[Prognostic factors in idiopathic pulmonary fibrosis in a tunisian cohort]. / Facteurs pronostiques au cours de la fibrose pulmonaire idiopathique : étude d'une cohorte tunisienne.

We present data on prognostic factors in a Tunisian cohort of people with Idiopathic pulmonary fibrosis. INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) has a poor prognosis, with a median survival in patients with the condition of only 3 to 5 years. Previous studies have identified a number of prognostic factors in this chronic pulmonary disease. METHODS: We conducted a retrospective study, including patients with idiopathic pulmonary fibrosis (IPF) who were diagnosed at the Pneumology Department of the University Hospital Fattouma-Bourguiba, Monastir, between 1991 and 2014. The aim of this study was to compare clinical, radiological, pulmonary functional predictors of survival in IPF in a Tunisian cohort with those of previous studies. RESULTS: This study included 126 patients. Their mean age was 66 years, with a male predominance (68.3%). Respiratory function tests revealed a restrictive ventilatory deficit in 72.6% of cases. The median survival of our study population was 22.5 months [6.7-49.5]. In univariate analysis, factors associated with a poor prognosis were: lower baseline values of TLC, FCV and DLco, level of dyspnea assessed by mMRC scale, hypoxemia at diagnosis, the degree of desaturation during exercise, a higher annual decline of FVC and DLco, acute respiratory distress and also the GAP score. In multivariate analysis, independent prognostic factors were: baseline DLco, level of dyspnea, desaturation at exertion and the annual decline of the DLco. CONCLUSION: Lower baseline DLco, the level of dyspnea, desaturation on exercise, and annual decline in DLco are all associated with a poor prognosis in IPF.

[Contribution of high fidelity simulation to training in respiratory medicine]. / Apport de l'apprentissage par la simulation dans l'enseignement de la pneumologie.

INTRODUCTION: Simulation maintains patient safety by limiting the risk of errors. In the medical field, simulation is a method of learning that is developing more and more in the acute specialties but it is still not widespread in the field of respiratory medicine. OBJECTIVE: To evaluate the efficacy of high fidelity simulation as a teaching tool in respiratory medicine. METHODS AND POPULATION STUDIED: This was a prospective and descriptive study including students who had high fidelity simulation training sessions during their traineeship in respiratory medicine. Simulation learning took the form of four teaching sessions. The included students were assessed at the beginning and end of each session by a pre-test and post-test. Student satisfaction was assessed at the end of each session. RESULTS: Comparing the average student score before and after the simulation session showed an improvement in post-test scores. This improvement was statistically significant for all four scenarios. The majority of students, 60% (N=13), were satisfied with the progress of their internship in the Respiratory Department. CONCLUSION: High fidelity simulation is a teaching method that allows the acquisition and/or optimization of several skills. Nevertheless, this method remains undeveloped in respiratory medicine.

[Asthma control and quality of life]. / Contrôle de l'asthme et qualité de vie.

INTRODUCTION: The assessment of asthma control is based on objective measures: clinical, pharmacological and spirometry. However subjective component may be also necessary for assessing asthma control. OBJECTIVES: To study the feasibility and clinical value of the assessment of the quality of life of patients with asthma by the SF-36 (Medical Outcomes Study Short Form) and the possible existence of a correlation between controlled asthma and a better quality of life. PATIENTS AND METHODS: A prospective study that included 167 patients with asthma in a stable condition. Control of asthma and SF-36 were established three months after the inclusion of patients. RESULTS: The SF-36 was lower in the uncontrolled group in all areas of the physical component and the difference was significant in the "limitation related to physical activity" and "perceived health". In the mental component, the score was lower in "mental health" and the "limitation due to mental state" in the group with uncontrolled asthma and the difference was significant only in the limitation due to mental state (P=0.043). CONCLUSION: The quality of life of asthmatic patients is correlated to the control of this disease.

[Obesity and the severity of asthma crisis]. / Obésité et sévérité de la crise d'asthme.

