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Obesity triggers the development of adipokines such as leptin, resistin, and visfatin, which have been associated with the development of diabetic nephropathy and other vascular disorders. The main purpose of the current investigation was to identify the physiological impact of visfatin on immunological response and its inflammatory effects on nephropathy. Fifty Iraqi patients with chronic kidney disease (CKD) at various stages, as described by the National Kidney Foundation (NKF) and ranging in age from 48.367.56 to 53.68 8.46 years on average were considered. Prior to the start of the investigation, informed consent was obtained from all participants, and the ethics committee approved the study. Patients were classified into two groups: Group (A) comprised patients with a GFR higher than 60 mL/minute, and Group (B) comprised patients with a GFR of less than 60 mL/min. There was no considerable variance between the groups as regards visfatin, but a highly significant correlation between serum visfatin and CRP was observed. The results of the current investigation indicated that serum visfatin levels are significantly correlated with CRP in CKD patients; it is also correlated with deterioration of kidney function. Moreover, higher visfatin levels were accompanied by increased serum triglyceride and cholesterol levels. These findings would suggest that visfatin may perform an essential function in uremia-related inflammation and may serve as a potential target for treatment and prevention of renal associated complications. Future studies may delineate whether visfatin is a marker of disease activity and severity as well as a predictor of outcome in CKD.

The risk of acute renal failure (ARF) following iodinated contrast media injection has long been overestimated because of the previous use of more toxic ICPs and uncontrolled studies. Nowadays, this concept is being questioned. Patients with severe renal failure and/or ARF are the only group still considered at risk. In these patients, it is necessary to discuss an alternative without an iodinated contrast agent. Contrast-enhanced ultrasound, MRI, spectral CT or PET-CT scan can be used instead of contrast-enhanced CT. Preventive measures should be applied when appropriate substitute to CT is not available or not diagnosed (minimum necessary dose of ICP, interruption of some treatments and prior hydration). These recommendations formalized by the European Society of Urogenital Radiology (ESUR) in 2018 address most situations faced by clinicians. In complex situations, an opinion from a nephrologist remains necessary after asking the radiologist about the availability of acceptable substitutes., (Copyright © 2022 Société Française du Cancer. Published by Elsevier Masson SAS. All rights reserved.)

Henoch-Schonlein purpura (HSP) is a small-vessel vasculitis secondary to IgA deposits. We conducted a retrospective study of 14 cases of HSP in adults based on EULAR/PRINTO/PRES classification criteria for HSP over a period of 18 years (1996 to 2014) in Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, South Tunisia. The purpose of our study was to highlight the epidemiological, clinical, therapeutic and evolutionary features of HSP in our adult patients. The average age of patients was 33 years (ranging from 17-64 years) with male predominance (sex ratio of 1.8). Vascular purpura with petechiae was constant. Articular manifestations (arthralgia and/or arthritis) were reported in 9 patients (64.2%). Gastrointestinal involvement was reported in 13 patients (92.8%). Renal failure was found in 11 patients (78.5%) revealed by nephrotic proteinuria in 7 cases, microscopic haematuria in 9 cases, high blood pressure in 4 cases and renal impairment in 1 case. The most common histological type was diffuse proliferative endocapillary glomerulonephritis (36.3%). High dose corticotherapy was initiated in 7 patients with proliferative renal impairment, bolus of Solu-Medrol in 4 cases, associated with cyclophosphamide in one case. Two patients with severe gastrointestinal involvement received corticotherapy. After a mean follow-up period of 18.5 months (4-36 months), outcome was favorable in all cases without relapse and chronic renal failure was reported only in one case. [ABSTRACT FROM AUTHOR]

Renal needle biopsy (RNB) is the gold standard in the diagnosis of kidney diseases. It is an invasive technique causing several complications, in particular hemorrhagic events. This study aims to evaluate our RNB practice, to update the current understanding of the technique for percutaneous renal biopsy, to assess complications and to determine the prevalence of kidney diseases diagnosed in our region. We conducted a retrospective study between January 2015 and June 2017. Sixty-nine patients hospitalized in the Division of Nephrology at the University Hospital Mohamed VI Oujda having undergone native kidney biopsy were included in the study. The average age of patients, at the time of RNB, was 38.16 ±13 years [12-77 years], with a sex ratio (M/F) of 0.86. Nephrotic syndrome was the most frequent indication for RNB in all age groups. Glomerular kidney diseases accounted for 81% of kidney diseases, divided as follows: extra-membranous glomerulonephritis in 19% of patients, focal and segmental hyalinosis in 18%, lupus nephritisin in 11%, chronic glomerulonephrites in 11%, membranoproliferative glomerulonephritis in 7%, extracapillary glomerulonephritis in 5% shared equally with renal amyloidosis. Four percent of RNB showed diabetic nephropathy. Histopathological examination revealed acute post-infectious glomerulonephritis, minimal glomerular lesion, immunoglobulin A (IgA) nephropathy, vasculitis, tubulo-interstizial nephropathies as well as thrombotic microangiopathy, with an estimated rate of 2% respectively. Five percent of RNB showed non-glomerular disease. Macroscopic haematuria was the main complication observed in our case series, with a rate of 2.8%. RNB is the gold standard in the diagnosis of renal disease. However, syndromic diagnosis enables clinician to identify the most probable renal disease and to guide any emergency treatment. [ABSTRACT FROM AUTHOR]

IgA vasculitis nephritis affects the prognosis of this disease in adult patients. This study aimed to examine the clinical characteristics of this renal involvement in adults and to identify factors influencing renal prognosis. We conducted a retrospective monocentric study of patients with histologically confirmed IgA vasculitis nephritis (rheumatoid purpura) (EULAR classification criteria) with renal involvement classified according to Pillebout classification. We analyzed renal survival and identified the factors influencing renal prognosis. Twenty-five patients were included (sex ratio M/F = 2.57), their average age at diagnosis of rheumatoid purpura was 35,76 years. Purpura was diagnosed in 100% of cases, with articular involvement in 28% of cases. Renal failure was identified in 44% of cases. The most common histological classification was IgA vasculitis nephritis (class II). Clinical remission was observed in 44% of cases and an evolution toward chronic renal failure (end-stage renal disease) in 36% of cases. Renal survival at 195 months was 57%. The identified prognostic factors were digestive involvement (p = 0.022), early renal failure (p = 0.0004), glomerular classification (P=0,001) and the severity of the histological lesions, renin-angiotensin system blocker treatment (p = 0.01) and plasma exchanges (p = 0.03). Our study shows that renal involvement during IgA vasculites can be relatively severe with poor renal prognosis. The identification of clinical and histological prognostic factors may be useful as guidance for the development of prospective therapeutic studies. [ABSTRACT FROM AUTHOR]

