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Le dilatazioni bronchiali dell’adulto sono sempre piu spesso diagnosticate con una TC toracica in sezioni sottili di fronte a una sintomatologia suggestiva (tosse cronica produttiva, emottisi, ecc.). Le loro eziologie sono diverse e un’indagine eziologica ha un impatto terapeutico in quasi un quarto dei casi. Tuttavia, le dilatazioni bronchiali idiopatiche rappresentano il 25-50% delle eziologie, secondo le serie. La ripercussione funzionale respiratoria e valutata mediante le prove funzionali respiratorie, che evidenziano un disturbo ventilatorio ostruttivo nell’80% dei casi. Lo studio regolare della flora bronchiale mediante esame citobatteriologico dell’espettorato e un elemento importante nel follow-up dei pazienti, consentendo di ricercare una colonizzazione bronchiale e di guidare la terapia antibiotica durante le esacerbazioni. La gestione terapeutica dei pazienti affetti da dilatazioni bronchiali si basa sulla sospensione di un eventuale tabagismo, sulla vaccinazione antinfluenzale e antipneumococcica, sul drenaggio bronchiale durante la fisioterapia respiratoria, sulla riabilitazione respiratoria, sulla terapia antibiotica delle esacerbazioni e sul trattamento delle infezioni croniche da Pseudomonas aeruginosa. Nuovi trattamenti, come i macrolidi e gli aerosol di antibiotici per via inalatoria, benche non abbiano l’autorizzazione all’immissione in commercio, sono, ora, valutati meglio, consentendo di prescriverli in determinate situazioni ben definite. E, ora, disponibile un punteggio di gravita della malattia, strumento utile per valutare i pazienti in clinica e anche per i futuri studi terapeutici.

Las dilataciones de los bronquios del adulto se diagnostican cada vez con mayor frecuencia mediante una tomografia computarizada (TC) toracica en cortes finos ante una sintomatologia sugerente (tos cronica productiva, hemoptisis, etc.). Sus etiologias son diversas, y un estudio etiologico influye en el tratamiento en cerca del 25% de los casos. A pesar de todo, las dilataciones idiopaticas de los bronquios representan el 25-50% de las etiologias segun las series. Se valora la repercusion funcional respiratoria mediante pruebas funcionales respiratorias que muestran un trastorno ventilatorio obstructivo en el 80% de los casos. El estudio regular de la flora bronquial mediante estudio citobacteriologico de los esputos es un elemento importante en el seguimiento de los pacientes. Permite identificar una colonizacion bronquial y orientar la antibioticoterapia en las exacerbaciones. El tratamiento de los pacientes con dilataciones de los bronquios se basa en la suspension de un posible tabaquismo, la vacunacion antigripal y antineumococica, el drenaje bronquial en la fisioterapia respiratoria, la rehabilitacion respiratoria, la antibioticoterapia de las exacerbaciones y el tratamiento de las infecciones cronicas por Pseudomonas aeruginosa. Nuevos tratamientos, como los macrolidos y los aerosoles de antibioticos inhalados, a pesar de carecer de autorizacion de comercializacion, estan en la actualidad mejor valorados y pueden prescribirse en algunos casos bien determinados. En la actualidad se dispone de una puntuacion de gravedad de la enfermedad, herramienta util para valorar a los pacientes en la clinica y para los proximos ensayos terapeuticos.

International audience; Introduction: Primary ciliary dyskinesia (PCD) is a genetic disease characterised by abnormalities in ciliary function, responsible for chronic pulmonary and sinonasal diseases. Adult clinical features and outcome are poorly described.Objectives: To assess the clinical characteristics and disease progression in adults with PCD.Methods: Bicentric retrospective study, focusing on adults (≥18 years) with an asserted diagnosis of PCD based on the presence of bronchiectasis with typical ultrastructural defect of cilia and/or situs inversus (SI). Clinical symptoms, respiratory function, extent of bronchiectasis, microbiology and molecular analysis were assessed. Results are expressed as median (25th; 75th centile).Results: 78 patients were included with a median follow-up of 8.1 years. 91% of patients had respiratory symptoms and 95% had chronic rhinosinusitis. Half of ultrastructural defects concerned dynein arms. Respiratory function was significantly lower in women (FEV1=60% predicted (50; 76), vs 77% (62; 95), p=0.009) and in patients with chronic airway Pseudomonas aeruginosa (PA, n=21) infection (FEV1=60% (48; 71) vs 75% (55; 89), p=0.04). FEV1 was associated with gender (regression coefficient for men =13.8, p=0.009), chest CT score (r=-0.42, p

