Long-term adherence to ambulatory initiated continuous positive airway pressure in non-syndromic OSA children.

PURPOSE: In children, the usual indications for continuous positive airway pressure (CPAP) are residual OSA after adenotonsillectomy and/or persistent OSA due to obesity. Data concerning adherence (hours/night) following ambulatory CPAP initiation are scarce. METHODS: An observational cohort of 78 children was followed over 2 years. All exhibited sleep-disordered breathing (SDB) symptoms, were assessed by polysomnography, and prescribed CPAP. CPAP was initiated at hospital for 10 children. RESULTS: OSA children, mean age 10.4 ± 3.2 years, were mostly males (75.6%), with a mean body mass index of 21.2 ± 7.3 kg/m2, and mean apnea+hypopnea index of 12.2 ± 10.6 events/hour. Seventy-two children were still on CPAP at 3 months, 63 at 6 months, 55 at 1 year, and 34 at 2 years. CPAP was discontinued thanks to rehabilitation programs, dento-facial orthopedics, and/or weight loss. Mean CPAP adherence at 1, 3, 6, 12, and 24 months was respectively 6.1 ± 2.8, 6.2 ± 2.6, 6.2 ± 2.8, 6.3 ± 2.8, and 7.0 ± 2.7 h/night. There was a trend towards higher CPAP adherence and younger age, primary versus middle/high school attendance, higher baseline apnea+hypopnea index, and neurocognitive disorders. CONCLUSION: In our population, mean CPAP adherence defined in hours per night was high and did not decrease during the 24-month follow-up. These findings support the feasibility of ambulatory CPAP initiation in non-syndromic OSA. The high CPAP adherence is expected to be associated with improvements in neurocognition, and in metabolic and cardiovascular parameters.

[Hereditary multiple exostosis complicated by spontaneous haemothorax]. / Hémothorax spontané compliquant une maladie des exostoses multiples.

Spontaneous haemothorax is a rare event in the general population. However some diseases, including hereditary multiple exostosis, have an increased incidence of this complication. We report the case of a 22 year old patient with hereditary multiple exostosis who presented with a right sided haemothorax due to an exostosis of the seventh rib. The pathophysiology of spontaneous haemothorax in the setting of hereditary multiple exostosis is discussed together with surgical treatment and long term follow up.

[Factors predicting mortality during an outbreak of Legionnaire's disease in the north of France]. / Epidémie de légionellose dans le Pas-de-Calais (2003--2004): analyse descriptive et facteurs prédictifs d'une évolution défavorable.

Between November 2003 and January 2004 in the North of France a large outbreak of legionnaire's disease affected 85 patients. The clinical, biological and radiological characteristics of the patients were investigated to determine factors associated with mortality. Two populations were defined and compared: patients who died within 28 days and those who survived. Eighty-five patients were included in this study. The median age was 75 years. The median fever was 39.3 +/- 0.1 degrees. Fifteen patients (17.6%) had at least 3 underlying co-morbidities. Cough, dyspnoea, confusion and diarrhoea were found in respectively 46, 68, 47, and 15% of the patients. The median of urea was 0.7 +/- 0.05 g/L, creatinine 16 +/- 1.5 mg/L, CRP 332 +/- 15 mg/L. On the chest X-ray, lung infiltrates were present in 64% and multilobar in 40%. The overall mortality rate was 21%. In univariate analysis, diabetes mellitus, dyspnoea, urea>0.90 g/l and CRP>350 mg/l were predictive factors of mortality. In multivariate analysis, diabetes mellitus, urea>0.90 g/l, and bilateral infiltrates on chest X ray were retained as independent risk factors for death.

[Carotid surgery with cervical plexus block: personal experience]. / Chirurgie carotidienne sous plexus cervical: expérience personnelle.

Although this is an unfrequently used technique, the authors report their early experience concerning twenty-seven cases of carotid artery revascularization under cervical plexus anesthesia. They express their surgical judgement about the advantages and the limits of the simplest and most reliable way of cerebral monitoring during the surgical procedure, but it might have only a limited indication.

[Allergic bronchopulmonary aspergillosis]. / Aspergillose bronchopulmonaire allergique.

Allergic bronchopulmonary aspergillosis (ABPA) is an immunologic bronchopulmonary inflammation due to an immune response of the lower respiratory tract against Aspergillus fumigatus. The major clinical features of ABPA include asthma, recurrent pulmonary infiltrates, immediate wheal and flare skin reactivity to A. fumigatus, elevated total serum IgE levels, detectable serum precipitating antibodies to A. fumigatus, peripheral blood eosinophilia, elevated levels of Aspergillus-specific serum IgE and central bronchiectasis with normal distal structures. The diagnosis of ABPA should be considered in asthmatics of all ages. Evolution of the disease comprises five stages from the acute to the fibrotic stage including pulmonary fibrosis and respiratory insufficiency. The goals of ABPA treatment are to control patient's asthma and prevent exacerbations of ABPA. During the acute stage, prednisone should be administered. Antifungals agents (itraconazole) may be useful to prevent exacerbations of the disease.

