No benefit of longer eradication therapy of Pseudomonas aeruginosa primoinfections in pediatric cystic fibrosis.

Patients with cystic fibrosis are more susceptible than members of the general population to lung infections. Infections with Pseudomonas aeruginosa require particular attention, because they may accelerate the deterioration of lung function if not adequately treated. This study assessed the eradication rate of P. aeruginosa primoinfections, with a protocol of inhaled tobramycin and oral ciprofloxacin over a 3 months’ period. Retrospective single-center study from June 1st, 2007 to December 31st, 2015. Inclusion of 28 pediatric patients (11 females, 17 males), with a total of 49 primoinfections. Overall success rate of 67.3%, which is similar or even inferior to figures published in the literature

Stop using the flotation technique and start weighing salbutamol pressurised metered-dose inhalers without dose counters.

Salbutamol pressurised metered-dose inhalers (pMDIs) are not equipped with dose counters outside the USA. The aim of this study was to describe a simple reproducible method for determining the number of doses remaining in a pMDI based on scale weight. With a laboratory scale, the mean weight of the canisters was 28.61 ± 0.10 g after priming and 14.84 ± 0.23 g after 200 puffs. Similar results were obtained with two common digital scales. We recommend weighing salbutamol canisters on a common digital scale, and replacing an old pMDI with a new one when the weight falls to ≤15 g

Lack of concordance in parapneumonic effusion management in children in central Europe.

Treatment of parapneumonic effusion in children remains controversial in the literature and in clinical practice. The aim of this study was to determine whether mutual consensus exists in the diagnosis and treatment of parapneumonic effusion in Central European countries. A questionnaire was sent to all directors of pediatric respiratory units in four adjacent Central European countries (Austria, France, Germany, Switzerland). The response rate was 61.8%. Responses reflected acceptable agreement regarding initial diagnostic procedures, as most centers performed chest X-ray and biological exams, followed by ultrasound, thoracocentesis, or computed tomography. However, antibiotic regimens were very heterogeneous, and the survey revealed complete lack of agreement on the indications and effusion volume threshold for invasive procedures, such as fibrinolytic instillation and thoracoscopy. In conclusion, apart from initial diagnostic procedures, this study showed a lack of mutual consensus among the four countries regarding the management of pediatric parapneumonic effusion. Multicenter prospective trials are clearly needed to acquire more evidence on the management of childhood parapneumonic effusion, enabling the development of evidence-based algorithms that could help to avoid unnecessary examinations with potential long-term side effects, such as radiation exposure at a young age. Pediatr Pulmonol. 2016;51:411-417. © 2015 Wiley Periodicals, Inc

Allergic bronchopulmonary aspergillosis: the hunt for a diagnostic serological marker in cystic fibrosis patients.

The diagnosis of allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis patients remains challenging, mainly owing to overlapping symptoms of the underlying lung disease with clinical symptoms of ABPA. In addition, a varying mixture of diagnostic criteria, including clinical status, radiological findings and immunological measurements, has led to confusion and differing recommendations. In order to help simplify as well as standardize the diagnostic criteria for ABPA, different serological markers have been evaluated in the last 20 years and their usefulness has been assessed in many clinical studies. This review presents current diagnostic criteria of ABPA, with a special focus on serum markers supporting the diagnosis and explains why the hunt for a serological marker for ABPA is still ongoing

Death after cessation of treatment by cystic fibrosis patients: An international survey of clinicians.

Little is known about cystic fibrosis patients, who are not considered to be terminally ill, and who die after voluntary cessation of treatment. This study was undertaken to provide an international snapshot of this issue. An online survey was distributed across three continents. Distribution to the medical directors of the cystic fibrosis centres affiliated with the US Cystic Fibrosis Foundation, Cystic Fibrosis Australia (inclusion of New Zealand) and to every clinician member of the European Cystic Fibrosis Society. More than 200 cystic fibrosis patients not considered to be terminally ill and, who voluntarily ceased treatment, were reported by the clinicians surveyed. Detailed data were reported in 102 patients (4 children, 25 adolescents and 73 adults). Only one child, six adolescents and one adult were judged by clinicians not to be competent to make the decision to stop treatment. Time-consuming and low immediate-impact therapies, such as respiratory physiotherapy, were most frequently discontinued. Resignation was the main reported reason for discontinuing treatment, followed by reactive depression and lack of familial support. A total of 69% of the patients received palliative care and 72% died in the 6 months following cessation of treatment. Death of cystic fibrosis patients, not considered to be terminally ill, is reported in Europe, the United States and Australia due to voluntary cessation of treatment

Newborn screening for cystic fibrosis - The parent perspective.

Newborn screening for CF started 01/2011 in Switzerland. We investigated the parents' opinions about the information received, their feelings, and overall approval of the screening. This is a prospective questionnaire survey of all parents of positively screened children. Parents were phoned by CF-centres and invited for diagnostic investigations. They completed a questionnaire after the visit to the CF-centre. From 2011-2013, 246 families received the questionnaire and 138 (56%) replied. Of these 77 (60%) found the information received at birth satisfactory; 124 (91%) found the information provided in the CF-centre satisfactory. Most parents (n=98, 78%) felt troubled or anxious when the CF-centre called, 51 (38%) remained anxious after the visit. Most parents (n=122; 88%) were satisfied with the screening, 4 (3%) were not, and 12 (9%) were unsure. The smooth organisation of the screening process, with personal information by a CF specialist and short delays between this information and the final diagnostic testing, might have contributed to reduce anxiety among parents. Most families were grateful that their child had been screened, and are happy with the process