Aim In 2019 new cystic fibrosis transmembrane conductance regulator (CFTR) modulators were commissioned by NHS England for a large proportion of people with Cystic Fibrosis (CF) and the paediatric pharmacy team adopted mechanisms to support their initiation integrating into CF multidisciplinary (MDT) clinic. Although driver change at time was this roll-out, NHSE specialised commissioning servic...

Allergic bronchopulmonary aspergillosis (ABPA) is a complex hypersensitivity reaction in patients with asthma or cystic fibrosis (CF), which is associated with bronchi colonized by the fungus Aspergillus species, most often Aspergillus fumigatus. ABPA is an important consideration for asthmatics that do not respond to asthma management or with recurrent chest infections and deteriorating lung f...

References 1 Speich R, van der Bij W. Epidemiology and management of infections after lung transplantation. Clin Infect Dis 2001; 33: Suppl. 1, S58–S65. 2 Gottlieb J, Mattner F, Weissbrodt H, et al. Impact of graft colonization with gram-negative bacteria after lung transplantation on the development of bronchiolitis obliterans syndrome in recipients with cystic fibrosis. Respir Med 2009; 103: ...

BACKGROUND Cystic fibrosis is a multisystem disease where the main problems are existing in the respiratory system. Aerobic exercise programs are effective in increasing physical fitness and muscle endurance in addition to chest physiotherapy. OBJECTIVE The aim of this study was to evaluate the effects of chest physiotherapy and aerobic exercise training on physical fitness in young children ...

BACKGROUND Quality of life is recognized as an important additional outcome measure in clinical trials and health economic evaluations. The EQ-5D is an important generic health outcome instrument often used for economic evaluations as a complement with disease-specific outcome measures. In this study quality of life data was assessed using the EQ-5D-Y (new EQ-5D version for children and adolesc...

INTRODUCTION Pancreatitis is a rare manifestation of cystic fibrosis (CF) and may rarely be the presenting symptom in adolescent or adult patients with CF. We report a case of a 4 year-old female who initially presented with recurrent pancreatitis, a normal sweat test, and a diagnosis of pancreas divisum. She was subsequently diagnosed with cystic fibrosis at the age of 6 years, despite normal ...

1 De Boeck K, Kent L, Davies J, et al. CFTR biomarkers: time for promotion to surrogate end-point? Eur Respir J 2013; 41: 203–216. 2 Durmowicz AG, Witzmann KA, Rosebraugh CJ, et al. Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience. Chest 2013; 143: 14–18. 3 Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with ...

The availability of non-invasive oxygen saturation (SaO2) measurement could prove to be a useful tool for following up the progress of patients with cystic fibrosis. The present study was undertaken to compare its use with other routine measurements in the clinic. A total of 100 patients with cystic fibrosis were compared with 50 patients with stable asthma. The children were aged between 5 and...

Early insulin response to rapid intravenous injection of glucose was studied in 7 cases of cystic fibrosis aged 8 months to 9 1/2 years. Plasma insulin was measured with a radioimmunological method. Blood glucose values were determined and the glucose disappearance rate (kG) calculated. In all children except the youngest one the early insulin response values were low compared with normal child...

Abstract Background Cystic fibrosis (CF) is a genetic disorder, which caused by the CFTR protein defects. Along with dysfunction, inflammation plays key role in disease outcomes. Inflammation may develop due to internal dysfunction of or external factors. Curcumin affects function primarily as corrector and potentiator secondary an anti-inflammatory antimicrobial agent. The present study aims a...