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Le retentissement cardiaque de la fistule artérioveineuse (FAV) chez les dialysés reste un sujet de controverse. Il s'agit d'une étude transversale incluant tous les hémodialysés chroniques via une FAV proximale. Cette étude a pour objectif d'étudier l'évolution clinique, biologique et écho cardiographique après la confection d'une FAV proximale. Nous avons colligé 24 patients dont l'âge moyen était 55,08 ans et un sex-ratio H/F de 1,4. Les néphropathies diabétiques et vasculaires constituaient les principales néphropathies initiales. La durée moyenne en hémodialyse via la FAV était de 66,75 mois (4–156 mois). La pression artérielle systolique baissait significativement après la confection de la FAV (p = 0,001). L'évolution des paramètres échocardiographiques était marquée une augmentation de diamètre télé systolique et télé diastolique de ventricule gauche (p = 0,004) une diminution de la fraction d'éjection de VG (FEVG) (p = 0,014). Un épaississement du septum interventriculaire et une augmentation des pressions de l'artère pulmonaire étaient observées mais sans différence statistiquement significative. Les principales complications après la confection d'une FAV proximale étaient un syndrome coronarien aigu chez 5 patients, une fibrillation auriculaire chez 4 patients, une péricardite chez 2 patients, et une cardiomyopathie dilatée chez un patient après un délai moyen de 60 mois, 35 mois, 60 mois et 48 mois respectivement. Les complications cardiovasculaires constituent la première cause de morbimortalité chez les hémodialysés chroniques. La création de la FAV peut avoir un retentissement cardiaque à cause de passage de sang de circulation artérielle vers la circulation veineuse. Une surveillance clinique et écho cardiographique régulière est nécessaire. [ABSTRACT FROM AUTHOR]

Introduction La tuberculose demeure un problème de santé publique en Tunisie. La fréquence particulière de cette infection au cours de l’insuffisance rénale chronique et en dialyse est due aux anomalies de l’immunité cellulaire, la dénutrition et l’inflammation. La non-spécificité de la symptomatologie, mimant souvent les signes d’urémie est à l’origine du retard diagnostique chez ces malades. La difficulté diagnostique de la tuberculose en dialyse est multifactorielle. Le caractère multifocal, la fréquence des localisations extrapulmonaires et l’immunodépression expliquent la négativité des prélèvements bactériologiques et histologiques chez les dialysés. Patients et méthodes Il s’agit d’une étude rétrospective réalisée sur une période de 10 ans intéressant 53 pris en charge pour une tuberculose survenue en dialyse. L’objectif de cette étude est d’analyser la démarche diagnostique de la tuberculose en dialyse (TBC). Résultats Le diagnostic de la TBC était retenu chez 53 patients. La moyenne d’âge était de 54 ans avec un sex-ratio H/F de 0,96 (27 femmes et 26 hommes). Quarante-sept malades (88,5 %) étaient en hémodialyse et six (11,5 %) en dialyse péritonéale. Le délai moyen de la survenue de la TBC par rapport au début de l’épuration extra rénale était de 17,7 mois [1 ; 26,3]. Un niveau socioéconomique défavorable était noté dans 71 % des cas. Trente-trois pour cent n’étaient pas vacciné contre le bacille de Koch et 12 % avaient des antécédents de tuberculose pulmonaire avant la dialyse. La symptomatologie clinique et biologique était très variable et non spécifique. En effet, le tableau était dominé par les signes généraux avec une altération de l’état général dans 50 cas (49 %), un amaigrissement non expliqué dans 45 cas (84,9 %), une fièvre prolongée dans 26 cas (49 %), une toux dans 19 cas (35,8 %), des douleurs thoraciques chez 15 patients (28,3 %) et une dyspnée dans 30 cas (56,6 %). La biologie avait relevé un syndrome inflammatoire biologique inexpliqué chez 48 patients (90,6 %), une anémie récente ou une résistance à l’érythropoïétine dans 47 cas (88,7 %), une hyperferritinémie chez 18 malades (56,3 %), une calcémie élevée ou anormalement normale chez 27 malades (50,9 %) associée à un taux de parathormone relativement bas dans 16 cas (30,8 %). L’IDR à la tuberculine était positive dans 13 % des cas. Le diagnostic positif était retenu sur des éléments bactériologiques dans 17 cas (32,07 %) : le Mycobacterium tuberculosis était isolé dans les crachats chez 6 patients (11,3 %), dans les urines dans 4 cas (7,5 %), dans le liquide d’ascite chez 2 malades (3,7 %), dans le liquide pleural chez un patient (1,88 %), dans la ponction ganglionnaire dans 3 cas (5,66 %) et dans un prélèvement cutané chez un malade (1,88 %). L’étude anatomopathologique était en faveur de la TBC dans 11 % des cas (6 patients). Il s’agit de granulomes tuberculoïdes avec une nécrose caséeuse dans 4 cas et d’un granulome sans nécrose chez 2 malades. L’étude histologique avait concerné des adénopathies cervicales dans 4 cas, du parenchyme pulmonaire et pleural dans respectivement un cas. Devant la négativité des prélèvements bactériologiques et histologiques, le diagnostic de la TBC était retenu sur des éléments de présomption clinicobiologiques et radiologiques chez 28 malades (56 %). Parmi ces patients, 21 malades (75 %) avaient évolués favorablement sous traitement antituberculeux. Conclusion Le pronostic des malades atteints de TBC est étroitement lié à la précocité de la prise en charge et la de mise en route du traitement dans les plus brefs délais. Une démarche basée sur l’analyse du terrain, de la symptomatologie clinicobiologique, la répétition des prélèvements et les nouvelles méthodes diagnostiques est la clé du diagnostic de cette maladie. [ABSTRACT FROM AUTHOR]

