Concerning one case of rupture of a flow regulator: How patient safety procedures contribute to the correct use of medical devices.

Flow regulators are widely used in hospitals to assist with intravenous (IV) infusion of medication. The rupture of a flow regulator at the base of the clamp was observed during parenteral nutrition. This rupture resulted in fluid leakage and an inlet of air, responsible for an air embolism in a fragile patient who had undergone a bilateral lung transplant.

Discordant courses of COVID-19 in a cohabiting couple of lung transplant recipients.

COVID-19 is a novel infectious disease caused by SARS-CoV-2 that emerged in late 2019 and which is now a pandemic. Solid organ transplant recipients are perceived to be at increased risk of severe COVID-19 due to their chronic use of immunosuppressive drugs (ISDs) and to their associated conditions. Scarce data are available on the optimized management of ISDs in these patients and on its impact on presentation, clinical course, viral shedding, and outcome.

Prevalence of Fecal Incontinence in Adults with Cystic Fibrosis.

Background: Patients with cystic fibrosis (CF) are deemed at risk of developing urinary incontinence (UI) due to repeated coughing and other factors causing increased pressure on the pelvic floor. Fecal incontinence (FI) is probably derived from the same mechanism, but only very few data are available on its frequency.

Aims: The aim of this study was to determine the prevalence of FI in an adult population with CF.

Real-life initiation of lumacaftor/ivacaftor combination in adults with cystic fibrosis homozygous for the Phe508del CFTR mutation and severe lung disease.

Objective: To investigate the short-term adverse events and effectiveness of lumacaftor/ivacaftor combination treatment in adults with cystic fibrosis (CF) and severe lung disease in a real life setting.

Methods: A multicentre observational study investigated adverse events, treatment discontinuation, FEV and body mass index (BMI) one month and three months after lumacaftor/ivacaftor initiation in adults with CF and FEV below 40% predicted.

Results: Respiratory adverse events (AEs) were reported by 27 of 53 subjects (51%) and 16 (30%) discontinued treatment.

[Inhaled treatments in cystic fibrosis: what's new in 2013?].

In the past few years some new inhaled drugs and inhalation devices have been proposed for the treatment of cystic fibrosis. Breath-controlled nebulizers allow increased pulmonary deposition, with a lower variability and a shorter delivery time. The new dry powder formulations of tobramycin, colistine and mannitol require a change in the inhalation technique which must be slow and deep.

How to minimize toxic exposure to pyridine during continuous infusion of ceftazidime in patients with cystic fibrosis?

Ceftazidime is particularly efficient against Pseudomonas aeruginosa in cystic fibrosis patients. Thus, the spontaneous production of pyridine, which is a toxic product, raises some concern. Our aim was to examine the kinetics of degradation of ceftazidime in portable infusion pumps either at 4°C, 22°C, or 33°C and to propose some recommendations in order to reduce the pyridine exposure.

Liver disease in adult patients with cystic fibrosis: a frequent and independent prognostic factor associated with death or lung transplantation.

Background & Aims: Increased life expectancy in patients with cystic fibrosis (CF) allows better knowledge of non-pulmonary complications like liver disease (CFLD). However, few data have been published in large adult cohorts. The aim of this study was to estimate the prevalence and the prognosis of CFLD in adult CF patients.

[Management of acute and severe complications in adults with cystic fibrosis].

The natural history of cystic fibrosis (CF) may be associated both with acute respiratory complications (respiratory exacerbations, haemoptysis, pneumothorax) and with non-respiratory complications (distal intestinal obstruction syndrome, dehydration) that may result in hospitalizations. The aim of this article is to describe the main therapeutic approaches that are adopted in the management of acute complications occurring in CF adults, and to discuss indications for admission of these patients to intensive care units. Adult CF patients admitted to intensive care unit often benefit from antibiotic courses adapted to their chronic bronchial infection, especially when the hospitalization is related to respiratory disease (including haemoptysis and pneumothorax).

Mediastinal tuberculosis in an adult patient with cystic fibrosis.

Tuberculosis (TB) is rarely observed in cystic fibrosis (CF) patients. We report the first case of mediastinal TB, associated with leg pain and skin rash, in an adult patient with CF, and discuss factors suggestive of TB in the course of CF.

[The diagnosis of cystic fibrosis in adults: lessons from a family story].

Introduction: Cystic fibrosis is usually diagnosed during the first years of life. Diagnosis may be achieved in adults with milder forms of the disease at any age.

Case Reports: We report the diagnosis of cystic fibrosis in three adults within the same family.

Clinical changes of patients with cystic fibrosis during transition from pediatric to adult care.

Purpose: To evaluate the clinical changes of adults with cystic fibrosis (CF) during transition from a pediatric to adult CF center.

Methods: Data were collected at the time of transfer, 1 year earlier and 1 year later, for all patients in our adult CF center arriving from one of the three pediatric CF centers in Paris between January 2001 and June 2004.

Results: Sixty-three of the 68 patients (transferred at a median age of 21.

[Severe dehydration and August 2003 heat wave in a cohort of adults with cystic fibrosis].

Objective: To study dehydration related to the August 2003 heat wave in France in a cohort of adults with cystic fibrosis. Method Retrospective study of the telephone calls received from and hospital admissions of all adult cystic fibrosis patients (n=245) regularly followed in our specialized clinic.

