[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.

[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.

[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.

[Submaximal exercise capacity and quality of life in exclusive water-pipe smokers]. / Aptitude aérobie sous-maximale et qualité de vie des fumeurs exclusifs de narguilé

BACKGROUND: It is well known that oxidative stress is increased significantly by regular water-pipe smoking (WPS). This could lead to muscle dysfunction and thus to impairments of exercise and quality of life (QOL). Considering the impressive number of WP smokers, we intend to investigate the potential effect of WPS on submaximal exercise capacity and QOL. AIMS: (1). To evaluate the submaximal exercise capacity by the 6-minutes walking test (6-MWT). (2). To compare the deficiency, incapacity and QOL data of exclusive WPS with those of two control groups (never smokers and exclusive cigarette smokers). (3). To determine the factors influencing the 6-minutes walk distance (6-MWD) of WPS subjects. METHODS: A multicentre study including 180 exclusive WPS [> or =5 WP-year] men aged > or =40 years. Cigar or cigarette smoking, contraindications to the 6-MWT or cortico-steroid therapy will be exclusion criteria. QOL evaluation, spirometry, electrocardiogram and two 6-MWT will be performed. Signs of exercise impairment will be: 6-MWD< or =lower limit of normal, end of walk dyspnoea > or =5/10, haemoglobin saturation fall > or =5 points. Data from WPS subjects will be compared with those from 90 never smoking subjects and 90 exclusives cigarettes smokers. EXPECTED RESULTS: (1). WPS will affect significantly the submaximal exercise capacity. (2). Resting spirometric, 6-MWT and QOL data of exclusive WPS subjects will be significantly reduced compared to never smoking subjects. (3). The 6-MWD's of exclusive WPS subjects will be significantly influenced by cumulative WP consumption, by resting spirometric data, by obesity and by physical activity score.

Relationship between glutathione S-transferase P1 polymorphisms and chronic obstructive pulmonary disease in a Tunisian population.

Chronic obstructive pulmonary disease (COPD) is a multifactorial disease with possible genetic predisposition and involvement of various environmental factors. Several candidate genes have been reported as potentially associated with this lung disease. The glutathione S-transferase P1 gene (GSTP1) was proposed to be involved in susceptibility to develop COPD. It belongs to the GST family, which is a group of phase II enzymes that catalyze the glutathione conjugation of many endogenous and exogenous electrophilic compounds, such as carcinogens, therapeutic drugs, environmental toxins, and oxidative stress products. We conducted a case-control study to investigate genetic polymorphisms of this enzyme [exon 5 (Ile105Val) and exon 6 (Ala114Val)] in 234 unrelated COPD cases and 182 healthy controls from a Tunisian population. Genotyping was carried out using polymerase chain reaction and restriction fragment length polymorphism methods. GSTP1 Ala114/Val114 and Val114/Val114 genotypes were not found in either patients or healthy controls. However, there were differences in the distribution of various exon 5 GSTP1 genotypes between COPD patients and healthy controls. GSTP1 Val105/Val105 was significantly more common in patients compared to controls (OR = 2.67; 95%CI = 1.45-4.92; P = 0.0013). Multivariate logistic regression analysis confirmed a significant relationship between the mutant genotype and COPD (OR = 2.58; 95%CI = 1.31-5.09; P = 0.026), after adjustment for classic risk factors. Analysis of variance showed no correlation between age, body-mass index, pack-years, percentage of predicted FEV1 values, and any of the GSTP1 genotypes. We conclude that subjects with GSTP1 Val105 allele are at higher risk of COPD.

[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.