Delayed hepatic necrosis in a cystic fibrosis patient taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio).

The introduction of Cystic Fibrosis Trans Regulatory modulator (CFTRm) drugs has seen a transformation in Cystic Fibrosis (CF) treatment. This has led to a significant improvement in lung function and quality of life with the potential for a real impact on life expectancy. Transient mild to moderate hepatic transaminitis is a well-known side effect of CFTRm drugs, which often improves on cessation and may not recur following the re-institution of the drug.

ERS/TSANZ Task Force Statement on the management of reproduction and pregnancy in women with airways diseases.

This European Respiratory Society/Thoracic Society of Australia and New Zealand statement outlines a review of the literature and expert opinion concerning the management of reproduction and pregnancy in women with airways diseases: asthma, cystic fibrosis (CF) and non-CF bronchiectasis. Many women with these diseases are now living into reproductive age, with some developing moderate-to-severe impairment of lung function in early adulthood. The statement covers aspects of fertility, management during pregnancy, effects of drugs, issues during delivery and the post-partum period, and patients' views about family planning, pregnancy and parenthood.

Intravenous antibiotic use and exacerbation events in an adult cystic fibrosis centre: A prospective observational study.

Introduction: In CF, people with higher FEV are less aggressively treated with intravenous (IV) antibiotics, with resultant negative impact on their health outcomes. This could be entirely clinician-driven, but patient choice may also influence IV use. In this prospective observational study, we explored IV recommendations by clinicians and IV acceptance by adults with CF to understand how clinical presentations consistent with exacerbations resulted in IV use.

Understanding Pseudomonas status among adults with cystic fibrosis: a real-world comparison of the Leeds criteria against clinicians' decision.

Pseudomonas aeruginosa status influences cystic fibrosis (CF) clinical management but no 'gold standard' definition exists. The Leeds criteria are commonly used but may lack sensitivity for chronic P. aeruginosa.

Mesalazine-induced lung fibrosis.

The medical management of a patient with Crohn's disease should take into account the activity, site and behaviour of disease, and should be discussed with the patient, and 5-aminosalicylates are a group of medications which have been commonly used. Sulfasalazine is a combination of 5-aminosalicylic acid and sulfapyridine which acts only as a carrier to the colonic site of action but can still cause systemic side-effects including lung disease. In mesalazine the specific sulfapyridine-related side-effects, especially pulmonary reactions, are avoided.

Lung deposition of inhaled tobramycin with eFlow rapid/LC Plus jet nebuliser in healthy and cystic fibrosis subjects.

Background: Reducing nebulisation times for tobramycin solution for inhalation in cystic fibrosis (CF) may improve compliance.

Methods: In this single-dose, open-label, two-way crossover study, 13 subjects (7 CF, 6 healthy) were randomised to receive tobramycin via eFlow rapid or LC Plus jet nebuliser. Drug deposition in the lung using gamma scintigraphic imaging, nebulisation times, pharmacokinetics, and safety were evaluated.

Decision making about reproduction and pregnancy by women with cystic fibrosis.

With significant improvements in longevity, women with cystic fibrosis are increasingly likely to consider pregnancy and parenthood. This article reviews the literature relating to medical and psychosocial research that informs the decision-making process these women undertake.

Guidelines for the management of pregnancy in women with cystic fibrosis.

Women with cystic fibrosis (CF) now regularly survive into their reproductive years in good health and wish to have a baby. Many pregnancies have been reported in the literature and it is clear that whilst the outcome for the baby is generally good and some mothers do very well, others find either their CF complicates the pregnancy or is adversely affected by the pregnancy. For some, pregnancy may only become possible after transplantation.

Pregnancy and chronic progressive pulmonary disease.

Progressive pulmonary disease may preclude the option of pregnancy for a number of women in their child-bearing years due to the severity of the disease. For a subset of women with chronic lung disease including cystic fibrosis, pregnancy is possible, but can have a devastating effect both on the prospective mother and fetus. The potential hazards of pregnancy in cystic fibrosis or other progressive pulmonary diseases may trigger a moral conflict between physician and patient.

Genotyping of Pseudomonas aeruginosa in cystic fibrosis suggests need for segregation.

Background: Emerging resistance of Pseudomonas aeruginosa within cystic fibrosis (CF) populations is attributed to antibiotic pressure and spread of transmissible strains. We describe increasing resistance of P. aeruginosa isolates, resulting in the identification of two multiresistant strains and their impact on morbidity.

Cystic fibrosis-related diabetes.

Diabetes mellitus (DM) has been recognized as a complication of cystic fibrosis (CF) for almost 50 years and commonly develops around 20 years of age. The prevalence increases with age and, with improved survival of those with CF, approaches 30% in certain centres. Its development appears to have a significant impact on pulmonary function and may increase mortality by up to six-fold.

Management of respiratory failure with ventilation via intranasal stents in cystic fibrosis.

The case history is presented of a patient with acute respiratory failure complicated by nasal obstruction resulting in intolerance of nasal ventilation. Urgent insertion of nasal stents permitted restoration of ventilation with resolution of breathlessness and stabilisation of arterial blood gases.

The outcome of 72 pregnancies in 55 women with cystic fibrosis in the United Kingdom 1977-1996.

Objective: To identify pregnancies in women with cystic fibrosis and describe obstetric, infant and maternal medical outcomes in relation to the severity of maternal disease.

Design: Retrospective study, based on casenotes.

Setting: Eleven cystic fibrosis centres in the United Kingdom.

Long-term nasal intermittent positive pressure ventilation in patients with cystic fibrosis and hypercapnic respiratory failure (1991-1996).

In patients with cystic fibrosis (CF), nasal intermittent positive pressure ventilation (NIPPV) is currently used as a short-term bridge to transplantation but its precise role has yet to be determined. Patients were offered a therapeutic trial of NIPPV when candidates for lung transplantation, with respiratory failure unresponsive to medical treatment. Twelve patients, six male of mean age of 26 +/- 1.