Sweet syndrome revealing systemic lupus erythematosus.

Sweet Syndrome is an acute inflammatory skin eruption which is rare in children. We report a case of childhood Systemic Lupus Erythematosus (SLE) that presented with Sweet syndrome. This case is a unique presentation of a common disorder which provides a new facet for the differential diagnosis of SLE in children. It is also the first paediatric case to be reported in a Caucasian child.

Management of parenteral nutrition associated hyperglycaemia: a comparison of subcutaneous and intravenous insulin regimen.

PN is associated with significant hyperglycaemia, which may be detrimental to clinical outcome. There are few data on the management of this phenomenon outside of intensive care units. In our unit, we studied the efficacy of protocol-based intravenous insulin delivery as compared to subcutaneous insulin prescribed individually outside of the critical care setting. In a retrospective review over a two-year period, we compared patients with PN-associated hyperglycaemia who had received both modes of insulin therapy. A total of 122 who developed PN-associated hyperglycaemia were identified. Those on the intravenous insulin regimen were within glycaemic target for more time than those on the subcutaneous regimen (62% Vs 43%, p = 0.008). We therefore conclude that outside of the critical care setting, intravenous insulin delivers better glycaemic control and should therefore be considered optimum therapy for patients with PN-associated hyperglycaemia.

Are there clinical features of a sensitized cough reflex?

Cough reflex hypersensitization is a key feature in patients with troublesome cough. The clinical consequence of this hypersensitive state is typified by bouts of coughing often triggered by low threshold stimuli encountered by the patient during normal daily activities including exposure to aerosols, scents and odours, a change in air temperature and when talking or laughing. These features are often perceived by cough patients to be the most disruptive aspect of their condition and undoubtedly contribute to impaired quality of life. Patients with troublesome cough may describe a range of additional symptoms and sensations including an 'urge to cough' or the feeling of an 'itch' at the back of the throat, or a choking sensation and occasionally chest pain or breathlessness. It is uncertain if these features arise due to the processes responsible for cough reflex sensitization or as a direct consequence of the underlying cough aetiology. In an attempt to understand the clinical features of a sensitized cough reflex, the spectrum of symptoms typically described by cough patients will be reviewed and possible underlying mechanisms considered. Since an intact cough reflex is crucial to airway protection, anti-tussive treatment that attenuates the hypersensitive cough state rather than abolishing the cough reflex completely would be preferable. Identifying such agents remains a clinical, scientific and pharmacological challenge.

Pulmonary rehabilitation and follow-on services: a Northern Ireland survey.

There should be a clear pathway through pulmonary rehabilitation and follow-on services. The aim of this survey was to determine the characteristics of the different components of the patient pathway, that is, pulmonary rehabilitation programs, ongoing exercise facilities, and support networks in Northern Ireland. Questionnaires were sent to current providers of pulmonary rehabilitation, providers of ongoing exercise, and support groups in Northern Ireland. Findings relating to the current status of pulmonary rehabilitation in Northern Ireland up to January 2007 are reported. There are currently 23 pulmonary rehabilitation programs in Northern Ireland. There appears to be a pathway through the short-term pulmonary rehabilitation program (6-8 weeks). Programs met standards for structure and format, except for the frequency of supervised exercise. Not all programs have links for the provision of ongoing exercise, but a range of exercise programs are available in leisure centers in Northern Ireland that include people with respiratory disease. There are 13 support groups for patients with respiratory disease in Northern Ireland and their function is diverse. Pulmonary rehabilitation is established in Northern Ireland, although not all patients are able to access these. Facilities for ongoing exercise and support groups are less developed. Improvements could be facilitated by better communication within the patient pathway and a strategic coordinated approach.

Common causes and current guidelines.

Chronic cough is a common and disabling symptom. Recent guidelines have attempted to provide direction in the clinical management of cough in both primary and secondary care. They have also provided a critical review of the available literature and identified gaps in current knowledge. Despite this they have been criticized for a reliance on a low quality evidence base. In this review, we summarize the current consensus on the clinical management of chronic cough and attempt to rationalize this based on recent evidence. We have also provided an overview of the likely pathophysiological mechanisms responsible for cough and highlighted areas, where knowledge deficits exist and suggest directions for future research. Such progress will be critical in the search for new and effective treatments for cough.

The challenge of patients' unmet palliative care needs in the final stages of chronic illness.

