Le cyclophosphamide dans le syndrome néphrotique idiopathique : résultats et perspectives

IntroductionCyclophosphamide (CYP) has been used for over 40 years in patients with steroid-sensitive nephrotic syndrome (NSSS) presenting frequent relapses (NSRF) or steroid dependence (NSSD). However, the long-term success of treatment with cyclophosphamide is difficult to predict. The objectives of this study are to determine long-term outcomes of cyclophosphamide and identify the factors associated with sustained remission.MethodsWe retrospectively studied the data from 50 patients with idiopathic nephrotic syndrome, treated by oral cyclophosphamide and followed at service of pediatric for more than 8 years for idiopathic nephrotic syndrome and related factors for survival without relapse were evaluated by univariate analysis.ResultsThe median age at the time of diagnosis was 4.3 years, and median follow-up time was 1.7 years with the median of 8 years at the first use of CYC. Patients had received a median cumulative dose of 168 mg/kg. At the end of follow-up, 38% of patients entered into remission after using CYC while 62% failed to respond and further relapses then occur. The median time of stopping corticosteroid therapy was three month. The survival without relapse was respectively 56% (28 patients), 52% (26 patients), 48% (24 patients), and 38% (19 patients), at 6 months, one year, two years and more than two years. In univariate analysis, the survival without relapse was related to the age at the moment of starting the therapy par CYC (the median was 5 months for an age  <  8 years and 41 months for an age ≥ 8 years; P = 0.049), the type of nephrotic syndrome [36 months for SNRF, 4 months for NSSD and nephrothic syndrome steroid resistant (NSSR); P = 0.068], and the histological lesion (6 months for diffuse mesangial proliferation, 2 months for segmental glomerulosclerosis; P = 0.009). The age at the moment of diagnosis, the sex and the cumulative dose of CYC did not have significant influence.ConclusionThe results presented in this study suggest the use of oral cyclophosphamide for short period remain second line effective therapy. Further well-designed trials are required to evaluate the efficacy of other steroid-sparing agents.     

Évaluation de l’élève en difficulté et degré de précision nécessaire dans le jeu de langage enseignant

While research has identified effective practices to be implemented with students which present difficulties, the acceptability of these practices by the teacher is not warranted. The specificity of the profession and the context in which he intervenes also explain that the transfer of research data in the classroom is not a simple thing. This study helps to overcome this difficulty by an initial theoretical positioning, which is part of a larger project on finding promising ways in harmony with the context in which teachers work. The assessment of student's particular needs may be a very demanding operation, the following question motivates our approach: what a teacher needs to know in order to intervene efficiently with a student who presents difficulties? To the propensity to want to draw a complete picture of the situation of the student, the philosopher Ludwig Wittgenstein would rather invite the teacher to stick to what is necessary for him to know, by adopting a good focal adapted to its work context.     

Motifs de primo-consultations de néphrologie en 2014 dans le territoire de santé 5 de Bretagne

BackgroundIn France, the coordinated healthcare circuit means that patients should be referred to specialists, for example nephrologists, by another physician. However, there are no recommendations concerning the reasons justifying the referral to a nephrologist. The main purpose of our study is to describe the motif of first consultations in nephrology in the health area 5 of Brittany.MethodsWe retrospectively collected medical reports of first consultations by 17 nephrologists in the 4 centers of the study area, during the year 2014. In these letters, we noted the consultation motif, the specialty of the physician who refers the patient, and main characteristics of patients.ResultsWe included 662 first consultations. The main reason for consultations was chronic kidney disease (68.7%), including chronic renal insufficiency (56.9%), proteinuria (7.3%), microscopic hematuria (3.3%) and searching for chronic kidney disease in the presence of risk factor (1.2%). Other frequent reasons were the follow-up consultation after a pregnancy complicated by preeclampsia (9.5%), urinary lithiasis (5.7%), hypertension (3.8%) and hydroelectrolytic disorder (3.5%). Non-nephrology reasons represent 3.2% of first consultations. Almost all patients have been referred by a physician (99.7%), mainly a general practitioner (71.9%).ConclusionNephrology first consultations are realized according to the coordinated healthcare circuit since almost all are requested by another physician. The reasons are adapted to the specialty. The main reason is chronic kidney disease, often already associated with renal insufficiency chronic.     

Les nouveaux traitements médicamenteux dans le syndrome de grêle court de l’adulte

The European Society for Clinical Nutrition and Metabolism has recently published recommendations on the “definition and classification of intestinal failure (IF) in adults”. Two criteria must be simultaneously present to diagnose IF: a “decreased absorption of macronutrients and/or water and electrolytes due to a loss of gut function” and the “need for parenteral support”. Home parenteral support (HPS) is the primary treatment for chronic intestinal failure (CIF).The principal cause of CIF is the short bowel syndrome (SBS). The aim of treatments is to maximize intestinal absorption and avoid, reduce or eliminate the need for HPS to achieve the best possible quality of life for the patient. Teduglutide, an analog of glucagon-like peptide 2, improves intestinal rehabilitation by promoting mucosal growth thereby reducing intestinal losses and promoting intestinal absorption. Thus, several studies showed that the GLP2 decreases parenteral calorie and fluid requirements in SBS patients with CIF.     

