BACKGROUND:Acute tubulointerstitial nephritis (ATIN) is a rare condition in children. The etiology, treatment, and outcome of childhood ATIN remain poorly understood. The long-term prognosis seems to be favorable; however, chronic kidney disease has been reported. This article describes clinical outcomes in a series of children with biopsy-proven ATIN. METHODS:All medical records with biopsy-proven ATIN between January 2006 and 2016 were retrospectively analyzed. The incidence, clinical features, etiology, treatment, and outcome were recorded for each patient. RESULTS:Over 10 years, ATIN was diagnosed in 25 cases (8%) based on 306 renal needle biopsies. The most frequent clinical signs were abdominal pain, asthenia/weight loss, and fever. A median glomerular filtration rate estimated at 30.1mL/min/1.73 m2 (16.5; 45.5). Drug-induced toxicity was the main etiology (eight patients). Other causes were TINU syndrome (tubulointerstitial nephritis and uveitis) (seven patients), infection (two patients), and toxic agents other than medication (one patient). No etiology was found in seven patients (idiopathic cases). Eighteen patients (72%) were treated with steroids. At the end of follow-up, eight patients presented chronic kidney disease, three hypertension, and three tubular dysfunction. Overall, renal function was highest in the idiopathic ATIN group and in children treated without delay. CONCLUSIONS:In a single-center 10-year series of biopsy-confirmed ATIN in children, drugs and TINU syndrome were the main etiologies of ATIN. This study suggests that children with idiopathic ATIN and prompt treatment have a better prognosis. In this series, occurrence of chronic kidney disease justified long-term follow-up.

Les patients pédiatriques hyperimmunisés contre les antigènes HLA ont un accès extrêmement limité à la transplantation rénale. Le recours à une greffe ABO incompatible en cas de proposition de donneur vivant HLA compatible peut être une solution pour ces patients. Nous rapportons le cas d’une jeune fille greffée à 2 ans et demi pour IRCT (hypoplasie rénale), avec retour en dialyse 11 ans après. À l’inscription pour une seconde greffe, cette jeune fille avait un FAG à 0 et un taux de greffons incompatibles à 95 % du à un large profil d’immunisation anti-HLA classe I et II. La recherche de DVA dans la famille a permis d’identifier son père comme étant le seul donneur sans DSA mais ABO incompatible (receveur O et donneur A). Les autres donneurs proposés présentaient des DSA cytotoxiques ne permettant pas d’envisager une désimmunisation HLA. Après deux ans d’attente, la décision de proposer une greffe ABO incompatible a été prise. Les titres initiaux d’anti-A étaient de 1/128 (IgM)(technique saline ne tube) et 1/64 (IgG)(support filtration AGH), avec présence d’anticorps immuns. La préparation à la greffe (18 séances d’IA, 8 EP, Tacrolimus, Cellcept et corticoïdes et 2 injections de rituximab) a permis d’atteindre les titres cibles (1/8). La transplantation s’est déroulée sans complication notamment hémorragique. En post-greffe la créatinine s’est stabilisée à 90 μmol/L. La réascension rapide des titres d’anti-A ont motivé 4 EP post-greffe. Les biopsies réalisées à M1 et M3 ne montrent aucun signe de rejet humoral, et comme attendu, un marquage C4d diffus. Actuellement, à un an post-greffe, les titres sont stables à 1/64 (IgM) et 1/16 (IgG) et la créatinine est à 95 μmol/L sous tacrolimus, MMF et corticoïdes. La greffe ABO incompatible avec DVA permet d’offrir un accès à la transplantation rénale à des enfants hyperimmunisés en l’absence de DVA HLA compatible, avec la perspective d’une meilleure survie au long court que la greffe HLA incompatible.