BACKGROUND: There is consensus in the literature that the end of life care for patients with chronic illness is suboptimal, but research on the specific needs of this population is limited. AIM: This study aimed to use a mixed methodology and case study approach to explore the palliative care needs of patients with a non-cancer diagnosis from the perspectives of the patient, their significant other and the clinical team responsible for their care. Patients (n = 18) had a diagnosis of either end-stage heart failure, renal failure or respiratory disease. METHODS: The Short Form 36 and Hospital and Anxiety and Depression Questionnaire were completed by all patients. Unstructured interviews were (n = 35) were conducted separately with each patient and then their significant other. These were followed by a focus group discussion (n = 18) with the multiprofessional clinical team. Quantitative data were analysed using simple descriptive statistics and simple descriptive statistics. All qualitative data were taped, transcribed and analysed using Colaizzi's approach to qualitative analysis. FINDINGS: Deteriorating health status was the central theme derived from this analysis. It led to decreased independence, social isolation and family burden. These problems were mitigated by the limited resources at the individual's disposal and the availability of support from hospital and community services. Generally resources and support were perceived as lacking. All participants in this study expressed concerns regarding the patients' future and some patients described feelings of depression or acceptance of the inevitability of imminent death. CONCLUSION: Patients dying from chronic illness in this study had many concerns and unmet clinical needs. Care teams were frustrated by the lack of resources available to them and admitted they were ill-equipped to provide for the individual's holistic needs. Some clinicians described difficulty in talking openly with the patient and family regarding the palliative nature of their treatment. An earlier and more effective implementation of the palliative care approach is necessary if the needs of patients in the final stages of chronic illness are to be adequately addressed.

Do patients prescribed short-burst oxygen therapy meet criteria for ambulatory oxygen?

The aim of this study was to determine whether patients (n = 40) with chronic obstructive pulmonary disease (COPD) currently receiving short-burst oxygen therapy meet existing criteria for ambulatory oxygen. A prospective randomised balanced double-blind crossover design was used. Criterion (i) for ambulatory oxygen (desaturation by 4% to below 90% on the practice walk) was met in 21 patients; 15 of these 21 patients also met criterion (ii) (10% improvement in distance and/or breathlessness score with oxygen). Despite no evidence of desaturation in 19/40 patients, 9/19 improved their distance and/or breathlessness score with oxygen. This study has shown that many patients on short-burst oxygen meet existing criteria for ambulatory oxygen and may have been receiving suboptimal therapy. It supports recent proposals for the development of comprehensive assessment services which incorporate assessment for all categories of oxygen.

Short burst oxygen therapy in chronic obstructive pulmonary disease: a patient survey and cost analysis.

The prescription of home oxygen cylinders is substantial. This study aimed to establish patient's current use of short burst oxygen therapy in chronic obstructive pulmonary disease (COPD) and to examine potential cost savings if cylinder use had been replaced by a concentrator. An interviewer-administered questionnaire was completed by 100 patients currently receiving short burst oxygen therapy. Patients reported that they used their oxygen before exercise/activity (26%), during exercise (19%), after exercise/activity (87%) and at rest (46%) and mostly for the relief of symptomatic breathlessness. The length of time [mean (SD)] patients had oxygen at home was 27.42 (29.31) months. Of those patients using cylinders, savings could have been made by transferring from cylinders to concentrators. While withdrawal of oxygen may be difficult, an oxygen assessment service could ensure that future prescription is aimed at those who benefit and is delivered by the most cost-effective method.

Oesophageal pH monitoring is of limited value in the diagnosis of "reflux-cough".

Reflux-cough is a diagnosis based on demonstrating both gastro-oesophageal reflux and a positive response to anti-reflux therapy. The authors sought to determine early and long-term response to therapy in patients with a "positive" 24 h oesophageal pH study, and identify any features which might predict response. Patients with chronic cough were recruited from July 1998 to July 2002. Those with a positive pH study were given dietary advice and an 8-week trial of omeprazole (20 mg b.i.d.). Response was judged after 8 weeks (clinical follow-up), and at long-term follow-up (telephone questionnaire). A total of 146 patients underwent pH monitoring with 82 (56.2%) "positive" studies. Follow-up data was available in 60 patients, with 49 receiving anti-reflux therapy, of which 20 (40.8%) reported a positive treatment response. At long-term follow-up (median 30 months), there was a significantly lower response (14 out of 49, 28.5%), with no significant difference in either acid exposure times (distal/proximal) or symptom correlation between responders and nonresponders at early or long-term follow-up. In conclusion, despite "positive" pH studies, over half of the patients (55.1%) failed to respond to therapy. No features on pH monitoring accurately predicted response. Short-term response did not predict long-term response. The precise role for pH monitoring in the assessment of chronic cough remains to be defined.