Introduction Les complications cardiovasculaires constitient un enjeu majeur au cours du lupus érythémateux systémique (LES). Le risque cardiovasculaire observé au cours du LES est multifactoriel. Il résulte de l’effet synergique des facteurs de risque cardiovasculaires classiques, de la maladie elle-même et de ses traitements. La rigidité aortique par mesure de la vitesse de l’onde de pouls (VOP) constitue désormais un outil validé de l’évaluation du risque cardiovasculaire. L’objectif de notre étude est de savoir si la VOP est augmentée chez les patients atteints de LES asymptomatiques pour une maladie cardiovasculaire comparée aux patients atteints de néphropathie tubulo-interstitielle chronique, et de trouver une corrélation entre la VOP chez les patients atteints de SLE et l’activité de la maladie, la prise de corticoïdes et les facteurs de risque traditionnels de l’athérosclérose. Patients et méthodes Nous avons inclus 30 patients suivis pour LES (2 hommes et 28 femmes) qui ont au moins 4 des critères ACR pour le LES. Dix-sept témoins (2 hommes et 15 femmes) suivis pour néphropathie tubulo-interstitielle appariés pour l’âge et le sexe ont été sélectionnés. Nous avons analysé des données épidémiologiques, anamnestiques, cliniques et biologiques sanguins des deux populations. La rigidité aortique a été évaluée en mesurant la VOP à l’aide d’un dispositif Complior. Les données ont été comparées entre les patients atteints de LES et les témoins en utilisant le test Chi 2 pour les variables dichotomiques et le test U de Mann-Whitney pour les données quantitatives avec une distribution non gaussienne. Une différence significative a été définie par une p < 0,05. Résultats La VOP n’était pas statistiquement plus élevé chez les patients atteints de LES comparativement aux témoins ( p = 0,801). Chez les patients atteints de LES, la VOP était corrélée positivement à l’âge, à la pression artérielle systolique et à l’indice SLEDAI et négativement au débit de filtaration glomérulaire. Aucune corrélation n’a été observée entre la VOP et la cholestérolémie et la durée du traitement par les corticoïdes. La VOP était positivement corrélée à la pression artérielle chez les patients atteints de SLE, même chez les patients normotendu. Conclusion La rigidité aortique évaluée par la mesure de la VOP permet le dépistage de la maladie athéromateuse de manière précoce au cours du LES. Ces résultats soulignent l’importance de la mesure de la VOP dans la prise en charge du LES. La mesure de la rigidité aortique chez ces patients améliore l’estimation du risque cardiovasculaire permettant ainsi l’instauration d’une prévention plus efficace. [ABSTRACT FROM AUTHOR]

Introduction L’atteinte rénale est très fréquente lors des vascularites pauci-immune. Elle est caractérisée histologiquement par la présence d’une prolifération extracapillaire en microscopie optique et par l’absence de dépôts en immunofluorescence. Patients et méthodes Afin d’identifier les corrélations entre les manifestations cliniques les plus fréquentes des glomérulonéphrites extracapillaires et les atteintes histologiques, nous avons mené une étude univariée portant sur 37 dossiers. Tous les patients étaient biopsiés au service de néphrologie La Rabta durant la période de 2007 à 2016. Un nombre minimum de 10 glomérules était nécessaire pour interpréter les biopsies rénales. Résultats Trente-sept patients (18 hommes et 19 femmes) ont fait l’objet de cette étude. L’âge moyen était de 54 ans (17–80). La diurèse était conservée chez 22 patients (59,5 %). L’oligoanurie était associée à la présence d’un infiltrat interstitiel de 25–50 % ( p = 0,06). La protéinurie de 24 heures était en moyenne de 2,25 g/l. Elle était associée à la présence d’un granulome périglomérulaire ( p = 0,04) et la présence d’une prolifération endocapillaire ( p = 0,04) et d’une fibrose interstitielle ( p = 0,03). L’hématurie était présente dans 97,3 %. Elle était associée à la présence d’un infiltrat interstitiel ( p = 0,02). Seize malades soit 43,24 % ont évolué vers l’insuffisance rénale terminale après un délai moyen de 8 mois. Parmi les facteurs de mauvais pronostic rénal, on a noté la présence de glomérules scléreux ( p = 0,004). Conclusion La fibrose glomérulaire ainsi que la fibrose tubulo-interstitielle constituent les facteurs irréversibles de mauvais pronostic rénal. La prise en charge impose une orientation rapide afin d’adapter le traitement et d’éviter l’évolution vers la fibrose rénale. [ABSTRACT FROM AUTHOR]

The interplay between human microbiota and various physiological systems has garnered significant attention in recent years. The gut microbiota plays a critical role in maintaining physiological homeostasis and influences various aspects of human health, particularly via the gut brain axis. Since 2017, the challenging concept of the gut-retina axis has emerged thanks to a network analysis emphasizing the potential role of the gut microbiota disruption in the development of the age-related macular degeneration and further retinal damages. Many other ocular disorders have been linked to the dysbiosis of the gut microbiota, including uveitis and glaucoma. It has been shown that age related macular degeneration can be prevented or reversed using a diet that induces changes in the gut microbiota. The potential link between the gut microbiota as well as others types of microbiota such as the ocular surface microbiota and the development/progression of age related as well as inherited retinal degenerations and other degenerative eye diseases, has recently been broadened. Therefore, the pathogenesis of several eye diseases has recently been associated with a larger perception called the gut eye axis. This mini-review examines the potential mechanisms underlying the gut eye axis and suggests implications for the management of eye diseases. By understanding the modulation of the gut microbiota and its impact on eye disease, this mini-review provides insight into potential therapeutic interventions and avenues for future research., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Kammoun, Rekik, Dlensi, Aloulou, Smaoui, Sellami, Trigui, Gargouri, Chaari, Sellami, Elatoui, Khemakhem, Hadrich, Neji, Abdelmoula and Bouayed Abdelmoula.)

Chronic kidney disease (CKD) treated by hemodialysis (HD) is a worldwide major public health problem. Its incidence is getting higher and higher, leading to an alarming social and economic impact. The survival of these patients is significantly low, especially during the first year of treatment. The purpose of our study was to identify the epidemiological and clinico-biological characteristics of patients at the HD initiation and to reveal the predictive factors of mortality at three months and one year of HD. This is a prospective, analytical, and descriptive study dealing with 229 patients with an end-stage renal disease (ESRD), followed up in the Nephrology Department of Charles Nicolle Hospital and La Rabta Hospital in Tunisia, that was started HD between January and June 2017. A multivariate logistic regression analysis allowed us to identify the independent predictors of mortality at three months and one year. The average age was 60.2 ± 15.3 years, with a gender ratio of 1.41. Seventy-eight percent of patients had more than two comorbidities, 59% had diabetes, and 88% had hypertension. Diabetic nephropathy was the leading etiology of kidney disease (48.9%), while 11% of nephropathies were of unknown etiology. Only 58% were early referred to a nephrologist. The average glomerular filtration rate at HD initiation was 6.06 ± 2.33 mL/min/1.73 m 2 . Hypocalcemia and hyperphosphatemia were noted, respectively, in 60.8% and 84.9% of cases. Anemia was objectified in 98.6% of cases. HD was started in an emergency in 56.8% of cases. One of the most urgent indications was acute pulmonary edema (APE) for 43.8% of patients. Only 10.5% of patients had functional arteriovenous fistula at the dialysis initiation. Patients were hemodialyzed one, two, or three sessions per week, respectively, in 23.2%, 26.6%, and 50.2% of cases. The crude mortality rate was 25% and 13% in, respectively, one year and three months of HD. On multivariate analysis, we identified heart failure and insufficient dialysis dose per week as predictive factors of mortality at the 1 st year of HD. C-reactive protein more than 21 mg/L, insufficient dialysis per week, modified Charlson Comorbidity Index less than 6, and APE at the dialysis initiation were identified as predictive factors of three-month mortality. Despite the short period of study, this work revealed the alarming conditions of patients at HD initiation. This critical situation is due to the delay in CKD diagnosis, the late nephrologist referral, and the lack of preparation before HD initiation.

