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.

The effects of flosequinan on hemodynamics and oxygen delivery in cor pulmonale.

The hemodynamic effects of a new orally active vasodilator, flosequinan, were compared with placebo (single blind) over 24 h in eight patients with pulmonary hypertension secondary to severe chronic obstructive pulmonary disease. Mean pulmonary artery pressure was reduced by 5.1 (3.4, 6.7) mm Hg (mean 95 percent CI) (p < 0.003) and pulmonary vascular resistance was reduced by 70 (23, 189) dynes.s.cm-5 (p < 0.013) by active drug compared with placebo. Cardiac output increased significantly with flosequinan by 0.47 (0.03, 0.91) L/min (p < 0.04) and systemic oxygen delivery increased by 90 (50, 120) ml/min/m2) (p < 0.05). A significant reduction in systemic vascular resistance was observed, 132 (35,230) dynes.s.cm-5 (p < 0.02) but no significant changes were seen in systemic arterial blood pressure or arterial blood gas tensions. Flosequinan favorably altered pulmonary hemodynamics relative to systemic and resulted in a significant improvement in oxygen delivery. The hemodynamic and blood gas effects of this compound suggest that it is a promising vasodilator for the treatment of pulmonary hypertension.

Is there an association between angiotensin-converting enzyme gene variants and chronic nonproductive cough?

BACKGROUND: It is unclear why some patients develop a chronic nonproductive cough. Angiotensin-converting enzyme (ACE) inactivates tussive peptides in the airways such as bradykinin and tachykinins. An insertion/deletion polymorphism in the ACE gene accounts for variation in ACE levels, and patients with the II genotype have lowest serum ACE levels compared with ID and DD genotypes. We hypothesized that the II genotype would be associated with increased risk of developing a chronic cough. MATERIALS AND METHODS: We recruited 47 patients (33 women), referred for evaluation of cough (median cough duration, 24 months; range, 2 to 240 months). Cough patients were evaluated using a comprehensive diagnostic protocol, and cough reflex sensitivity was measured using a capsaicin inhalation challenge. ACE genotyping was performed on DNA samples from patients using the polymerase chain reaction followed by agarose gel electrophoresis. ACE genotypes in patients with chronic cough were compared with those in 199 healthy control subjects. Serum ACE levels were determined using a colorimetric assay. RESULTS: Genotype frequencies for the ACE gene were similar between patients and control subjects. There was no correlation between capsaicin sensitivity and ACE genotypes or serum ACE levels. CONCLUSION: Susceptibility to develop chronic cough is not associated with ACE genotype.

The natural history and clinical consequences of aspiration in acute stroke.

Sixty consecutive patients admitted to a teaching hospital with acute stroke were studied prospectively for 3 months to define the natural history and consequences of lung aspiration. Using videofluoroscopy, aspiration was identified in 25 patients (42%) within 72 h of stroke onset, and had resolved in all but three patients (8%) after 3 months. It was closely related to the presence of dysphagia, which itself resolved within 2 weeks in all but the persistent aspirators. Lower respiratory tract infection (LRTI) was more common in aspirating patients (68%) than non-aspirators (6%). The use of intravenous fluids without oral intake did not appear to prevent LRTI in aspirating patients who were also dysphagic. Pneumonia occurred after 2 weeks in the three patients subsequently found to aspirate persistently. Aspiration is a transient phenomenon in most cases of acute stroke; it is associated with a high incidence of LRTI, but mortality in this series was not significantly associated either with respiratory tract infection or aspiration itself.

The psychological benefits of exercise and the treatment of delinquent adolescents.

The conviction that athletic participation imparts desirable educational, social and personal values has been the basis for including recreational sports in the remedial programme for juvenile delinquents. More recently, the psychological benefits of aerobic exercise and increased fitness have been investigated. Changes following intensive exercise include reductions in anxiety, tension and depression, and increased self-esteem. These effects have been variously hypothesised as due to a sense of increased control or mastery, a meditative effect or to alterations in neurotransmitter-levels. Delinquent adolescents comprise a heterogeneous population characterised by clusters of risk factors, handicapping conditions and psychopathology, with a prevalence of depression and low self-esteem far exceeding that of the population at large. Intervention which can benefit these characteristics may be especially useful in preventive or therapeutic programmes in this group. While less intensive recreational play or physically challenging 'Outward Bound' programmes can be effective in improving social attitudes and self-esteem, intensive aerobic exercise may produce greater improvements in self-esteem and depression. Future research must identify the mechanisms by which exercise confers these benefits, whether it enhances other areas, such as social skills or academic performance, and which populations are benefitted by such programmes.

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.

Native seed preferences of shrub-steppe rodents, birds and ants: the relationships of seed attributes and seed use.