Occupational asthma after exposure to plaster casts containing methylene diphenyl diisocyanate.

BACKGROUND: The case of a 34-year-old female nurse is presented. She worked in an accident and emergency department in a district general hospital, with methylene diphenyl diisocyanate (MDI)-containing synthetic plaster casts. She worked with MDI on a daily basis for 4 years. She was out of the department for 1 year and on her return developed cough, wheeze and dyspnoea within 5 min of exposure to MDI- containing synthetic casts. METHOD: A bronchial provocation test was performed and confirmed an early asthmatic response. RESULTS: There was a 39% decrease in the forced expiratory volume in 1 s 15 min after exposure, which required the administration of a bronchodilator on two occasions. The patient has subsequently avoided MDI-containing synthetic plaster casts and has experienced no further respiratory symptoms. CONCLUSION: This case illustrates that respiratory sensitization can occur as a result of exposure to MDI-containing synthetic casts and highlights the need for vigilance when health care workers are using isocyanate-containing synthetic casts.

Subjective benefit of inhaled therapies in patients with bronchiectasis: a questionnaire study.

The objectives of this study were to obtain information about the use and subjective benefits of inhaled therapies in patients with bronchiectasis and to determine if these benefits were related to the presence of airflow obstruction. One hundred and twenty patients completed a questionnaire on inhaled therapies and performed spirometry. Inhalers were used daily by 85/120 patients with bronchiectasis and 42/120 patients had a nebuliser. Inhaled therapies were used extensively in patients with (FEV1 < 80%) and without (FEV1 > 80%) evidence of airflow obstruction. The majority of patients reported subjective benefits from their inhaled therapies. Chi2 analysis showed that there was no association between perceived benefits of short acting bronchodilators (SAB), long acting bronchodilators (LAB), corticosteroids or nebulisers and degree of airflow obstruction. This study provides information about the subjective benefits of inhaled therapies which could facilitate a more comprehensive assessment of the net benefit of inhaled therapies in patients with bronchiectasis with and without airflow obstruction.

Prescribing practice for intermittent oxygen therapy: a GP survey.

INTRODUCTION: UK figures show that the prescription of home oxygen cylinders for intermittent use is substantial. AIM: To examine GP assessment criteria and prescribing practice for intermittent oxygen therapy in patients with a diagnosis of chronic obstructive pulmonary disease (COPD) in Northern Ireland. METHODS: A postal questionnaire was sent to all GPs (n = 534) in two health boards who had prescribed cylinder oxygen in a six month period prior to the study. The questionnaire was piloted to establish reliability and validity. RESULTS: Completed questionnaires were returned by 52% (280/534) of GPs. GPs 'most frequently' used advice from hospital specialists [82% (230/280)] to determine the need for intermittent oxygen. Criteria such as breathlessness score on exercise (e.g., BORG), oximetry on exercise, local guidelines or national guidelines were used less frequently or never. CONCLUSIONS: Most patients are likely to have been prescribed intermittent oxygen without any objective assessment. Implementation of evidence-based guidelines and a formal oxygen assessment service, would rationalize the use of intermittent oxygen therapy and enable better targeting of this expensive resource.

Lung cancer survival in Northern Ireland.

Lung cancer causes more deaths than any other cancer in Northern Ireland. Survival, and factors which could influence survival, were examined for the 4,458 patients diagnosed with lung cancer from 1992 to 1996. Overall five-year relative survival was low, but better for females (9%) than males (7%). Survival improved each year although this could not be attributed to treatment changes. Survival was better for men under 65 years, than older men (p<0.01), while survival from non-small cell type lung cancer was better than small cell (p<0.001). The proportion of patients having bronchoscopy and CT scan increased over the study period. Although the proportion of patients receiving surgery or radiotherapy remained unchanged over the period there was an increase in the proportion receiving chemotherapy (8.7% vs. 9.4%, P<0.05). Survival rates, although slightly better than in the rest of the United Kingdom, were poorer than in several other countries, including the Republic of Ireland and the United States of America. High mortality rates from lung cancer and, at best, modest improvements in survival, point to the need for increased efforts to reduce levels of lung cancer by tackling the main cause, smoking.

Smoking, atopy and certain furry pets are major determinants of respiratory symptoms in children: the International Study of Asthma and Allergies in Childhood Study (Ireland).