[The common mucosal immune system in respiratory disease]. / Le système immunitaire muqueux commun en pathologie respiratoire.

The principal function of the mucosal immune system is to protect the mucosa from exogenous aggression. It also involves a lymphocyte recirculation phenomenon allowing activated lymphocytes to migrate to the aggressed site, for example the bronchi, and to recirculate and colonize other sites of the mucosal immune system. In asthma, analysis of the other sites of the common mucosal immune system demonstrates asthma-like inflammatory reactions in the accessory salivary glands and the gut: lymphocyte infiltrate, mast cell activation, thickening of the basal membrane, accumulation and activation of eosinophils (gut), activation of endothelial cells expressing ICAM-1. Lymphocyte, eosinophil and mast cell infiltration is observed in the digestive tract as well as increased expression of IL-3, IL-5 and GM-CSF. The similarity of the anomalies observed in BALT and GALT tissues would suggest the entire mucosal immune system is implicated in asthma.

[Interstitial lung disease linked to fluoxetine]. / Pneumopathie interstitielle liée à la prise de fluoxétine.

Fluoxetine (Prozac) is a new anti-depressant introduced on the marked in France since 1989. We report a case of a patient who developed progressive dyspnea without any other symptoms who had been treated with Prozac for nine months. Clinical examination found bilateral basal crepitations. Laboratory assessments showed evidence of inflammation (ESR: 50 mm per hour; CRP 8.9 mg/l), the pulmonary radiograph and thoracic CT scan showed evidence of bilateral diffused interstitial disease. There was also a restrictive ventilatory defect (TLC 59 per cent predicted; FEV1 79 per cent predicted; FVC 70 per cent predicted), effort hypoxaemia and alveolar hyperlymphocytosis (35 per cent). The clinical, radiological and pulmonary function improved and the bronchoalveolar lavage became normal three months after stopping Prozac suggesting a role for this drug in the genesis of this interstitial pneumonia.

[Pulmonary hemorrhagic syndrome after inhalation of cocaine]. / Syndrome hémorragique pulmonaire après inhalation de cocaïne.

Frequency of recreational abuse of cocaine is increasing. We report the case of a patient who developed an acute anemia associated with an alveolar hemorrhage after repeated inhalation of cocaine. He presented with daily hemoptysis and dyspnea. Chest-X ray and CT scan showed bilateral micronodular opacities. The fibroscopic alveolar lavage fluid showed fresh blood. DLCO/VA was 117% predicted. With oxygen therapy and no blood transfusion the evolution was positive within 48 hours, without relapse. The occurrence of hemoptysis in subjects who inhale cocaine must lead to suspect cocaine induced-pulmonary hemorrhage.

[Severe varicella zoster pneumonia during the course of treatment with azathioprine for Crohn's disease]. / Pneumopathie varicelleuse sévère au cours d'un traitement par azathioprine pour une maladie de Cröhn.

INTRODUCTION: Disseminated varicella zoster infection has only rarely been reported in patients with inflammatory bowel disease, despite the frequent use of azathioprine for this disorder. CASE REPORT: We report the case of an 18-year-old woman who developed severe varicella zoster pneumonia 9 months after starting azathioprine for Crohn's disease. The patient recovered after prompt treatment with acyclovir and discontinuation of the azathioprine. CONCLUSIONS: Strategies concerning the treatment and the prevention of varicella infection in the immunocompromised patient are discussed.

[Respiratory bronchiolitis with diffuse interstitial lung disease]. / Bronchiolite respiratoire avec pneumopathie interstitielle diffuse.

Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) is a disease that exclusively affects cigarette smokers. Long-term prognosis is good with cessation of smoking, in combination or not with corticosteroid therapy. We report here the case of a 50-year-old patient with RB-IL diagnosed on lung biopsy. Despite corticosteroid and cyclophosphamide therapy, no functional or radiological improvement was obtained. In contrast, cessation of smoking was associated with the disappearance of the infiltrative opacities. Clinical and radiological parameters remained stable during follow-up (13 years) while a moderate obstructive pattern appeared.

[Erasmus syndrome: clinical, tomographic, respiratory function and bronchoalveolar lavage characteristics]. / Syndrome d'Erasmus: caractéristiques cliniques, tomodensitométriques, fonctionnelles respiratoires et biologiques du lavage bronchoalvéolaire.