Introduction Les dilatations des bronches (DDB) sont caracterisees par une inflammation locale et systemique, parfois associee a une hypoxemie chronique ou repetee ; ces phenomenes peuvent favoriser la survenue de comorbidites cardio-metaboliques. Methodes Cette etude monocentrique observationnelle incluait des patients adultes suivis pour une DDB, hors mucoviscidose. Les facteurs de risque cardiovasculaires, les maladies cardiovasculaires et les comorbidites metaboliques comme l’osteoporose ou la sarcopenie etaient recherchees par un double interrogatoire puis par un depistage associant un bilan biologique, une mesure de la vitesse de l’onde de pouls, une osteodensitometrie, une impedancemetrie corporelle, une mesure du score calcique coronaire et une echocardiographie de repos, voire d’effort. L’association entre la severite de la DDB et la presence de comorbidites etait evaluee. Les patients etaient ensuite compares a trois groupes « controle » : BPCO et sujets sains, fumeurs et non-fumeurs. Resultats Entre octobre 2016 et avril 2019, 89 patients ont ete inclus (70,8 % de femmes, 37,4 % de tabagiques). Le taux de colonisation bronchique bacterienne etait de 22,4 % et le VEMS median de 84 % (IQR 66–99 %). Selon les scores de severite valides BSI et BACI, les patients etaient plutot a faible risque (respectivement 42,6 % et 58,4 %) ou a risque intermediaire (34,8 % et 36,0 %) de mortalite et/ou d’hospitalisation. La prevalence des facteurs de risque cardiovasculaires etait de 75,3 %, celles des maladies cardiovasculaires de 19,1 % apres double interrogatoire, et de 23,5 % a l’issue du depistage systematique. L’osteoporose et la sarcopenie ont ete mis en evidence respectivement chez 32,5 % et 24,7 % des patients. La presence de maladies cardiovasculaires et de l’osteoporose n’etait pas significativement associee a la severite de la maladie bronchique selon le score BSI (p respectifs 0,727 et 0,053). Apres ajustement, la prevalence de l’osteoporose et de la sarcopenie etaient significativement plus elevee chez les patients DDB que chez les sujets sains non fumeurs (p = 0,005 et p = 0,009). Cette difference n’etait pas retrouvee en comparaison avec les patients BPCO (p = 0,078 et p = 0,556). Conclusion La prevalence des maladies cardiovasculaires est plutot faible chez les patients atteints de DDB non mucoviscidosiques. En revanche, la prevalence elevee de l’osteoporose et de la sarcopenie, quel que soit le niveau de severite de la maladie, pourrait conduire a des mesures de depistage systematique et de prevention.

Background The present study aimed to develop an automated computed tomography (CT) score based on the CT quantification of high-attenuating lung structures, in order to provide a quantitative assessment of lung structural abnormalities in patients with Primary Ciliary Dyskinesia (PCD). Methods Adult (≥18 years) PCD patients who underwent both chest CT and spirometry within a 6-month period were retrospectively included. Commercially available lung segmentation software was used to isolate the lungs from the mediastinum and chest wall and obtain histograms of lung density. CT-density scores were calculated using fixed and adapted thresholds based on various combinations of histogram characteristics, such as mean lung density (MLD), skewness, and standard deviation (SD). Additionally, visual scoring using the Bhalla score was performed by 2 independent radiologists. Correlations between CT scores, forced expiratory volume in 1 s (FEV1) and forced vital capacity (FVC) were evaluated. Results Sixty-two adult patients with PCD were included. Of all histogram characteristics, those showing good positive or negative correlations to both FEV1 and FVC were SD (R = − 0.63 and − 0.67; p