Cardiovascular disease is the leading cause of death of dialyzed patients. Aortic stiffness, evaluated by the carotid-femoral pulse wave velocity (cfPWV), is now considered as a prognostic factor for cardiovascular mortality in patients with chronic kidney diseases. The peritoneal dialysis (PD) patients had significantly stiffer arteries. cfPWV was 9.12 ± 2.7 m/s in PD patients without significant correlation compared to hemodialysis (HD) patients (8.97 ± 2.52 m/s). In the univariate study, we found a statistically significant correlation between PWV and age (P = 0), between the pulse wave velocity and phosphorus (P = 0.46), between the VOP and PTH (P = 0.013) and between PWV and dyslipidemia (P = 0.014). Other variables such as phospho-calcic product, hemoglobin, total cholesterol, and KT/V were not significant. To identify the risk factors independently linked to the event, we conducted a multi-varied analysis. A correlation was found between VOP and dyslipidemia (P = 0.008). The other variables were insignificant.

Carotid-femoral pulse wave velocity (cf-PWV) is the noninvasive gold standard technique for measuring aortic stiffness. Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic renal disease in adults. It is associated with a high risk of cardiovascular complications. We aimed to assess the prevalence of increased arterial stiffness and its predicting factors in a population of ADPKD patients. Sixty-two patients with ADPKD underwent noninvasive measurement of cf-PWV using a COMPLIOR Analyse device. Recruitment period was 17 months and we used the cut-off of 10 m/s to define a high cf-PWV. Mean age was 51 ± 12.7 years. Gender ratio male/female was 0.63. Smoking, hypertension (HTN), and dyslipidemia were reported in 14%, 66%, and 27% of the cases, respectively. Mean glomerular filtration rate (GFR) was 47.7 ± 44 mL/min/1.73 m 2 . Among our patients, 39% had chronic kidney disease stages 1 or 2 and 45% stage 5 (40% stage 5D). Mean cf-PWV was 9 ± 2.4 m/s, and 31% of the patients had a high cf-PWV. In univariate analysis of all our patients, cf- PWV correlated with age (r = 0.565; P <10 -3 ), GFR (r = -0.268;P = 0.035), C-reactive protein (r = 0.447; P = 0.007), peripheral systolic arterial pressure (r = 0.309; P = 0.015), and peripheral pulse pressure (r = 0.335; P = 0.008). Patients with high cf-PWV were on average nine years older than the others. Patients with HTN were 3.84 times more likely to have high cf-PWV (P = 0.046). cf-PWV did not seem to be lower with any antihypertensive treatment. A level of C-reactive protein higher than 10 mg/L was the only independent predicting factor of a high cf-PWV in multivariate analysis (P = 0.043). Our study confirmed the relationship between cf-PWV and age, renal failure, and HTN in patients with ADPKD. It also emphasized the close relationship between systemic inflammation and arterial stiffness in this nephropathy.

Introduction: Mechanisms underlying bone fragility in patients under dialysis are various. The assessment of bone disorder is not yet codified in these patients. Our study aimed to determine the relationship between the serum fibroblast growth factor 23 (FGF23) level and bone fragility. We also aimed to assess the bone alkaline phosphatase (bAP) to the C-terminal telopeptide of type I (CTX) ratio and the FGF23*bAP product to CTX ratio in patients under hemodialysis., Methodology: We conducted a cross-sectional study, including 76 patients under hemodialysis. To assess bone fragility, we measured bAP, CTX, and FGF 23. We calculated the bAP to the CTX ratio (bAP/CTX) and the FGF23*bAP product to the CTX ratio (FGF23*bAP/CTX). We defined bone fragility as the existence of osteoporosis or fragility fractures. Receiver operating characteristic (ROC) curves were evaluated for each biological using the existence of osteoporosis or fragility fracture as the gold standard for bone fragility., Results: There were 51 men. The mean age was 53.36 ± 14.27 years. Bone fragility was noted in 25 cases. Patients with osteoporosis had higher FGF*bAP/CTX and bAP/CTX ratios. The ability of the ratio (bAP/CTX) to distinguish patients with osteoporosis from those without osteoporosis was good, with a ROC AUC of 0.707. The optimal ratio cut-off value with the highest accuracy was 9.72. The ability of the ratio (FGF23*bAP/CTX) to distinguish patients with bone fragility was good, with a ROC AUC of 0.701. The optimal ratio cut-off value with the highest accuracy was 1621.89 (sensitivity 60%, specificity 78.4%)., Conclusion: Our study showed FGF23, FGF23*bAP product to CTX ratio, and the bAP to CTX ratio can be used as markers of bone fragility in hemodialysis patients. Therefore, these noninvasive and relatively inexpensive methods may serve to diagnose bone fragility in patients under hemodialysis., (Copyright © 2020 The International Society for Clinical Densitometry. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Conflict of interest The authors declare that there is no conflict of interests.

Immunoglobulin A nephropathy is the most common primary glomerular disease. The main challenge in this disease is the evaluation of prognostic factors for end-stage renal disease. The aim of our study was to describe the characteristics of immunoglobulin A nephropathy, to evaluate the histological data according to Oxford classification, and to identify factors associated with renal survival. This was a retrospective study, including adults with primary immunoglobulin A nephropathy. The study was conducted over a period of 10 years. Renal biopsies were scored according to Oxford classification. Oxford score, based on the sum of the different histological lesions of Oxford classification, was calculated for each patient. We included 50 patients with a gender ratio (male:female) of 2.8. The average age was 35.6 ± 10.6 years. Fifty-eight percent of the patients had hypertension (HTN). The median proteinuria was 1.9 g/day. The median of the glomerular filtration rate was 47.6 mL/min/1.73 m 2 . According to Oxford classification, mesangial proliferation, endocapillary proliferation, glomerulosclerosis, interstitial fibrosis, and/or tubular atrophy and crescents were present in 40%, 38%, 88%, 36%, and 22% of the cases, respectively. The median Oxford score was 2. The median follow-up duration was 30 months. Ten patients (20%) reached end-stage renal disease. At univariate analysis, HTN, glomerular filtration rate, proteinuria, tubular involvement, and Oxford score >3 were associated with progression to end-stage renal disease (ESRD). Tubular involvement was an independent risk factor for ESRD. Our study confirms the prognostic value of the Oxford classification in immunoglobulin A nephropathy.