This study established the preferences of shrubsteppe granivores among seeds of 6 common sagebrushsteppe plants and related the preferences observed to physical and nutritional attributes of the seeds. Seeds of big sagebrush (Artemisia tridentata), cheatgrass (Bromus tectorum), Indian ricegrass (Oryzopsis hymenoides), western wheatgrass (Pascopyrum smithii), bitterbrush (Purshia tridentata) and green needlegrass (Stipa viridula) were placed in groups of petri dishes designed such that seed removal could be ascribed to either diurnal vertebrates, nocturnal vertebrates or ants. Though absolute quantities of seeds removed varied among the 3 granivore classes, calculations of preference based on weights of each seed species removed by each granivore class indicated that all 3 ranked the seeds similarly. Preference hierarchies of the 3 granivore classes were highly positively correlated with both calories per seed and % soluble carbohydrate of the seeds. The first correlation supports a basic prediction of optimal foraging theory -that foragers should maximize energy intake per unit time spent foraging. Both correlations emphasize the role of seed nutritional qualities in granivore seed selectivity in that soluble carbohydrate is a water-efficient energy source and its percentage is a good indicator of the digestible energy available in a food item. A corollary experiment comparing granivore use of an exotic seed (millet [Panicum miliaceum]) and a preferred native seed (Oryzopsis) demonstrated a distinct preference for the exotic. Since millet seeds are particularly high in % soluble carbohydrate, this result reinforced the apparent value of this nutritional attribute as a predictor of granivore seed preference. Among many seed resource characteristics upon which granivore seed selectivity might operate, our results indicate that individual species' nutritional composition may be particularly important. Thus, inferences about seed selectivity and resource partitioning among arid-land granivores should be interpreted with caution, especially those based on experiments using seed introductions, since the influence of seed nutritional attributes has not been widely acknowledged heretofore.

Ankyloglossia: incidence and associated feeding difficulties.

OBJECTIVES: To determine the incidence of ankyloglossia (tongue-tie) in the well-baby population, and to determine whether patients with ankyloglossia experience breastfeeding difficulties. DESIGN: Prospective controlled study. SETTING: Tertiary care children's hospital. PATIENTS: A total of 1041 neonates in the well-baby nursery were screened for ankyloglossia. Those positively identified were invited to participate in the study. Mothers of newborns with ankyloglossia and mothers of a matched control group of unaffected newborns were contacted by telephone on a monthly basis for 6 months after their children were discharged from the hospital to determine the presence of breastfeeding difficulties. MAIN OUTCOME MEASURES: Incidence of ankyloglossia, percentage of infants successfully breastfed, and incidence of breastfeeding difficulties. RESULTS: Fifty newborns were identified with ankyloglossia, for an incidence of 4.8% The male-female ratio was 2.6:1.0. Of the 36 mothers of affected infants who were followed up and who intended to breastfeed, 30 (83%) successfully breastfed their infants for at least 2 months, compared with 33 (92%) of the 36 mothers of infants in the matched control group (P = .29). Breastfeeding difficulties were experienced by 9 (25%) of the mothers of infants with ankyloglossia compared with 1 (3%) of the control mothers (P<.01). CONCLUSION: Ankyloglossia, which is a relatively common finding in the newborn population, adversely affects breastfeeding in selected infants.

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 nine 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 breast-fed 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 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 > or =95th percentile. When infants with STB > or =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 two 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.

Physical and psychological effects of aerobic exercise in boys with learning disabilities.

Aerobic exercise has been associated with improvements in psychological status and physical fitness in adults, but its effects on children are less clear. The purpose of this study was to evaluate the effects of an aerobic exercise program on the self-concept, academic achievement, motor proficiency, and cardiovascular fitness of boys with learning disabilities. Fifty-four boys were randomly assigned to one of two exercise programs lasting 20 weeks. One program emphasized aerobic exercise and the other consisted of similar but less vigorous activities. The self-concept, academic achievement, motor proficiency, and physical fitness of each subject was assessed before and after the exercise programs. The results from group comparisons demonstrated an association between the aerobic exercise program and improvement of self-concept and physical fitness. No effect on academic achievement or motor proficiency could be attributed to the aerobic exercise program.

Improved harness crutch to reduce upper limb effort in swing-through gait.

A novel crutch, the harness crutch, is described which reduces loading on the arms during the swing phase of swing-through gait. The device was fabricated by attaching a modified mountaineering harness by two side straps, to modified axillary crutches. The harness crutch was compared with the saddle crutch, described by Taylor in 1883. The saddle crutch produced pressures in excess of 500 mmHg in the perineal area; no pressures were produced in this area with the harness crutch. Ischial pressures produced by both systems were similar. In six out of eight non-impaired subjects, significantly more force was transmitted to the harness crutch (an average 47% of body weight) than to the saddle crutch (an average 40% of body weight). A comparison of the oxygen cost of swing-through gait was made between the harness crutch and unmodified axillary crutches; with the harness crutch oxygen cost was significantly lower (p < 0.01) and there were no significant differences in speed and stride length.

Stenting and superior vena caval syndrome.

The use of intravascular stents in the management of Superior Vena Caval Obstruction is a relatively recent development. We describe our experience with six patients, aged between 64 and 85 years who developed SVC obstruction secondary to malignant disease and who had one or more intravascular stents inserted. Five of the patients had lung cancer and one metastatic breast carcinoma. Positive histology was available in three cases. A partial or complete clinical response was seen in all patients and two patients developed recurrent symptoms. Stenting appears to provide effective palliation of SVC obstruction symptoms.