Variabilité décisionnelle dans le choix thérapeutique des anévrismes intracrâniens

ObjectivesTo measure the variability in choosing treatment options in different clinical situations involving intracranial aneurysms.Materials and methodsA questionnaire regarding 25 clinical cases was presented via visual projection, to attendees of the Congrès de la Société française de neurochirurgie et de la Société de langue française de neurochirurgie held in Toulouse from May 9th to May 12th, 2012. The audience (n = 59) consisted of 58 neurosurgeons and one neuroradiologist. A minority of them (29.2%) stated that they specialized in vascular neurosurgery. The questionnaire dealt with 25 illustrative clinical cases, in which age, gender, and clinical context were provided along with the corresponding angiographic image of the aneurysm. The questionnaire asked whether the physician would have proposed treatment, and if so, which type of treatment (clip, coil or other). In addition, the physician had to indicate, on a scale of one to ten, the degree of confidence they had in their decision.ResultsFor one-third of the cases, there was at least 10% of the respondents who opted for a decision opposite to the one of the majority. For 41.7% of the cases, the proportion of respondents who opted for the clip was not significantly different from the proportion of those who opted for the coil.ConclusionEven in a relatively homogenous group of physicians, there were significantly diverging opinions regarding the management of cerebral aneurysms. This demonstrates the need for evidence-based data from controlled randomized studies.     

Implication du pharmacien d’officine dans le parcours de soins de la maladie rénale chronique

Chronic kidney disease (CKD) is a major concern of public health. The pharmacist is known as a health practitioner involved in prevention and therapeutic education. Our study aimed at defining the impact of community pharmacists’ interventions for preventing and screening CKD. In our observational prospective study of 5 months conducted in 109 community pharmacy, we included 2 groups of patients: A (therapeutic optimization): CKD patients and B (CKD screening): population at risk. In group A, we included 354 patients, mainly women (51.2%), in stage 3 of CKD, mean age 73 years old, with hypertension alone (40.6%) or associated with diabetes (44%). About 70% of the patients had a follow up by a nephrologist and 45% of them were good adherent according to the Morisky-Green self-report. However, approximately 20% of patients did not have nephroprotective treatments in their regimen although they were on stage 3 or 4 CKD patients, and about half of them were not aware of medical situations at risk. Concerning group B, 532 patients were included. The pharmaceutical interventions screened 10% of patients with a GFR < 60 mL/min/1.73 m². The community pharmacists’ interventions helped to optimize the therapeutic management of CKD patients and in the early screening of patients at risk. More studies are needed to extrapolate our observations to a larger population.     

Thrombose artérielle du membre inférieur sur un syndrome néphrotique

Thromboembolic complications are frequent in the nephrotic syndrome. Arterial localizations have been rarely reported. There is no consensus on their management; it depends on the location and hypercoagulable state. We report a case of acute lower limb ischemia occurring in children with a history of nephrotic syndrome, complicated by toes necrosis. The diagnosis was made by Doppler ultrasonography. Thrombectomy was performed and the patient received an anticoagulant treatment. Local cares have improved the local state, avoiding surgical necrosectomy.     

Recommandations vaccinales pour les enfants avec un syndrome néphrotique idiopathique

The specific treatment of idiopathic nephrotic syndrome is based on corticosteroid therapy and/or steroid-sparing immunosuppressive agents in children who are steroid-dependant or frequent relapsers (60–70 %). Patients have an increased infectious risk not only related to the disease during relapses (hypogammaglobulinemia and urinary leakage of opsonins) but also to treatments (corticosteroids or immunosuppressive agents) in period of remission. Vaccination is therefore particularly recommended in these patients. Potential vaccine risks are ineffectiveness, induction of vaccine disease and relapse of idiopathic nephrotic syndrome. Only live vaccines expose to the risk of vaccine disease: they are in general contra-indicated under immunosuppressive treatment. The immunogenicity of inactivated vaccines is reduced but persists. The immunogenic stimulus of vaccination may in theory trigger a relapse of the nephrotic syndrome. Nevertheless, this risk is low in the literature, and even absent in some studies. The benefit-risk ratio is therefore in favor of vaccination with respect to the vaccination schedule for inactivated vaccines, with wide vaccination against pneumococcus and influenza annually. Depending on the context and after expert advice, immunization with live vaccines could be discussed if residual doses/levels of immunosuppressive treatments are moderate and immunity preserved.