Background: Epithelioid angiomyolipoma (EAML) is a rare potentially malignant variant of renal angiomyolipoma (RAML). This study aims to determine whether RAML clinico-pathologic and molecular features (i.e. p53 gene abnormalities) differ significantly with regards to its histologic variant or to the presence of an epithelioid component within it., Methods: Consecutively resected RAML were reviewed, tumours comprising at least 80% of epithelioid cells were considered as EAML according to the 2016 World Health Organization classification of tumours of the kidney. P53 gene abnormalities were investigated using both immunohistochemical and molecular analysis., Results: A total of 3 EAML among 17 RAML were identified, accounting for 3.9% of the total AML cases. Fatty aspect on imaging was more observed within tumours devoid of an epithelioid component. EAML showed a higher mitotic rate and a stronger p53 staining, no renal poles involvement and was not treated by nephron sparing surgeries. RAML comprising an epithelioid component demonstrated severer nuclear atypia as well as stronger p53 staining. P53 gene sequencing revealed a missense mutation (c.747G > C) in one classic AML harbouring a strong labelling with p53., Conclusions: Strong p53 staining in a RAML, even in the absence of gene mutation, may suggest the presence of an epithelioid component or of a truly EAML. To the best of our knowledge, c.747G > C p53 gene mutation is being reported for the first time in a RAML, although its role in AML pathogenesis is still unknown., Competing Interests: Declaration of competing interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Dialysis patients have higher rates of sudden cardiac death. The study of the electrocardiogram could identify patients at risk of developing rhythm disorders. The aim of this study was to evaluate the electrocardiographic findings before and after the hemodialysis (HD) session and to examine associations of clinical and serum electrolytes with electrocardiogram findings. We conducted a multicentric transversal study, including chronic HD patients during January 2018. Standard 12-lead electrocardiogram was recorded, before and after the HD session. A medical history was documented. It included age, gender, initial nephropathy, and comorbidities. Serum potassium and total serum calcium were measured before a routine HD session. Serum potassium was measured after HD session. Corrected QT for heart rate was calculated using Bazett's formula. The study included 66 patients. Nineteen patients (28.8%) had hyperkalemia before the HD session and 44 (66.7%) patients had hypokalemia after the HD session. Seventeen patients had prolonged QTc interval (25.7%). On multiple regression analysis, only the prolonged QTc interval was significantly correlated with the serum potassium (P = 0.046).When comparing the mean values of electrocardiogram parameters before and after the HD session, we noted a significant change of heart rate (P = 0.001), R wave (P = 0.016), T wave (P = 0.001), and T/R (P = 0.001) wave. Delta K+ did not correlate with the change in T wave amplitude (r = 0.23, P = 0.59), R wave amplitude (r = -0.16, P = 0.2), T/R wave (r = 0.055, P = 0.65), or QRS duration (r = 0.023, P = 0.85). Delta QTc was correlated to ΔK+. We conclude that usual electrographic manifestations of hyperkalemia are less pronounced in HD patients. Our results confirmed the unstable status of cardiac electrophysiology during HD session.

Background: Bone disease is common in patients undergoing hemodialysis. It is the result of bone turnover abnormalities and the decrease of bone mineral density (BMD). We aimed to determine the usefulness of serum bone turnover markers and BMD measurement by dual-energy x-ray absorptiometry (DXA) in hemodialysis patients., Methods: We conducted a cross-sectional study including 90 hemodialysis for more than 12 months. Bone mineral density was assessed by DXA. Peripheral blood samples were obtained from each patient before dialysis in a fasting state within a week of the DXA. Biochemical variables of calcium and phosphate were measured. One bone formation marker (bone-specific alkaline phosphatase (bAP), one bone resorption marker (carboxy-terminal telopeptides of type 1 collagen (CTX)) were measured. Total alkaline phosphatase (TAP), intact parathyroid hormone (PTH) and fibroblast growth factor 23 (FGF23) which is a bone-derived hormone were also measured., Results: CTX values were 6.25 times higher than the normal limit of the assay. Bone alkaline phosphatase levels were less than 10 ng/mL in 28.8% of cases. 23% of patients have osteoporosis and 45% have osteopenia. Femoral BMD had negative correlations with age and PTH levels. FGF23 levels were significantly increased in patients with osteoporosis affecting the lumbar. The levels of bAP and CTX showed a positive correlation. Both circulating bAP and CTX levels showed also positive correlations with PTH levels. Fractures, observed in 12.2% of cases, were associated with low PTH values and the existence of osteoporosis., Conclusions: Our study showed that osteoporosis and fracture are common in dialysis patients. The reduced BMD was associated with advanced age and elevated levels of PTH. Markers of bone turnover and FGF23 may play a role in the diagnosis of bone disease in hemodialysis patients. DXA measurement is necessary for the monitoring for bone loss.

Introduction: Iatrogenic Kaposi's sarcoma is widely reported after transplantation. Less commonly, it occurs in patients receiving immunosuppressive therapy for ANCA associated vasculitis. We report here the rare association of Kaposi's sarcoma, prurigo nodularis and ANCA associated vasculitis in a hemodialysis patient., Case Report: We describe a 58-year-old woman who presented granulomatosis with polyangeiitis with alveolar hemorrhage and renal failure requiring hemodialysis. She developed cutaneous Kaposi's sarcoma seven weeks after the beginning of immunosuppressive therapy. Biological tests showed negative HHV8 virus infection. Lesions of Kaposi's sarcoma responded to a discontinuation of immunosuppressive drugs and a decreasing dosage of corticosteroids., Conclusion: Our case showed that the immunosuppressed state related to multiple factors such as underlying disease, immunosuppressive therapy and hemodialysis may all have contributed to the development of this neoplastic disorder in our patient., (Copyright © 2018. Published by Elsevier Masson SAS.)