We report clinical and renal histological data, treatment modalities and outcome of 43 Tunisian children with biopsy-proven lupus nephritis seen over a 23-year period. There were 39 girls and 4 boys with a mean age of 12.5 years at diagnosis of lupus nephritis and followed for a mean period of 77 months. Renal symptoms included urinary abnormalities in all patients, hypertension in 40% of cases, nephrotic syndrome in 60% of cases and renal failure in 25% of cases. Class IV and class III nephritis were observed in 48.8 % and 30.2 % respectively. Corticosteroids were used in all cases, associated to immunosuppressive therapy in 23%. Overall survival was 86% at 5 years and 74% at 10 and 15 years. Renal survival was 83% at 5 and 10 years and 63% at 15 years. Initial renal failure and tubulointerstitial fibrosis were significantly increased risk for the development of end-stage renal disease in our study group. Renal histological findings provide the basis for treatment recommendations. Timely performed renal biopsy is greatly needed to accurately determine the prognosis and to guide treatment in children lupus nephritis.

Résumé Introduction La néphropathie, induite par les produits de contraste (NIPC), est une complication fréquente et grave en milieu de cardiologie interventionnelle. Objectif Le but de notre étude était de comparer l’incidence de NIPC selon deux protocoles d’hydratation accélérés : le premier par du sérum bicarbonaté seul et le deuxième associant le sérum bicarbonaté à la vitamine C orale. Méthodes Il s’agit d’une étude multicentrique prospective, randomisée menée entre octobre 2012 et mai 2013, incluant 160 patients. Résultats L’âge moyen de notre population d’étude était de 60,8 ± 9,3 ans (36 à 83 ans). Les deux groupes de l’étude étaient comparables à l’état de base en termes de facteurs de risques cardiovasculaires, de la médication concomitante, et du taux de créatinémie basale. La NIPC était estimée à 6,3 % dans le groupe vitamine C et à 10 % dans le groupe témoin, avec une différence non significative ( p = 0,38). L’analyse de sous-groupes selon le protocole utilisé n’a pas montré de différence significative quant à l’incidence de NIPC. Cependant, une forte tendance à un moindre taux de NIPC a été notée dans le sous-groupe vitamine C en cas d’utilisation de dose toxique de produit de contraste. De même, aucune NIPC n’a été notée dans le sous-groupe de patients âgés de plus de 75 ans sous vitamine C. Conclusion Selon notre étude, l’acide ascorbique administré par voie orale dans le cadre d’un protocole de réhydratation accéléré ne permet pas de réduire l’incidence de NIPC. Introduction Contrast-induced nephropathy (CIN) is a common and severe complication in interventional cardiology. Objective The aim of our study was to compare the incidence of contrast-induced nephropathy in two accelerated hydration protocols: the first one by the serum bicarbonate and the second combining the serum bicarbonate and oral vitamin C. Methods This is a multicenter prospective, randomized study conducted between October 2012 and May 2013, including 160 patients. Results The mean age of our study population was 60.8 ± 9.3 years (36–83 years). The two study groups were comparable in terms of cardiovascular risk factors, concomitant medication, and baseline serum creatinine. The CIN incidence was 6.3% in the vitamin C group and 10% in the control group ( P = 0.38). No significant difference was observed in terms of CIN incidence between the different subgroups analyzed. Conclusion According to our study, ascorbic acid administered orally as part of an accelerated hydration protocol does not reduce the incidence of CIN. [ABSTRACT FROM AUTHOR]

Résumé L’amylose AA reste l’une des trois grandes variétés d’amylose multisystémique, avec l’amylose AL et les formes héréditaires. Son incidence semble toutefois diminuer dans les pays occidentaux, où les maladies inflammatoires sont la principale cause d’amylose, au premier rang desquelles se tient la polyarthrite rhumatoïde, suivie par la spondylarthrite ankylosante et les syndromes auto-inflammatoires. Parmi les tumeurs, il faut signaler la maladie de Castleman, dont l’ablation chirurgicale permet parfois la résolution des symptômes de l’amylose. Une inflammation prolongée est un prérequis au développement d’une amylose AA ; la protéine majoritaire des dépôts est un fragment de la protéine serum amyloid A (SAA), l’une des protéines de la réaction inflammatoire. Cependant, d’autres facteurs, notamment génétiques, sont impliqués dans la susceptibilité à la survenue de l’amylose AA. La néphropathie est la principale manifestation clinique de l’amylose de type AA. La recherche de protéinurie et la mesure de la créatinine plasmatique restent les éléments de dépistage de l’amylose au cours de toute maladie qui comporte une inflammation chronique. Le diagnostic précis de l’amylose AA nécessite de confronter l’ensemble des données cliniques et histologiques et notamment de l’immunohistochimie, afin de ne pas la confondre avec l’une des autres variétés. Une fois installée, l’amylose AA reste de mauvais pronostic en raison de l’insuffisance rénale terminale, qui est le terme ultime de la néphropathie, et de l’atteinte digestive qui s’accompagne de dénutrition profonde. Les traitements actuels sont ciblés sur la maîtrise de l’inflammation ; des médicaments inhibant l’interaction de la protéine AA aux composants communs de l’amylose (composant amyloïde P et héparane sulfate) sont en cours d’évaluation. AA amyloidosis remains one of the three main types of systemic amyloidosis with AL and ATTR. Its incidence has been however decreasing recently in Western countries. Chronic inflammatory diseases are currently the first cause of AA amyloidosis, including rheumatoid arthritis, spondyloarthritis and autoinflammatory diseases. Castleman's disease is a specific cause of AA amyloidosis that can be cured by surgery. A chronic inflammatory response is required to develop amyloidosis. Other genetic and environmental factors are also involved. The first clinical manifestation is a chronic glomerular nephropathy, which can be detected by urine examination and serum creatinine measure. Immunohistochemistry is mandatory to confirm the clinical diagnosis of AA amyloidosis and to avoid misdiagnosis. Long-term prognosis remains poor on chronic dialysis in case of clinical gut involvement. Current treatment is based on the control of the inflammatory response. Specific treatment aimed at inhibiting amyloid formation targeting serum amyloid P component and heparan sulphate are currently evaluated. [ABSTRACT FROM AUTHOR]

