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Biomedical subjects

S P Conway

Publications and source records attributed to S P Conway.

At least 19 recordsLinked to original sources
PubMed2007

Low gammaglobulin subclass 2 levels in paediatric cystic fibrosis patients followed over a 2-year period.

The aim of this study was to relate serum immunoglobulin G2 subclass levels in a large paediatric population with cystic fibrosis, to clinical status and antibody levels to Haemophilus influenzae type b and Streptococcus pneumoniae and to observe any changes over a 2-year period. IgG subclasses were measured in 131 patients. Results were compared with levels from age-related normal population data. The following clinical data were collected at baseline and 2 years later; genotype: height, weight, and BMI z-scores: FEV1 (as percent predicted): Shwachman-Kulczcyki and Northern chest X-ray scores: Pseudomonas aeruginosa status. Antibody levels to H. influenzae type b and S. pneumoniae measured at baseline were related to IgG2 level. There was a reduction in the prevalence of low levels of IgG2 from 29% to 10% over the 2-year period. Low levels of IgG2 were not associated with any decline in clinical well-being. Low levels of IgG2 alone were associated with low antibody levels to S. pneumoniae. Low levels of IgG2 and low levels of antibody to H. influenzae and S. pneumoniae were not associated with any decline in clinical well-being. Children with high levels of IgG2 had worse lung function, worse Shwachman-Kulczcyki and Northern chest X-ray scores and higher levels of P. aeruginosa infection. Children with low IgG2 levels were not worse clinically compared to those with normal or high IgG2 levels. High IgG2 levels were associated with a worse clinical status.

Adolescent↗
PubMed2007

Paediatric Northern Score centile charts for the chest radiograph in cystic fibrosis.

AIM: To create the first national centile charts for the chest radiograph Northern Score using the UK Cystic Fibrosis (CF) Database (UKCFD). MATERIALS AND METHODS: All active patients for 2002 from the UKCFD were analysed in 1-year cohorts from 0 to 18 years. Northern Score results from the annual review forms were used to construct centile lines for the 5th, 25th, 50th, 75th, 95th centiles. RESULTS: There were 1806 patients with recorded Northern Score data for 2002 (927 male patients, male:female ratio 1.05). The centile chart demonstrates a quasi-linear rise throughout childhood. A Northern Score in excess of age in years equates to >95th centile in school-aged CF patients. CONCLUSION: This centile chart provides a disease-specific reference range for monitoring individual patients or for evaluating therapeutic change using the dominant chest radiograph scoring system in the UK. Patients, parents and clinicians may find these useful during the annual review process.

Aging↗
PubMed2006

Measurement of urinary N-acetyl-b-D-glucosaminidase in adult patients with cystic fibrosis: before, during and after treatment with intravenous antibiotics.