INTRODUCTION: Obesity is associated with inflammatory processes, which could influence the airway inflammation that is found in patients with asthma. Obesity may thus have a role in the development of asthma. However, the role of obesity in the severity of acute asthma has not been well described. PATIENTS AND METHODS: We performed a retrospective study, which included 77 patients hospitalized for acute asthma. Two groups of patients were formed according to their body mass index (BMI): group 1 consisting of 59 patients with a BMI inferior to 30 kg/m(2) and group 2 consisting of 18 patients with a BMI superior or equal to 30 kg/m(2). These two groups were compared according to demographic factors, clinical features and the spirometric severity of asthma. RESULTS: The mean age was 43 ± 17.4 years with a sex-ratio 0.57 (28 men/49 women). Thirty-one percent of these patients had a severe asthma attack requiring hospitalization in intensive care in four patients with the use of mechanical ventilation in two patients. The comparison between obese and non-obese patients did not show a significant difference in the severity of asthma. CONCLUSION: Although a contribution of obesity to the manifestation and severity of asthma is commonly recognized, the present data to not confirm the impact of obesity on the severity of acute attacks.

[Anxiety-depressive disorders and bronchiectasis]. / Troubles anxiodépressifs et dilatation des bronches.

INTRODUCTION: Bronchiectasis is a chronic lung disease that may be associated with anxiety-depressive disorders affecting the quality of life of patients. Detecting these anxiety-depressive disorders may be necessary in the support and overall management of a patient with bronchiectasis. AIMS: To evaluate the prevalence of anxiety and depression in patients with bronchiectasis and to investigate the possible correlation between the severity of the disease and the importance of these psychological disorders. PATIENTS AND METHODS: This was a prospective study that included 53 patients with stable bronchiectasis and without other comorbidities. All the patients underwent a complete clinical examination, spirometry and chest computed tomography. All the 53 patients responded to the Hospital Anxiety and Depression Scale (HAD) questionnaire. RESULTS: Anxiety was present in 22.7% of patients and depression in 20.8%. Subjects who had an anxiety disorder had symptoms primarily of dyspnoea (P=0.001), a low FEV (P=0.04) and respiratory failure at a stage requiring home oxygen therapy (P=0.009). A similar comparison of patients with and without depressive disorder again found a high prevalence of dyspnea (P=0.003), a low FEV (P=0.04), and chronic respiratory failure in the depressive patients. CONCLUSION: Symptoms of depression and anxiety are common in patients with bronchiectasis and appear to be associated with dyspnoea. Early detection is necessary in the context of the overall management of these patients.

[Right upper lobe pulmonary agenesis]. / Agénésie pulmonaire lobaire supérieure droite.

INTRODUCTION: Pulmonary agenesis is a rare congenital anomaly. Other cardiovascular, gastrointestinal, musculoskeletal, and urogenital system anomalies can be observed in association with it. CASE REPORT: A 24-year-old female patient presented to our clinic with a history of cough and chest pain with an abnormal chest X-ray. Physical examination was normal. Chest X-ray, CT-Scan and endoscopy led to the diagnosis of right upper lobe pulmonary agenesis. CONCLUSION: The diagnosis of pulmonary malformations like agenesis or hypoplasia can be delayed and may not occur until adulthood. Once the diagnosis has been established medical follow up is mandatory.

[Smoking habits and severity of obstructive sleep apnea hypopnea syndrome]. / Tabagisme et sévérité du syndrome d'apnées hypopnées obstructives du sommeil.

INTRODUCTION: Smoking habits have many adverse health effects. The effect of tobacco on obstructive sleep apnea hypopnea syndrome is well-documented but the effect on the severity of this syndrome has not been completely established. AIM OF THIS STUDY: Evaluate the effect of tobacco on the severity of sleep apnea hypopnea syndrome. PATIENTS AND METHODS: During 4 years 307, patients were referred to our department for a suspicion of obstructive sleep apnea hypopnea syndrome. Among these patients, only 151 patients had an obstructive sleep apnea syndrome confirmed by nocturnal polygraph. Smoking status, clinical and spirometric data were determined in these 151 patients. RESULTS: Among these 151 patients, 101 were non-smokers and 50 current smokers. The apnea and hypopnea index (AHI) were higher among current smokers than non-smokers (42.96/h versus 28.77/h; P<0.0001). The percent of patients who had a not severe obstructive sleep apnea syndrome were higher among non-smokers than current smokers patients (P=0.009). Current smokers were 3.7 times more likely having severe obstructive sleep apnea hypopnea syndrome than non-smokers (OR=3.7; P=0.001). CONCLUSION: Smoking habits seems to be associated with the severity of obstructive sleep apnea syndrome that's why smoking cessation is very important in the treatment of obstructive sleep apnea hypopnea syndrome.

[Rare primary chest wall sarcoma: the synovialosarcoma]. / Sarcome primitif rare de la paroi thoracique: le synovialosarcome.

