Particularités de l'amylose rénale au Togo

Objectifs :analyser la démarche diagnostique, thérapeutique, et l'évolution des trois cas d'amylose rénale diagnostiqués avec une revue de la littérature. Méthodologie : Il s'agissait d'une étude rétrospective et descriptive portant sur trois cas cliniques observés sur une période de 27 mois, avec une revue de la littérature. Résultats : Nos patients, adultes jeunes (17, 15 et 31ans) ont été tous admis pour un syndrome né-phrotique. L'examen clinique a retrouvé dans tous les cas des œdèmes des membres inférieurs de type rénaux. La biologie a retrouvé une protéinurie massive sans hématurie et une hypoprotidémie dans tous les cas et une fonction rénale préservée au début. Aucune étiologie n'a pu être trouvée pour ce syndrome néphrotique qui s'est avéré cortico-résistant dans tous les cas d'où une amylose rénale avait été suspectée. Une Biopsie des Glandes Salivaires Accessoires réalisée était en faveur d'une amyloseavec des signes d'inflammation. Le typage de l'amylose n'a pas été fait, compte tenu de la pauvreté de notre plateau technique, ce qui a empêché l'instauration d'un traitement curatif de cette pathologie. L'évolution a été marquée dans un cas par une insuffisance rénale terminale, plu-sieurs épisodes de septicémie sévère avec décès de la patiente. Dans un autre cas, nous avons as-sisté à un arrêt de grossesse. Conclusion :L'amylose rénale est une maladie rare. Son diagnostic est histologique et il faut y pen-ser devant tout syndrome néphrotique surtout ceux cortico-résistants. Son traitement doit être précoce pour éviter l'évolution vers les complications

Profil évolutif d'un cas de syndrome néphrotique infantile par mutation génétique NPHS3 à Dakar

Les auteurs rapportent un cas de syndrome néphrotique (SN) infantile génétique révélé chez un garçon à l'âge de huit mois. Il présentait un syndrome néphrotique impur avec hématurie et insuffisance rénale. Il s'agissait d'un SN corticorésistant. La biopsie rénale avait permis de noter une hyalinose segmentaire et focale (HSF) classique. L'étude génétique a permis de retrouver une mutation dans le gène NPHS3. L'évolution était marquée par une persistance de la protéinurie, un retard staturopondéral et la surve aigu du poumon (OAP) entre deux séances d'hémodialyse. Le protocole néphrectomie-dialyse-transplantation rénale aurait permis de prendre en charge cet enfant

Cyclophosphamide versus cyclosporine in children with frequent-relapsing and steroid-dependent nephrotic syndrome in Khartoum State, Sudan

Background: in children with frequent-relapsing and steroid-dependent (FR/SD) nephrotic syndrome (NS) remission can be achieved with either cyclophosphamide (CPM) or cyclosporine(CSA). Our objective was to compare the efficacy and safety of these agents.Methodology: Records of all children with FR/SD NS who received CPM or CSA at the Pediatric Renal Unit, Soba Hospital, Khartoum, during the period 2005­2015 were retrospectively reviewed.Main outcomes were: remission rate, relapse rate, and renal outcome.Results: We studied 82 children with FR/SD NS treated with CPM (59.8%) or CSA (40.2%). Males were 69.5% and females 30.5%. The mean admission age was 5 ± 3.10 years. At 6 months,77.6% children on CPM and 60.3% on CSA were in complete remission (CR), (P=0.012) whereas 22.4% versus 39.4% relapsed respectively (P=0.012). At 12 months, 57.5% on CPM and 72.7%on CSA were in CR, (P=0.013) whereas 42.5% versus 27.3% relapsed respectively, (P=0.013). At 24 months, 16.6% on CPM and 29% on CSA were in CR, (P=0.030) whereas 83.4% versus 71%relapsed respectively, (P=0.030). The mean number of relapses per 24 months were 1.7± 0.86 inCPM group versus 2.2 ±0.85 in CSA group, (P=0.72). Mild complications were recorded in 12.4% of patients on CPM group versus 33.3% on CSA, (P=0.031). At the latest follow- up, there was no significant change from basal levels of TWBC, mean serum creatinine, GFR, or BMI, (P>0.05 for all parameters). Conclusion: In children with FR/SD NS, both CPM and CSA were effective and safe in achieving remission with less risk of serious side- effects. However, long-term remission was less stable with both agents

Pattern and outcome of renal diseases in hospitalized children in Tikur Anbessa specialized teaching hospital, Addis Ababa, Ethiopia