Survival rates of diabetic patients on maintenance hemodialysis (HD) are reported to be poorer in Tunisia than in developed countries. This study aims to specify the epidemio-logical, clinical, and biological characteristics of diabetic disease, chronic kidney disease (CKD), and comorbidities at the initiation of HD and investigate factors associated with mortality during the dialysis period. We retrospectively analyzed the outcome of diabetic patients who were initiated on HD from 2007 to 2012 at the Rabta Hospital of Tunis. During the follow-up period, all morbid events and deaths were recorded. Univariate analysis and multivariate analysis were performed to identify risk factors associated with mortality in our population. The study population included 142 patients. The mean age was 58.7 ± 11.9 years. The sex ratio male/female was 1.4. One hundred and twenty-seven patients were type 2 diabetics (89.4%). Diabetic nephropathy was the main initial nephropathy (87.3%). CKD was diagnosed at Stages 4 or 5 in 95.1% of cases. HD was started in emergency conditions in 68.6% of cases. The one-year and the five-year survival rates were 42% and 17%, respectively. At initiation of HD, low socioeconomic status (P = 0.001), advanced age (P = 0.008), low body mass index (P = 0.04), history of stroke (P = 0.04), peripheral neuropathy (P = 0.02), initial vascular access (P = 0.03), secondary hyperpara-thyroidism (P = 0.03), nephrotic-range proteinuria (P = 0.01), and glycated hemoglobin ≤7% (P = 0.03) were associated with higher mortality rate. During dialysis period, cardiovascular events (P = 0.02), infectious complications (P = 0.04), and secondary hyperparathyroidism (P = 0.04) were significantly more noticeable among deceased patients. Due to poor survival rates of diabetic patients on HD, prevention, early detection, and management of diabetic CKD patients should be the way to go forward., Competing Interests: None

The arteriovenous fistula (AVF) is the vascular access of the first choice for hemodialysis (HD). Studies on patency of AVF and its affecting factors reveal a high risk for access failure. The aim of this study was to assess the primary and secondary AVF patency and their determinant factors. It was a retrospective, descriptive study conducted in the HD facility of the Nephrology Department in Rabta University Hospital. We included AVF created before December 2009 in end-stage renal disease (ESRD) patients. The end of the follow-up was fixed in December 2013. We included 126 AVFs created in 111 patients; 22.5% were aged >65 years, 39.6% were diabetic, 68.5% were hypertensive, and 26.1% had peripheral vascular disease. The primary patency rates were 78% at one year and 42% at five years. The secondary patency rates were 80% at one year and 69% at five years. Multivariate analysis revealed that the factors affecting the primary patency of AVF were: the use of jugular catheter for longer than three months (odds ratio (OR):1.91, P = 0.044) and a C-reactive protein >5 mg/L (OR: 1.7, P = 0.049). Aging (>65 years) (OR: 2.46, P = 0.042), referral time to a nephrologist <6 months before onset of ESRD (OR: 2.87, P = 0.015), absence of an antiplatelet therapy (OR: 4.47, P = 0.005), and serum phosphorus <45 mg/L (OR: 2.07, P = 0.045) were the significant impairing risk factors for secondary AVF patency. Our study suggests that early referral and creation of AVF and maturation before ESRD as well as its adequate monitoring are essential for maintaining patency., Competing Interests: None declared

Fluoroquinolones are usually well tolerated with a minimum of serious adverse effects; renal toxicity is uncommon. Apart from the renal side effects of ciprofloxacin, we aimed to highlight the renal impact of a ciprofloxacin overdose, and thus conducted a prospective study in the Department of Nephrology at La Rabta Hospital between 2010 and 2015. The cohort database was continually updated until the inclusion of five patients who were subjected to an overdose and who were initially admitted to the medical intensive care unit and then transferred to our department for acute renal failure (ARF) due to ciprofloxacin ingestion requiring urgent hemodialysis. All patients developed ARF after 12-36 h of ingestion. Renal ultrasound was normal in all cases. Twenty-four-hour proteinuria was present but not significant in one case, while microscopic hematuria was present in one case. Treatment consisted of supportive therapy and extrarenal purification by conventional intermittent hemodialysis. Four patients recovered normal renal function within 3 weeks and the remaining patient eventually had chronic kidney failure.

Objectives: The aim of this study was to evaluate the association between two VDR SNPs FokI and BsmI and mineral status in ESRD patients., Design and Methods: Our case-control study included 100 patients with chronic renal failure in ESRD and 149 healthy subjects. We measured the serum Vitamin D levels and the serum intact PTH level by Electrochemiluminescence Technology (cobas E411 analyzer). We evaluated the serum FGF23 levels by indirect ELISA method. The genotyping of two VDR gene variants FokI and BsmI was carried out by PCR-RFLP technique., Results: In our study, the FokI TT genotype was associated with lower risk of ESRD development (OR = 0.176, Padj = 0.039). The difference in PTH and FGF23 levels between cases and controls was statistically significant. The expression of FokI CT genotype in subjects with diabetic nephropathy was associated with a negative correlation between VD and PTH levels (r = -0.620, P = 0.032) and a positive correlation between VD and FGF23 levels (r = 0.967, P = 0.012). A significant differences in VD levels between patients and controls was observed in the presence of FokI TT (P = 0.044) and CT (P = 0.036) genotypes. The expression of FGF23 serum level was significantly elevated in patients than in controls in the presence of the FokI CC and BsmI AG genotypes., Conclusions: In conclusion, our study shows the existence of an association between VDR FokI, BsmI polymorphisms and mineral status in ESRD patients. The presence of VDR variants affect the protein expression of VD, phosphorus, FGF23 and PTH., (Copyright © 2018 The Canadian Society of Clinical Chemists. Published by Elsevier Inc. All rights reserved.)

In the era of the diseasomes and interactome networks, linking genetics with phenotypic traits in Turner syndrome should be studied thoroughly. As a part of this stratagem, mosaicism of both X and Y chromosome which is a common finding in TS and an evaluation of congenital heart diseases in the different situations of mosaic TS types, can be helpful in the identification of disturbed sex chromosomes, genes and signaling pathway actors. Here we report the case of a mosaic TS associated to four left-sided CHD, including BAV, COA, aortic aneurysms and dissections at an early age. The mosaicism included two cell lines, well-defined at the cytogenetic and molecular levels: a cell line which is monosomic for Xp and Xq genes (45,X) and another which is trisomic for pseudoautosomal genes that are present on the X and Y chromosomes and escape X inactivation: 45,X[8]/46,X,idic(Y)(pter→q11.2::q11.2→pter)[42]. This case generates two hypotheses about the contribution of genes linked to the sex chromosomes and the signaling pathways involving these genes, in left-sided heart diseases. The first hypothesis suggests the interaction between X chromosome and autosomal genes or loci of aortic development, possibly dose-dependent, and which could be in the framework of TGF-β-SMAD signaling pathways. The second implies that left-sided congenital heart lesions involve sex chromosomes loci. The reduced dosage of X chromosome gene(s), escaping X inactivation during development, contributes to this type of CHD. Regarding our case, these X chromosome genes may have homologues at the Y chromosome, but the process of inactivation of the centromeres of the isodicentric Y spreads to the concerned Y chromosome genes. Therefore, this case emerges as an invitation to consider the mosaics of Turner syndrome and to study their phenotypes in correlation with their genotypes to discover the underlying developmental and genetic mechanisms, especially the ones related to sex chromosomes.

