermont-Ferrand, Grenoble, Besancon et Strasbourg. Resultats obtenus ou attendus Cette enquete retrouve de fortes disparites regionales d’enseignement pratique et theorique en DP. Elle souligne un manque de formation des internes par rapport a ce traitement de suppleance. La grande majorite des internes choisirait cette technique s’ils devaient dialyser. Plus de la moitie des internes interroges a pris en charge moins de dix patients traites par dialyse peritoneale et le plus souvent, lors de complications liees, ou non, a la DP. Tres peu d’internes ont vu plus de cinq patients lors d’un suivi programme. Moins de la moitie des internes se sentirait capable de gerer seule une infection du liquide de dialyse peritoneale, complication aigue la plus frequente. Conclusion Heureusement, plus la formation theorique et pratique des internes en dialyse peritoneale est developpee, plus les internes de nephrologie se sentent capables de gerer les patients en dialyse peritoneale.

Since a couple of years, a new paradigm of care has taken place where the patient plays a major role in the choice of his treatment. Patient information in a context where the doctor-patient relationship has drastically changed and where knowledge is continuously growing, is a challenge for the nephrologist. For the patient who decides to undergo home dialysis, numerous questions arise during the decision-making process. How should treatment alternatives be discussed with the patient? What are the current data available on the different methods? As comfort, quality of life and level of autonomy often drive the choice of care management, home dialysis can be promoted more with appropriate assistance from both the nephrologist and the healthcare team. Cet article fait partie du numero supplement Innovations en Nephrologie realise avec le soutien institutionnel de Vifor Fresenius Medical Care Renal Pharma.

Objective: Systemic lupus erythematosus (SLE) is a prototype autoimmune disease, assumed to occur due to a complex interplay of environmental and genetic factors. Rare causes of monogenic SLE have been described, providing unique insights into fundamental mechanisms of immune tolerance. Here, our objective was to identify the cause of an autosomal recessive form of SLE. Methods: We investigated three siblings from one consanguineous kindred with juvenile-onset SLE and used next generation sequencing to identify mutations in the disease-associated gene. We performed extensive biochemical, immunological and functional assays to assess the impact of the identified mutations on B cell biology. Results: We identified a homozygous missense mutation in PRKCD, encoding protein kinase delta (PKC?) in all three affected siblings. Mutation of PRKCD resulted in reduced expression and activity of the encoded protein PKC?, involved in the deletion of autoreactive B cells, leading to a resistance to BCR- and calcium-dependent apoptosis and increased B cell proliferation. Thus, as for mice deficient in PKC?, which exhibit an SLE phenotype and B cell expansion, we observed an increase of immature B cells in affected patients, and a developmental shift toward an immature phenotype of na�ve B cells. Conclusion: Our findings indicate that PKC? is crucial in regulating B cell tolerance and preventing self-reactivity in humans, and that PKC? deficiency represents a novel genetic defect of apoptosis leading to SLE. � 2013 American College of Rheumatology.

Many quantitative clinical, biological and radiological parameters are useful in medicine for guiding diagnosis and therapy. The nephrologists use such parameters on a daily basis, particularly to assess renal function. In order to use the measurement methods of these parameters in clinical practice, it is mandatory to establish the agreement of the values provided, comparatively to the value obtained by using the reference measurement method of this parameter. This review details the methodology for the validation of methods for estimating quantitative parameters.