The Erasmus syndrome describes the association of generalised progressive scleroderma following exposure to silica with or without silicosis. This is a case report on four patients presenting with the Erasmus syndrome who were admitted to hospital. The analysis of the four cases enables an assessment of the cause of the dyspnoea during the course of the Erasmus syndrome. The dyspnoea presents more as scleroderma (pulmonary fibrosis in two cases, pulmonary artery hypertension in one case and localised thoracic skin disease in one case) than of pneumoconiosis. Pulmonary fibrosis should be considered where there is an association of progressive effort dyspnoea, fine crackles on auscultation and a radiological appearance either of honeycombing and/or a ground glass appearance predominantly in the posterior regions which does not exist in isolated cases of silicosis. The functional repercussion of the fibrosis is evident by a restrictive ventilatory defect which is not specific but more severe than in a case of silicosis alone. Bronchoalveolar lavage showed, in two cases of pulmonary fibrosis, an unusual polymorphonuclear neutrophilia during the course of the silicosis. The presence of ausculatory anomalies, the atypical aspects of pneumoconiosis on computed tomography and an unusual form of LBA should suggest the existence of pulmonary fibrosis associated with pneumoconiosis.

[Acute silicosis. Clinical, radiologic, functional, and cytologic characteristics of the broncho-alveolar fluids. Observations of 6 cases]. / Silicose aiguë. Caractéristiques cliniques, radiologiques, fonctionnelles et cytologiques du liquide broncho-alvéolaire. A propos de 6 observations.

We describe six cases of acute silicosis which were characterised by an intense exposure to pure silica; there was a rapid onset with diffuse abnormalities on the radiological image and severe respiratory abnormalities. The duration of occupational exposure to silica was four to eighteen years. Three patients presented with a cough associated with disabling dyspnoea (stage III and IV). The physical examination showed finger clubbing in three patients and rales and crepitations on auscultation in two patients. There was general debility in four of the cases with weight loss. The chest x-ray showed a rapid progress with confluence of the diffuse nodular opacities. Respiratory function tests showed a restrictive ventilatory defect (mean +/- standard error of mean: TLC 67 +/- 5%, Vital Capacity +/- 7%, FEV1 63.5 +/- 6%) and alteration in the transfer factor for carbon monoxide (DLCO: 49.5 +/- 5%). The initial broncho-alveolar lavage showed a lymphocytosis (23.6 +/- 4.2%) associated with an alveolar neutrophilia (7 +/- 2.5%). A lung biopsy was carried out in three patients and a transbronchial biopsy in one patient confirming the diagnosis of silicosis in three cases and of silicoproteinosis in one case. After a period of eighteen months to 14 years, four patients died either due to cardiopulmonary complications of their disease (two cases) or to intercurrent disorders (two cases). The last two patients are currently stable.

[Interstitial lung disease induced by timolol eye solution]. / Pneumopathie interstitielle induite par un collyre de timolol.

Timolol maleate is a beta-blocker widely used in topical administration for open angle glaucoma. We report a case of interstitial pneumopathy which developed in a 58-year-old patient who was given timolol eyedrops (2 drops b.i.d. since 1986). The patient presented with cough and dyspnea and was found to have a restrictive disorder leading to hypoxemia (68 mmHg at rest and 51 mmHg during exercise). Bronchial lavage fluid had high lymphocyte (28%) and neutrophil (7%) counts. Transbronchial biopsies demonstrated moderate fibrous thickening of the interalveolar walls. Timolol eyedrops were stopped. Three months later, the patient was asymptomatic with normal lung function, chest x-ray and thoracic CT-scan.

[The anti-synthetase syndrome]. / Le syndrome des anti-synthétases.

The anti-synthetase syndrome comprises the association of an inflammatory myopathy (polymyositis, dermatomyositis), interstitial pneumonitis, skin lesions characteristic of "mechanics hands", Raynaud's phenomena, inflammatory polyarthritis and, at the biological level, antinuclear antibodies known as anti-synthetases. We report our observations of two patients, one with a typical anti-synthetase syndrome and one with an incomplete form. Two men aged 49 and 47 presented with increasing dyspnoea upon effort, muscular weakness, arthralgia, bilateral pulmonary crackles and, in the first case, typical hairless skin lesions. In both cases the chest x-rays and CT scans confirmed the presence of interstitial lesions, predominantly in the lower lobes. Lung function tests showed a restrictive pattern with reduced gas transfer. At the biological level both patients presented an inflammatory picture with elevated muscle enzymes and anti-Jo-1 antibodies. Immuno-suppressive treatment with cortico-steroids and cyclophosphamide lead to a symptomatic improvement, regression of the radiological changes and improvement in the measurements of pulmonary function.

[Idiopathic pulmonary fibrosis: from biopathology to treatment]. / Fibrose pulmonaire idiopathique: de la biopathologie au traitement.

Alveolitis, accumulation of immune and inflammatory cells within the lower respiratory tract, is the key to the development of pulmonary fibrosis. Alveolitis is responsible for lung injury (due to release of oxidant and proteolytic enzymes) and exaggerated lung repair (due to proliferation of mesenchymal cells and imbalance of collagen synthesis). Rationale for the treatment of pulmonary fibrosis implies the knowledge of pathophysiology of the different steps of the fibrotic process.