Atypical hemolytic uremic syndrome (aHUS) is characterized by microvascular thrombosis resulting in thrombocytopenia, hemolytic anemia, and multiorgan dysfunction. It is associated with genetic or acquired disorders of regulatory components of the complement system. For our study, we collected data from 16 patients diagnosed with aHUS between January 2010 and January 2014. The mean age was 33.6 years. The female-to-male ratio was 3. The median follow-up duration was 27 ± 3.5 months. The most common clinical presentation was hypertension. Renal involvement was noted in all cases. Ten patients had extrarenal manifestations. Semi-quantitative dysfunction of the alternative pathway of complement was found in all cases. A genetic study was not available for our patients. During the acute stage, all patients received plasma therapy, and among them, seven required dialysis and five were still on dialysis at the time of discharge. One patient underwent renal transplantation. None of our patients received eculizumab perfusion. The renal survival was inversely correlated to young age (<30 years) (P = 0.001), presence of anti-factor H antibodies (P = 0.003) and serum creatinine at diagnosis >5 mg/dL (P = 0.02). Mortality rate was significantly correlated to young age (<30 years old) (P = 0.01). Collecting multicentric data on adult patients with aHUS will enable better characterization of the spectrum of adult aHUS in our country and the evaluation of current treatments and different outcomes., Competing Interests: None declared

Obstetric cortical renal necrosis is a serious complication that can lead to chronic renal failure and the need for chronic dialysis. The aim of renal cortical necrosis therapy is to restore hemodynamic stability, institute early dialytic therapy, and treat the underlying cause of the disease. Most cases of renal cortical necrosis do not recover a normal renal function despite intensive care. We describe the course of a patient who was diagnosed with acute renal cortical necrosis in pregnancy treated with hemodialysis for three years but then she recovered her renal function., (Copyright © 2017 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.)

The incidence of tuberculosis (TB) is high in patients undergoing chronic dialysis than it is in the general population. The diagnosis of TB is often difficult and extrapulmonary involvement is predominant. This study investigates the spectrum of clinical presentations and outcome in dialysis patients during a nine-year period. TB was diagnosed in 41 patients. Anti-TB drugs, adverse effects of therapy, and outcome were noted. Thirty-eight patients (92.6%) were on hemodialysis and three were on peritoneal dialysis (7.3%). The mean age at diagnosis was 50.8 years and the male/female ratio was 1.16. Four patients had a history of pulmonary TB. Extrapulmonary involvement was observed in 32 (78 %) patients. The bacteriological confirmation was made in 41.46% and histological confirmation was made in 26.83%, and in the rest, the diagnosis was retained on the criterion presumption. Nineteen patients (46.34%) developed adverse effects of antitubercular drugs. Eight patients (19.51%) died during the study from TB or adverse effects of treatment. Low urea reduction ratio and female sex were associated with poor prognosis in our study. The clinical manifestations of TB in patients on dialysis are quite nonspecific, making timely diagnosis difficult, and delaying the initiation of curative treatment, which is a major determinant of the outcome.

Purpose: To evaluate the impact of lymphovascular invasion on the prognosis of patients treated for upper urinary tract urothelial carcinomas., Materials and Methods: Clinical records of 49 patients treated surgically at our institute for upper urinary tract urothelial carcinomas were reviewed retrospectively. LVI was defined as the presence of cancer cells within an endotheluim-lined space without underlying muscular walls. Actuarial survival curves were analysed by Kaplan-Meier method. Multivariate analysis was performed using Cox's proportional hazard model., Results: Median follow-up was 32 months. Lymphovascular invasion was present in 26 (53%) patients. Lymphovascular invasion was associated with higher pathological tumor stage (pT) and higher tumor grade. The disease-free and overall survival rates of the patients with lymphovascular invasion were significantly worse than those of the patients without lymphovascular invasion (p < 0.001 and p = 0.027 respectively). Multivariate analysis revealed that lymphovascular invasion as well as tumor grade and pathological tumor stage were significant prognosticfactors for disease-free and overall survival., Conclusion: The presence of lymphovascular invasion was a strong predictor of a poor outcome for UTUC. This finding could help identify patients at greater risk for disease recurrence who would benefit from close follow-up and early adjuvant therapy.

Transurethral resection of the prostate is currently the gold standard for the surgical treatment of the benign prostatic hyperplasia. This surgery may lead transurethral resection of the prostate (TURP) syndrome and in some cases, acute tubular necrosis can develop. We report a patient who developed hyponatremia, hemolysis and oliguric acute renal failure as a major complication following TURP using glycine as irrigating fluid.A 64-year-old man was admitted for a prostate resection procedure. Physical examination revealed a healthy elderly man. Preoperative laboratory data showed serum sodium 140 mEq/L, blood urea nitrogen (BUN) 0.6 g/L, creatinine 0.7 mg/dL and hemoglobin 12.9 g/dL. Few hours after, the patient becomes incoherent and developed oliguria, nausea and vomiting. The laboratory data revealed rapidly elevating BUN and creatinine levels (BUN 2.4 g/L; creatinine 6.1 mg/dL), the serum sodium concentration decreased by 14 meq/L. A decreased hemoglobin level (7.4 g/dL) with an elevated lactate dehydrogenase level (665 U/L) was observed. Renal ultrasonography was normal. The diagnosis of acute tubular necrosis complicating TURP syndrome was retained. The hyponatremia was slowly corrected to 132 mmol/L by diuresis and fluid restriction. The renal function recovered after four hemodialysis sessions. Using glycine as an irrigant for TURP may cause hyponatremia, hemolysis and also acute renal failure, especially in patients with longer resection time. It is necessary to carry out every effort to shorten resection time and avoid extravasation during surgery.