BACKGROUND: Patients with cystic fibrosis (CF) are at high risk from the nephrotoxic effects of intravenous antibiotics due to repeated and prolonged courses of therapy. Routine methods of monitoring renal injury are insensitive. N-acetyl-b-d-glucosaminidase (NAG) is a lysosomal enzyme present in the renal proximal tubular cells, with increased excretion an indicator of renal tubular dysfunction. METHODS: Urinary NAG, creatinine, serum creatinine, electrolytes and BUN were measured on days 1, 14 and at the first out-patient visit following treatment with tobramycin or colistin. Urinary NAG levels were corrected for urinary creatinine and expressed as a NAG ratio. Patients who received>1 course of intravenous antibiotics during the study period were included in a separate analysis of the cumulative effect of treatment. RESULTS: 88 patients (44 female, 31 with CFRD) completed a single course of intravenous antibiotics. 71 patients had urinary NAG levels at follow-up. The median time to follow-up was 50 days. Serum electrolytes, creatinine and BUN were normal throughout. A 3.5-fold increase in urinary NAG excretion was observed between day 1 and 14 and 46% of patients had an elevated NAG level at follow-up. A highly significant difference in NAG excretion was observed on day 14 for tobramycin vs. colistin (median 2.24 vs. 0.98, p<0.001). A significant difference in NAG excretion was seen in patients with CFRD at all measured time points. Patients with CFRD had a significantly worse clinical status and had received more days of intravenous antibiotics over the previous 6 years. In 20 (80%) of 25 patients who received>1 course of treatment during the study period, baseline NAG levels were significantly higher in subsequent courses (p<0.001). There was a significant correlation between previous exposure to colistin and baseline NAG levels (r=0.389, p<0.001). CONCLUSIONS: Both tobramycin and colistin cause acute renal tubular injury with a significant rise in urinary NAG excretion. Patients with CFRD seem to be at greatest risk of renal tubular damage. Cumulative damage is evident with repeated dosing. Previous exposure to nephrotoxic antibiotics, especially colistin, is associated with elevated baseline NAG levels. We recommend that colistin is reserved for patients with resistant Pseudomonas aeruginosa or those who are intolerant to tobramycin. Serial longitudinal NAG measurements may be useful in patients with CF, especially those with CFRD, to identify patients at risk of developing renal disease.

Acetylglucosaminidase↗
PubMed2005

Delayed diagnosis of cystic fibrosis associated with R117H on a background of 7T polythymidine tract at intron 8.

We report late diagnoses of cystic fibrosis (CF) in two men aged 61 and 65 years. At the time of presentation, both patients had significant pulmonary disease. In each case two CFTR gene mutations were identified, including R117H on a background of a poly T genotype of 7T/9T. Patients with two identified CFTR mutations which include the R117H/7T anomaly should be followed up routinely as they remain susceptible to severe lung disease.

Cystic Fibrosis↗
PubMed2005

Immunoglobulin and IgG subclass levels in a regional pediatric cystic fibrosis clinic.

The aim of this study was to report serum immunoglobulin (Ig) and IgG subclass levels in a large pediatric population with cystic fibrosis, and relate these to measures of disease severity. Total immunoglobulin levels were measured in 154 patients, and IgG subclass levels were measured in 136 patients and compared to age-related normal population data and to levels reported in previously published studies of children with cystic fibrosis. Clinical data were also collected: genotype; height, weight, and BMI standard deviation scores; FEV(1) (as percent predicted); Shwachmann-Kulczycki (S-K) and Northern chest X-ray scores; and Pseudomonas aeruginosa infection status. The clinical well-being of patients with hypo- or hyper-gammaglobulinemia was compared with age- and sex-matched control patients who had normal levels of gammaglobulin. IgG subclass levels were measured, and the results were compared with previous studies. Eleven patients had hypergammaglobulinemia (7.8% compared with 0-69% in the published literature). Patients with hypergammaglobulinemia had lower FEV(1) percent-predicted values, and worse S-K and Northern chest X-ray scores than controls. Three patients had hypogammaglobulinemia (1.9% compared with 0-10.8% in the published literature). There was no difference in any clinical parameter between controls and those with hypogammaglobulinemia. Nineteen patients (14%) had low levels of IgG1, and 40 patients (29%) had low levels of IgG2. The low percentage of patients with abnormally high immunoglobulin levels probably reflects the improved respiratory status of today's children with CF. The low percentage of those with low IgG probably reflects better nutritional status. The finding of worse lung function and clinical scores in patients with hypergammaglobulinemia agrees with the published literature. The high percentage of patients with low IgG2 was unexpected and was not previously reported. The clinical significance of this in patients with CF is unknown.

Adolescent↗
PubMed2005

Nebulized antibiotic therapy: the evidence.