Purpose To evaluate long-term results of endovascular procedures in treatment of venous juxta-anastomotic stenoses (JASs) of native forearm radiocephalic arteriovenous fistulas (AVFs) and to identify prognostic factors influencing these results. Materials and Methods During a 124-month period, 147 endovascular interventions were performed in 75 forearm radiocephalic AVFs with JASs defined as stenoses located within the first 5 cm of the outflow vein. Prognostic factors included patient characteristics (age, sex, diabetes), AVF-related characteristics (location on forearm, age, maturity), stenosis-related characteristics (position relative to anastomosis, length, and degree), and degree of residual stenosis and delay of restenosis after the first endovascular procedure. Results At 1 and 3 years, access primary patency (PP) rates were 46.6% (95% confidence interval [CI], 36.3%–59.9%) and 25.5% (95% CI, 15.7%–41.6%) and assisted PP (APP) rates were 81.3% (95% CI, 72.6%–91.1%) and 63.2% (95% CI, 50.6%–79.0%), respectively. Stenosis degree of 50%–75% ( P = .017), stenosis length of 10 mm or more ( P = .017), and time before first restenosis of less than 6 months ( P = .03) significantly increased the frequency of endovascular procedures during follow-up. However, only the degree of residual stenosis after the first endovascular treatment significantly affected long-term APP ( P = .039). When residual stenosis was less than 50%, 1- and 2-year access APP rates were 84.6% (95% CI, 75.8%–94.4%) and 76.1% (95% CI, 64.6%–89.6%), respectively. When it was at least 50%, the respective APP rates were 62.3% (95% CI, 38.9%–99.9%) and 46.8% (95% CI, 22.4%–97.7%). Conclusions Endovascular treatment of JASs in forearm radiocephalic AVFs provides good long-term results except when the residual stenosis after the first procedure is 50% or more. In that case, the optimal treatment remains to be determined.

Introduction Chronic hepatitis C virus (HCV) infection is the most common chronic liver disease in patients with end stage renal disease (ESRD) and is well known as a frequent cause of mortality and graft loss among haemodialysed and kidney transplant patients. Up to now, there are no data on antiviral efficacy and tolerability of available protease inhibitors (telaprevir and boceprevir) in HCV infected haemodialysed patients. Methods We report 4 cases of HCV infected haemodialysed patients, who have not responded to a prior course of pegylated interferon (Peg-IFN) and ribavirin (RBV) and who were listed for kidney transplantation (KTx). These 4 patients received a second-line antiviral treatment with Peg-IFN, RBV and telaprevir. Results After 12 weeks of triple therapy, tolerability was acceptable and HCV-RNA became undetectable in 3/4 patients. Mild side-effects included anaemia leading to increasing the doses of erythropoietin (EPO). Dose of RBV ranged from 200 mg three times a week to 200 mg/day. Conclusion Triple therapy with a first generation protease inhibitor could be the new standard for the treatment of HCV patients with ESRD. This needs to be confirmed by larger series.

There are few epidemiologic data on Chronic Kidney Disease management before replacement therapy. The two objectives of the PREPARE study were (1) to describe the characteristics of these patients and accordance to clinical practice guidelines (2) to study nephrologists preference for renal replacement therapy in case of progression to end stage renal disease. PREPARE is a non-interventional cross-sectional study. All the French nephrologists had been solicited to collect information about CKD outpatients not on dialysis, not transplanted, with glomerular filtration rate lower than 60mL/min/1,73m(2), followed on any day between 23 and 27 November 2009. Three hundred and eight investigators included 2089 patients, 59% of them were male, they were on average 69 years old, 15, 37 and 48% had respectively a CKD stage V, stage IV and stage III, the nephropathy was the most often (43%) vascular. The most frequently reported cardiovascular risk factors were hypertension (88%), hypercholesterolemia (53%), diabetes (37%). The average time between diagnosis of nephropathy and the first nephrology consultation was too long 1,5 years. The implementation measures of nephroprotection and treatment of complications of CKD were generally satisfactory. However, preparation for replacement therapy was often too late, haemodialysis was more likely scheduled instead of peritoneal dialysis and without preparation for renal transplantation. PREPARE can therefore highlight the qualities of the current management of CKD by nephrologists in France. Nevertheless, PREPARE also shows weaknesses in preparation for replacement therapy. One can suggest that they could be reduced by systematic access of patients with risk of progression to stage V, as soon as the stage IV, to structured multidisciplinary care.

