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Abstract: Non-immediate hypersensitivity reactions to antibiotics in patients with CF represent a real-life challenge for clinicians. Desensitization is often performed in patients who have exhausted all therapeutic options. Whilst desensitization is an established procedure for immediate reactions we assessed the outcomes and safety of desensitization for non-immediate reactions. We retrospectively reviewed 275 desensitization procedures in 42 patients with a range of non-immediate reactions to six commonly used antibiotics. Desensitization was performed using a 7-step rapid intravenous protocol on a normal medical ward. 250 (91%) of overall desensitization procedures were successful; however, this figure incorporates certain individuals having multiple successful procedures. Individual patient success ranged from 55% with tazocin through to 88% with tobramycin. In the 25 patients who failed desensitization the reactions were mild and the majority occurred within 48h of starting treatment. Prophylactic anti-histamines and steroids did not reduce the risk of reaction. Whilst the mechanisms remain uncertain we can confirm that rapid desensitization is a safe and effective way of re-introducing an antibiotic to a patient with a non-immediate reaction. [Copyright &y& Elsevier]

Objectives Hypersensitivity reactions to beta-lactam antibiotics remain a major clinical problem in the management of patients with CF. Whilst reactions are more likely to occur in sicker patients receiving more frequent treatment this is not always the case and genetic predisposition may play a role. Methods Patients were recruited from the Leeds Adult CF Centre. High resolution sequence based typing was performed on HLA A, B, C, DRB1, DQA1, and DQB1. The data was compared to our electronic patient records. Results 263 patients took part in the study. 163 had a history of beta-lactam reactions and 100 were tolerant controls. 97 patients had a history of piperacillin hypersensitivity. No statistical differences in frequencies of single HLA alleles were seen between hypersensitive patients and controls. One haplotype (A*0201:B*4402:DRB1*0401) occurred at a higher frequency in patients with piperacillin reactions (OR 15.3; p = 0.0006). Conclusion The high frequency of beta-lactam reactions in patients with CF makes it unlikely that a single HLA allele could account for increased predisposition alone. This study shows interesting data supporting an increased genetic risk in certain individuals and more work is needed to refine this further. [ABSTRACT FROM AUTHOR]

Abstract: Introduction: Extra-pulmonary complications of Burkholderia cepacia complex (Bcc) infection in patients with cystic fibrosis are unusual. To the best of the authors'' knowledge no case of pyomyositis secondary to Bcc infection has been reported previously. Case presentation: We report a case of pyomyositis of the forearm caused by Bcc infection in a patient with CF. We also briefly discuss the management of pyomyositis. Conclusion: Pyomyositis is a potential extra-pulmonary complication of Bcc infection in patients with CF. A high index of clinical suspicion is required to make a prompt diagnosis. Final diagnosis may need MRI. An early diagnosis, aggressive medical therapy, multidisciplinary care and timely surgical intervention are all essential for proper management of this condition. [Copyright &y& Elsevier]

Objective Procalcitonin (PCT), a precursor of calcitonin is selectively raised in bacterial infections. The aim of this pilot study was to investigate the role of PCT as a surrogate marker of infection in adults with CF and to discriminate between infection and inflammation in patients with elevated C reactive protein (CRP). Methods Adults with CF receiving hospital intravenous antibiotics for a pulmonary exacerbation were recruited. PCT levels were prospectively measured in patients with elevated C reactive protein (CRP). Results To date 11 subjects, median (range) age, 32 (21–51) yrs have been investigated. CRP and Procalcitonin levels were 185 (69–283) mg/L and 0.3 (<0.01–49.8) ug/L respectively. In five cases, PCT levels were ≤0.2 ug/L despite significant elevation in CRP. In two cases PCT levels were ≤0.1 ug/L and a clinical diagnosis of viral illness or a Tazocin reaction were suspected. In both cases full resolution occurred after discontinuing antibiotics. The highest PCT levels occurred in a patient with a CRP of 381 mg/L and following severe Influenza A H3 (seasonal) infection. Conclusion PCT is a more specific marker of severe infection than conventional markers such as CRP. Preliminary data suggests that PCT may be useful in discriminating between severe pulmonary and endobronchial infection as well as noninfectious inflammation. This study is ongoing. [ABSTRACT FROM AUTHOR]

Objectives Metabolic alkalosis is a recognised complication of CF. The condition appears to occur during both clinical stability and during pulmonary exacerbations. The aim of this study was to explore the relationship between metabolic alkalosis, clinical status and blood biochemistry. Methods A retrospective review of electronic patient records (2007–2013) was undertaken for all patients attending the Leeds Adult CF Unit who had a blood gas analysed. Demographic data, diabetic status, pancreatic status, CF liver disease, sputum microbiology and blood electrolytes were extracted for the most recent blood gas for each individual. Results A total of 568 samples were analysed from 124 subjects. Median age was 30 years (range 17–69). pH was normally distributed but the curve was shifted to the right with a median pH of 7.44 (range 7.1–7.66). Full data was available for 120 subjects. Reduced FEV1% (p ≤ 0.0001), elevated MRC dyspnoea score (p ≤ 0.0001), low BMI (p = 0.02), chronic Pseudomonas status (p<0.05) and use of intravenous antibiotics (p ≤ 0.01) were associated with higher blood gas bicarbonate levels. Although there was no association between bicarbonate and potassium, magnesium or sodium plasma levels, there was an inverse correlation with plasma chloride (r = –0.557, p<0.0001). Conclusion Metabolic alkalosis is a common complication of CF and is associated with worse clinical status, antibiotic therapy and reduced plasma chloride. The aetiology remains unclear and cannot be explained by simple electrolyte depletion. [ABSTRACT FROM AUTHOR]