Résumé But de l’étude Ce travail étudie les relations entre le gain baroréflexe (GBR), la pression pulsée (PP) et le débit de filtration glomérulaire (DFG) chez le patient diabétique de type 2 (DT2). Patients et méthodes Un enregistrement continu (Finapres ® ) de la pression artérielle (PA) et de la fréquence cardiaque (FC) lors d’un test postural standardisé (« squatting » : debout-accroupi-debout) est réalisé chez 64 patients DT2 permettant, notamment, de calculer GBR durant la phase de redressement et l’augmentation de PP durant l’accroupissement. Le DFG est calculé par la formule MDRD avant et après un suivi moyen de 12 ± 5 années. Résultats Par rapport aux patients avec un GBR élevé ( n = 30), les patients avec un GBR bas ( n = 34) sont légèrement plus âgés, mais ont un sex-ratio, une durée du DT2, un taux d’HbA 1c et des valeurs de PA comparables. Ils ont une FC de base plus élevée, une élévation de PP plus grande en position accroupie, ainsi qu’une chute de PA plus importante, avec un retard à la récupération, et une tachycardie moindre lors du redressement. Si DFG est significativement plus bas au départ chez les patients avec GBR abaissé, la diminution durant le suivi de 12 ans est comparable dans les deux groupes. Conclusion Un GBR abaissé, marqueur de la neuropathie autonome cardiovasculaire, est associé à une PP élevée en position accroupie (marqueur indirect de rigidité artérielle) et une diminution du DFG (néphropathie), mais ne permet pas de prédire la dégradation ultérieure de la fonction rénale. Aim of the study The present work is studying the relationships between baroreflex gain (BRG), pulse pressure (PP) and estimated glomerular filtration rate (eGFR) in type 2 diabetic (T2D) patients. Patients and methods A continuous non-invasive monitoring (Finapres ® ) of blood pressure (BP) and heart rate (HR) during a standardized postural manoeuvre (squatting test) was performed in 64 T2D patients allowing the calculation of BRG during the squatting to standing transition and the increase in PP during the standing to squatting transition. eGFR was calculated using the MDRD formula and glycated haemoglobin (HbA 1c ) was measured at baseline and after a follow-up of 12 ± 5 years. Results When compared to patients with a high BRG ( n = 30), patients with a low BRG ( n = 34) were slightly older, but had a similar sex ratio and comparable duration of diabetes, HbA 1c level and BP values. They had a higher baseline HR, a greater elevation of PP during squatting and a more pronounced BP fall when standing up, with delayed BP recovery and a less marked tachycardia. While eGFR was significantly lower at baseline in patients with low BRG, the subsequent reduction in eGFR during the 12 years of follow-up was comparable between the two groups. Conclusion A low BRG, a marker of cardiac autonomic neuropathy, is associated with a higher PP in squatting position, a marker of arterial fitness, and a reduction in eGFR (nephropathy), but does not predict the subsequent deterioration of renal function. [ABSTRACT FROM AUTHOR]

Résumé La néphropathie à immunoglobulines A (IgA) est la glomérulonéphrite primitive la plus répandue au monde. Elle est responsable d’une insuffisance rénale progressive évoluant vers l’insuffisance rénale terminale dans près d’un tiers des cas. Les mécanismes physiopathologiques de cette pathologie médiée par des complexes immuns restent encore non élucidés. La présentation et l’évolution clinique, ainsi que l’aspect en microscopie optique de la biopsie rénale peuvent être extrêmement variables, rendant toute classification histologique difficile. La plupart des études thérapeutiques n’incluent donc jusqu’à présent les patients que sur des critères cliniques de gravité. La nouvelle classification d’Oxford devrait changer cette situation. Seule la prise en charge des patients ayant une néphropathie avec lésions glomérulaires minimes et syndrome néphrotique, ou une glomérulonéphrite extracapillaire et une insuffisance rénale rapidement progressive, est consensuelle, avec traitement par corticoïdes seuls pour les premiers et associés à des immunosuppresseurs pour les seconds. Une corticothérapie peut être discutée chez les patients ayant une protéinurie supérieure à 1 g/j sans insuffisance rénale. Tous les patients atteints de néphropathie à IgA doivent bénéficier de la prise en charge globale d’une glomérulopathie chronique, comprenant notamment un traitement par bloqueur du système rénine-angiotensine en présence d’une hypertension artérielle ou d’une protéinurie. IgA nephropathy is the most common form of primary glomerulonephritis worldwide and an important cause of chronic kidney disease and end-stage kidney failure. Its pathophysiology remains in part unsolved but it is recognized as an immune complex disease. Recent years have brought progress in the field through the discovery of several genetic susceptibility loci and the formulation of the multi-hit pathogenesis model. Presentation, clinical course and histology can be extremely variable, making any histological classification still difficult. Indeed, most therapeutic studies until now include patients based only on the severity of clinical criteria but the new classification of Oxford should change that. Only the management of patients with nephropathy with minimal change glomerular lesions and nephrotic syndrome, or extra-capillary glomerulonephritis and rapidly progressive renal failure, is consensual: Corticosteroids alone for the first and associated with immunosuppressive drugs for the latter. The recent Kidney Disease Improving Global Outcomes (KDIGO) consensus treatment guideline is still controversial, especially in light of the last clinical studies. Corticosteroid therapy can be discussed in patients with proteinuria greater than 1 g/day without renal failure. All IgA nephropathy patients should benefit from the global management of chronic glomerular disease, including a renin-angiotensin system blocker in the presence of hypertension or proteinuria. [ABSTRACT FROM AUTHOR]

Résumé La néphropathie induite par les produits de contraste (NIPC) représente une complication fréquente chez les patients hospitalisés. Sa survenue est associée à une durée et un coût d’hospitalisation accrus, ainsi qu’une morbidité et une mortalité majorées. De ce fait, les moyens de préventions font partie des enjeux thérapeutiques majeurs. Certains patients admis en salle de cathétérisme cardiaque sont particulièrement à risque de développer une NIPC en raison de leurs comorbidités. De surcroît, les moyens de prévention ne peuvent pas être initiés dans certaines situations d’urgences. Après un rappel sur les propriétés des différents produits de contraste iodés, l’objectif de ce travail est de fournir des informations sur la définition, la physiopathologie, le diagnostic et les stratégies de préventions de la NIPC. Contrast-induced nephropathy (CIN) is common in hospitalized patients. Its occurrence is associated with an increased hospitalization stay and cost, morbidity and mortality. Thus, preventives strategies remain a major issue. Patients that are referred for cardiac catheterization are among the most vulnerable to develop CIN due to their comorbidities. Moreover, in some cases, such preventives measures cannot be introduced due to emergent clinical settings. After a summary regarding the properties of iodinated contrast medium, the aim of this work was to review the definition, pathophysiology, diagnosis and preventive strategies related to CIN. [ABSTRACT FROM AUTHOR]