The main indications for nebulized antibiotic use are as maintenance therapy for patients with chronic Pseudomonas aeruginosa infection and in treatment protocols aimed at eradicating early P. aeruginosa infection. Daily nebulized antibiotic therapy has been used extensively in Europe for the last 25 years and recently in North America following the introduction of tobramycin solution for inhalation (TSI). The antibiotic is delivered directly to the site of infection, maximizing its efficacy and reducing its potential for toxicity. The efficacy of nebulized antibiotic therapy has been confirmed by meta-analyses of early studies which usually involved only small numbers of patients, and recently by large scale randomized control trials. These studies have shown that regular aerosolized antibiotic treatment results in improved respiratory function, less hospital admissions and respiratory exacerbations, and a significant reduction in the load of P. aeruginosa respiratory tract infection. Concerns about increasing bacterial resistance do not yet seem to have had any clinical impact. Successful eradication of early P. aeruginosa infection has been reported with nebulized colistin (in combination with oral ciprofloxacin), tobramycin and TSI. No advantage has been shown in studies comparing nebulized and intravenous antibiotics versus intravenous antibiotics alone in the treatment of acute respiratory exacerbations. Inhalation of antibiotics may provoke bronchospasm and patients should be assessed before and after treatment prior to continuing long-term therapy at home.

Administration, Inhalation↗
PubMed2004

Scoliosis in cystic fibrosis: is it idiopathic?

STUDY DESIGN: This is a retrospective study of all the patients registered with the Yorkshire Regional Cystic Fibrosis unit from 1982 to 1997. Of the 316 patients, there were 184 adults (age 17 years and above) and 132 children (age 0-6 years). OBJECTIVES: This study was aimed at determining the prevalence of scoliosis in people with cystic fibrosis and describes the characteristics and progression of scoliosis in these patients and highlights predictive factors, which account for high prevalence of scoliosis in this condition. SUMMARY OF THE BACKGROUND DATA: Two previous North American studies (1978 and 1982) have indicated a high prevalence of scoliosis in patients with cystic fibrosis. METHODS: The patients were divided into 3 groups based on their chronologic age as on January 1998. Chest, abdomen, and whole spine radiographs were studied for the presence of any spinal deformity, and measurements were made using the Oxford Cobbmeter (Oxford Metrics). The extent, apex, and the side of the curves were described. The disease-specific scores, ie, Chrispin-Norman score (score for radiologic severity of lung disease) and Shwachman score (score for general condition), were noted from the patient follow-up database maintained by the Regional Cystic Fibrosis Unit. Multiple linear regression analysis was used to study the correlation between Cobb angle and the previously mentioned scores. RESULTS: In the 4- to 16-year age group, the prevalence of scoliosis was 15.6%, which is 20 times the prevalence in 15,793 school children with a similar age and sex distribution from the same geographic area. The majority of curves were single-thoracic, apical around T6-T8 with no side predilection. In the adult population (above 16 years), the prevalence was 9.8%, which is higher than that of the general population. These curves were thoracic, apical around T7-T8, and approximately two thirds of them were right-sided. Infantile curves are described for the first time in our study; these tend to be nonprogressive, right-sided, upper thoracic curves. CONCLUSION: Our study shows a high prevalence of scoliosis in people with cystic fibrosis. These are benign short midthoracic curves, apical between T6-T8 with no side predilection.

Adolescent↗
PubMed2004

Clinical value of obtaining sputum and cough swab samples following inhaled hypertonic saline in children with cystic fibrosis.

Prompt detection and treatment of lower respiratory tract infection are essential in the management of patients with cystic fibrosis (CF), who often have signs or symptoms of respiratory infection without any pathogens being isolated from sputum or cough swab specimens. The aims of this study were to assess the efficacy and clinical value of obtaining sputum and oropharyngeal cough swab samples following induction with hypertonic saline (HS) in this group of patients. Forty-three outpatients with CF, mean age 7.2 years (range, 1.8-12.9 years), were recruited over a 2-year period. Nebulized salbutamol was administered, followed by 6% HS. Sputum was preferentially obtained before and after HS induction if possible. If the patient was not able to expectorate, oropharyngeal cough swabs were taken instead. Four patients were able to expectorate sputum before and 19 after HS induction. The procedure was tolerated in 41 of 43 patients. Pathogens were isolated from 13 patients' HS-induced samples, but not from their corresponding preinduced specimens, and 4 patients' preinduced specimens cultured organisms which were not identified from their HS-induced samples. Significant changes were made in the management of 13 (30.2%) patients directly resulting from the positive culture of pathogens only from HS-induced samples. Cultures from oropharyngeal cough swab or expectorated sputum specimens following inhalation of HS provide additional microbiological information which is of clinical value and may lead to changes in patient management.