Objectives: Several studies, especially in Europe, have recently reported the emerging phenomenon of azole resistance in Aspergillus fumigatus, but very few data are available in France. Our study aimed to determine the resistance prevalence in A. fumigatus isolates recovered from clinical samples over a 1-year period in two university hospital centers. Methods :A llA. fumigatus isolates were screened for azole resistance using RPMI agar plates supplemented with itraconazole and voriconazole. Resistance was then confirmed by the EUCAST method. A part of the beta-tubulin gene was amplified for resistant isolates to confirm the A. fumigatus species, and the Cyp51A gene and its promoter were afterward sequenced to detect mutations potentially responsible for this resistance. Results: One hundred sixty-five A. fumigatus isolates were recovered from 134 patients. Three isolates recovered from three patients were found resistant with MICs of >8 mg/l, 4 mg/l, and 1 mg/l for itraconazole, voriconazole, and posaconazole, respectively. The TR34/L98H mutation, previously and largely described in other countries, was detected in the three isolates. Conclusion: Our study demonstrated the occurrence of azole resistance among unselected A. fumigatus clinical isolates, with an overall prevalence of 1.8%.

Infertility, observed in 75% of male and 61% of female PCD patients, is dependent on ultrastructural and gene defects http://ow.ly/P4K030fPnPp

Resume Les etiologies des syndromes cavitaires pulmonaires sont multiples, notamment neoplasiques, infectieuses ou inflammatoires. Une etiologie particuliere est celle d’infarctus pulmonaires multiples secondaires a des embolies pulmonaires. Ce diagnostic peut etre suspecte devant la localisation sous-pleurale des lesions et l’association avec un thrombus arteriel pulmonaire. Nous rapportons le cas d’une patiente atteinte d’une hypertension pulmonaire post-embolique (HTP PE) chronique associee a une thrombophilie familiale revelee par des infarctus pulmonaires excaves. L’HTP PE chronique est parfois diagnostiquee lors d’une recidive embolique sur des lesions anciennes passees inapercues. Parmi les conditions medicales associees au developpement d’une HTP PE, figurent certaines thrombophilies telles que l’elevation isolee du facteur VIII.

Mycobacterium chelonae (M. chelonae) is rarely responsible for respiratory infection. This report concerns the case of an 81-year-old man with previously asymptomatic bronchiectasis, colonised by M. chelonae for 3 years. He was hospitalised for acute dyspnoea and fever due to a right hydro-pneumothorax with cavitated alveolar opacities of the right lung. Pleural fluid and bronchial aspiration were positive for M. chelonae and no other microorganisms were detected. The effusion was drained and the patient treated with clarythromycin and amikacin. The radiological abnormalities improved but the patient's general condition remained poor. Treatment was continued for 11 months. Because of the absence of any other bacteria, clinical deterioration following broad-spectrum antibiotics and stabilisation of the lesions after anti-mycobacterial treatment, our diagnosis was severe M. chelonae pleuro-pneumonia in an immunocompetent patient.

We investigated whether mutations in the genes that code for the different subunits of the amiloride-sensitive epithelial sodium channel (ENaC) might result in cystic fibrosis (CF)-like disease. In a small fraction of the patients, the disease could be potentially explained by an ENaC mutation by a Mendelian mechanism, such as p.V114I and p.F61L in SCNN1A. More importantly, a more than three-fold significant increase in incidence of several rare ENaC polymorphisms was found in the patient group (30% vs. 9% in controls), indicating an involvement of ENaC in some patients by a polygenetic mechanism. Specifically, a significantly higher number of patients carried c.-55+5G>C or p.W493R in SCNN1A in the heterozygous state, with odds ratios (ORs) of 13.5 and 2.7, respectively.The p.W493R-SCNN1A polymorphism was even found to result in a four-fold more active ENaC channel when heterologously expressed in Xenopus laevis oocytes. About 1 in 975 individuals in the general population will be heterozygous for the hyperactive p.W493R-SCNN1A mutation and a cystic fibrosis transmembrane conductance regulator (CFTR) gene that results in very low amounts (0-10%) functional CFTR. These ENaC/CFTR genotypes may play a hitherto unrecognized role in lung diseases.