Thrombotic microangiopathy is a rare but severe complication of treatment with gemcitabine. Its prevalence increases because gemcitabine's indications are growing. We report four cases, which presented with common clinical and biological manifestations, i.e. high blood pressure, proteinuria and increasing plasmatic creatinine level. However, severity was not similar, hemodialysis was inconstant. There is no consensus on treatment for this condition. Stopping gemcitabine is essential. Treatment was dispensed considering the severity of the presentation: plasma exchange therapy of variable outcome, and eculizumab, which was efficient when used. It's important to note that this syndrome includes common and frequent signs in patients receiving chemotherapies. But they must encourage the research of most specific signs, such as hypertension, mechanic hemolysis signs, proteinuria or hematuria, in order to recognize thrombotic microangiopathy as early as possible to treat it precociously, and to prevent additional gemcitabine injections.

Kidney dysfunction during congestive heart failure, although frequent, is often neglected. Yet, it represents a life-threatening condition, oven when the kidney dysfunction is moderate. The initial approach involvus strict application of recommendations, cardiologic and nephrologic joined management and close follow-up involving patient's general practitioner. Cases of true diuretics resistance are infrequent and late. Yet, it represents a significant turning point. Mortality is high, with a major individual unpredictability. A multidisciplinary approach is needed, which has to take into account patient's preferences. Several treatments may be discussed and are sometimes joined: cardiac transplantation, water and salt extraction (using ultrafiltration, hemodialysis or peritoneal dialysis), vasoconstrictive drugs, ventricular assistance devices and palliative care. Water and salt extraction techniques seem to space out hospitalizations and to provide symptomatic relief even though no benefit on patient survival has been demonstrated to date. The need for randomized clinical trials is mandatory.

International audience; Of the 600-700 mg inorganic phosphate (Pi) removed during a 4-hour hemodialysis session, a maximum of 10% may be extracted from the extracellular space. The origin of the other 90% of removed phosphate is unknown. This study tested the hypothesis that the main source of phosphate removed during hemodialysis is the intracellular compartment. Six binephrectomized pigs each underwent one 3-hour hemodialysis session, during which the extracorporeal circulation blood flow was maintained between 100 and 150 ml/min. To determine in vivo phosphate metabolism, we performed phosphorous (P-31) magnetic resonance spectroscopy using a 1.5-Tesla system and a surface coil placed over the gluteal muscle region. P-31 magnetic resonance spectra (repetition time = 10 s; echo time = 0.35 ms) were acquired every 160 seconds before, during, and after dialysis. During the dialysis sessions, plasma phosphate concentrations decreased rapidly (-30.4 %; P=0.003) and then, plateaued before increasing approximately 30 minutes before the end of the sessions; 16 mmol phosphate was removed in each session. When extracellular phosphate levels plateaued, intracellular Pi content increased significantly (11%; P\textless0.001). Moreover, beta ATP decreased significantly (P\textless0.001); however, calcium levels remained balanced. Results of this study show that intracellular Pi is the source of Pi removed during dialysis. The intracellular Pi increase may reflect cellular stress induced by hemodialysis and/or strong intracellular phosphate regulation.

International audience; We report a case of a 37-year-old man with Maturity Onset Diabetes of the Youth (MODY) type 5, admitted for an episode of cholestasis and a simultaneous acute kidney injury (AKI). Chronic liver disease was due to a mutation in the transcription factor 2 (TCF2) gene, thus highlighting the need for a close liver follow-up in these patients. AKI was attributed to a cholemic nephropathy based on the following rationale: (1) alternative diagnoses were actively ruled out; (2) the onset of AKI coincided with the onset of severe hyperbilirubinaemia; (3) renal pathology showed large bile tubular casts and a marked tubular necrosis and (4) creatinine serum dramatically decreased when bilirubin levels improved after the first sessions of extracorporeal albumin dialysis (ECAD), thus suggesting its role in renal recovery. Even though cholestasis can precipitate renal injury, the diagnosis of cholemic nephropathy could require a renal biopsy at times. Future studies should confirm the benefits of ECAD in cholemic nephropathy.