Background: Renal diseases are major causes of morbidity and mortality in pediatric practice. Pediatric patients with renal disease, especially younger ones may present with nonspecific signs and symptoms unrelated to the urinary tract. Unexplained fever or failure to thrive may be the only manifestation. Most children with renal diseases in our hospital arrive very late either because of inadequate health awareness among the parents or failure of recognizing the symptoms of renal diseases at a lower health care level. This review will highlight the symptoms of renal diseases at presentation and outcomes of treatment in children in a major referral hospital.Methods: A cross-sectional retrospective chart review was done over a period of 3 years (June, 2012 to May, 2015) in 381 admitted children (Birth-17 years) at Tikur Anbessa Specialized Teaching Hospital in Addis Ababa, Ethiopia.Results: Out of 14521 pediatric ward admissions in the study period, kidney diseases accounted for 473 admissions in 381 children, accounting for 3.3% of all admissions. The three most common renal diseases observed were congenital anomalies of the kidney and urinary tract (CAKUT) seen in 127 children (26.8%), followed by nephrotic syndrome in 80 children 16.9% and acute glomerulonephritis in 58 children (12.2%). Other renal diseases observed were urinary tract infection 8.0%, urolithiasis 6.7%, Wilm's tumor 6.3%, acute kidney injury 4.2% and chronic kidney disease 4.0%. Other less frequently detected diseases were bladder exstrophy, lupus nephritis, Henock shonlein Purpura nephritis and prune-belly syndrome.Out of 381 children 207 (54.3%) recovered normal renal function, 20(5.2%) remained with proteinuria, 13(3.4%) progressed to chronic kidney disease and 11(2.9%) died. Sixty one nephrotic children (76.3%) achieved remission but 17 children (21.3%) remained with proteinuria; one steroid resistant child died of end stage renal disease. Ten children (2.6%) with different renal diseases were lost to follow-up and 5 (1.3%) discharged against medical advice.Conclusions: This data reflects that many of the renal diseases are preventable or potentially curable. Therefore, improvement of pediatric renal services and training of health workers would help in early detection and treatment of these conditions leading to reduction in their morbidity and mortality

A descriptive retrospective study on children with newly diagnosed nephrotic syndrome presented to Tripoli Children Hospital during the period between Jan. to Dec. 2014

Introduction: Nephrotic syndrome is a clinical picture characterized by severe proteinuria, hypoalbuminemia, edema and hypercholesterolemia. A retrospective study was carried out in order to describe disease pattern in newly diagnosed nephrotic syndrome of children admitted to Tripoli children hospital during the year 2014.Methods: The medical data of 56 patients aged between 1 year and 11 years diagnosed with idiopathic nephrotic syndrome were analysed using SPSS software. The data included gender differences, sensitivity to steroid therapy, relapses during six months of follow up and the effect of variable factors such as family history, hypertension, hematuria, serum urea on the degree of relapse.Results: Out of 56 patients with newly diagnosed nephrotic syndrome (NS), 60.7% were boys and 39.3% were girls, with a mean age 4.2±2.2 years. Age was related significantly to the response to steroid therapy, where 79.5% of patients aged between 2-8 years (group 1) had steroid sensitive nephrotic syndrome (SSNS) compared with only 41.7% of patients aged less than 2 years or more than 8 years (group 2) (P<0.001). Although girls relapsed more than boys (70.5% versus 57.1%) during six months of therapy, this difference was not statistically significant. Similarly, no other factors measured such as family history of NS, hypertension, hematuria, serum complement and urea had any effect on the percentage of relapse in patients with newly diagnosed NS. Conclusion: NS is one of the commonest reasons for admission to nephrology ward. It is more common in boys than girls. The age at presentation related significantly to the response to steroidal therapy. Regarding relapses, girls seems to relapse more frequent than boys and relapses was seen more in age group 1 than group 2, however, these differences were not significant. Other factors studied seems to have no effect on the relapse rate of children with newly diagnosed NS