PURPOSE The clinical indications and economic consequences of human granulocyte colony-stimulating factor (G-CSF) prescription during small-cell lung cancer (SCLC) chemotherapy remain controversial. The aim of this study, based on a Markov model, was to assess the impact of routine G-CSF use in the treatment of SCLC on costs and patient comfort. Markov models allow the modeling SCLC chemotherapy, in which the risk of febrile neutropenia (FN) is continuous over time and may occur more than once. PATIENTS AND METHODS We used a Markov model to compare three strategies: a chemotherapy dose reduction after FN and nonuse of G-CSF ("never" strategy), secondary use of G-CSF ("CSF if FN" strategy) and primary use of G-CSF ("systematic CSF" strategy). Model baseline probabilities were based on a review of medical records for all patients (n = 39) treated for SCLC in our unit during 1993 (when G-CSF was not used) and on published reductions in the incidence of FN obtained by using G-CSF. Two different types of rewards were used: a cost-utility scale that took into account the costs of FN (CFN) episodes and G-CSF (CCSF) courses; and a comfort-utility scale that took into account the discomfort of FN and G-CSF therapy. Costs were analyzed from the health care payer's perspective and utilities were assessed prospectively in standardized interviews with treated SCLC patients. RESULTS The never strategy was the least costly ($4,875 [United States] versus $5,816 and $7,690 for CSF if FN and systematic CSF) and gave the highest comfort value (378 U v 365 and 327 for CSF if FN and systematic CSF). Sensitivity analyses showed that the never strategy remains the less costly when the probability of a first FN episode was less than 49%, the probability of FN recurrence was less than 60%, or the CFN was less than $6,077, or the CCSF was greater than $863. In terms of patient comfort, the never strategy was the best choice, except for patients who considered that a course of G-CSF caused little or no discomfort, whether or not it prevented FN. CONCLUSION Routine use of G-CSF during SCLC chemotherapy is not justified by clinical benefits, improved patient comfort, or economic considerations.

Several studies, especially in Europe, have recently reported the emerging phenomenon of azole resistance in Aspergillus fumigatus, but very few data are available in France. Our study aimed to determine the resistance prevalence in A. fumigatus isolates recovered from clinical samples over a 1-year period in two university hospital centers.All A. fumigatus isolates were screened for azole resistance using RPMI agar plates supplemented with itraconazole and voriconazole. Resistance was then confirmed by the EUCAST method. A part of the beta-tubulin gene was amplified for resistant isolates to confirm the A. fumigatus species, and the Cyp51A gene and its promoter were afterward sequenced to detect mutations potentially responsible for this resistance.One hundred sixty-five A. fumigatus isolates were recovered from 134 patients. Three isolates recovered from three patients were found resistant with MICs of8 mg/l, 4 mg/l, and 1 mg/l for itraconazole, voriconazole, and posaconazole, respectively. The TR34/L98H mutation, previously and largely described in other countries, was detected in the three isolates.Our study demonstrated the occurrence of azole resistance among unselected A. fumigatus clinical isolates, with an overall prevalence of 1.8%.

Resume La coqueluche est une infection respiratoire humaine, gravissime pour le nouveau-ne, et dont les premieres descriptions remontent au XVIe siecle. Des la mise en evidence de son agent etiologique, la bacterie Bordetella pertussis, des vaccins dits a germes entiers, car composes de suspensions bacteriennes inactivees, ont ete mis au point. Ils peuvent etre tres efficaces mais sont difficiles a produire et mal toleres. Grâce a la caracterisation des proteines intervenant dans la pathogenicite de Bordetella pertussis, un autre type de vaccin a ete developpe. Ce nouveau type de vaccin est dit acellulaire car compose de proteines bacteriennes purifiees. La generalisation de l’utilisation des vaccins a germes entiers, en association avec l’amelioration des conditions de vie, a conduit a une diminution drastique de la morbidite et de la mortalite liees a la coqueluche. Mais trente ans apres l’introduction de la vaccination, une resurgence est observee, secondaire a la duree limitee de l’immunite vaccinale ou naturelle. La maladie touche alors les adolescents et jeunes adultes presentant une toux dont les caracteristiques cliniques sont atypiques et de diagnostic difficile. Ceux-ci contaminent les nouveau-nes non vaccines exposes aux formes mortelles de la coqueluche. Des rappels vaccinaux sont necessaires et sont heureusement maintenant possibles grâce a la mise sur le marche des vaccins acellulaires depuis 1998. La France a ete le premier pays a introduire un vaccin pour les adolescents. Un rappel pour les jeunes adultes est en cours de discussion. Neanmoins, une surveillance active est toujours indispensable en raison du danger de contamination des nourrissons non vaccines. Cette surveillance passe par l’utilisation de diagnostics biologiques car la clinique est souvent atypique chez les jeunes adultes anciennement vaccines.