La dysplasie fibromusculaire artérielle (DFM) est la principale cause d’HTA d’origine rénovasculaire chez l’enfant. Les troncs supra-aortiques (TSA) sont parfois concernés. Évaluer la prévalence et décrire l’atteinte des TSA chez des enfants ayant une sténose de l’artère rénale (SAR). Un questionnaire a été envoyé aux centres pédiatriques. L’évaluation de la prévalence de l’atteinte des TSA n’a été faite qu’à l’hôpital Necker sur la période 1985–2015. Dix-neuf patients ont été suivis dans 7 centres pour une HTA en rapport avec une SAR associée à une sténose des TSA. L’âge moyen au diagnostic était de 3,4 ans (10 jours-11 ans). L’HTA était le mode de révélation dans 7 cas (63 %). Chez 2 patients (37 %) la maladie est révélée par un accident vasculaire cérébral. La sténose est située sur l’artère rénale principale dans 15 cas (80 %) (10/15 unilatérale et 5/15 bilatérale). Quinze patients sur 19 (79 %) avaient une atteinte de l’artère carotide interne (7/15 atteinte isolée). Douze patients sur 19 (63 %) avaient une atteinte du polygone de Willis. Un aspect Moyamoya est associé dans 8 cas (42 %). Cinq patients sur 19 sont décédés, 4 fois par ischémie cérébrale (1/4 pendant une procédure anesthésique, 3/4 par instabilité hémodynamique durant la période postopératoire) et un patient est décédé d’infarctus du myocarde. À l’hôpital Necker parmi les 35 patients ayant une SAR, 14 (40 %) avaient une atteinte des TSA. Dix patients sur 14 (71 %) avaient une DFM isolée, 3/14 (22 %) une maladie de Takayasu et 1/14 (7 %) une neurofibromatose NF-1. Le pronostique est défavorable chez les patients avec atteinte diffuse des artères intracrânienes. La pression artérielle doit être maintenue à un niveau relativement élevé spécialement au cours des procédures anesthésiques et en période post-opératoire.

Asteraceae family members are well-known for their medicinal potential, comprising several properties that make them unique among plants. Here we focus on Santolina semidentata, an endemic plant from the Iberian Peninsula, not yet described for its medicinal properties. Phytochemical characterization of S. semidentata was performed, concerning total phenol content, flavonoid content, antioxidant capacity, HPLC-DAD profile, acetylcholinesterase inhibitory capacity, cytotoxicity and neuroprotective effect in a human neurodegeneration cell model. Moreover, essential oil composition and antifungal activity were also analised. This oil might be useful for therapeutical purposes, particularly in the treatment of dermatophytosis. S. semidentata potential for neuroprotection was revealed by acetylcholinesterase inhibitory capacity and also by an effective protective effect in human neuronal cells. Furthermore, different seed conservation protocols, as well as successful in vitro propagation were established which may be useful when integrated in a broad strategy for the conservation of these endemic plants and their sustainable use for potential biotechnological applications. The results presented here greatly contribute to value this species regarding its potential as a source of phytochemicals with prospective neuroprotective health benefits, either as alternative neuroprotective drugs or as leads for synthetizing more effective molecules.

Le syndrome hémolytique et urémique atypique (SHUa) à anticorps anti-facteur H (anti-FH) est associé à un risque élevé de rechutes et de complications malgré la plasmathérapie. Ce travail vise à montrer qu'un traitement court par cyclophosphamide et prednisone peut permettre de prévenir les rechutes à long terme. Nous présentons le suivi à long-terme (1–7 ans) de 4 enfants traités par 2–4 bolus de cyclophosphamide (500 mg/m2), échanges plasmatiques (EPs) et prednisone (6 mois). Les patients avaient présenté un SHUa avec des titres d'anticorps anti-FH très élevés. Le Patient 1 avait rechuté 2 fois après EPs+Rituximab puis a reçu de la prednisone+4 bolus de cyclophosphamide. Les 3 patients suivants ont été traités dès le diagnostic par 2 EPs+prednisone+2 bolus de cyclophosphamide permettant d'obtenir une rémission rapide et prolongée jusqu'à 7 ans après l'arrêt de tout traitement. La fonction rénale est restée normale et les titres d'anti-FH ont diminué tout en restant détectables sans rechute. Aucun effet indésirable du cyclophosphamide n'a été observé. Ce travail confirme l'efficacité à long-terme et la bonne tolérance des bolus de cyclophosphamide associés aux EPs et à la prednisone dans le SHUa avec anticorps anti-FH.