Background Electronic care records (ECRs) for cystic fibrosis (CF) provide a basis for accurate, reliable capture of clinical measures and interventions, and epidemiological trends, providing the basis for improved efficiency and patient safety. Methods A primary care system was modified for hospital use and clinical codes devised for all aspects of CF care. Performance and usability were assessed. Results Of a total of 620 patients 619 consented to their data being recorded in the system. Five hundred and twenty three new codes were created and embedded behind 60 new templates. Following introduction of ECR, completion of annual assessments increased from 43% to 92%, retrieval of drug costs rose significantly and time to correspondence with primary care fell from 34 days to < 2 days. Staff satisfaction was high. Conclusion The system is fully operational allowing the unit to function as a paperless service. Efficiencies of staffing activity, process management and cost retrievals are evident. Sharing of coding structures is important in future care. [ABSTRACT FROM AUTHOR]

Abstract: Background: Respiratory viruses have become increasingly recognised as important agents in pulmonary exacerbations in infants and children with CF. The aim of this study was to determine the prevalence of respiratory viruses during acute pulmonary exacerbations in adults and compare the severity of these exacerbations with non-viral associated exacerbations. Methods: This was a retrospective case control study. Viral throat swabs were taken from all patients presenting with an acute pulmonary exacerbation requiring intravenous antibiotic treatment over a 12month period. Results: There were 432 pulmonary exacerbations in 180 adults. A positive viral PCR in 42 exacerbations indicated a prevalence of 9.7%. The commonest virus isolated was rhinovirus (n =29, 69%) with influenza A/H1N1 in seven patients (16.7%). Exacerbations associated with a positive viral PCR had a greater fall in lung function at presentation with higher levels of inflammatory markers. They received more days of intravenous antibiotics, showed less response to treatment and had a shorter time to next pulmonary exacerbation compared to matched controls. Conclusion: Viral associated pulmonary exacerbations in adults with CF are associated with more severe pulmonary involvement and respond less well to standard treatment. [Copyright &y& Elsevier]

Abstract: There is a trend today to develop zirconia toughened alumina (ZTA) composites for orthopaedic applications. So far, there is limited data concerning their sensitivity to ageing, especially considering tests performed on implants produced on an industrial scale. Here, complementary tools were used to assess the ageing resistance of ZTA femoral heads. The results were compared to femoral heads processed under the same industrial process with monolithic 3Y-TZP. As expected, monolithic 3Y-TZP implants exhibited significant ageing. In contrast ZTA femoral head showed no sign of degradation even over a period equivalent to that of a human life. The potential impact of coupling effects between wear and ageing is assessed. Monolithic zirconia and ZTA femoral heads were thus first worn in a hip joint simulator, and then aged in autoclave. The kinetics of transformation of the worn monolithic zirconia implants is accelerated while that of worn ZTA femoral heads remains almost unchanged. [Copyright &y& Elsevier]

Objectives Cystic fibrosis arthropathy (CFA) is a term commonly used for joint pain with and without swelling seen in some patients with CF. Early studies into CFA focused on the presence of rheumatoid factor and immunological changes on synovial biopsy, with parallels drawn between respiratory and joint activity. Identification of anti-cyclic citrullinated peptide antibodies (anti-CCP) as a marker of rheumatoid arthritis (RA), along with increased access to sensitive imaging techniques including ultrasound (US) and magnetic resonance imaging (MRI), offer great potential to investigate and more accurately understand the type(s) of inflammatory arthritis that may underlie CFA. The aim of this study was to phenotype an active CFA cohort using serology and imaging, as a basis for further work in this understudied area. Methods This was a prospective observational cohort study of symptomatic CFA patients presenting with joint pain. Participants underwent serological testing, clinical and US joint and entheseal assessment, as well as MRI of the most symptomatic joint/joint area. Results Ten symptomatic patients were studied with 9/10 having positive clinical findings. Inflammatory changes on US were seen in 8/10 cases. Five patients had positive findings on MRI (3 of whom had received IV gadolinium contrast). This included patients with significant erosive changes. One patient was anti-CCP positive suggestive of RA, and two were anti-nuclear antibody positive. Conclusion Imaging, and to a lesser extent serology, identified inflammatory joint pathology in a proportion of cases, providing important data to explore in a large CFA cohort examining the clinical and imaging phenotype of this group. [ABSTRACT FROM AUTHOR]

Objective Cystic Fibrosis Arthropathy (CFA) is a term commonly used for joint pain +/– swelling seen in some patients. Early studies into CFA focused on presence of rheumatoid factor and immunological changes on synovial biopsy, with parallels drawn between respiratory and joint activity. Identification of anticyclic citrullinated peptide antibodies (anti-CCP) as a marker of rheumatoid arthritis (RA), along with increased access to sensitive imaging techniques including ultrasound (US) and magnetic resonance imaging (MRI), offer great potential to more accurately understand the type(s) of inflammatory arthritis that may underlie CFA. The aim of this pilot study was to phenotype an active CFA cohort using serology and imaging, as a basis for further work in this understudied area. Methods This was a prospective observational cohort study of symptomatic CFA patients presenting with joint pain +/– joint swelling. Participants underwent serological testing, clinical and US joint and entheseal assessment, and MRI of the most symptomatic joint. Results Ten symptomatic patients were studied with 9/10 having positive clinical findings. Five patients had positive findings on MRI (3 of whom had received IV gadolinium contrast). This included patients with significant erosive changes. Inflammatory changes on US were seen in 8/10 cases. One patient was CCP positive suggestive of RA, and two were anti-nuclear antibody positive. Conclusion In CFA, imaging, and to a lesser extent serology, identified inflammatory joint pathology in a proportion of cases, providing important pilot data to explore in a large CFA cohort examining the clinical and imaging phenotype of this group. [ABSTRACT FROM AUTHOR]