Diagnosis, treatment and recurrence of a mandibular Langerhans cell histiocytosis: a three-year follow-up case report

Introduction: Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells secondary to immune process, mutation of oncogene or genetic predispositions. It preferentially affects bone, lung and skin. The incidence is 2–6 cases per million per year. Prognosis is variable and depends on number and location of lesions, and impact of the initial treatment. Oral lesions may be the first sign of LCH as illustrated by the present case. Observation: A 24-year-old male consulted first for severe gingival inflammation, teeth mobilities and alveolar bone loss with a suspicion of LCH. A pulmonary involvement was secondarily revealed by tomodensitometry. Histological examination, from gingival biopsy, confirmed the diagnostic of LCH, showing cells positive for the anti-CD1A antibody. The patient was managed by oral surgery and chemotherapy approaches. Alveolar bone loss significantly reduced. But 2 years and a half after the diagnosis, a recurrence was noted and managed by surgical approach. After a three-year follow-up, no recurrence was noted. Conclusion: Oral lesions can be inaugural manifestations of LCH. The dentist has an essential role in the early detection of these lesions

Celesio® et " OCP point " (présentation, description et étude en 2005)

Cette thèse présente le groupe Celesio®, grossiste répartiteur européen, qui utilise l'informatique et l'Internet au débit (ADSL) pour moderniser l'activité officinale dans le cadre d'une harmonisation du système de santé européen. La première partie est consacrée à la technique de l'ADSL. Des données sur la situation actuelle au niveau français et au niveau mondial de l'Internet seront fournies. La seconde partie est une présentation du complexe industriel Celesio® et de l'OCP, le grossiste répartiteur de Celesio® sur le territoire national. La troisième partie est consacrée à " OCP point ", un site professionnel pour les pharmaciens clients chez OCP. Un descriptif complet des options d'OCP point a été réalisé. La conclusion porte sur l'avenir de la pharmacie d'officine en Europe à travers un grossiste répartiteur, le groupe Celesio® et l'utilisation des nouvelles informatiques tel que les réseaux virtuels personnels qui permettent à une entreprise d'utilisé la technique de l'ADSL pour un usage purement privé. (Ce qui signifie qu'une entreprise pourra développer un " réseau Intranet via le réseau Internet ".NANTES-BU Médecine pharmacie (441092101) / SudocSudocFranceF

Vasculitides induced by cocaine and/or levamisole

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Characterization and in vivo functional analysis of the Schizosaccharomyces pombe ICLN gene

During the early steps of snRNP biogenesis, the survival motor neuron (SMN) complex acts together with the methylosome, an entity formed by the pICln protein, WD45, and the PRMT5 methyltransferase. To expand our understanding of the functional relationship between pICln and SMN in vivo, we performed a genetic analysis of an uncharacterized Schizosaccharomyces pombe pICln homolog. Although not essential, the S. pombe ICln (SpICln) protein is important for optimal yeast cell growth. The human ICLN gene complements the Deltaicln slow-growth phenotype, demonstrating that the identified SpICln sequence is the bona fide human homolog. Consistent with the role of human pICln inferred from in vitro experiments, we found that the SpICln protein is required for optimal production of the spliceosomal snRNPs and for efficient splicing in vivo. Genetic interaction approaches further demonstrate that modulation of ICln activity is unable to compensate for growth defects of SMN-deficient cells. Using a genome-wide approach and reverse transcription (RT)-PCR validation tests, we also show that splicing is differentially altered in Deltaicln cells. Our data are consistent with the notion that splice site selection and spliceosome kinetics are highly dependent on the concentration of core spliceosomal components

Diagnosis, treatment and recurrence of a mandibular Langerhans cell histiocytosis: a three-year follow-up case report

Introduction: Langerhans cell histiocytosis (LCH) is an abnormal clonal proliferation of Langerhans cells secondary to immune process, mutation of oncogene or genetic predispositions. It preferentially affects bone, lung and skin. The incidence is 2–6 cases per million per year. Prognosis is variable and depends on number and location of lesions, and impact of the initial treatment. Oral lesions may be the first sign of LCH as illustrated by the present case. Observation: A 24-year-old male consulted first for severe gingival inflammation, teeth mobilities and alveolar bone loss with a suspicion of LCH. A pulmonary involvement was secondarily revealed by tomodensitometry. Histological examination, from gingival biopsy, confirmed the diagnostic of LCH, showing cells positive for the anti-CD1A antibody. The patient was managed by oral surgery and chemotherapy approaches. Alveolar bone loss significantly reduced. But 2 years and a half after the diagnosis, a recurrence was noted and managed by surgical approach. After a three-year follow-up, no recurrence was noted. Conclusion: Oral lesions can be inaugural manifestations of LCH. The dentist has an essential role in the early detection of these lesions

Clinical contribution of myositis-related antibodies detected by immunoblot to idiopathic inflammatory myositis: A one-year retrospective study

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Bronchiectasis is highly prevalent in anti-MPO ANCA-associated vasculitis and is associated with a distinct disease presentation.