INTRODUCTION: Malignant primary tumours occurring in the thorax encompass a large group of tumours which may arise from the lung, mediastinal structures, the pleura or the chest wall. OBSERVATION: We report the case of a 37 year old patient, who presented with left sided chest pain. On clinical examination a right sided chest wall mass was identified. Chest X Ray showed a left sided upper mediastinal opacity, associated with a left sided pleural opacity. Thoracic CT scan revealed a large mass arising from the chest wall and infiltrating the mediastinum associated with a second chest wall mass at the level of the 8(th) and 9(th) right ribs. The biopsy of the chest wall mass revealed it to be a parietal synovialosarcoma. The patient responded to chemotherapy based on ifosfamid and doxorubicin as well as mediastino-pulmonary radiotherapy. There was an improvement in the patient's clinical and radiological state but the patient died by pulmonary embolism after the 3(rd) cause of treatment. CONCLUSION: Chest wall synovialosarcoma has a poor prognosis, however, its chemosensitivity means that treatment may initially be effective.

[Enigmatic evolution of an association of pulmonary tuberculosis and amyloidosis]. / Évolution énigmatique d'une association de tuberculose et d'amylose pulmonaire.

INTRODUCTION: Amyloidosis is characterized by tissue deposits of amyloid material. Secondary amyloidosis can occur as a sequel to pulmonary tuberculosis over a relatively long period. However, this was not the case with our patient. Subsequently we conducted a literature review to try to explain the unusual course of AL amyloidosis in our patient. CASE REPORT: A 36- year-old patient was admitted to our department for investigation of haemoptysis. A diagnosis of primary pulmonary tuberculosis was made and antituberculous treatment was started. On the second day of treatment, a haematoma appeared on the sole of the right foot, which spread down to the toes during the following days. Renal investigations showed a 24h proteinuria of 9 g/L and serum protein electrophoresis revealed an albumin level of 11.8 g/L. A diagnosis of nephrotic syndrome was made. A renal biopsy was indicated but this was not possible on account of a marked worsening of the patient's condition after 14 days of treatment. The patient's level of consciousness deteriorated and he was transferred to the intensive care unit for ventilation. He died 48 hours later. Post-mortem histological examination of pulmonary and cutaneous tissue revealed AL amyloid deposits. CONCLUSION: In view of the association of active pulmonary tuberculosis and a pulmonary localisation of amyloidosis, a causal relationship is not definite. Coexistence of active pulmonary tuberculosis and primary amyloidosis must also be considered, particularly as the immunohistochemical characterisation revealed AL amyloidosis.

[Unusual alveolar proteinosis]. / Protéinose alvéolaire atypique.

Pulmonary alveolar proteinosis (PAP), a rare infiltrative disease of unknown aetiology, is characterized by an accumulation of abnormal lung surfactant in the alveoli. The diagnosis is based on the results of the bronchoalveolar lavage (BAL) and sometimes on the lung biopsy. The authors report the case of a 49-year-old woman who was hospitalized for chronic expectoration of the membranes. The chest X-ray revealed alveolar opacities in the lowest part of the right lung. The chest CT scan detected alveolar ground glass opacities with interlobular thickening involving the middle lobe. The BAL was opaque with periodic acid-Schiff stain-positive acellular material. The anatomopathology analysis of the membranes concludes as to the presence of granular eosinophilic material and the absence of neoplasic cells or hydatidous membranes. The diagnosis of PAP was established. Since functional deterioration was not detected, therapy was based on physiotherapy alone. The evolution was favourable, with the disappearance of the symptomatology and the normalisation of the chest X-ray. This observation shows an unusual presentation of PAP based on membrane expectoration and unusual localized lesions.

[Neuropsychiatric side effects of antituberculosis agents]. / Les effets indésirables neuropsychiatriques des antituberculeux.

UNLABELLED: Accurency of neurological or psychiatric complications secondary to the administration of antituberculosis may be at the origin of diagnosis and therapeutic problems. This work aims at studying the frequency of these manifestations, their clinical presentations and their therapeutic approach. PATIENTS AND METHODS: This retrospective study was carried on from January 1990 to June 2008 at Ibn Nafis Pulmonary Department of Abderrahmen Mami Hospital in Ariana. It dealt with 18 in-patients with a neurologic or a psychiatric complication due to antituberculous drugs. Peripheral neuropathy was noted in 6 patients (33%). One of them had a history of chronic alcoolism, another one had a history of diabetes mellitus and 2 other patients were more than 72 years old. We had definitively stopped isoniazide in 2 cases and decreased the dosage in 4 other patients. However, all patients received B6 vitamin. Convulsions occured in 2 women without any history of epileptic status. Anticonvulsivant treatment was prescribed and isoniazid definitively stopped. Hallucinosis was noted in 4 patients, with one having a history of chronic alcoolism. Isoniazide was stopped in all cases. Agressivity, insomnia and memory problems were noted in 6 patients. Isoniazid was interrupted in only one woman who had history of depression. In 5 other patients, anxiolytics were prescribed. Isoniazide was incrimined in all cases and evolution was favorable for the 18 patients. A close monitoring of patients on antituberculous treatment is required to detect the onset of any neuropsychiatric complications incriminating usually isoniazid. Definitive interruption or decrease of the dose of isoniazid depending of the acetylation test were necessary.