Administration, Inhalation↗
PubMed2004

Dietary intakes in adult patients with cystic fibrosis--do they achieve guidelines?

BACKGROUND: Most patients with cystic fibrosis (CF) require a higher energy and protein intake than their healthy peer group. There are few data on dietary intakes of adult patients. The aim of this study was to determine nutritional intakes in an adult population with CF. The impact of nutritional intervention and disease on macronutrient intake was examined. METHODS: Retrospective cross-sectional analysis of 94 unweighed food diaries at annual review (1995-2000). Energy and protein intakes were compared to the estimated average requirement (EAR) for energy and reference nutrient intake (RNI) for protein. The effect of diet alone, oral supplements, enteral tube feeding, and cystic fibrosis related diabetes (CFRD), on macronutrient intake was examined and impact of pancreatic sufficency (PS) and lung transplantation. RESULTS: Mean energy and protein intakes approached recommended CF guidelines, but in 72% of assessments these values were not achieved. Mean energy and protein intakes for patients on diet alone and protein intake for those with CFRD failed to meet recommendations. Oral supplementation and enteral tube feeding regimens increased energy and protein intake above recommended levels. No group achieved 40% total energy from fat. Patients receiving enteral tube feeds had the highest mean energy and protein intakes but lowest body mass index (BMI) and lung function. CONCLUSION: Adequate mean energy and protein intakes in adult patients with CF mask subgroups of patients who fail to meet recommendations ie. diet alone, diabetic. Oral supplementation and enteral tube feeding increase energy and protein intake but fail to achieve an adequate BMI level in subjects with a decreased clinical status. Individual nutritional assessment remains essential.

Adolescent↗
PubMed2004

Treatment of resistant distal intestinal obstruction syndrome with a modified antegrade continence enema procedure.

We report a case of a patient with CF who had a long history of recurrent distal intestinal obstruction syndrome. She had been treated with conventional treatment including gastrografin, n-acetyl cysteine, Klean prep and Picolax. She underwent a modified antegrade continence enema procedure. She currently irrigates her conduit every 2-3 days. She has had no further symptoms of distal intestinal obstruction syndrome.

Adolescent↗
PubMed2004

Effect of oral bisphosphonates on bone mineral density and body composition in adult patients with cystic fibrosis: a pilot study.

BACKGROUND: Approximately two thirds of adult patients with cystic fibrosis have reduced bone mineral density and up to one quarter have osteoporosis at one or more sites. Any bone mineral deficits are likely to be exacerbated in patients following lung transplantation by their immunosuppressive regimen. Vertebral collapse and rib fractures will impair the ability to cough and the efficacy of physiotherapy treatments. METHODS: Patients attending the Leeds Regional Adult Cystic Fibrosis Unit with either osteopenia or osteoporosis on dual energy x ray absorptiometry (DXA) scanning were offered treatment with oral bisphosphonates after exclusion of abnormal vitamin D, calcium, or phosphate levels, abnormal thyroid function, or hypogonadism. Those declining treatment or patients with a normal initial DXA scan formed the control group. A second DXA scan was performed after a mean of 2.4 years in the treatment group and 2.9 years in the non-treatment group. Patients in the active group were asked to complete a short questionnaire detailing their adherence to treatment. RESULTS: The medians of the differences in annual changes in bone parameters between treatment and control groups showed significant differences in bone mineralisation in favour of the treatment group at the lumbar spine (L2-L4), the femoral neck, and for total body measurements. There were no significant differences in weight, height, or body composition in either patient group. Most treated patients stated that they adhered to treatment most of the time. CONCLUSION: Treatment with oral bisphosphonates may improve bone mineralisation in adult patients with cystic fibrosis. The results of this pilot study need to be further explored in a randomised controlled trial.