Résumé Parmi les hémoglobinopathies congénitales, la drépanocytose reste la plus fréquente et la plus grave. Elle se présente sous la forme d’une anémie hémolytique de transmission autosomique récessive liée à une mutation du gène de la β-globine porté par le chromosome 11. La mutation confère à l’hémoglobine ainsi formée (ααβ S β S ), encore appelée hémoglobine S (HbS), une propriété de polymérisation anormale favorisant la falciformation des globules rouges, qui perdent ainsi leurs propriétés de déformabilité, et viennent obstruer les vaisseaux de petits calibres, ce qui se traduit cliniquement par la survenue de crises vaso-occlusives. Dans cet article, les atteintes rénales sont particulièrement étudiées chez ces patients, dont la qualité de la prise en charge de ces dernières années a permis une meilleure survie, mais aussi de laisser émerger des complications rénales plus fréquentes, voire plus graves. Les auteurs revoient la physiopathologie, les différentes présentations cliniques et les thérapeutiques préventives et curatives de cette complication. Sickle-cell disease (SCD), one of the most common severe monogenic disorders into the world, is associated with an increased frequency of chronic kidney disease. SCD is caused by a point mutation in the gene encoding β globin gene which leads to the formation of hemoglobin S that polymerises after deoxygenation. HbS polymerisation is associated with erythrocyte rigidity and vaso-occlusive episodes that play a central role into SCD pathogenesis. The spectrum of renal diseases during SCD is broad and includes various renal manifestations which become more apparent with increasing age. Underlying pathophysiological processes involved in sickle cell nephropathy are multifactorial but endothelial dysfunction related to chronic hemolysis is a key factor contributing to renal involvement. Our review focuses on the pathogenesis and on the spectrum of renal manifestations occurring in SCD patients. [ABSTRACT FROM AUTHOR]

BACKGROUND: In Sudan limited data is available regarding the prevalence of diabetes mellitus (DM), its complications as well as patients' awareness and practice patterns. This study aimed to evaluate the knowledge and practice regarding control of DM and occurrence of diabetic nephropathy (DN), among Sudanese diabetics with diabetic foot. METHODS: A cross-sectional study conducted in Khartoum State - Sudan, during the period from February to April 2013. It targeted adult diabetics admitted to hospital with diabetic foot. Data was obtained from the Hospital Medical Records as well as by direct interviewing of patients. Descriptive analysis of data was done using SPSS computer software. RESULTS: A total of 76 patients were included, their mean age was 58 - 1.01 years and 59.2% were males. Diabetic retinopathy and nephropathy were seen in 56.6% and 27.6% of patients, respectively. Regular follow-up was evident in 59.2% of patient. Following hospital admission and the diagnosis of diabetic foot, 73.7% were unable to tell why they had diabetic complications. Prior screening for DN was done in 31.6% of patients mostly in the form of urine dipstick for protein, and measurement of serum creatinine; whereas 53.9% of patients were not aware of prior screening for DN. CONCLUSION: A substantial proportions of Sudanese patients with diabetes remain far from achieving their glycemic goals. A multidisciplinary set-up for screening and management of diabetes, and its complications remains essential.

Résumé Les spondyloarthrites (SpA) sont parfois associées à des manifestations extra-articulaires, dont la néphropathie, retrouvée chez 3 à 6 % des patients. Dans les séries historiques, l’amylose AA, secondaire au syndrome inflammatoire chronique, était la cause la plus commune de maladie rénale au cours des SpA. Avec l’utilisation des biothérapies et la prise en charge rhumatologique précoce, la prévalence de l’amylose AA secondaire diminue en Europe, mais reste encore une pathologie fréquente et sévère dans d’autres populations, responsable de syndrome néphrotique et d’insuffisance rénale parfois rapidement progressive, aboutissant à la dialyse. La néphropathie à dépôts mésangiaux d’IgA est le second type de glomérulonéphrite rencontrée dans ce contexte. Dans la majorité des cas, il s’agit de la découverte fortuite d’une hématurie ou d’une protéinurie lors du suivi d’une SpA, mais l’évolution vers l’insuffisance rénale, parfois sévère, n’est pas rare et ce malgré les traitements anti-inflammatoires (corticoïdes/agents anti-TNF). Pour finir, de nombreuses causes médicamenteuses peuvent causer ou aggraver une néphropathie au cours de la SpA, au premier rang desquelles les AINS. Le dépistage systématique de la néphropathie (prise de la pression artérielle, bandelette urinaire, dosage de la créatinine et estimation du DFG) doit être réalisée de façon annuelle chez le patient suivi pour une spondyloarthrite, afin d’approfondir les explorations néphrologiques et ajuster le traitement néphroprotecteur en cas de néphropathie avérée. Une lithiase urinaire, source d’hématurie microscopique, doit être discutée parmi les diagnostics différentiels. Nephropathy is one of the extra-articular manifestations associated with spondyloarthritis (SpA), and is observed in up to 6 % of all patients. In historical series, AA amyloidosis, secondary to the chronic inflammatory syndrome, was the most frequent cause of renal disease encountered in this setting. The use of TNF blockers therapies and early diagnosis of the rheumatic disease has induced a significant decrease in the incidence of amyloidosis in Europe, but this renal complication remains one of the main concerns in other populations, being responsible for nephrotic syndrome, rapidly progressive renal failure and chronic dialysis. IgA nephropathy (IgAN) is the second major type of glomerulopathy associated with SpA. In most cases, IgAN is revealed by asymptomatic hypertension, proteinuria and/or haematuria but this silent nephropathy can sometimes progress to end-stage renal disease (ESRD) despite anti-inflammatory treatment with anti-TNF drugs or corticosteroids. Finally, several nephrotoxic drugs are prescribed to SpA patients, such as NSAID, which may deteriorate renal function. Systematic and regular screening of kidney disease, including blood pressure monitoring, serum creatinine measurement and urine dipstick analysis, must be proposed to all SpA patients. Confirmation of chronic kidney disease should lead to etiological investigations and initiation of nephroprotective therapy to stop progression towards ESRD. [ABSTRACT FROM AUTHOR]