Streptococcus pyogenes or group A streptococcus (GAS) is responsible for serious invasive infections with a risk of secondary infection in patients with more contact than in the general population. Regardless of clustering, few intrafamilial invasive infections have been reported despite a recent increase in the incidence of invasive GAS disease. We report the cases of two brothers, one a boy of 8.5 years with toxic shock syndrome with no bacteria identified and the second, 1 week later, his 14.5-year-old brother in hospital for sepsis due to GAS. The occurrence of a confirmed case of invasive GAS and a probable case within such a short period met the definition of clustered cases. Both brothers showed no risk factors for invasive disease and no gateway including skin was found. Antibiotic therapy was initiated in the family as recommended by the French Higher Council of Public Hygiene. (C) 2011 Elsevier Masson SAS. All rights reserved.

Kawasaki disease is a well-known disease in young children. However, it can also affect older children. The aim of this study was to determine the different characteristics of Kawasaki disease in older children and young adults. This is a descriptive, retrospective, and multicenter study including all cases of Kawasaki disease occurring in children over 8 years and adults hospitalized at children's or adult Hospitals, in Marseille, France, between 1999 and 2009. The clinical, biological, prognostic, and therapeutic data were reviewed for each case. Over a 10-year period, 98 patients were hospitalized for Kawasaki disease. Six cases were aged between 8 years and 1 month and 21 years and 7 months. All patients showed a classic form of the disease with associated organ damage in 5 patients. A cardiac problem was present in 5 cases with 2 patients needing intensive care. The median time to diagnosis and treatment was 11.2 days, with all patients initially diagnosed erroneously. Current treatment guidelines were applied in 2 patients. Kawasaki disease in children over 8 years and adults under 30 years has a worse prognosis than in young children even though clinical features are atypical. It is rarely seen by clinicians in this age group, causing a delay in diagnosis, the main factor of the poor prognosis. The diagnosis of Kawasaki disease must be raised when predisposing factors are present in this group. (C) 2011 Elsevier Masson SAS. All rights reserved.

This study considered the relation between self-image posts (i.e., selfies, posies) on Instagram and the personality and self-perception attributions made by unfamiliar perceivers based on those posts. Phase 1 involved 30 undergraduates who completed self-report inventories and whose Instagram posts were coded and then screenshot for the second phase. Phase 2 included 119 undergraduates from a different university. Phase 2 participants (perceivers) rated Phase 1 participants (targets) on 13 attributes (e.g., self-absorption, low self-esteem, extraversion, successfulness) based on these screenshots. Targets who posted more selfies were rated more negatively (e.g., more lonely, less successful). Although self-image posts on social media may not be clearly indicative of personality/self-perception, they may be cues for how the depicted person is perceived by others.

Mushroom intoxication due to Amanita proxima poisoning is characterized by moderate gastrointestinal symptoms, followed by severe acute renal failure and sometimes by hepatic cytolysis. This syndrome was described in the 1990s in the southeast of France; we report here the first pediatric case, requiring dialysis but achieving complete recovery. The mother of this 11-year-old boy, who had eaten the same mushrooms but in smaller quantities, had only biological renal and hepatic involvement. (C) 2011 Published by Elsevier Masson SAS.

Mushroom intoxication due to Amanita proxima poisoning is characterized by moderate gastrointestinal symptoms, followed by severe acute renal failure and sometimes by hepatic cytolysis. This syndrome was described in the 1990s in the southeast of France; we report here the first pediatric case, requiring dialysis but achieving complete recovery. The mother of this 11-year-old boy, who had eaten the same mushrooms but in smaller quantities, had only biological renal and hepatic involvement.

We report 2 cases of children with group A streptococcus pyogenes pleuropneumonia, in one child associated with Kawasaki disease and in the other with streptococcal toxic shock syndrome. These 2 features, with theoretically well-defined clinical and biological criteria, are difficult to differentiate in clinical practice, however, likely due to their pathophysiological links. In case of clinical doubt, an echocardiography needs to be performed to search for coronary involvement and treatment including intravenous immunoglobulins, and an antibiotic with an anti-toxin effect such as clindamycin has to be started early.