Absorptiometry, Photon↗
PubMed2003

Antibiotic treatment of multidrug-resistant organisms in cystic fibrosis.

Respiratory tract infection with eventual respiratory failure is the major cause of morbidity and mortality in cystic fibrosis (CF). Infective exacerbations need to be treated promptly and effectively to minimize potentially accelerated attrition of lung function. The choice of antibiotic depends on in vitro sensitivity patterns. However, physicians treating patients with CF are increasingly faced with infection with multidrug-resistant isolates of Pseudomonas aeruginosa. In addition, innately resistant organisms such as Burkholderia cepacia complex, Stenotrophomonas maltophilia and Achromobacter (Alcaligenes) xylosoxidans are becoming more prevalent. Infection with methicillin-resistant Staphylococcus aureus (MRSA) is also a problem. These changing patterns probably result from greater patient longevity and increased antibiotic use for acute exacerbations and maintenance care. Multidrug-resistant P. aeruginosa infection may be treated successfully by using two antibiotics with different mechanisms of action. In practice antibiotic choices have usually been made on a best-guess basis, but recent research suggests that more directed therapy can be achieved through the application of multiple-combination bactericidal testing (MCBT). Aerosol delivery of tobramycin for inhalation solution achieves high endobronchial concentrations that may overcome bacterial resistance as defined by standard laboratory protocols. Resistance to colistin is rare and this antibiotic should be seen as a valuable second-line drug to be reserved for multidrug-resistant P. aeruginosa. The efficacy of new antibiotic groups such as the macrolides needs to be evaluated.CF units should adopt strict segregation policies to interrupt person-to-person spread of B. cepacia complex. Treatment of panresistant strains is difficult and often arbitrary. Combination antibiotic therapy is recommended, usually tobramycin and high-dose meropenem and/or ceftazidime, but the choice of treatment regimen should always be guided by the clinical response.The clinical significance of S. maltophilia, A. xylosoxidans and MRSA infection in CF lung disease remains uncertain. If patients show clinical decline and are chronically colonized/infected with either of the former two pathogens, treatment is recommended but efficacy data are lacking. There are defined microbiological reasons for attempting eradication of MRSA but there are no proven deleterious effects of this infection on lung function in patients with CF. Various treatment protocols exist but none has been subject to a randomized, controlled trial. Multidrug-resistant microorganisms are an important and growing issue in the care of patients with CF. Each patient infected with such strains should be assessed individually and antibiotic treatment planned according to in vitro sensitivity, patient drug tolerance, and results of in vitro studies which may direct the physician to antibiotic combinations most likely to succeed.

Anti-Bacterial Agents↗
PubMed2003

Stenotrophomonas maltophilia contamination of nebulizers used to deliver aerosolized therapy to inpatients with cystic fibrosis.

There is circumstantial evidence that nebulizer equipment may be a source of Stenotrophomonas maltophilia for patients with cystic fibrosis. Eighty-nine inpatient nebulizers were examined for evidence of S. maltophilia contamination of which nine (10%) yielded 14 strains of the bacterium. Environmental samples were obtained from 73 different sites on the ward, of which 17 (23%) yielded a further 21 strains. Positive sites included taps, sink drains, and potable water. Genotyping using ERIC-PCR and pulsed-field gel electrophoresis revealed that two pairs of patients' nebulizers were contaminated with closely related strains. None of the S. maltophilia isolates obtained from the ward environment shared genotypes with those obtained from the nebulizers. The frequency of isolation of S. maltophilia from potable water sources on the ward suggests that contamination may result from using it to clean reusable nebulizer equipment, particularly if this is followed by inadequate drying. Although the actual source of S. maltophilia contamination of hospital-use nebulizer equipment in this study remained elusive, these results have important infection control implications.