Objective: To evaluate the influence of continent external urinary diversion type Mitrofanoff on male sexuality., Material and Methods: Between 1992 and 2011, 140 patients underwent continent urinary diversion type Mitrofanoff at an academic hospital. Among 76 men, 46 were interviewed about their sexuality after this operation. This study was performed using a set of validated questionnaires (IIEF, DAN PSS and Urolife), grouped by the model of the CTMH. Patients were divided according to their marital status: group 1: patients married before surgery (15 cases), group 2: patients married after surgery (7 cases) and group 3: singles (24 cases)., Results: In the first group, the functional dimension of sexuality was positive with an overall score of 81%, the sexual discomfort score was assessed at 26 % and the sexual satisfaction score was 77%. In the second group, sexual function was considered conserved in all cases with a satisfaction score estimated at 98%. These patients reported a feeling of well-being following the disappearance of urinary incontinence with integrity of their body images. In contrast, in the last group, relatively impaired sexual function was noted (65%) with a satisfaction score estimated at 59%. These disorders were multifactorial, mainly related to neurological causal pathology., Conclusion: To our knowledge, this is the first study about male sexuality in patients with a continent urinary diversion type Mitrofanoff. Marital status has a major role in the sexuality of these patients. A prospective study with pre- and postoperative evaluation will better clarify the factors affecting sexuality in these patients., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Cocaine is one of the most commonly used illicit drugs with distribution and consumption throughout the world. Acute renal failure associated with rhabdomyolysis, direct vasoconstriction and hemodynamic alteration is well described in patients with cocaine intoxication. Cocaine use is associated with high blood pressure and may rarely induce malignant hypertension associated with thrombotic microangiopathy. We report the case of a patient who developed malignant hypertension associated with thrombotic microangiopathy after chronic consumption of cocaine. A kidney biopsy revealed thrombotic microangiopathy with fibrinoid necrosis of arterioles and glomerular tufts. He required dialysis sessions. Cocaine-mediated endothelial injury and platelet activation may play important pathogenetic roles in cocaine abusers who develop malignant hypertension associated with thrombotic microangiopathy. Clinicians need to be aware of this rare feature of cocaine intoxication.

Background: Vascular access is a basic and essential tool required for performing renal replacement therapy in end stage renal disease., Aim: To study the indications of tunneled catheter (KTT) in hemodialysis (HD), identify complications related to the use of KTT and contributing factors, assess the survival and performance of the technique., Introduction: The making of a vascular access is an angular piece for adequate HD and in good conditions. In this context the KTT may be an alternative., Methods: A retrospective study of 52 KTT placed in 49 patients collected in the department of Nephrology Dialysis and Renal Transplantation in RABTA Tunis between 2008 and 2011., Results: The average age of our patients was 55.58 years ± 13.5 years, their Sex ratio was 0.79. The Thirty of our patients were diabetic, 46.2% had hypertension and 21.2% had underlying cardiac disease. The mean duration of HD was 1111.35 days or 37 months. The most common indication of KTT was the absence of arteriovenous fistula in 65.4% of cases, other indications were: short survival (30.7%), the exhaustion of venous capital (34.6%), mediacalcosis (34.6%) and immunosuppression (36.5%). The right internal jugular vein was the choice of insertion site with 78.8%. The overall incidence of immediate complications was 19.2%. Among our patients, 31.4% had a dysfunction. The period of HD represent the risk factor for dysfunction KTT (p = 0.006).An infectious complication was observed in 29% of cases. The median time to onset of infection was 190.83 days. Staphylococcus was isolated in 40% of cases. The average duration of use of KTT was 238 days. The only single factor determining the survival of KTT was the number of KTT put in the same patient., Conclusion: More than a quarter of the population are dialyzed through a catheter. Despite concerted efforts, much remains to be done for the confection at time of a permanent vascular access.

Crow-Fukase syndrome, also known as POEMS syndrome, is a rare plasma dyscrasia characterized by monoclonal gammopathy and various combinations of polyneuropathy, organomegaly, endocrinopathy and dermatological changes, and their initials stand for the acronym POEMS. Substantial kidney involvement is rarely related to this disease. Our report is about five patients suffering from the POEMS syndrome with kidney involvement that rapidly progressed to end-stage renal disease. Our report is about three females and two males with a mean age of 60.6 years. Neuropathy was noted in all the cases. Endocrinopathy included hypothyroidism and/or diabetes. Skin changes were noted in one case, and included peri-orbital hyperpigmentation. Monoclonal gammopathy was present in all the cases and was related to multiple myeloma in three cases. Kidney involvement presented in all the five cases. Treatment included Melphalan, Thalidomid, steroids and hemodialysis. Survival was short for three patients, from five to 34 months.

Subcutis calcinosis, characterized by abnormal calcium deposits in the skin, is a rare complication of using calcium-containing heparin occurring in patients with advanced renal failure. We report the case of an 83-year-old female, a known case of chronic kidney disease (CKD) for four years with recent worsening of renal failure requiring hospitalization and hemodialysis. She developed subcutis calcinosis following injection of calcium-containing heparin. Biochemical tests showed serum parathormone level at 400 pg/dL, hypercalcemia, elevated calcium-phosphate product and monoclonal gammopathy related to multiple myeloma. She developed firm subcutaneous nodules in the abdomen and the thighs, the injection sites of Calciparin ® (calcium nadroparin) that was given as a preventive measure against deep vein thrombosis. The diagnosis of subcutis calcinosis was confirmed by the histological examination showing calcium deposit in the dermis and hypodermis. These lesions completely disappeared after discontinuing calcium nadroparin injections. Subcutis calcinosis caused by injections of calcium-containing heparin is rare, and, to the best our knowledge, not more than 12 cases have been reported in the literature. Pathogenesis is not well established but is attributed to the calcium disorders usually seen in advanced renal failure. Diagnosis is confirmed by histological tests. Outcome is mostly favorable. The main differential diagnosis is calciphylaxis, which has a poor prognosis. Even though rarely reported, we should be aware that CKD patients with elevated calcium-phosphorus product can develop subcutis calcinosis induced by calcium-containing heparin. When it occurs, fortunately and unlike calciphylaxis, outcome is favorable.