BACKGROUND: The association between blood pressure (BP) and diabetic kidney diseases in Africans has been less well investigated. We assessed and compared the strength of the association and discriminatory capability of systolic (SBP), diastolic (DBP) BP, pulse pressure (PP) and mean arterial blood pressure (MAP) for nephropathy risk in sub-Saharan Africans with type 2 diabetes. METHODS: Participants were 420 consecutive individuals (49% men) with type 2 diabetes receiving chronic care in two main referral centres in the two major cities of Cameroon. Logistic regression models were used to compute the odd ratio (OR) and 95% confidence interval (95% CI) for a standard deviation (SD) higher level of SBP (25 mmHg), DBP (13), PP (18) and MAP (16) with nephropathy risk. Discrimination was assessed and compared with c-statistics and relative integrated discrimination improvement (RIDI, %). RESULTS: The adjusted OR (95% CI) for nephropathy with each SD higher BP variable were: 1.45 (1.15-1.84) for SBP, 1.33 (1.06-1.66) for DBP, 1.35 (1.06-1.71) for PP and 1.42 (1.13-1.78) for MAP. C-statistic comparison showed no difference in discrimination of models with each of the BP variables (p-valuesless than 0.69 for c-statistics comparison). However, RIDI statistic always showed and enhancement in models discrimination when other BP variables were replaced with SBP, although such an enhancement was marginal for MAP. Using BP combination modestly improved models, discrimination. CONCLUSION: SBP was the best predictor of prevalent nephropathy in this population, while DBP was the less effective. This may have implication for kidney disease risk stratification and protection.

Résumé: HNF1B est un gène codant pour un facteur de transcription impliqué dans le développement de l’appareil urogénital, du pancréas et du foie. Transmise sur le mode autosomique dominant, la maladie par mutation de HNF1B résulte d’une délétion complète du gène (≈50 %), ou de mutations ponctuelles (≈50 %). Une néomutation est décelée dans la moitié des familles. La maladie combine atteinte rénale et manifestations extrarénales. La néphropathie constitue l’anomalie la plus fréquente. Hétérogène, elle peut être décelée par échographie anténatale (reins hyperéchogènes ou dysplasie kystique bilatérale), ou dans l’enfance. À l’âge adulte, il s’agit d’une néphropathie tubulo-interstitielle chronique, avec perte lente de fonction rénale (−2mL/min par an), et des kystes corticaux peu nombreux, des anomalies pyélocalicielles ou un rein unique. Une hypomagnésémie et une hypokaliémie par fuite rénale sont fréquentes. Plus rarement, un syndrome de Fanconi ou un cancer chromophobe du rein est possible. Ce dernier justifie un dépistage échographique régulier. L’atteinte extrarénale concerne le pancréas (diabète MODY-5, insuffisance ou atrophie pancréatique), le foie (anomalies fluctuantes des tests hépatiques) et l’appareil génital (utérus bicorne chez la femme, infertilité par agénésie déférentielle chez l’homme). La fréquence d’un retard mental modéré semble accrue. L’hétérogénéité phénotypique intrafamiliale est notable. Au stade terminal de l’insuffisance rénale une transplantation est possible, ou une transplantation rein–pancréas en cas de diabète associé. Inconnue il y a une décennie, la néphropathie liée à HNF1B vient en fréquence au second rang des maladies rénales génétiques à transmission dominante. Les informations colligées permettent désormais une prise en charge appropriée, incluant un conseil génétique précis. [Copyright &y& Elsevier]

Résumé: Le tabagisme reste un problème majeur de santé publique. Il est associé à un nombre considérable de décès dans la population mondiale. Fumer est au même titre que l’hypertension artérielle, un facteur indépendant de progression pour toute néphropathie primaire ainsi que chez le transplanté rénal. Il semble que l’arrêt du tabac ralentit la progression de l’insuffisance rénale chez les fumeurs. Les données de la littérature sont parfois à ce sujet contradictoires en raison de certaines faiblesses méthodologiques. En revanche, les modèles expérimentaux mettent en lumière les effets néfastes du tabac par des facteurs hémodynamiques et non hémodynamiques. La conclusion est qu’un effort important supplémentaire doit être produit par la communauté néphrologique afin de motiver nos patients de cesser de fumer. [Copyright &y& Elsevier]

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Copyright of Néphrologie & Thérapeutique is the property of John Libbey Eurotext Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Néphrologie & Thérapeutique is the property of John Libbey Eurotext Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: Cadmium is a metallic impurity in various minerals. The two main cadmium exposure sources in general population are food and tobacco smoking. Its industrial exploitation has grown in the early twentieth century. Cadmium is used in accumulators or alkaline batteries (80%) and in pigments for paints or plastics (10%), in electrolytic process by deposit or by cadmium plating on metals or to reduce melting points (welding rods…). Cadmium is a cumulative toxic substance whose half-time for elimination is about 20 to 40 years and it is mainly stored in the liver and kidneys. Inhalation of cadmium oxide fumes may cause inhalation fevers or chemical pneumonitis. Cadmium chronic poisoning causes mainly renal tubulopathy and could be the cause of osteomalacia and diffuse osteoporosis. Cadmium is classified as certain carcinogen agent for humans by International Agency for Research on Cancer (IARC). The most relevant biological index exposure is the urinary cadmium. According to literature, no chelating agent can be still used in human cadmium poisonings. In France, some diseases caused by occupational exposure to cadmium may be compensated. [Copyright &y& Elsevier]

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Copyright of Obésité is the property of Lavoisier and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: Henoch-Shonlein purpura is a systemic vasculitis with predominance in children; its diagnosis is clinical and prognosis is often favourable. The aim of this study was to precise the epidemiological, clinical profiles and course of disease. The authors studied retrospectively 67 cases enrolled in the paediatrics department of the Fattouma Bourguiba university hospital in Monastir (Tunisia) during 14 years (1990–2003). Children were aged from 3 to 13 years (mean age: 7 years and half) with male predominance (sex-ratio: 1.18). Purpura was observed in all cases. Articular and digestive manifestations were noted respectively in 88.4% and 67.3% of cases. Renal involvement was noted in 17 cases (25.3%); one of them had nephropathy. The Henoch-Sconlein purpura is generally a benign disease. Prognosis depends on the risk digestive complications at the acute phase and renal damage at long-term. [Copyright &y& Elsevier]