Urinary tract infection in children with nephrotic syndrome in Kano, Nigeria

Background: Nephrotic syndrome is a common childhood renal disorder; the prevalence of Urinary tract infection (UTI) in these patients is high. The increased prevalence of UTI are due to immunoglobulin loss; defective T cell function; presence of ascites and relative malnutrition. Objective: The study is to evaluate the prevalence of UTI ; its etiological agents; antibiotics; sensitivity pattern and the outcome in children with nephrotic syndrome. Methods: A prospective study of all patients with diagnosis of nephrotic syndrome from January 2003 to December 2006. Urine specimen were routinely obtained by clean catch method following careful preparation urethral orifices. The specimens were processed immediately. Five millimeters (5mml) loopful of the sample were inoculated on a blood agar and CLED agar plates. Identification of the organism to species level was by using stokes disc diffusion technique. Results : Forty two patients were studied. The mean age and SEM for males was 8.2 + 0.5 years and females with 7.9 + 0.8years. The age range was two to fifteen years UTI was caused predominantly by Staphylococcus aureus in 67.9; Klebsiella species (17.9) and Pseudomonas (14.2). There was high invitro resistance of these organisms to nalidixic acid and ampicillin but sensitive to cefotaxime; ceftriazone and ciprofloxacin. Conclusion: It is recommend that UTI should be sought for in patients with nephrotic syndrome and treatment should be prompt and appropriate

Selective protein index and clinical features of childhood nephrotic syndrome in Kampala [see comment]

A cross sectional study was carried out to determine Selective Protein Index (SPI) and relate it to some clinical features that might be useful in deciding on the treatment of children with the nephrotic syndrome in four hospital in Kampala; Uganda. Radial immunodiffusion technique in commercially prepared plates was used for the determination of SPI. High selective protein index was found in 23 out of 60(38) and low selective protein index was found in 37 out of 60(62) of the patient studies. In patients who were not on antihypertensive or diuretic therapy; the clinical features found to be significantly associated with high Selective Protein Index (SPI) were: normal blood pressure and generalised oedema. When these features occurred together in a patient; their positive predictive value for high SPI was found to be 71. Normal blood pressure and generalised oedema appear to be the clinical features that may be used in combination to select children with nephrotic syndrome who are likely to have high Selective Protein Index and therefore to respond to steroid therapy

Protéinurie et syndrome néphrotique de l'adulte dans le service de néphrologie de l'unité d'hémodialyse de l'hôpital du Point G :à propos de 65 cas

Le syndrome néphrotique est une affection assez fréquente dans le monde en général et dans les pays sous-développés en particulier, lié au bas niveau socio-économique. En Afrique, en particulier au Mali, plusieurs facteurs interviennent dans la pathogénie de la maladie modifiant son aspect caricatural. Nous avons réalisé une étude rétrospective portant sur un échantillon de 65 patients âgés de 15 à 63 ans. L'objectif était d'étudier la protéinurie au cours du syndrome néphrotique de l'adulte, afin de déterminer les aspects quantitatifs, les signes cliniques et biologiques associés et les aspects thérapeutiques. Il ressort que la protéinurie était supérieure à 3g/24h dans 70,76% des cas, comprise entre 1 et 3 g/24 h dans 21,54% des cas et entre 0,5 et 1g/24h dans 7,69% des cas. Les signes cliniques étaient plus fréquents chez les patients présentant une protéinurie supérieure à 3g/24h. Quant aux signes biologiques, ils étaient diversement observés selon le taux de protéinurie. Le traitement spécifique de la protéinurie a consisté en une corticothérapie, utilisée dans 93,85% des cas, seule ou associée aux IEC et aux immunosuppresseurs. Le traitement non spécifique a fait appel aux IEC dans 6,15% des cas

Another look at the nephrotic syndrome in adult Nigerians : pathological and immunological findings

One hundred and twenty adult Nigerians with the nephrotic syndrome were subjected to similar pathological investigations which included light microscopy and immunoperoxidase study of renal biopsy specimens; serum immunoglobulins and C3c levels. This study shows the high incidence of minimal change nephropathy in our adult population

Nephrotic syndrome in Port Harcourt - clinical presentation and response to steroids

Thirty children who presented at the University of Port Harcourt Teaching Hospital with nephrotic syndrome were studied retrospectively. The peak incidence was between the ages of 3 and 5 years. All showed heavy urinary protein excretion (40mg/m2/hr); hypoalbuminaemia (25g/1) and hypercholesterolaemia (6mMol/1) Plasmodium falciparum was isolated from the blood of 2 patients (6.7). Seven patients who had no evidence of sickle cell anaemia; hepatitis; hypertension or impaired renal function were treated with prednisolone 60mg/m2/day and went into remission in 10 - 30 days after starting therapy; (p = 0.001). Renal biopsies; performed in 3 frequent relapsers showed hypercellularity of mesangial cells with normal glomerular capillary walls; on light microscopy. In conclusion; steroid sensitive nephrotic syndrome is not uncommon in the Rivers State of Nigeria and although the aetiology is unknown; it appears worthwhile treating such patients with steroids prior to a renal biopy