Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease.

Background: Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports.

Mepolizumab and benralizumab in patients with severe asthma and a history of eosinophilic granulomatosis with polyangiitis.

Introduction: Asthma associated with eosinophilic granulomatosis with polyangiitis (EGPA) is often severe and corticosteroid-dependent, leading to significant morbidity. Mepolizumab and benralizumab are humanized monoclonal antibodies targeting interleukin 5 (IL-5) and its receptor, respectively. They have been shown to be effective in steroid-sparing in patients with severe eosinophilic asthma.

Pulmonary function test results are correlated with 6-minute walk distance, distance-saturation product, and 6-minute walk work in patients with lymphangioleiomyomatosis.

Background And Objective: Lymphangioleiomyomatosis (LAM) is a rare multicystic lung disease. Although a correlation between pulmonary function test (PFT) results and exercise capacity appears probable, it has not yet been demonstrated. The aim of this study was to assess whether PFT results correlate with 6-minute walk test (6MWT) results in patients with LAM.

[Genetic diffuse cystic lung disease in adults].

Multiple cystic lung diseases comprise a wide range of various diseases, some of them of genetic origin. Lymphangioleiomyomatosis (LAM) is a disease occurring almost exclusively in women, sporadically or in association with tuberous sclerosis complex (TSC). Patients with LAM present with lymphatic complications, renal angiomyolipomas and cystic lung disease responsible for spontaneous pneumothoraces and progressive respiratory insufficiency.

Elaboration of a French version of the Duke Activity Status Index questionnaire and performance to predict functional capacity.

Background: Guidelines recommend detecting poor functional capacity (VO < 14 ml.kg.min) to assess preoperative cardiac risk.

Concurrence of 1- and 3-Min Sit-to-Stand Tests with the 6-Min Walk Test in Idiopathic Pulmonary Fibrosis.

Background: In idiopathic pulmonary fibrosis (IPF), some physiological parameters measured during a 6-min walk test (6-MWT) impart reliable prognostic information. Sit-to-stand tests (STSTs) are field exercise tests that are easier to implement than the 6-MWT in daily practice.

Objectives: The aims of the study were to test the reproducibility and compare 2 STSTs (the 1-min STST [1-STST] and the semi-paced 3-min chair rise test [3-CRT]) in IPF, and to determine if selected physiological parameters (speed of displacement and changes in pulse oxygen saturation [SpO2]) are interchangeable between the STSTs and the 6-MWT.

Impact of Lung Biopsy on Lung Function in Idiopathic Pulmonary Fibrosis.

Background: Video-assisted surgical lung biopsy (SLB) is performed in 10-30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF).

Objectives: The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF.

Methods: This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center.

Impact of comorbidities and delay in diagnosis in elderly patients with pulmonary hypertension.

Patient age at diagnosis of pulmonary hypertension is steadily increasing. The present study sought to analyse clinical characteristics, time to diagnosis and prognosis of pulmonary hypertension in elderly and very elderly patients. A study was conducted in a French regional referral centre for pulmonary hypertension.

Safety of right heart catheterization for pulmonary hypertension in very elderly patients.

Right heart catheterization (RHC) is the reference test in diagnosing pulmonary hypertension (PH). The increasing age of patients at the time of diagnosis raises the issue of the morbidity of this invasive test in elderly individuals. We hypothesized that the morbidity associated with RHC would be increased in elderly patients and highlight differences in hemodynamic characteristics compared to younger patients.

Prognostic value of right ventricular ejection fraction in pulmonary arterial hypertension.

Right ventricle ejection fraction (RVEF) evaluated with magnetic resonance imaging is a strong determinant of patient outcomes in pulmonary arterial hypertension. We evaluated the prognostic value of RVEF assessed with conventional planar equilibrium radionuclide angiography at baseline and change 3-6 months after initiating pulmonary arterial hypertension-specific therapy. In a prospective cohort of newly diagnosed patients with idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension, RVEF was measured at baseline (n=100) and 3-6 months after initiation of therapy (n=78).

Hypereosinophilic obliterative bronchiolitis: a distinct, unrecognised syndrome.

Biopsy-proven cases of eosinophilic bronchiolitis have only been reported in isolation, and all come from Japan. We present six patients with hypereosinophilic obliterative bronchiolitis (HOB), defined by the following criteria: 1) blood eosinophil cell count >1 G·L(-1) and/or bronchoalveolar lavage eosinophil count >25%; 2) persistent airflow obstruction despite high-dose inhaled bronchodilators and corticosteroids; and 3) eosinophilic bronchiolitis at lung biopsy (n=1) and/or direct signs of bronchiolitis (centrilobular nodules and branching opacities) on computed tomography (n=6). Chronic dyspnoea and cough which was often severe, without the characteristic features of asthma, were the main clinical manifestations.

Conditions associated with severe carbon monoxide diffusion coefficient reduction.

Objective: To identify conditions associated with severe carbon monoxide transfer coefficient reduction (Kco < 40% of predicted values).

Patients And Methods: We retrospectively reviewed pulmonary function tests, such as Kco measurement, in consecutive patients evaluated in the physiology unit of a University hospital over a 6-year period. Patients were included if they had at least 1 measure of severe Kco reduction, with further detailed pulmonary function tests, clinical data, and diagnostic procedures conducted within 6 months.

Right-to-left shunt with hypoxemia in pulmonary hypertension.

Background: Hypoxemia is common in pulmonary hypertension (PH) and may be partly related to ventilation/perfusion mismatch, low diffusion capacity, low cardiac output, and/or right-to-left (RL) shunting.