Resumen La tos ferina o coqueluche es una infeccion respiratoria humana, gravisima para el recien nacido, y cuyas primeras descripciones se remontan al siglo xvi . Tras el descubrimiento del agente etiologico, la bacteria Bordetella pertussis, se desarrollaron vacunas denominadas de «germenes enteros» al estar constituidas por suspensiones bacterianas inactivadas. Es posible que estas vacunas sean muy eficaces, pero resultan dificiles de producir y son mal toleradas. Gracias a la caracterizacion de las proteinas responsables de la capacidad patogenica de la Bordetella pertussis, se ha desarrollado otro tipo de vacunas. Este nuevo tipo se llama acelular, al estar formado por proteinas bacterianas purificadas. La utilizacion generalizada de vacunas con germenes enteros, junto con la mejoria de las condiciones de vida, han conseguido una disminucion drastica de la morbilidad y de la mortalidad por tos ferina. Sin embargo, treinta anos despues de la introduccion de la vacuna, se ha observado un nuevo resurgimiento de esta enfermedad, secundario a la duracion limitada de la inmunidad natural o de la vacuna. Esta nueva enfermedad afecta a adolescentes y a adultos jovenes, que presentan una tos de caracteristicas atipicas, lo que dificulta su diagnostico. A su vez, estos enfermos contaminan a los recien nacidos no vacunados, que desarrollan formas mortales de la tos ferina. Son necesarias por tanto las dosis de refuerzo. Afortunadamente, esto es posible gracias a la comercializacion de vacunas acelulares desde 1998. Francia ha sido el primer pais que ha introducido la vacuna para los adolescentes. La conveniencia de una dosis de refuerzo en los adultos jovenes se discute actualmente. No obstante, dado el riesgo de contaminacion de los lactantes no vacunados, es fundamental una vigilancia activa. Esta vigilancia conlleva la utilizacion de pruebas de laboratorio complementarias ya que la clinica en estos adultos jovenes vacunados anteriormente suele ser atipica.

Resume Des etudes recentes ont montre que la prevalence de la coqueluche serait de l'ordre de 10 a 20 % chez les patients adultes souffrant d'une toux chronique d'une duree superieure a huit jours et inferieure a deux mois. Confronte a ce symptome tres banal, le clinicien doit penser a la coqueluche et tenter d'etayer ce diagnostic par cinq elements cles : 1) le caractere paroxystique et astheniant de la toux ; 2) la chronologie particuliere des symptomes, la toux s'aggravant apres une banale phase catarrhale initiale ; 3) la normalite de l'examen physique entre les acces de toux ; 4) l'identification dans l'entourage d'un possible contaminateur ; 5) la normalite de la radiographie thoracique. Une suspicion clinique renforcee doit en effet conduire a demander les examens biologiques susceptibles d'affirmer le diagnostic (PCR chez l'adulte vaccine, serologie par immunoempreinte) et a discuter une antibiotherapie adequate (macrolides).

