A case of hairy cell leukaemia complicated as a terminal event by massive retroperitoneal lymphadenopathy is described. The patient had recently been treated with lithium carbonate and had previously been demonstrated to suffer from a systemic vasculitis, either or both of which may have contributed to the development of this rare complication.
IntroductionAllergic bronchopulmonary aspergillosis (ABPA) is increasingly prevalent among cystic fibrosis (CF) patients. Atopy, sex, season at diagnosis and cystic fibrosis transmembrane conductance regulator (CFTR) mutation are suggested risk factors for ABPA. Studies indicate that centres using a high amount of intravenous antibiotics have better outcomes, but this has also been linked to the increase in ABPA. The objective of this retrospective case-control study was to assess potential risk factors for ABPA, in particular intravenous antibiotic use.MethodsOf 150 paediatric patients, 13 fulfilled the CF foundation consensus diagnostic criteria for ABPA. These were compared to 130 patients without evidence of ABPA. Seven patients were excluded from the study as they showed evidence of ABPA but did not fulfil the diagnostic criteria. The intravenous antibiotic data for the ABPA group was compared to 80 patients without ABPA using extended matching by age of diagnosis. The data collected included age, sex, CFTR mutation, atopy (history or family history of diagnosed hay fever, eczema or asthma), and weeks of intravenous antibiotics administered. Season of diagnosis was noted for the ABPA group.ResultsThe median duration of use of intravenous antipseudomonal antibiotics in the ABPA group was 38 weeks, compared to 5 weeks in the non-ABPA group (p<0.05). No difference was found in the duration of use of non-antipseudomonal antibiotics (median 12 weeks in ABPA group, 2 weeks in non-ABPA group). The average time since diagnosis of CF was higher in the ABPA group by 3 years (p=0.01). 12/13 (92%) of ABPA patients had a history of atopy compared to 50/105 (47%) of the non-ABPA group (p=0.05). There was no difference in CFTR mutation, sex or season of ABPA diagnosis.ConclusionIntravenous antipseudomonal antibiotic use was substantially higher in the ABPA population. This could be a factor in the increase in ABPA, suggesting that tighter microbiological guidance may be useful before initiating antibiotic treatment. However, this could also be that IV use and ABPA are both markers for increased disease severity. Atopy was also significantly higher in the ABPA population. This could potentially be used to highlight those CF patients at risk from this disease.
had asthma (n = 53) with 37% COPD (n = 31), with a mean age 56.3 and 67.0 years respectively. Table 1 shows a significant proportion of patients had uncontrolled disease based on their quality of life scores (QoL), use of reliever inhalers and frequency of exacerbations. Despite use of high dose medicines, this was likely to be due to poor adherence to maintenance inhalers and poor inhaler technique in a large proportion of patients. Interventions were made in most patients, including reducing the beclomethasone dipropionate (BDP) equivalence in the asthma group by 60.4%. Follow up showed that despite a reduction in inhaler therapy, QoL, peak flow measurements and reliever use all improved. Conclusions Tailored reviews by the pharmacist resulted in significant interventions that improved QoL, adherenc to therapy, reduced unnecessary over prescribing of high dose medicines and resulted in a large proportion of patients to successfully stop smoking. Introduction Lung function tests and BMI are widely-used outcome measures in children with CF, and their preservation requires adherence to exacting treatment regimens. From screening to treatment of children with chronic lung disease P79 THE IMPACT OF SOCIAL DEPRIVATION ON CLINICAL OUTCOMES IN CHILDREN WITH CYSTIC FIBROSIS (CF) INWe hypothesised that deprivation might affect the adherence to therapy of children in a deprived area of Scotland, thus reducing BMI and FEV 1 scores and increasing clinical input required.Schechter (2003) demonstrated a link between deprivation and mortality in children with CF, although the mechanism remained unclear, and the study has not been repeated elsewhere. In an adult CF population in an affluent area of England, Jarad (2005) found no correlation between deprivation and FEV 1 . This question has not previously been examined in a deprived paediatric population. Method In February 2013, RHSC Glasgow had 95 children (4946) with CF under its care. All children over 2 years had their BMI centile calculated. In all children over four years, mean FEV1% predicted was calculated. The number of outpatient clinics and inpatient admissions over a three year period was noted for all children over three years old.The Scottish Index of Multiple Deprivation (SIMD) ranks the 6505 postcode districts in Scotland in order of deprivation, and a rank was obtained for each child' postcode.SMID rank was plotted against BMI, mean FEV1% predicted, outpatient appointments and inpatient admissions, with any correlations noted. No correlation was shown between SMID rank and BMI centile (r 2 = 0.0025), mean FEV1% predicted (r 2 = 0.0025), respiratory clinics attended (r 2 = 0.0121), total outpatients attended (r 2 = 0.0263) and inpatient admissions (r 2 = 0.0078). Conclusion Relative deprivation does not correlate with BMI centile or FEV1% predicted in this population of CF children and is not associated with the level of clinical input required.
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