Methods: To determine whether true RL shunting causing hypoxemia is caused by intracardiac shunting, as classically considered, a retrospective single center study was conducted in consecutive patients with precapillary PH, with hypoxemia at rest (PaO2 < 10 kPa), shunt fraction (Qs/Qt) greater than 5%, elevated alveolar-arterial difference of PO2 (AaPO2), and with transthoracic contrast echocardiography performed within 3 months.

Results: Among 263 patients with precapillary PH, 34 patients were included: pulmonary arterial hypertension, 21%; PH associated with lung disease, 47% (chronic obstructive pulmonary disease, 23%; interstitial lung disease, 9%; other, 15%); chronic thromboembolic PH, 26%; miscellaneous causes, 6%.

Sarcoidosis and the kidney: not only the granulomatous interstitial nephritis.

Renal involvement in sarcoidosis displays a wide range of manifestations, and kidney dysfunction may involve all three mechanisms of renal failure. We report a new case of systemic sarcoidosis presenting as a severe renal failure due to hypercalcemia, sarcoidosis-related bilateral nephrolithiasis and granulomatous interstitial nephritis. A prostate adenocarcinoma was also diagnosed, but has to be regarded as an unrelated disease.

Persistent airflow obstruction in asthma of patients with Churg-Strauss syndrome and long-term follow-up.

Background: Little is known about the long-term outcome of airflow obstruction in asthma of patients with Churg-Strauss syndrome (CSS).

Methods: We conducted a retrospective study of 24 consecutive patients (aged 41.1 +/- 13.

Noninvasive ventilation using a mouthpiece in patients with chronic obstructive pulmonary disease and acute respiratory failure.

Background: Noninvasive positive pressure ventilation (NPPV) delivered via a mouthpiece (mNPPV) has been successfully used in stable chronic restrictive respiratory insufficiency, but not in patients with chronic obstructive pulmonary disease (COPD) and acute respiratory failure (ARF).

Objectives: The purpose of this matched case-control study was to compare the usefulness of mNPPV to noninvasive ventilation using a nasal or oronasal mask (nNPPV) or standard medical treatment (SMT) in COPD patients with ARF.

Methods: Twenty-nine patients receiving mNPPV were matched with 29 patients receiving nNPPV and 29 patients receiving SMT regarding age, SAPSII, admission PaCO(2) and pH.

Assist-control ventilation vs. low levels of pressure support ventilation on sleep quality in intubated ICU patients.

Objective: To compare the impact of assist-control ventilation (ACV) and pressure support ventilation with 6 cmH2O inspiratory pressure (low PSV) on sleep quality.

Design: Prospective randomized cross-over study.

Patients: Twenty intubated and mechanically ventilated patients for acute on chronic respiratory failure.

Diaphragm electromyograms recorded from multiple surface electrodes following magnetic stimulation.

The diaphragm compound-muscle action potential (CMAPdi), elicited by unilateral magnetic stimulation (UMS) of the phrenic nerve can be recorded using surface electrodes. However, there is no consensus on the best positioning of surface electrodes and there are no data on the reproducibility of the signal. Using 36 surface electrode pairs, in five healthy subjects, the CMAPdi elicited by UMS and electrical stimulation (ES) were compared and 12 pairs were identified as providing acceptable signals.

[Results of an ambulatory respiratory rehabilitation program: observations in 100 COPD patients].

Introduction: Respiratory rehabilitation is a multidisciplinary approach enabling personalized management adapted for each patient's handicap.

Method: Our ambulatory program includes twenty sessions per week (four half-days/week) for respiratory training at the ventilatory threshold, respiratory physical therapy, and health education. One hundred COPD patients participated in this retrospective study: mean age 62.

Vagal pulmonary afferents and central respiratory effects of 5-HT in newborn rats.

In decerebrate newborn rats, serotonin (5-HT) is a respiratory depressant via activation of 5-HT2 receptors, whereas it evokes respiratory stimulant effects when applied to the isolated brainstem obtained from the newborn rat. This discrepancy could be due to deafferentation in the in vitro preparation. The aim of our study was to analyse the role of vagal afferents in the modulation of central respiratory effects of 5-HT.

Comparison of 2 maintenance doses (100 microg vs 200 microg) in Hymenoptera venom immunotherapy: influence of the maintenance dose on the immunologic response.

Background: In Hymenoptera venom immunotherapy, the maintenance dose is usually 100 microg. However, persistent systemic reactions to sting challenges could be treated by an increase in the maintenance dose to 200 microg with success, suggesting greater efficiency.

Objective: To compare the effects of 2 monthly maintenance doses (100 microg vs 200 microg) on skin test sensitivity and venom specific IgE antibody levels.

Oxygen consumption and PEEPe in ventilated COPD patients.

The intrinsic positive-end-expiratory pressure (PEEPi) increases the inspiratory load, the cost of breathing and thus oxygen consumption (V(O2)). It has been shown that applying an extrinsic positive-end-expiratory pressure (PEEPe) reduces the inspiratory threshold load but the optimal PEEPe level is still in debate. We hypothesize that the best level of PEEPe could induce a decrease in V(O2) by reducing the V(O2) demands from PEEPi.

[Transudative pleural effusions and the ovarian hyperstimulation syndrome].

Introduction: Pleural effusions occurring in the ovarian hyperstimulation syndrome (OHS) are frequently associated with haemoconcentration.

Observations: We report three cases of symptomatic pleurisy with ascites, requiring therapeutic pleural aspiration. Biochemical analysis of these effusions may give rise to confusion as consideration of the pleural fluid protein levels in isolation may lead to the incorrect diagnosis of an exudate.