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Copyright of EMC-Nephrologie is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of EMC-Medecine is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purpose. – To analyze anatomoclinic and evolutive aspects of the renal involvement associated to the Behc¸et’s disease through 6 observations collected in the nephrology department from 1985 to 2000 and to make a review of the literature.Methods. – Retrospective study, diagnosis of Behc¸et’s disease according to the Classification of the International Group Study on the Behc¸et’s disease and renal damage confirmed by histology.Results. – Our patients all male are aged between 25 to 55 years with a mean at 34 years old. The renal damage was revelated by a nephrotic syndrome in 3 cases and by a proteinuria at 1 to 2.7 g/day in 3 cases. Microscopic hematuria was present in 2 cases and arterial hypertension in 2 cases. The renal insufficiency has been noted in 2 cases of which severe in one of them. The renal biopsy showed an amyloidosis AA type in 3 cases, a segmental and focal glomerulonephritis in 2 cases and a thrombotic microangiopathy associated to a moderate tubulo-interstitiel lesions by toxicity of ciclosporine in 1 case. The extrarenal signs were dominated by bipolar aphtosis in all cases, necrotic pseudofolliculitis and the no specific cutaneous hyperreactivity in 5 cases and the erythema nodosum in 1 case. The ocular manifestation has been noted in 4 cases and articular manifestation in 3 cases. The vascular manifestation has been noted in one case. The treatment was colchicine in 4 cases and prednisone and cyclophosphamide in 1 case. Three patients were lost of view and a patient died in hemodialysis. The 2 other patients with amylosis had persistent proteinuria with a normal renal function.Conclusion. – The kidney is one of organs that can alter the prognosis of the Behc¸et’s disease; so, its screening must be realised in each patient with this disease. [Copyright &y& Elsevier]

Purpose. – Though currently asymptomatic, a sickle cell trait can be responsible for renal abnormalities with macroscopic hematuria.Methods. – Ten patients were admitted to our department with gross hematuria in sickle trait.Results. – Six blacks men and four blacks women, aged from 17 to 53 years, had recurrent episodes of gross hematuria with clots in five patients and lumbar pain in four patients. Duration of gross hematuria varied from two weeks to two years. Imaging findings were normal. Urinary concentration ability was abnormally low in all patients but none had an impairement of the ability to dilute the urine or tubular dysfunction. Resolution of hematuria was obtained by abundant and alkaline hydratation in three patients. In six patients, aminocaproic acid was successfully employed, with a complete (3/6) or partial (3/6) efficiency. In one last patient, oral urea permitted a partial improvement.Conclusion. – Combination of chemical and physical factors in renal medullary of sickle cell trait are reponsible for gross hematuria and impaired capacity to concentrate the urine. A cautious care must still be given to make this association a diagnostic of exclusion. Clinical is usually benign. When resting and alkaline hydration are not sufficient to resolve hematuria, antifibrinolytic agents such as aprotinin, oral urea, urologic technical are sometimes necessary. [Copyright &y& Elsevier]

Gout secondary to lead-induced nephropathy is a long-term complication of occupational lead exposure. We report a case of this now-rare condition. The patient was a 63-year-old man who had been a miner from 1950 to 1970. Thirty years after cessation of his exposure to lead, he experienced onset of inflammatory symmetric polyarthritis with joint deformities. Hyperuricemia, moderately severe renal failure, and tubular acidosis were found, indicating gouty polyarthritis. Blood lead levels were high, establishing that the cause was lead poisoning. EDTA chelation therapy was effective. Lead poisoning is frequently under-recognized because the clinical manifestations are often minimal and the diagnosis difficult to establish. We suggest that lead bound to bone may result in continued exposure to lead after cessation of industrial or environmental exposure. Chelating agents are valuable for the diagnosis and can ensure a full recovery. [ABSTRACT FROM AUTHOR]

Abstract: Alport syndrome is a rare progressive hematuric nephropathy associated with sensorineural deafness. Leiomyomatosis associated with Alport syndrome is quite rare. We report a particular case of Alport syndrome which was diagnosed in the setting of an oesophageal leiomyomatosis. Alport syndrome and leiomyomatosis are caused by mutation of the genes encoding for the alpha chain of type IV collagen. In view of the important clinical and genetic implications, renal function and urinary status should be controlled in any patient with oesophageal leiomyomatosis. [Copyright &y& Elsevier]

Henoch-Schonlein purpura (HSP) is a small-vessel vasculitis secondary to IgA deposits. We conducted a retrospective study of 14 cases of HSP in adults based on EULAR/PRINTO/PRES classification criteria for HSP over a period of 18 years (1996 to 2014) in Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, South Tunisia. The purpose of our study was to highlight the epidemiological, clinical, therapeutic and evolutionary features of HSP in our adult patients. The average age of patients was 33 years (ranging from 17-64 years) with male predominance (sex ratio of 1.8). Vascular purpura with petechiae was constant. Articular manifestations (arthralgia and/or arthritis) were reported in 9 patients (64.2%). Gastrointestinal involvement was reported in 13 patients (92.8%). Renal failure was found in 11 patients (78.5%) revealed by nephrotic proteinuria in 7 cases, microscopic haematuria in 9 cases, high blood pressure in 4 cases and renal impairment in 1 case. The most common histological type was diffuse proliferative endocapillary glomerulonephritis (36.3%). High dose corticotherapy was initiated in 7 patients with proliferative renal impairment, bolus of Solu-Medrol in 4 cases, associated with cyclophosphamide in one case. Two patients with severe gastrointestinal involvement received corticotherapy. After a mean follow-up period of 18.5 months (4-36 months), outcome was favorable in all cases without relapse and chronic renal failure was reported only in one case., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts., (© Hanen Loukil et al.)