L’omalizumab (OMZ) a une AMM depuis 2005 dans l’asthme severe allergique avec un allergene perannuel retrouve. Ce travail a pour objectif d’evaluer l’adequation de la prescription de l’OMZ et son efficacite en utilisation courante dans un centre de pneumologie. Patient et methodes Etude retrospective monocentrique. Tous les pts (n = 55) ayant recu de l’OMZ entre 2003 et 2014 ont ete inclus. Les criteres de severite, l’adequation de la prescription par rapport aux recommandations de l’AMM et l’efficacite du traitement ont ete analyses. Resultats Tous les pts avaient un asthme severe (VEMS moyen 61 %, ACT moyen 11,6, Nombre moyen d’EA : 5,44/an). Cinquante-quatre pour cent des patients recevaient une corticotherapie orale, tous avaient des doses elevees de CSI (> 1000 mcg/j). Quatre-vingt-cinq pour cent avaient un allergene perannuel. 10 pts avaient des taux d’IgE ou un poids hors des tables d’AMM : IgE 1500 : n = 3, IgE > 30 et Conclusion La prescription d’OMZ n’etait pas conforme aux recommandations dans 15 % des cas du fait de l’absence d’allergene perannuel identifie et dans 18 % des cas car ils etaient hors des tables de dosage recommande. L’efficacite de 66 % est proche de la litterature, mais 18 % ont presente un EI imposant l’arret du traitement.

In the past few years some new inhaled drugs and inhalation devices have been proposed for the treatment of cystic fibrosis. Breath-controlled nebulizers allow increased pulmonary deposition, with a lower variability and a shorter delivery time. The new dry powder formulations of tobramycin, colistine and mannitol require a change in the inhalation technique which must be slow and deep. In the field of the inhaled mucolytic drugs, hypertonic saline and mannitol have an indication in some patients. With regard to antibiotics, dry-powder tobramycin and colistine can be substituted for the same drug delivered by nebulization. Nebulized aztreonam needs more studies to determine its place. These new treatments represent a definite advance for cystic fibrosis patients and need to be known by all practitioners. Their position in our therapeutic arsenal remains to be accurately defined.

Background Primary Ciliary Dyskinesia (PCD) is a inherited disorder characterized by abnormal ciliary structure/function, responsible for impaired muco-ciliary transport and infertility. The diagnosis is often delayed for several years though PCD is a rare (~1/20,000) and heterogeneous disease. We elaborated recommendations aiming at improving the quality and efficacy of PCD diagnosis. Methods A multicentric working group, created in 2009 within the French National Centre for Rare Respiratory Diseases, elaborated precise and practical algorithm for PCD diagnosis, based on their experience and the recent literature. It includes adults and paediatric physicians, biologists and technicians involved in PCD, dealing since many years with the difficulties to confirm this diagnosis.

Background Primary Ciliary Dyskinesia (PCD) is a rare inherited disease (~1/20,000), characterized by ciliary structure/function abnormalities, responsible for impaired muco-ciliary transport, leading to recurrent upper and lower airways infections early in life and infertility. The diagnosis is confirmed on ciliate cell samples, collected by nasal and/or bronchial endoscopy. Patients usually need several samples, due to difficulties to get reliable results, especially during respiratory tract infections which are frequent in PCD. We created national guidelines aimed at obtaining the more efficient quality of ciliate cell samples for children and adults.

Several etiologies are involved in the pathogenesis of cavitating pulmonary disease including neoplastic, infectious or inflammatory processes. Another is pulmonary infarction associated with venous thromboembolism. The lung cavities tend to be located peripherally and are the result of pulmonary embolism. We report the case of a woman with chronic thromboembolic pulmonary hypertension (CTEPH), associated with familial thrombophilia, revealed by cavitating pulmonary infarcts. CTEPH is sometimes diagnosed during an episode of recurrent pulmonary embolism following previously unnoticed lesions. Thrombophilias such as isolated elevated factor VIII are risk factors for CTEPH.

International audience; BACKGROUND:The identification by CFTR mRNA studies of a new deep-intronic splicing mutation, c.870-1113_1110delGAAT, in one patient of our series with mild CF symptoms and in three CF patients of an Italian study, led us to evaluate the mutation frequency and phenotype/genotype correlations.METHODS:266 patients with CF and related disorders and having at least one undetected mutation, were tested at the gDNA level in three French reference laboratories.RESULTS:In total, the mutation was found in 13 unrelated patients (5% of those already carrying a mutation) plus 4 siblings, including one homozygote and 12 heterozygotes having a severe CF mutation. The sweat test was positive in 10/14 documented cases, the diagnosis was delayed after 20 years in 9/15 and pancreatic insufficiency was present in 5/16.CONCLUSION:c.870-1113_1110delGAAT should be considered as CF-causing with phenotype variability and overall delayed diagnosis. Its frequency highlights the potential of mRNA studies.