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.

Cardiopulmonary exercise testing in the pre-operative assessment of patients for repair of abdominal aortic aneurysm.

We have investigated the value of cardiopulmonary exercise testing in the pre-operative assessment to patients for abdominal aortic aneurysm repair. Thirty-six patients were entered into the study. All had a pre-operative clinical assessment and investigations including chest radiograph, electrocardiograph, spirometry and echocardiogram with measurement of left ventricular ejection fraction. Each patient performed a symptom limited treadmill exercise test using a STEEP protocol with on-line measurement of respiratory gas exchange. Patients were followed up for 12 months post-operatively by review of casenotes. Thirty out of 36 patients had surgical repair of abdominal aortic aneurysm. There was 1 death in the perioperative period and 2 deaths in the following 12 months. Seven other patients suffered post-operative complications. There were no significant differences in left ventricular ejection fraction, spirometry and peak achieved oxygen consumption (PVO2) between those patients who died or who had post-operative complications and those who had not. However, PVO2 < 20 ml/min/kg was found in 70 per cent of patients who had complications compared with 50 per cent of those who had not. Also 4 patients considered medically unfit for surgery all had PVO2 < 20 ml/min/kg. Cardiopulmonary exercise testing with measurement of PVO2 may be helpful in identifying patients more at risk of post-operative complications but should not be used in isolation without through clinical assessment.

Cost minimisation analysis of provision of oxygen at home: are the drug tariff guidelines cost effective?

OBJECTIVES: To determine the level of oxygen cylinder use at which it becomes more cost effective to provide oxygen by concentrator at home in Northern Ireland, and to examine potential cost savings if cylinder use above this level had been replaced by concentrator in 1996. DESIGN: Cost minimisation analysis. SETTING: Area health boards in Northern Ireland. MAIN OUTCOME MEASURES: Cost effective cut off point for switch to provision of oxygen from cylinder to concentrator. Potential maximum and minimum savings in Northern Ireland (sensitivity analysis) owing to switch to more cost effective strategy on the basis of provision of cylinders in 1996. RESULTS: In Northern Ireland it is currently cost effective to provide oxygen by concentrator when the patient is using three or more cylinders per month independent of the duration of the prescription. More widespread use of concentrators at this level of provision is likely to lead to a cost saving. CONCLUSIONS: The Drug Tariff prescribing guidelines, advocating that provision of oxygen by concentrator becomes cheaper when 21 cylinders are being used per month-are currently inaccurate in Northern Ireland. Regional health authorities should review their current arrangements for provision of oxygen at home and perform a cost analysis to determine at what level it becomes more cost effective to provide oxygen by concentrator.

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.

Transplantation for chronic pulmonary disease: referral and outcome in Northern Ireland, 1986-1990.

Eighteen patients have been referred for lung transplantations from Northern Ireland in 1986-1990. Fourteen were accepted but only four achieved transplantation. These rates are lower than for comparable regions in the North of England. The lung donation rate from Northern Ireland during the same period was similar to that for the United Kingdom as a whole. The low referral and transplant rates for Northern Ireland require reassessment of the procedures involved.

Home ventilation in Northern Ireland.

Thirteen patients were identified as receiving assisted ventilation at home in Northern Ireland in 1994. Two patients have since died. An increasing number of patients are starting home ventilation, especially by nasal mask. Recognition of the needs of these patients and provision of care require further consideration.

Prescription of oxygen concentrators and survival in Northern Ireland.

Long-term oxygen therapy (LTOT) has been shown to prolong survival and to improve quality of life in patients with chronic obstructive pulmonary disease (COPD) and in respiratory failure. In Northern Ireland oxygen concentrators have been available on prescription since August 1986, initially on a restricted basis from hospital physicians only. This was followed by open prescribing from April 1989, when concentrators could be prescribed by general practitioners. This study examined prescribing habits of LTOT during both periods, and patient survival. Case notes of all prescriptions of oxygen concentrators in Northern Ireland (to April 1991) were reviewed. Prescription criteria and advice regarding usage during both periods were analysed. A questionnaire survey of subjects during open prescribing documented the advice given at the time of prescription and current usage. 164 charts of 178 total installations were available for review. During both periods many concentrators were installed without adherence to the prescribing criteria at the time (75% restricted, 48% open). The majority of these were on the advice of a consultant respiratory physician and only 14 were prescribed directly by GPs. 89 of 91 subjects receiving current LTOT during the study period completed questionnaires. Of the subjects prescribed LTOT during the restricted period, 2 subjects are still alive (median survival 19 m, range 0-104). From the open period, survival data was available on 107 of 129 subjects with 17 still alive (median survival 22 m, range 0-94). This study documents an inadequate rate of prescribing and a lack of conformity to guidelines in the provision of LTOT in Northern Ireland. We would suggest that familiarisation with the prescribing criteria, formal written advice at the time of prescription, appropriate follow up to ensure adequate supplementation and regular patient education on the use of LTOT would address these problems to a substantial degree.