Use of extracorporeal membrane oxygenation in cystic fibrosis in an Australian cystic fibrosis centre.

Extracorporeal membrane oxygenation (ECMO) support is used in selected patients with cystic fibrosis (CF) as a bridge to transplantation. Our aim was to describe briefly treatment and outcomes of six CF patients who received ECMO. One patient received a lung transplant and another recovered from acute respiratory failure.

Hypoglycaemia in cystic fibrosis: An analysis of a single centre adult cystic fibrosis clinic.

Background: Hypoglycaemia in cystic fibrosis (CF) is known to occur during oral glucose tolerance tests (OGTT) and continuous glucose monitoring, however demographic, clinical and mechanistic data are limited. The aims of this study were to review patient electronic medical records (EMR) in order to 1) describe patient characteristics of a university teaching hospital CF clinic, 2) determine the prevalence of hypoglycaemia on OGTT and explore associations with demographic and clinical characteristics, and 3) explore patient reported symptoms suggestive of hypoglycaemia documented in the EMR.

Methods: Adults who attended the RPA CF clinic between January 2009 to April 2016 were included in the study.

Pregnancy outcomes in the current era of cystic fibrosis care: a 15-year experience.

Background: With improvement in clinical care and longer survival of patients with cystic fibrosis (CF), pregnancy has become commonplace. However, the impact of pregnancy on maternal health and fetal outcomes requires ongoing review.

Methods: A retrospective study of 20 pregnancies from 18 women with CF during the period 1995-2009 was performed.

Clinical profile of adult cystic fibrosis patients with frequent epidemic clones of Pseudomonas aeruginosa.

Background And Objective: Earlier reports suggested that Pseudomonas aeruginosa frequent epidemic clones circulating in cystic fibrosis (CF) centres had increased virulence. However, recent data show no consistent associations with virulence, and suggest attenuation of virulence in chronic infection. Changes to infection control programmes in relation to frequent epidemic clones should be based on their frequency, virulence across all age groups and mode of acquisition.

Pregnancy and cystic fibrosis.

The management of a pregnancy in a woman with cystic fibrosis is usually achieved with successful outcomes for mother and child with appropriate multidisciplinary care. The process begins prior to conception and requires frequent monitoring of the mother's respiratory status, level of glycaemic control and obstetric wellbeing. Recent reports have suggested that pregnancy can be managed without a persisting decrement in lung function beyond what may be expected in women with cystic fibrosis who are not pregnant.

Contraception, communication and counseling for sexuality and reproductive health in adolescents and young adults with CF.

With survival now into the fourth decade and rapid growth of the adolescent and adult population of people with cystic fibrosis CF sexual and reproductive health issues are integral to the management of adolescents and adults with CF. Education and counseling for sexual health related issues must be included in the daily routine of CF care. With advances in genetic counseling, contraception, assisted reproductive technology and collaborative management adolescents and young adults with CF realizing their sexual and reproductive potentials safely and realistically can be possible .

Recombinant activated factor VII for massive hemoptysis in patients with cystic fibrosis.

Massive hemoptysis is a common complication in patients with cystic fibrosis (CF) and is associated with significant morbidity and mortality. Conventional treatment with antibiotic therapy and early bronchial artery embolization (BAE) is usually successful in achieving hemostasis in the majority of patients. Recombinant activated factor VII (rFVIIa), originally developed for use in patients with hemophilia, has emerged as a general hemostatic agent that is potentially useful in the management of many life-threatening bleeding conditions.

A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis.

Background: Inhaled hypertonic saline acutely increases mucociliary clearance and, in short-term trials, improves lung function in people with cystic fibrosis. We tested the safety and efficacy of inhaled hypertonic saline in a long-term trial.

Methods: In this double-blind, parallel-group trial, 164 patients with stable cystic fibrosis who were at least six years old were randomly assigned to inhale 4 ml of either 7 percent hypertonic saline or 0.

Improved outcomes of patients with cystic fibrosis admitted to the intensive care unit.

Background: Invasive mechanical ventilation (IMV) has been previously associated with a poor outcome for patients with cystic fibrosis (CF), but with improved survival and the availability of lung transplantation intensive care unit (ICU) admission is being increasingly considered. This study aimed to review the outcomes of adult CF patients admitted to ICU, and to identify factors that may have influenced outcomes.

Methods: A retrospective audit was conducted of CF patients admitted to ICU.

Granulomatous diseases in a patient with cystic fibrosis.

We report the case of an adult with Crohn's disease and pulmonary sarcoidosis on the background of cystic fibrosis (CF). There is a recognized association between Crohn's colitis and CF, but cases of pulmonary sarcoidosis in CF are rare. There may be a pathogenic link between the two granulomatous disorders and CF with chronic immune stimulation leading to hyperimmunoglobulinemia, circulating immune complexes and subsequent granuloma formation.

Effect of Burkholderia cepacia infection in the clinical course of patients with cystic fibrosis: a pilot study in a Sydney clinic.

Background: Colonisation with Burkholderia cepacia complex in patients with cystic fibrosis (CF) has been associated with adverse outcomes. The aim of the present study was to determine the actuarial survival of CF patients colonized with B. cepacia and to evaluate the efficacy of the Royal Prince Alfred Hospital segregation policy.