International audienceOBJECTIVES:To assess the prevalence of bronchiectasis in a Western cohort with ANCA-positive granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) and its correlations with disease presentation and outcome. METHODS:Retrospective study of ANCA-associated vasculitis (AAV) patients followed at Nantes University Hospital (2005-2015). Clinical, biological, and follow-up data were collected through chart review. Two experienced radiologists blinded to the clinical data interpreted chest high-resolution CTs according to the Feischner Society criteria. RESULTS:Fifty-eight patients were included: 30 had MPA (51.7%) and 28 had GPA (48.3%). The median age at AAV diagnosis was 65.5 years. Anti-MPO-ANCA and anti-PR3-ANCA were present in 39 (67.2%) and 19 (32.8%) patients, respectively. Overall, bronchiectasis was found in 22 patients (37.9%), all of whom had anti-MPO ANCA. In multivariate analysis, bronchiectasis was independently associated with anti-MPO-ANCA, female gender and age at AAV diagnosis. Furthermore, anti-MPO ANCA patients with bronchiectasis had more frequent peripheral nerve involvement (54.5 vs. 17.6%, p = 0.019) and less frequent renal involvement than those without bronchiectasis (40.9% vs. 82.3%, p = 0.009). Disease course, survival and risk of severe pulmonary infection were similar in patients with and without bronchiectasis on chest CT. CONCLUSIONS:This study shows that bronchiectasis is a highly prevalent pre-existing respiratory condition in Caucasian patients with anti-MPO AAV. This subset of patients exhibits a distinct presentation. Further studies are needed to confirm these findings and clarify the clinical implications of this association. Whether the respiratory tract could be the site of initiation of anti-MPO auto-immunity remains to be investigated

Vinblastine chemotherapy in adult patients with langerhans cell histiocytosis: a multicenter retrospective study

Abstract Background Vinblastine is the standard treatment for children with Langerhans cell histiocytosis (LCH). Whether this treatment could be extended to adults with LCH is questionable. This retrospective multicenter study included 35 adult patients (median age 33 years; 23 men; 80% with multisystem LCH) who were treated with vinblastine + steroids as a first-line chemotherapy and followed for a median time of 83 months. The objectives were to determine the overall response rate (based on the Histiocyte Society criteria), disease reactivation rate, toxicity, permanent consequences, and survival rate corresponding to this treatment. The lung involvement outcome was based on serial lung function tests. The distribution of right-censored end points was estimated by the Kaplan-Meier method. Univariate Cox model with time-fixed and time-varying covariates was used for the predictive analysis of reactivation in the responders. Univariate analyses of risk factors for neurotoxicity were based on nonparametric Wilcoxon rank sum tests and exact Fisher tests. Results The median duration of the first course of vinblastine was 7.6 months, with a median cumulative dose of 160 mg [IQR 120–212]. Seventy percent of the patients were responders at the end of this treatment. Subsequently, LCH reactivation occurred with a 5-year cumulative incidence of 40%. During the study, 27 reactivations were observed in 17 patients, and half of these episodes were retreated with vinblastine. At the end of the last vinblastine treatment, 70% of the patients were responders. None of the patients with impaired lung function improved. No grade 3–4 peripheral neuropathy was observed. At the final vinblastine treatment, permanent LCH consequences, primarily pituitary stalk involvement, were present in 15 (43%) patients, and all were present at the time of vinblastine initiation. The 10-year survival rate was 86.2% (95CI, 71.8–100%), and the 2 patients who died from LCH had risk organ localizations. Conclusions Vinblastine is an effective and well-tolerated first-line treatment for adult LCH except in patients with lung involvement and impaired lung function. However, a significant portion of patients experienced LCH reactivation during long-term follow up. As in childhood LCH, the presence of risk organ involvement has a negative impact on patient prognosis