Renal involvement in Sjogren's syndrome (SS) is not uncommon and may precede other complaints. Tubulointerstitial nephritis is the most common renal disease in SS and may lead to renal tubular acidosis (RTA), which in turn may cause osteomalacia. Nevertheless, osteomalacia rarely occurs as the first manifestation of a renal tubule disorder due to SS. We herewith describe a 43-year-old woman who was admitted to our hospital for weakness, lumbago and inability to walk. X-ray of the long bones showed extensive demineralization of the bones. Laboratory investigations revealed chronic kidney disease with serum creatinine of 2.3 mg/dL and creatinine clearance of 40 mL/min, hypokalemia (3.2 mmol/L), hypophosphatemia (0.4 mmol/L), hypocalcemia (2.14 mmol/L) and hyperchloremic metabolic acidosis (chlorine: 114 mmol/L; alkaline reserve: 14 mmol/L). The serum alkaline phosphatase levels were elevated. The serum levels of 25-hydroxyvitamin D and 1,25-dihydroxy vitamin D were low and borderline low, respectively, and the parathyroid hormone level was 70 pg/L. Urinalysis showed inappropriate alkaline urine (urinary PH: 7), glycosuria with normal blood glucose, phosphaturia and uricosuria. These values indicated the presence of both distal and proximal RTA. Our patient reported dryness of the mouth and eyes and Schirmer's test showed xerophthalmia. An accessory salivary gland biopsy showed changes corresponding to stage IV of Chisholm and Masson score. Kidney biopsy showed diffuse and severe tubulo-interstitial nephritis with dense lymphoplasmocyte infiltrates. Sicca syndrome and renal interstitial infiltrates indicated SS as the underlying cause of the RTA and osteomalacia. The patient received alkalinization, vitamin D (Sterogyl ®), calcium supplements and steroids in an initial dose of 1 mg/kg/day, tapered to 10 mg daily. The prognosis was favorable and the serum creatinine level was 1.7 mg/dL, calcium was 2.2 mmol/L and serum phosphate was 0.9 mmol/L.

The association between Kaposi's sarcoma (KS) and human herpes virus eight (HHV-8) infection is rarely reported in hemodialysis (HD) patients. We report here the rare association of KS, HHV-8 and hepatitis C virus (HCV) infection as well as syphilis in a HD patient. We report the case of a 72-year-old woman who presented with microscopic polyangiitis with alveolar hemorrhage and pauci-immune necrosing and crescentic glomerulonephritis as well as renal failure requiring HD. Biological tests showed positive HCV and syphilis tests. The patient was treated by HD and intravenous pulse, followed by oral corticosteroids and six cyclophosphamide monthly pulses with remission of the alveolar hemorrhage, but without renal functional recovery as the patient remained HD dependent. Five months after the first treatment administration, she developed extensive purpuric lesions on her lower limbs, abdomen face and neck. A skin biopsy showed KS. The HHV-8 test was positive, with positive polymerase chain reaction-HHV8 in the serum and skin. After immunosuppression withdrawal, the KS skin lesions regressed rapidly without relapse after 12 months of follow-up, but alveolar hemorrhage relapsed after 16 months of follow-up. Our case showed that the immunosuppressed state related to multiple factors such as aging, vasculitis, HHV-8, HCV, syphilis, immunosuppressive therapy and HD may all have contributed to the development of KS in our patient.

Background: Uremic pruritus is frequent in patients undergoing hemodialysis. It's multifactorial secondary in most cases to metabolic complications related to uremia., Aim: We report a rare cause of pruritus in an haemodialysed patient., Observation: It's a 48-year-old woman in periodic haemodialysis during 17 years. She suffered from generalized pruritus associated with psychiatric disturbance without neurologic deficits. CT scan and magnetic resonance imaging revealed extensive, diffuse and bilateral involvement of the white matter. The diagnosis of large low grade B cell lymphoma was confirmed by the histologic examination of stereotactic biopsy which leads to early intracranial hemorrhage and death., Conclusion: Before attributing pruritus to renal failure, one should exclude other causes such as the NHL even rarely reported especially when it associated with psychiatric disturbances. CT scan and magnetic resonance imaging contribute to diagnosis.

Background: The incidence of end-stage renal failure is high and it is responsible for the increase of the rate of morbidity and mortality rates among our patients., Aim: The objective is to study patient characteristics before starting hemodialysis and to evaluate factors influencing their short and long term survival., Methods: This is a prospective study of 127 patients starting hemodialysis between June and December 2001. On May 31, 2005, their survival was analyzed according to different parameters., Results: Patients were 77 males and 50 females. Their mean age was 51.4 +/- 16.1 years (15 to 78 years). Diabetes was observed in 33.9% of cases. Only 70.9% of patients were covered by a social service. Chronic renal failure was diagnosed at the end stage in 34.6% of cases. Before starting hemodialysis, only 4 patients were vaccinated against B hepatitis and arteriovenous fistula were not made in any patients. Pericarditis was observed in 9.4% of patients. Albuminemia was < 35 g/l in 60.5% of patients. First hemodialysis session was programmed in 53.5% of patients and realized urgently in 46.3% of patients. Patients were hemodialysed 4, 8 and 12 hours per week respectively in 16.5%, 15.8% and 67.7% of cases. On May 31, 2005, 35.4% of patients died. Their actuarial survivals at 3 months, 1 year and 4 years were respectively at 87.5%, 79.5% and 64.4%. Acturial survival was bad in patients with pericarditis, diabetes, hemodialysed less than 12 hours/week and when the first hemodialysis session was started urgently., Conclusion: The diagnosis of renal failure was frequently made at end-stage. There are no preparations before starting hemodialysis. We have to reinforce prevention programmes and increase the number of nephrologists and nephrology departments.

Background: Tuberous sclerosis is a rare inherited disease which can involve several organs. Renal involvement is one of the most severe manifestations of the disease. We analysed in this study renal involvement of tuberous sclerosis., Methods: We studied retrospectively nine cases of tuberous sclerosis with renal involvement diagnosed between 1994 and 2005. The diagnosis was based on the presence of typical extra-renal manifestations and familial history of tuberous sclerosis., Results: There were eight females and one male. Their mean age was 40.8+/-16.4 years (range: 15-62). At first presentation, we noted hypertension in three cases, proteinuria in seven cases, hematuria in eight cases and renal failure in five cases (end stage renal failure in four cases). By ultrasonography, we found angiomyolipoma in one case, cysts in four cases and angiomyolipoma associated to cysts in four cases. Nephrectomy was performed in two patients, in one case for hydronephrosis secondary to urolithiasis and in the other for hemorrhagic complication. In the later, pathological examination revealed renal carcinoma. At last evaluation, two patients were lost to follow-up, one had normal serum creatinine, five were on renal replacement therapy and the latter died from sepsis following kidney removal., Conclusions: In our patients, renal disease was diagnosed late at chronic renal failure stage. Patients with end-stage renal failure require dialysis and renal transplantation, but we recommend binephrectomy after starting dialysis and before transplantation due to the risk of cancer and bleeding related to angiomyolipomas.