Mycobacterium chelonae (M. chelonae) is rarely responsible for respiratory infection. This report concerns the case of an 81-year-old man with previously asymptomatic bronchiectasis, colonised by M. chelonae for 3 years. He was hospitalised for acute dyspnoea and fever due to a right hydro-pneumothorax with cavitated alveolar opacities of the right lung. Pleural fluid and bronchial aspiration were positive for M. chelonae and no other microorganisms were detected. The effusion was drained and the patient treated with clarythromycin and amikacin. The radiological abnormalities improved but the patient's general condition remained poor. Treatment was continued for 11 months. Because of the absence of any other bacteria, clinical deterioration following broad-spectrum antibiotics and stabilisation of the lesions after anti-mycobacterial treatment, our diagnosis was severe M. chelonae pleuro-pneumonia in an immunocompetent patient.

We report two cases of malignant lymphoma of B phenotype occurring after therapeutic pneumothorax for tuberculosis. In both cases, outcome was fatal without time for specific treatment. Mainly reported in Japan, this pathology seems to be less frequent in western countries. As for B phenotype lymphoma associated with immunodeficiency, association with Epstein Barr virus is reported. Definite diagnosis is difficult and requires surgical biopsy. Prognosis remains poor with a survival ranging from 3 to 6 month.

But de la presentation La dyskinesie ciliaire primitive (DCP) est une maladie genetique rare caracterisee par des anomalies ciliaires alterant la clairance mucociliaire. Les manifestations cliniques ORL sont bien connues chez les enfants mais la prevalence des symptomes et la description clinique sont mal connues chez les adultes. L’objectif de cette etude est de decrire l’atteinte ORL de la DCP chez l’adulte. Materiel et methodes Etude retrospective multicentrique sur 64 patients ayant une DCP confirmee par etude ciliaire et/ou genetique. Les patients etudies ont eu un examen ORL standardise (avec endoscopie nasale), une audiometrie + tympanometrie, des prelevements bacteriologiques des secretions nasales et un scanner des sinus. Resultats L’echantillon etudie comptait 63 % d’hommes, un âge median de 37 ans et 34 % de syndrome Kartagener. La consanguinite etait retrouvee dans 30 % des cas. Des antecedents de rhinosinusite et d’otite seromuqueuse (OSM) etaient presents chez 98 % et 73 % des patients, respectivement. La rhinorrhee (95 %) et l’hypoacousie (53 %) sont les principales plaintes. L’endoscopie nasale a montre une rhinosinusite chronique dans 60 % et des polypes dans 34 % des cas. Une OSM a ete diagnostiquee chez 48 % et une surdite de perception dans 15 % des cas. L’etude bacteriologique endonasale a montre la presence d’ Haemophilus influenzae , de Streptococcus pneumoniae et de Pseudomonas aeruginosa dans 28 %, 19 % et 19 % des cas, respectivement. Le scanner des sinus montrait des hypoplasies/agenesies sinusiennes dans 83 % des cas. Conclusion La DCP chez les adultes est caracterisee par l’importance des manifestations rhinosinusiennes. L’OSM reste cependant frequente et une surdite de perception n’est pas rare.

International audience; Occupational lung cancers are underestimated by the number of cases compensated in the French National Insurance System. Rules of compensation of occupational diseases were recently modified in France. Therefore a study was conducted on incident cases of lung cancer in a general hospital in the Paris area. The aim was to evaluate the exposure to carcinogens using data of a detailed specific occupational questionnaire, and to determine the number of cases who could receive compensation. Two hundred and seven subjects (171 males, 36 females, mean age = 64.5 years) were eligible in 1996, and 122 had an occupational questionnaire. Definite exposure to one or more occupational carcinogens in at least one job was identified in 50 subjects, the most frequent agent was asbestos (42 subjects). Claim for compensation was done in 32 subjects, mainly for asbestos (30 subjects). This study emphasizes the frequency of occupational exposure to carcinogens, and the usefulness of systematic occupational questionnaire in subjects having lung cancer. Social and financial consequences are important for these subjects. Further studies are needed, with recruitement of control subjects to allow calculation of the attributable risk to occupational factors in lung cancer.