Results: Six patients developed extracellular dehydration with functional kidney failure concomitant to intracellular dehydration with hypokalemia and hypochloremia.

Diagnosis of cystic fibrosis in adults with diffuse bronchiectasis.

We assessed the contribution of the sweat test, genotyping and nasal potential difference (NPD) in the diagnosis of cystic fibrosis (CF) in adults with diffuse bronchiectasis (DB). Among 601 adults referred for DB from 1992 to 2001, 46 were diagnosed with CF. The sweat test was positive in 37 patients and normal or intermediate in nine patients.

Malnutrition in adults with cystic fibrosis.

Objective: To determine the prevalence and clinical features of malnutrition and its relationship with the CFTR genotype in a cystic fibrosis (CF) adult population.

Design: Cross-sectional study.

Setting: Department of Pulmonology, Cochin Hospital, Paris, France.

Distal intestinal obstruction syndrome in adults with cystic fibrosis.

Background & Aims: With the improved survival of patients with cystic fibrosis (CF), gastrointestinal complications become more evident in adults with this condition. The aims of this study were to determine the prevalence and clinical features of distal intestinal obstruction syndrome (DIOS) and its relationship with the cystic fibrosis transmembrane conductance regulator (CFTR) genotype in an adult CF population.

Methods: Cross-sectional study was conducted in an adult CF cohort.

[First consultation for asthma].

The first medical visit is an essential moment of asthma management because it is a chronic disease needing a long term follow-up. This initial assessment which takes time, can be done gradually, on several days or even weeks. Once the differential diagnosis is excluded, the diagnosis of asthma is confirmed and its severity assessed.

[Characteristics and specificities of cystic fibrosis in adults: evolutive disease of childhood or recently diagnosed disease?].

We have studied the characteristics of 202 cystic fibrosis adult patients, all with chronic respiratory symptoms, with a median age of 27 yrs (18 to 55 yrs) and a male predominance (56%). At genetic analysis, delta F508 homozygotes were 41%, delta F508 heterozygotes 42% and 17% had no delta F508. The respiratory disease was more severe and complications were more frequent in adults: hemoptysis in 14%, pneumothorax in 15%, lung transplantation in 25 patients.

Increased frequency of cystic fibrosis deltaF508 mutation in bronchiectasis associated with rheumatoid arthritis.

This study investigated the clinical characteristics and the possible involvement of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in patients with symptomatic diffuse bronchiectasis (DB) associated with rheumatoid arthritis (RA). Twenty-six patients with both RA and DB (group RA+DB) and control groups of 29 consecutive patients with RA but no bronchiectasis (group RA) and 29 patients with symptomatic DB of unknown origin (group DB) were prospectively studied. Among the patients of the RA+DB group, four (15.

Genotype-phenotype relationships in a cohort of adult cystic fibrosis patients.

In cystic fibrosis (CF), relationships between genotype and phenotype have been shown for pancreatic status but not for pulmonary disease. One hundred and ten adult CF patients were classified according to the expected effect of their mutations on cystic fibrosis transmembrane conductance regulator (CFTR) protein: Group 1 (n=48) included deltaF508 homozygotes; Group 2 (n=26), patients with two "severe" mutations and no expected CFTR production; Group 3 (n=17), patients with expected partly functional CFTR corresponding to at least one "mild" mutation; Group 4 (n=19), patients with no mutation identified or only one identified "severe" mutation. As compared to Groups 1 and 2: patients from Groups 3 and 4 had higher arterial oxygen tension (Pa,O2) (9.

Evidence of a strong, positive association between atopy and the HLA class II alleles DR4 and DR7.

Background: Atopy, with or without associated asthma, provides a useful model for evaluating the genetic factors that control human immune responsiveness. HLA class II gene products are involved in the control of immune responses.

Objectives: We investigated whether susceptibility or resistance to the disease was associated with HLA class II genes.

Airway responsiveness to bradykinin is related to eosinophilic inflammation in asthma.

We investigated the relationship between airway inflammation and airway responsiveness, as assessed by PD15, to methacholine and to bradykinin in asthmatic patients. Bronchoalveolar lavage (BAL), bronchial biopsies, and methacholine and bradykinin challenges were performed in 18 nonsmoking subjects with mild or moderate perennial asthma. Bradykinin PD15 correlated negatively with eosinophil count in BAL (p < 0.

Nedocromil sodium reduces cigarette smoke-induced bronchoconstrictor hyperresponsiveness to substance P in the guinea-pig.

Acute exposure to cigarette smoke provokes airway hyperresponsiveness to substance P and inactivates neutral endopeptidase (NEP). To determine whether nedocromil sodium can prevent cigarette smoke-induced hyperresponsiveness to substance P, we studied two groups of anaesthetized guinea-pigs. One group of guinea-pigs was pretreated with aerosolized 0.

[Late recurrences of spontaneously resolved mediastinal-pulmonary sarcoidosis].

Five cases of recurrent thoracic sarcoidosis which happened 5.5 years on the average after spontaneous resolution of the disease are reported. Initially, these patients were characterized by the frequency of the presence of a Löfgren's syndrome and the absence of extrathoracic sarcoid localizations.