Endovascular revascularization as treatment of atherosclerotic renal-artery stenosis (aRAS) is controversial since 3 large and multicentric randomised trials (CORAL, ASTRAL, STAR) failed to prove the superiority of percutaneous transluminal renal-artery stenting (PTRAS) over medical treatment only (MT). However, considering the multiple bias of these trials, among which questionable inclusion criterias, these results must be extrapolated in clinical practice with caution. New pathophysiological data have been helping to understand why restoring blood flow does not necessarily lead to kidney function improvement. Today, the diagnostic approach must in one hand confirm the artery stenosis and on the other hand assess its severity and impact on the kidney. Therapeutic options still lie on the American guidelines published in 2006, since no study data can be reasonably used in everyday practice. However, particular sub-groups of patients who could benefit from revascularisation have been identified through recent cohort studies. Further prospective studies are needed in order to confirm the superiority of PTRAS in these populations. Meanwhile, multidisciplinary approach should be promoted, in order to provide the best treatment for each patient.

Atherosclerosis is an important predictor of mortality in patients with chronic kidney disease (CKD) and is associated with a wide inflammatory response. The aim of this study is to evaluate in vitro how different membranes can remove mediators associated with this pathology in a closed loop dialysis model. We performed experimental hemofiltration in vitro using three different membrane materials. Human plasma was preliminarily incubated with various inflammatory mediators and filtered in a closed loop circulation model for 240 min. Respective concentrations of 17 different mediators were measured over time to study the removal mechanisms of each membrane, including associated removal time course. The experiment was repeated three times for the assay of tumor necrosis factor (TNF)-α to document the model variability. Means were compared using Mann-Whitney test. Most of the investigated mediators were effectively removed with the different dialysis membranes. Adsorption mechanism was mainly at the origin of the decrease in mediators circulating concentrations and was maximized in the region 10 000-20 000 Da. Especially, the HeprAN membrane showed fast removal capacities of mediators with elevated isoelectric point including complement factors and chemokines or having basic groups located in the protein periphery, plasminogen activator inhibitor (PAI-1), and TNF-α-like. The latter was further significantly removed with HeprAN and polymethylmethacrylate (PMMA) compared to polyethersulfone (PES) material (P 0.01). We concluded that dialysis using ionic adsorptive membrane could have a beneficial impact for CKD patients with atherosclerosis and would deserve further clinical investigations.

International audience; PURPOSE: To evaluate clinical and multidetector computed tomography (MDCT) features associated with the presence and size of microaneurysms in renal angiomyolipomas (AMLs). MATERIALS AND METHODS: The MDCTs and digital subtraction angiographies (DSAs) of 31 patients who had further percutaneous arterial embolization of AMLs were retrospectively reviewed. They were 22 women and 9 men (mean age, 47.7+/-27.7 years). The medical files of the included patients were reviewed for age, gender and clinical features. MDCT and DSA images were analyzed by two readers working in consensus. RESULTS: Of the 31 patients, 15 had tuberous sclerosis complex (TSC) or lymphangioleiomyomatosis (LAM). In total, the 31 patients had 54 AMLs (5 ruptured). On DSA, 28 clusters of microaneurysms were found in 17 patients (21 AMLs). Four of the five ruptured AMLs had microaneurysms. None of the 12 AMLs\textless/=40mm and 21 of the 42 AMLs\textgreater40mm had microaneurysms. Among AMLs\textgreater40mm, history of TSC/LAM (P=0.5), RENAL score (P=0.7) and relative volume of fat (P=0.11) did not significantly predict the presence of microaneurysms. Microaneurysms were significantly larger in ruptured (9.5+/-5.7mm) than non-ruptured (3.9+/-1.9mm, P=0.02) AMLs. No associations were found between the size of microaneurysms and the size of AMLs. CONCLUSION: Microaneurysms were found in no AML \textless/=40mm and in 50%of AMLs\textgreater40mm. In AMLs \textgreater40mm, history of TSC/LAM, RENAL score and relative volume of fat did not significantly predict the presence of microaneurysms.