Objective: To investigate visceral comorbidities observed during gout., Patients and Method: A descriptive and analytical cross-sectional study of gout patients diagnosed according to the ACR 1977 criteria, followed between January 1, 2012 and January 31, 2017 in the Rheumatology Department of the Brazzaville University Teaching Hospital in Congo. Patients were assessed for visceral cardiovascular, renal, and cerebral comorbidities., Results: 105 patients with gout were identified during the study period, 75 (71.43%) of whom met the inclusion criteria, thus constituting the study population. There were 60 men and 15 women (sex ratio 4/1), mean age 60 +/- 11.47 years (range: 30-82 years). Visceral co-morbidities were represented in 50.67% of cases. These included renal failure (21.33%), coronary heart disease (14.67%), stroke (12%) and heart failure (2.67%). The presence of comorbidities was associated with irregular follow-up in 86.56% of cases (P = 0.025) and therapeutic nonobservance in 93.75% of cases (P = 0.006)., Conclusion: Gout is a serious disease because of the frequency of its visceral comorbidities. Irregular monitoring and therapeutic nonobservance are the main factors associated with this., (Le comitée de rédaction se réserve le droit de revoyer aux auteurs avant toute soumission à l'avis des lecteurs les manuscrits qui ne seraient pas conformes à ces modalités de présentation. En outre il leur conseille de sonserver un examplaire du manuscrit, des figures et des tableaux.)

Background: Sickle cell anemia is the most common hereditary hemopathy in the world. It is a disease that attacks all the systems of the organism. The kidneys are among the most sensitive organs of this disease. The main objective of this study is to detect sickle cell nephropathy in patients followed at the National Reference Center for Sickle Cell Disease in Niamey., Methods: It is a prospective study carried out over a period of one year (January to December 2016). It included patients aged at least two years who had not had a vaso-occlusive crisis (VOC) for 2 months and not transfused for at least 3 months. A questionnaire was sent to each patient to collect demographic, clinical and biological information. Each patient provided a fresh blood and urine sample to assess biological parameters. We used Schwartz's formula in children and CKD-EPI in adults to assess glomerular filtration rate. The Pearson correlation coefficient (r) was used to assess the relationship between the different parameters under study. The threshold for statistical significance was set at 0.05., Results: Two hundred and eighty participants were included in the study. Adolescents (≤17years) were 226 (80.71%) and adults were 54 (19.29%). The mean age of the patients was 11.94±4.70years. It was lower in SS subjects and higher in SC. The SS form accounted for 87.5% of the three sickle cell disease phenotypes encountered. The age group 6-15years was the most predominant (42.86%). The mean baseline hemoglobin in the sample was 7.59±1.15g/dL ; (6.8 ±0.9d/dL) in SS patients and higher (10.5±2.1g/dL) in SC patients. The average glomerular filtration rate was 201.04mL/min/1.73m 2 . Glomerular hyperfiltration was found in 90% of the sample, it was lower (77.6%) in Sβ thalassemia patients (P=0.000). In young subjects hyperfiltration was present in 84.95% against it was observed in only 29.63% of adults. Proteinuria was positive in 20% of patients. Positive proteinuria was found in 6.20% pediatric cases. The SC form was the most affected and in 74.6% of the patients it was positive at 1+. Chronic kidney disease (CKD) was found in 10% of the participants, of whom 75% were of the SS phenotype. Sβ thalassemia subjects had no CKD. The male sex was predominant in subjects with CKD (P=0.000). Patients over 15 years of age accounted for more than 95% of patients in both forms of the disease (P=0.0001)., Conclusion: The SS form of the disease, recurrent vaso-occlusive crisis, male sex and advanced age were the main precipitating factors in the development of renal function disorders., (Copyright © 2017 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.)

IgA vasculitis nephritis affects the prognosis of this disease in adult patients. This study aimed to examine the clinical characteristics of this renal involvement in adults and to identify factors influencing renal prognosis. We conducted a retrospective monocentric study of patients with histologically confirmed IgA vasculitis nephritis (rheumatoid purpura) (EULAR classification criteria) with renal involvement classified according to Pillebout classification. We analyzed renal survival and identified the factors influencing renal prognosis. Twenty-five patients were included (sex ratio M/F = 2.57), their average age at diagnosis of rheumatoid purpura was 35,76 years. Purpura was diagnosed in 100% of cases, with articular involvement in 28% of cases. Renal failure was identified in 44% of cases. The most common histological classification was IgA vasculitis nephritis (class II). Clinical remission was observed in 44% of cases and an evolution toward chronic renal failure (end-stage renal disease) in 36% of cases. Renal survival at 195 months was 57%. The identified prognostic factors were digestive involvement (p = 0.022), early renal failure (p = 0.0004), glomerular classification (P=0,001) and the severity of the histological lesions, renin-angiotensin system blocker treatment (p = 0.01) and plasma exchanges (p = 0.03). Our study shows that renal involvement during IgA vasculites can be relatively severe with poor renal prognosis. The identification of clinical and histological prognostic factors may be useful as guidance for the development of prospective therapeutic studies., Competing Interests: Les auteurs ne déclarent aucun conflit d’intérêts.

Résumé: Une femme âgée de 39 ans a développé une néphropathie dans le cadre d''un lupus érythémateux systémique après un voyage en Jamaïque. Un traitement par prednisone, azathioprine et aspirine fut prescrit. Comme cette patiente avait des préoccupations esthétiques, elle décida de ne pas prendre le traitement. À la place, elle prit les conseils de son entraîneur de bodybuilding qui lui suggéra l''administration intramusculaire de stanozolol pendant dix semaines pour augmenter son volume fessier. Une semaine après avoir administré la dernière injection, il n''existait aucune activité de la maladie. Le fait que le lupus soit en rémission spontanée ou grâce aux injections de stanozolol restera un mystère dans ce cas clinique dont l''issue fut favorable. [Copyright &y& Elsevier]

IgA nephropathy is the most common form of primary glomerulonephritis worldwide and an important cause of chronic kidney disease and end-stage kidney failure. Its pathophysiology remains in part unsolved but it is recognized as an immune complex disease. Recent years have brought progress in the field through the discovery of several genetic susceptibility loci and the formulation of the multi-hit pathogenesis model. Presentation, clinical course and histology can be extremely variable, making any histological classification still difficult. Indeed, most therapeutic studies until now include patients based only on the severity of clinical criteria but the new classification of Oxford should change that. Only the management of patients with nephropathy with minimal change glomerular lesions and nephrotic syndrome, or extra-capillary glomerulonephritis and rapidly progressive renal failure, is consensual: Corticosteroids alone for the first and associated with immunosuppressive drugs for the latter. The recent Kidney Disease Improving Global Outcomes (KDIGO) consensus treatment guideline is still controversial, especially in light of the last clinical studies. Corticosteroid therapy can be discussed in patients with proteinuria greater than 1 g/day without renal failure. All IgA nephropathy patients should benefit from the global management of chronic glomerular disease, including a renin-angiotensin system blocker in the presence of hypertension or proteinuria., (Copyright © 2016 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.)