BACKGROUND: Environmental, cultural and health care differences may account for variation among countries in the prevalence of asthma and respiratory symptoms in teenagers. OBJECTIVE: To examine the prevalence of respiratory symptoms and the level of diagnosis, and to compare determinants of asthma and severe wheeze in two countries. METHODS: Self-completion questionnaires based on the International Study of Asthma and Allergies in Childhood (ISAAC) protocol were provided to school children in Ireland (Republic and Northern Ireland). In the Republic of Ireland, all children in classes largely aged 13-14 years from 30 post-primary schools were selected by random sampling stratified by school size, composition and Health Board in Spring 1995. In Northern Ireland, all children largely aged 13-14 years of age from 26 post-primary schools were selected by random sampling stratified by school type, composition and Education and Library Board in Spring 1996. RESULTS: Questionnaires were completed by 2,364 children from Northern Ireland and 2,671 from the Republic, about 90% of those eligible to participate. The prevalences of wheeze at various levels of severity, of diagnosed asthma and of treated wheeze were very similar in Northern Ireland and the Republic of Ireland. A significant proportion of those reporting more severe symptomatology (four or more attacks of wheeze in the past 12 months and/or one or more nights disturbed and/or moderate or greater disruption of daily activities and/or speech restriction due to wheeze) had been neither diagnosed nor treated for asthma (20-37%). To investigate the determinants of the more severe symptomatology of asthma or treated wheeze a series of stepwise multiple regression analyses was performed. A history of atopy, cigarette smoking, the possession of a furry pet other than a dog or cat and age were each independently associated with severe wheeze, whilst atopy, a furry pet (as above) and gender were each independently associated with asthma or treated wheeze. CONCLUSIONS: Cigarette smoking is closely associated with the reporting of significant respiratory symptoms together with atopy and exposure to furry pets. Some 20-37% of severe symptoms were neither diagnosed nor treated as asthma.

The current physiotherapy management of patients with bronchiectasis: a UK survey.

The purpose of this survey was to ascertain which physiotherapy techniques are currently used, how interventions are assessed and to determine the views of physiotherapists in the UK on the need for further research. Senior physiotherapists in 100 randomly selected hospitals providing respiratory care in the UK were sent a previously piloted questionnaire on their bronchiectasis practice. The response rate was 82%. A variety of physiotherapy treatment techniques and outcome measures are used, and there are no national guidelines on the physiotherapy management of patients with bronchiectasis. There is a clear need for research into the effectiveness of physiotherapy techniques in bronchiectasis including a cost-benefit analysis which would enable targeting of resources and the development of evidence-based practice guidelines.

Cytokine concentrations and neutrophil elastase activity in bronchoalveolar lavage and induced sputum from patients with cystic fibrosis, mild asthma and healthy volunteers.

BACKGROUND: Induced sputum (IS) has been proposed as a non-invasive alternative to bronchoalveolar lavage (BAL) for the assessment and monitoring of airways inflammation. The aim of this study was to compare both methods in patients with cystic fibrosis (CF). The possible differences between subjects with CF, mild asthma and healthy volunteers (HV) was also assessed. METHOD: In a single centre, randomised, two way crossover study, 11 patients with CF, 9 mild asthmatics (MA) and 11 HV underwent BAL and hypertonic saline induction on consecutive days. Free neutrophil elastase (NE), neutrophil elastase/alpha(1)-anti-trypsin complex (NE-AAT), tumour necrosis factor receptor (p55) and interleukin-8 (IL-8) were measured in cell free supernatants. RESULTS: Three CF patients reported serious adverse events following BAL. NE was usually undetectable in both IS or BAL samples and NE-AAT concentrations did not differ consistently between the two sampling methods. IL-8 and p55 levels in the CF patients tended to be higher in IS samples compared with BAL samples (median 19,860 vs. 3,855 pg/ml and 2.55 vs. 0.29 ng/ml, respectively). There was a significant difference in mean p55 concentrations between CF, MA and HV in IS samples (P=0.003) but not in BAL samples (P=0.36). The difference in mean IL-8 concentrations in IS samples between subject groups was statistically different (P=0.023). CONCLUSIONS: IS samples can be safely obtained from CF patients. Analysis of IS samples can help to characterize the inflammatory process in the airways of CF patients. The serious adverse events following BAL in 3 CF patients highlight an inherent risk associated with this procedure.

Prediction of hyperbilirubinemia in near-term and term infants.