[Hypersensitivity reactions to antituberculous therapy]. / Manifestations d'hypersensibilité aux antituberculeux.

INTRODUCTION: Hypersensitivity reactions to antituberculous drugs pose both a diagnostic and a therapeutic problem. This work aims to study the frequency of allergic reactions to antituberculous drugs, their clinical presentations and the diagnostic approach required. PATIENTS AND METHODS: This retrospective study covered the period from January 1990 to June 2008 at the Ibn Nafis Pulmonary Department of Abderrahmen Mami Hospital in Ariana. It dealt with 30 in-patients who experienced an allergic reaction to antituberculous drugs. RESULTS: Cutaneous manifestations were the most frequent (80% of cases), predominantly urticarial. Thrombocytopenia was noted in two cases, anaphylactic shock in three cases, a systemic toxidermia in two cases and renal failure in one patient. Pyrazinamide was implicated in most cases (28%) when only one drug was considered to be responsible for the reaction. Interrupting either one drug or the whole treatment was necessary to define the cause of the reaction. The clinical evolution of hypersensitivity signs was favorable in all cases following definitive withdrawal of the responsible drug. Complete recovery from tuberculosis occurred in all cases. CONCLUSION: Close monitoring of patients on antituberculous treatment is required to detect the onset of any allergic reaction and ensure an adequate compliance with treatment.

[Tuberculosis in patients with asthma]. / Asthme et tuberculose: association bénéfique ou maléfique?

INTRODUCTION: The association of asthma and tuberculosis is rare but may raise particular issues around patient management. The aim of this study was to evaluate the clinical, therapeutic and progress of this association. PATIENTS AND METHODS: We describe a retrospective study, which included seven asthmatic patients hospitalized for pulmonary tuberculosis during the period between June 2001 and June 2006. RESULTS: Five men and two women were included. Mean age was 37 years. Two patients had mild asthma, four had moderate asthma and one had severe and corticosteroid-dependant asthma. Only four patients had controlled asthma when tuberculosis diagnosis was established. Asthma treatment was based on inhaled corticoids and long-acting beta-2-agonists. During antituberculosis treatment two patients developed near fatal asthma. Long-term stable asthma control was achieved over a time course of 3 to 8 years. CONCLUSION: The association of asthma and tuberculosis can lead to potential therapeutic difficulties because of pharmacologic interactions between antituberculosis therapies and treatments for asthma treatment. In addition asthma following treatment for tuberculosis appears to be well controlled.

[Effect of tobacco smoking on pulmonary tuberculosis]. / Effet du tabagisme sur la tuberculose pulmonaire.

Tobacco increases the risk of pulmonary infection, especially tuberculosis. We try by this study to analyse this action. It's a comparative study between two groups of patients hospitalized in our department between January 2006 and June 2008. The first group was made of 30 smokers patients hospitalized because of confirmed pulmonary tuberculosis. The second group consists of 30 non smokers patients and also hospitalized because of confirmed pulmonary tuberculosis. Delay of diagnosis was longer in the group of smokers (3, 1 +/- 3 months versus 2 +/- 1 month with p = 0.039). The most frequent symptoms in both groups were cough and loss of weight. The biological investigations showed a high level of white blood cells with predominance of neutrophil cells especially in smokers (p = 0.024). Chest X ray showed essentially nodules in both groups. These nodules were bilateral in smokers (P = 0.045). Evolution after antituberculosis treatment was favourable for all patients. A delay of recovery (time between symptoms and recovery) was longer in smokers than in non smoker patients (p = 0.043). Pulmonary Sequels such as dyspnoea (p = 0.016) and fibrosis (p = 0.041) were most frequent in smokers. No patients had tuberculosis relapse. Tobacco may delay the recovery of pulmonary tuberculosis and may induce pulmonary sequels in spite of correctly antituberculosis treatment.