Contrast-induced nephropathy (CIN) is common in hospitalized patients. Its occurrence is associated with an increased hospitalization stay and cost, morbidity and mortality. Thus, preventives strategies remain a major issue. Patients that are referred for cardiac catheterization are among the most vulnerable to develop CIN due to their comorbidities. Moreover, in some cases, such preventives measures cannot be introduced due to emergent clinical settings. After a summary regarding the properties of iodinated contrast medium, the aim of this work was to review the definition, pathophysiology, diagnosis and preventive strategies related to CIN., (Copyright © 2016. Published by Elsevier SAS.)

Sickle-cell disease (SCD), one of the most common severe monogenic disorders into the world, is associated with an increased frequency of chronic kidney disease. SCD is caused by a point mutation in the gene encoding β globin gene which leads to the formation of hemoglobin S that polymerises after deoxygenation. HbS polymerisation is associated with erythrocyte rigidity and vaso-occlusive episodes that play a central role into SCD pathogenesis. The spectrum of renal diseases during SCD is broad and includes various renal manifestations which become more apparent with increasing age. Underlying pathophysiological processes involved in sickle cell nephropathy are multifactorial but endothelial dysfunction related to chronic hemolysis is a key factor contributing to renal involvement. Our review focuses on the pathogenesis and on the spectrum of renal manifestations occurring in SCD patients., (Copyright © 2016. Published by Elsevier SAS.)

Abstract: Fabry disease and cystinosis are both lysosomal diseases. Some clinical features (such as renal and corneal involvement) are shared by both diseases whereas many other features are different (mode of inheritance, rate of progression, mechanism of lysosomal storage, therapeutic modalities etc.). Intermediary mechanisms that lead from lysosomal overload to lesions and disease are still incompletely understood. [ABSTRACT FROM AUTHOR]

Abstract: Fabry disease, an X-linked lysosomal storage disorder due to alpha-galactosidase A deficiency, leads to an accumulation of globotriaosylceramide resulting in a multisystemic disorder. The initial manifestations of the disease are not specific, leading to a delayed diagnosis. We report a patient in whom the diagnosis was obtained by family screening and the confrontation of clinical signs. We also present a 4 year follow-up under enzyme replacement therapy (agalsidase β, 1 mg/kg/14 days). [ABSTRACT FROM AUTHOR]

Permanent proteinuria is an early marker of the kidney dysfunction. Tracking by urinary strip, imposes a precise quantification by the laboratory. In front of the difficulties of urine collection during 24 hours, protein determination can be carried out on the urine of a miction and can be expressed as g per g of creatinine (uPCR). We analysed the impact of the expression of the proteinuria in g/L (uP) as compared to uPCR on the urinary electrophoretic profiles. A revision and a simplification of this in practice clinical interpretation is proposed. The proteinuria of 696 in-patients was quantified on an Olympus AU2700. The urinary electophoretic profiles (SDS-AGE) were interpreted by two biologists. uP and uPCR are well correlated (r=0.847, p< 0.0001). Data agreed for proteinuria > 1 g/L but concordance was obtained only in 74% of the subjects and 55% of the pathological patients. A repetition of the analyses is suggested. The interpretative diagram suggested with simplified comments improved interpretation. We advise an interpretation by two biologists. In conclusion, interpretation of the urinary electrophoretic profile rests on the rate of total proteinuria. Expression of the proteinuria as g/g of creatinine must be associated with the expression in g/L because of the analytical conditions. The SDS-AGE Technique does not allow the identification of the monoclonal compound but allows a quantitative follow-up under treatment and especially an early tracking of the type of renal dysfunction.

HNF1B encodes for a transcription factor involved in the early development of the kidney, pancreas, liver and genital tract. Mutations in HNF1B are dominantly inherited and consist of whole-gene deletion, or small mutation. De novo mutation occurs in half of tested kindreds. HNF1B-related disease combines renal and non-renal manifestations. Renal involvement is heterogeneous and may escape early recognition. During fetal life and childhood, it mostly consists of hyperechogenic kidneys or bilateral renal cystic hypodysplasia. The adult phenotype encompasses tubulointerstitial profile at presentation and slowly progressive renal decline (-2 ml/min/year). Renal involvement includes renal cysts (mostly few cortical cysts), a solitary kidney, pelvi-caliceal abnormalities, hypokalemia and hypomagnesemia related to tubular leak, and more rarely, Fanconi syndrome and chromophobe renal carcinoma. The latter warrants ultrasound screening. Extrarenal phenotype consists of diabetes mellitus (MODY-5), exocrine pancreas failure and pancreas atrophy; fluctuation liver tests abnormalities; diverse genital tract abnormalities in females or infertility in males; and mild mental retardation in rare individuals. Phenotype heterogeneity within families is striking. Individuals progressing to end-stage renal disease are eligible for kidney transplantation (or combined pancreas and kidney transplantation for diabetic individuals). While HNF1B disease was still unknown one decade ago, it has emerged as the second most prevalent dominantly inherited kidney disease. Data available pave the way for early recognition and improved specific management, including genetic counselling., (Copyright © 2013. Published by Elsevier SAS.)

Unlabelled: This study sought to assess the prevalence of lupus nephropathy and to determine its clinical, histological, therapeutic and outcome profiles., Patients and Methods: This retrospective study examined records covering a 10-year period from 1999 to 2009 at the nephrology department of the Aristide Le Dantec University Hospital Center., Results: The records showed 43 patients with systemic lupus erythematosus (SLE), and 72% had lupus nephritis. The patients' mean age was 32.9 years; 40 were women and 3 men, for a sex ratio of 0.075. The lupus nephritis led to discovery of SLE in 27.9%. The mean proteinuria concentration was 2.01 g/dL. Seventeen patients had impaired renal function, and 26 had renal biopsies. It found class II nephritis in 2 patients, class IV in 10, and class V in 12. In addition, fifteen patients received combined corticosteroid + immunosuppressant treatment. Four patients died during the study period, one from SLE activity, two from complications of renal failure, and the fourth from sepsis., Conclusion: A larger study would be useful to assess more acurately the prevalence of various classes and severity of lupus nephropathy among blacks living in sub-Saharan Africa.