OBJECTIVE: The purpose of this study was to determine whether end-tidal carbon monoxide (CO) corrected for ambient CO (ETCOc), as a single measurement or in combination with serum total bilirubin (STB) measurements, can predict the development of hyperbilirubinemia during the first 7 days of life. METHODS: From 9 multinational clinical sites, 1370 neonates completed this cohort study from February 20, 1998, through February 22, 1999. Measurements of both ETCOc and STB were performed at 30 +/- 6 hours of life; STB also was measured at 96 +/- 12 hours and subsequently following a flow diagram based on a table of hours of age-specific STB. An infant was defined as hyperbilirubinemic if the hours of age-specific STB was greater than or equal to the 95th percentile as defined by the table at any time during the study. RESULTS: A total of 120 (8.8%) of the enrolled infants became hyperbilirubinemic. Mean STB in breastfed infants was 8.92 +/- 4.37 mg/dL at 96 hours versus 7.63 +/- 3.58 mg/dL in those fed formula only. The mean ETCOc at 30 +/- 6 hours for the total population was 1.48 +/- 0.49 ppm, whereas those of nonhyperbilirubinemic and hyperbilirubinemic infants were 1.45 +/- 0.47 ppm and 1.81 +/- 0.59 ppm, respectively. Seventy-six percent (92 of 120) of hyperbilirubinemic infants had ETCOc greater than the population mean. An ETCOc greater than the population mean at 30 +/- 6 hours yielded a 13.0% positive predictive value (PPV) and a 95.8% negative predictive value (NPV) for STB >/=95th percentile. When infants with STB >95th percentile at <36 hours of age were excluded, the STB at 30 +/- 6 hours yielded a 16.7% PPV and a 98.1% NPV for STB >75th percentile. The combination of these 2 measurements at 30 +/- 6 hours (either ETCOc more than the population mean or STB >75th percentile) had a 6.4% PPV with a 99.0% NPV. Conclusions. This prospective cohort study supports previous observations that measuring STB before discharge may provide some assistance in predicting an infant's risk for developing hyperbilirubinemia. The addition of an ETCOc measurement provides insight into the processes that contribute to the condition but does not materially improve the predictive ability of an hours of age-specific STB in this study population. The combination of STB and ETCOc as early as 30 +/- 6 hours may identify infants with increased bilirubin production (eg, hemolysis) or decreased elimination (conjugation defects) as well as infants who require early follow-up after discharge for jaundice or other clinical problems such as late anemia. Depending on the incidence of hyperbilirubinemia within an institution, the criteria for decision making should vary according to its unique population.

Sleep complaints and risk factors for excessive daytime sleepiness in adult males in Northern Ireland.

The prevalence of sleep complaints in Northern Ireland is unknown. Sleep disruption can result in excessive daytime sleepiness (EDS), with significant socioeconomic consequences. The aim of this study was to assess the prevalence of sleep complaints and to determine risk factors for EDS in a Northern Irish community. From an urban and rural community of 499,111 people, a random sample of 3391 adult men were sent a questionnaire by mail. Questions were asked regarding sleep, EDS and medical history. There were 2364 completed questionnaires returned (response rate 70%). The mean age of respondents was 46.0 years (range 18--91 years). 26.7% of men were not satisfied with their usual night's sleep and 68% of men woke up at least once during the night. Based on pre-defined criteria, 24.6% of the population had insomnia and 19.8% had EDS. The strongest risk factor identified for EDS was a history of snoring loudly (odds ratio 2.62; 95% CI 1.82--3.77). Other risk factors included ankle swelling, feeling sad or depressed stopping sleep, experiencing vivid dreams while falling asleep, waking up feeling unrefreshed and age > 35 years. The prevalence rates of sleep complaints and EDS in this community-based study is high, although this does depend directly on the criteria used to define insomnia and EDS. Recognition of risk factors for EDS may help to identify and treat those affected.

Effect of once weekly pulmonary rehabilitation on exercise tolerance in patients with chronic lung disease.

BACKGROUND: Pulmonary rehabilitation (PR) programmes improve exercise tolerance and reduce breathlessness in patients with chronic obstructive pulmonary disease. AIM: A retrospective audit was undertaken to assess the effectiveness of the pulmonary rehabilitation programme at Belfast City Hospital (BCH). METHODS: Data from 74 patients who completed a six-week PR programme were reviewed. Exercise tolerance was assessed by the shuttle walk test (SWT), and breathlessness by the BORG scale. Paired t-tests were used for within group analysis. RESULTS: The mean (SD) exercise tolerance assessed by the SWT improved significantly as did duration of individual exercises. Patients were able to exercise more without increasing their breathlessness or heart rate. CONCLUSIONS: This paper highlights the effect of PR on exercise tolerance and breathlessness in patients with chronic lung disease. The results compare favourably with published literature where more frequently supervised exercise sessions were provided.