[Familial idiopathic pulmonary fibrosis associated with autoimmune polyendocrinopathy and epidermodysplasia verruciformis]. / Fibrose pulmonaire idiopathique familiale associée à une polyendocrinopathie auto-immune et à une épidermodysplasie verruciforme.

Familial idiopathic pulmonary fibrosis (IPF) is a very rare and progressively fatal disease. Its pathogenesis is not fully understood and involves damage to alveolar epithelial cells of possibly immunological, microbiological or chemical origin, leading to fibrosing healing. A genetic predisposition has been demonstrated. The authors report the case of a female patient whose brother died at the age of 29 from IPF. She had epidermodysplasia verruciformis since childhood, with the absence of pubertal development. At the age of 31, she presented diffuse interstitial pneumonia. A lung biopsy confirmed the diagnosis of IPF. Endocrine explorations detected hypogonadotropic hypogonadism, primary hypothyroidism and magnetic resonance imaging revealed an empty sella turcica. The association of familial IPF, autoimmune polyendocrinopathy and genetic dermatosis caused by a cellular immune deficiency supports the hypothesis of an immune dysfunction in the pathogenesis of IPF.

[Pleural empyema revealing osteopoikilosis]. / Pleurésie purulente révélatrice d'une ostéopoecilie.

INTRODUCTION: Osteopoikilosis is a rare, inherited and usually asymptomatic sclerosing bone dysplasia of unknown etiology which predominantly involves the appendicular and rarely the axial skeleton. CASE REPORT: We report the case of a 24 year old man who was hospitalized for pleural empyema and treated with antibiotics for six weeks in addition to pleural evacuation and physiotherapy. The diagnosis of osteopoikilosis associated with the pleural empyema was made on the radiological findings. In fact the chest X-Ray showed spherical areas of increased bone density in both humeral epiphyses. In order to explore these bone abnormalities further investigations were performed, including red and white blood cell counts, sedimentation rate and protein electrophoresis. There were no biological abnormalities. Radiography of the whole skeleton showed disseminated sclerotic lesions in the pelvis and the metacarpal and carpal bones of both hands. A neoplastic aetiology was excluded. In the light of these investigations, the diagnosis of osteopoikilosis was established. CONCLUSION: Widespread osteopoikilosis can be revealed on chest radiography.

[Echinococcosis of the rib with epidural extension]. / Kyste hydatique costovertébral : pathologie bénigne ou maligne ?

Osseous hydatidosis, especially when located in the rib, is a very rare disease. Less than 50 cases of costal echinococcosis have been reported in the literature to date. The authors report a case of echinococcosis of the rib with epidural extension in a 76-year-old patient presenting paraparesis. In addition, the patient presented a large posterior and thoracic soft tissue mass measuring about 30 centimetres in diameter. A chest x-ray, a CT thoracic scan and an MRI of the dorsal spine were performed. The imaging suggested echinococcosis of the rib with epidural extension. The cyst was completely resected. Histopathology of the resected specimen confirmed the diagnosis of echinococcosis. The patient died due to postoperative complications. Accurate presurgical diagnosis allows for appropriate management and helps eradicate the disease. This also prevents the dissemination of parasites and further complications.

[Broncholithiasis]. / Broncholithiase.

INTRODUCTION: Broncholithiasis is defined as the presence of calcified material in the bronchial lumen. The aim of our work was to study the clinical, radiological and therapeutic aspects of broncholithiasis. OBSERVATIONS: We report the histories of 6 patients identified over 14 years (1990-2004). They included 4 men and 2 women with an average age of 50 years. A past history of pulmonary tuberculosis was found in 2 cases. The presenting symptom was haemoptysis in 4 patients. Fibreoptic bronchoscopy was performed in all patients and broncholiths were found in two. Thoracic CT scan was performed in 5 patients and in 3 showed hilar and parenchymal calcification, suggesting the diagnosis of broncholithiasis. The diagnosis was confirmed in 2 patients by bronchoscopy, in 2 others by surgical biopsy and in the remaining 2 by the CT appearances. Three patients were operated on: 2 for diagnosis and the third for haemostasis. Pulmonary tuberculosis was discovered in 2 patients, one by culture and the other by surgical biopsy. The outcome was satisfactory in all cases. CONCLUSIONS: Broncholithiasis presents a problem of differential diagnosis from other pulmonary pathologies on account of misleading clinical, endoscopic and radiological features.