Lung function in Birt-Hogg-Dubé syndrome: a retrospective analysis of 96 patients.

BACKGROUND: Birt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant disorder caused by mutations in the FLCN gene coding for folliculin. Its clinical expression includes cutaneous fibrofolliculomas, renal tumors, multiple pulmonary cysts, and recurrent spontaneous pneumothoraces. Data on lung function in BHD are scarce and it is not known whether lung function declines over time. We retrospectively assessed lung function at baseline and during follow-up in 96 patients with BHD. RESULTS: Ninety-five percent of BHD patients had multiple pulmonary cysts on computed tomography and 59% had experienced at least one pneumothorax. Mean values of forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), FEV1/FVC ratio, and total lung capacity were normal at baseline. Mean (standard deviation) residual volume (RV) was moderately increased to 116 (36) %pred at baseline, and RV was elevated > 120%pred in 41% of cases. Mean (standard deviation) carbon monoxide transfer factor (DLco) was moderately decreased to 85 (18) %pred at baseline, and DLco was decreased < 80%pred in 33% of cases. When adjusted for age, gender, smoking and history of pleurodesis, lung function parameters did not significantly decline over a follow-up period of 6 years. CONCLUSIONS: Cystic lung disease in BHD does not affect respiratory function at baseline except for slightly increased RV and reduced DLco. No significant deterioration of lung function occurs in BHD over a follow-up period of 6 years.

[Selection of lung transplant candidates in France in 2019]. / Sélection des candidats à la transplantation pulmonaire en France en 2019.

INTRODUCTION: In 2015, the International Society for Heart and Lung Transplantation (ISHLT) published a consensus document for the selection of lung transplant candidates. In the absence of recent French recommendations, this guideline is useful in order to send lung transplant candidates to the transplantation centers and to list them for lung transplantation at the right time. BACKGROUND: The main indications for lung transplantation in adults are COPD and emphysema, idiopathic pulmonary fibrosis and interstitial diseases, cystic fibrosis and pulmonary arterial hypertension (PAH). The specific indications for each underlying disease as well as the general contraindications have been reviewed in 2015 by the ISHLT. For cystic fibrosis, the main factors are forced expiratory volume in one second, 6-MWD, PAH and clinical deterioration characterized by increased frequency of exacerbations; for emphysema progressive disease, the BODE score, hypercapnia and FEV1; for PAH progressive disease or the need of specific intravenous therapy and NYHA classification. Finally, the diagnosis of fibrosing interstitial lung disease is usually a sufficient indication for lung transplantation assessment. OUTLOOK AND CONCLUSION: These new recommendations, close to French practices, help clinicians to find the right time for referral of patients to transplantation centers. This is crucial for the prognosis of lung transplantation.

[Prediction of the efficiency of endoscopic lung volume reduction by valves in severe emphysema]. / Prédiction de l'efficacité de la réduction endoscopique d'emphysème pulmonaire par valve.

INTRODUCTION: In severe emphysema, endoscopic lung volume reduction with valves is an alternative to surgery with less morbidity and mortality. In 2015, selection of patients who will respond to this technique is based on emphysema heterogeneity, a complete fissure visible on the CT-scan and absence of collateral ventilation between lobes. Our case report highlights that individualized prediction is possible. CASE REPORT: A 58-year-old woman had severe, disabling pulmonary emphysema. A high resolution thoracic computed tomography scan showed that the emphysema was heterogeneous, predominantly in the upper lobes, integrity of the left greater fissure and no collateral ventilation with the left lower lobe. A valve was inserted in the left upper lobe bronchus. At one year, clinical and functional benefits were significant with complete atelectasis of the treated lobe. CONCLUSION: The success of endoscopic lung volume reduction with a valve can be predicted, an example of personalized medicine.

Abdominal tuberculosis in a low prevalence country.

OBJECTIVE: Abdominal tuberculosis is a rare disease. The clinical and radiological manifestations are non-specific and the diagnosis is difficult. Our objective was to describe the characteristics and treatment of patients presenting with abdominal tuberculosis in a low-incidence country. PATIENTS AND METHODS: We reviewed the clinical, diagnostic, treatment, and outcome features of patients presenting with abdominal tuberculosis diagnosed by bacteriological and/or histological results and managed in five French university hospitals from January 2000 to December 2009. RESULTS: We included 21 patients. The mean diagnostic delay was 13 months. Twelve patients (57%) came from a low-incidence area and only two had a known immunosuppressed condition. Eighteen patients (86%) presented with abdominal symptoms. The main organs involved were the peritoneum (n=14, 66%), the mesenteric lymph nodes (n=13, 62%), and the bowel (n=7, 33%). Sixteen patients (76%) underwent surgery, including two in an emergency setting. Seventeen patients (81%) received six months or more of anti-tuberculosis treatment. Finally, 16 patients (76%) had a positive outcome. CONCLUSION: New diagnostic procedures, and especially molecular biology, may help diagnose unusual clinical presentations of tuberculosis. Invasive procedures are frequently necessary to obtain samples but also for the treatment of digestive involvement.

[Residents in respiratory medicine: Assessment of the course and wishes regarding their career]. / Appréciation du cursus et vœux relatifs à l'exercice futur des internes en pneumologie.

INTRODUCTION: The aim of this study was to assess the feelings of residents in respiratory medicine regarding the quality and organization of their training and towards their career prospects. METHODS: A prospective survey conducted over the Internet among all the members of the French Young Pulmonologists Association (AJPO2). RESULTS: One hundred and thirty-two (71.5%) members responded. The rating given to theoretical training was 6 [5-7] whereas the practical training was rated at 7 [6-8] out of 10. The majority of the residents considered that the length of their course should be adapted (80.3%). Of them, 74.2% wanted to add a mandatory semester. The proposed mandatory semester was in bronchoscopy (40.3%). Seventy-two percent of the resident wanted to acquire a specialisation, the most common of which was in oncology (36.6%). Among the residents, 96.2% wanted to conduct a fellowship. The main reason for this was their feeling of inability to correctly handle patients at the end of their residency. Of the residents, 55.3% were considering working in a public hospital. CONCLUSION: There are opportunities to improve the French respiratory residency training both in its theory and practical aspects. The modalities of this training could also be adapted. Access to a fellowship is a major concern for the residents.