D. Nazareth

A review of renal disease in cystic fibrosis

Kidney disease is becoming increasingly common in CF. This review looks at the effect of CFTR on the kidney, the problems with measuring renal function effectively in CF, the causes and incidence of renal dysfunction, and its pathophysiology. Strategies to reduce aminoglycoside toxicity are discussed.

Coming of age in cystic fibrosis - transition from paediatric to adult care

Cystic fibrosis (CF) is the most common multi-system inherited disorder, with a UK population exceeding 9,000. There have been significant improvements in CF survival over the decades, attributed to improvements in therapies available, our understanding of the disease and better organisation of care. CF care providers have been early advocates for successful healthcare transition from the paediatric to adult sector and CF can be considered a model process where a paediatric disease has now become an adult one. This article looks at the transition process in CF and the future challenges CF physicians will face.

Cystic fibrosis related diabetes is not independently associated with increased Stenotrophomonas maltophilia infection: Longitudinal data from the UK CF Registry

INTRODUCTION Stenotrophomonas maltophilia is common in the sputum of people with cystic fibrosis related diabetes (CFRD), raising the question as to whether this is a risk factor for its acquisition. We investigated this at a population level. METHODS We analysed national Cystic Fibrosis Registry data 2011-2015 for 8047 people with CF > age 6 years, looking at demographics, diagnosis of CFRD, lung function and sputum microbiology; using descriptive and multivariate strategies to establish independent predictors for S. maltophilia culture and associated outcomes. RESULTS S. maltophilia was present in 1148 (14.1%). Although univariate analysis confirmed it was more prevalent in those with CFRD, when adjusted for other clinical parameters there was no longer a relationship. Markers of more severe lung disease were independent risk-factors for S. maltophilia. CONCLUSION Although S. maltophilia is more common in people with CFRD, it is not an independent risk-factor for S. maltophilia acquisition.

Continuous glucose monitoring guided insulin therapy is associated with improved clinical outcomes in cystic fibrosis-related diabetes

INTRODUCTION Continuous glucose monitoring (CGM) allows assessment of day to day glycaemic excursions and detects early glucose handling abnormalities that may not be apparent on oral glucose tolerance testing (OGTT). However, there is little published evidence as to whether these early dysglycaemic changes are amenable to treatment. We present outcomes following CGM guided insulin initiation at our centre. METHODS Adults without a prior diagnosis of cystic fibrosis related diabetes (CFRD) whom underwent >72 h CGM at our adult CF centre were included in the study. Clinical outcomes including weight and pulmonary function changes over the next 12 months were compared between groups based on CGM results and subsequent management. RESULTS CGM profiles for 59 patients were analysed. Insulin was commenced in 37 patients who had evidence of hyperglycaemia on CGM. Significant improvements in mean [95% confidence intervals] forced expiratory volume in 1 s (FEV1) (+4.3% predicted [1.06-7.48], p = 0.01) and weight (+1.2 kg [0.32-2.15], p = 0.01) were observed at 3 months in the insulin group. Annual rate of pulmonary function decline was also improved following insulin initiation. CONCLUSION Insulin treatment targeted towards glycaemic excursions seen on CGM is associated with improvements in lung function and weight with subsequent reduced pulmonary function decline.

Case Report: First report of Elizabethkingia miricola infection in a patient with cystic fibrosis

Elizabethkingia miricola is a rare non-fermenting Gram-negative rod that has previously been reported to be associated with blood stream and pulmonary abscess infections, but never before in cystic fibrosis (CF). Here we present the first reported case of Elizabethkingia miricola infection in a patient with CF and discuss the management options. We describe a patient with CF in whom we observed clinical and spirometric evidence of pulmonary exacerbation with the associated growth of E. miricola in sputum culture. The period of clinical instability was observed to coincide with the obtainment of four sputum samples from which E. miricola was cultured; improvement was seen following treatment with ciprofloxacin and the subsequent eradication of E. miricola. We conclude that E. miricola is able to survive in the CF lung and in this case was associated with pulmonary exacerbation. Empirical treatment with fluoroquinolones is appropriate, based on our experience.

Case Report: Haemolytic anaemia with ceftazidime use in a patient with cystic fibrosis

Drug-induced Immune Haemolytic Anaemia (DIIHA) is a rare but serious complication of cephalosporin use. Ceftazidime is recognized to be a rare cause of DIIHA. We report and discuss a case of DIIHA in a person with cystic fibrosis who developed severe haemolytic anaemia following use of ceftazidime in the management of an acute pseudomonal pulmonary exacerbation.

Pulmonary function testing is safe in patients with thoracic aortic aneurysms

Thoracic aortic aneurysms (TAA) occur in up to 16 patients per 100 000 of the population and are increasingly amenable to surgical correction [1]. However, low lung function is associated with poor post-operative outcomes, and a pre-operative assessment of pulmonary function is therefore important for prognostication [2]. Spirometry is safe as part of pre-operative work-up in patients with thoracic aortic aneurysms http://ow.ly/uwSe30lJ6Qn

Between a Rock and an Airspace: Pneumothorax After Extracorporeal Shock Wave Lithotripsy for Renal Stones in a Patient With Cystic Fibrosis

Renal disease is a well-recognized manifestation of cystic fibrosis (CF) and people with CF are at increased risk of nephrolithiasis. Lithotripsy is the preferred treatment but has occasionally been associated with pulmonary complications. Here we report the case of a person with CF who developed a pneumothorax soon after lithotripsy and discuss the potential mechanism of injury. We hope this case highlights some of the additional considerations clinicians should take into account when managing patients with advanced pulmonary disease in CF.

183 Intermittent exogenous insulin may prevent progression of dysglycemia in pre-diabetic adults

Objectives In prediabetes, pancreatic beta cell function may be preserved by intermittent exogenous insulin support, potentially delaying the onset of diabetes. We assessed whether this could be employed in CF, where the development CFRD greatly increases the treatment burden, morbidity and ultimately overall mortality. Methods We studied 5 male prediabetic (defined on the basis of an abnormal continuous glucose monitoring (CGM) result and normal 2-hour oral glucose tolerance test (OGTT)) CF patients who were in an otherwise clinically stable state. Their clinical and glycaemic parameters (based on CGM) were measured for 3 months after 14 days of Detemir treatment. Results CGM devices were well tolerated and only one data collection point was incomplete (day 18 CGM result). Glucose profiles improved in the first few weeks after treatment, and dysglycaemia had resolved entirely in 2 patients by day 64. There was no significant change in FEV1 or HbA1c. ScreeningDay 18Day 34Day 64Day 94Mean % time glucose profile raised17%12%7%12%9%Mean FEV1%76%75%77%76%73% Conclusion This study suggests that pre-diabetic adult CF patients treated with a short (2-week) course of insulin may experience extended periods of improved glucose control. These effects seem most pronounced for the immediate few weeks after treatment is received. This raises the prospect that intermittent insulin regimes may delay the progression of dysglycaemia in this increasingly common complication of the CF condition.

Abdominal trauma and lung nodules

Splenosis represents the deposition of viable splenic tissue in a different anatomic compartment, usually following trauma. The extent of deposition is related to the severity of trauma and amount of pulp tissue released into the abdominal and pelvic cavities. Thoracic splenosis, is the deposition of splenic tissue within the thoracic cavity and occurs in about 18% of cases.1 This splenic tissue is thought to perform normal splenic functions and draws its blood supply from surrounding tissue. The condition is usually discovered …