Adolescent↗
PubMed2003

A pilot study of zafirlukast as an anti-inflammatory agent in the treatment of adults with cystic fibrosis.

BACKGROUND: Persistent endobronchial inflammation is in part responsible for the attrition of lung function seen in cystic fibrosis. Leukotrienes act as pro-inflammatory mediators. The aim of this study was to assess the efficacy of the leukotriene receptor antagonist zafirlukast as a potential anti-inflammatory agent in the treatment of adult patients with cystic fibrosis. METHODS: Clinically stable patients were enrolled in the study if they had no history or clinical evidence of asthma, bronchial hyper-reactivity, or aspergillosis. They were randomised to receive zafirlukast 20 mg twice daily with all routine treatment for four months or routine treatment alone in an open cross-over design. Primary endpoints were changes in respiratory function tests and a modified NIH clinical score. RESULTS: Thirty patients were enrolled and 25 completed. There was a significant improvement in the modified NIH clinical score but no significant increase in respiratory function with zafirlukast. CONCLUSIONS: Patients receiving a leukotriene receptor antagonist in addition to routine treatments showed significant improvement in a clinical score which is a composite of clinical wellbeing, chest radiograph appearance, and physical examination. Respiratory function showed a non-significant trend towards improvement with treatment. Zafirlukast may benefit patients with CF. An adequately powered study is justified on the basis of these results.

Adult↗
PubMed2003

Quality of life in cystic fibrosis: the impact of gender, general health perceptions and disease severity.

BACKGROUND: Disease progression in cystic fibrosis (CF) is marked by deterioration across a number of physiological systems. In addition, there is evidence that females have a worse prognosis than males. The current work assesses the impact of both these factors on health related quality of life (HRQoL). METHODS: Two hundred and twenty-three adolescents and adults completed the cystic fibrosis quality of life (CFQoL) questionnaire with a further 185 approached and not responding by non-completion of the questionnaire. The CFQoL is divided into nine domains: physical, social, treatment, chest symptoms, emotional functioning, concerns for the future, relationships, body image, and career. Measurement of objective clinical status included, body mass index (BMI), and percentage of predicted forced expiratory volume in one second (FEV1). General health perceptions (GHP) were also measured. RESULTS: Patients were sub-divided by gender and disease severity (mild > 70% FEV1, moderate 40-69% and severe < 40%). Factorial analysis of variance indicated significant main effects for FEV1 (F = 587.98, P < or = 0.001) and BMI (F = 17.29, P < or = 0.001) as a function of disease severity. Post hoc tests revealed significant two-group differences for FEV1 and BMI between disease severity groups. No differences were observed for gender across FEV1 or BMI. Differences emerged across most CFQoL domains for disease severity, with the exception of concerns for the future, which was consistently low throughout. Gender differences emerged for chest symptoms, emotional functioning, concerns for the future, body image and career. With the exception of body image, females exhibited poorer HRQoL. Pearson correlations indicated that females' perception of health was more closely related to clinical status than males. CONCLUSIONS: Disease severity has an impact on HRQoL in adolescents and adults with CF. Some differences emerged between males and females, with females generally reporting poorer HRQoL. Evidence indicated that males and females perceived their health status differently, with females having a more accurate perception of objective clinical health status.

Adaptation, Psychological↗
PubMed2002

Validation of the SF-36 for the assessment of quality of life in adolescents and adults with cystic fibrosis.

BACKGROUND: Generic health-related quality of life measures are often applied to disease groups without assessment of their psychometric properties. The current work assesses the properties of the Short Form 36-item (SF-36) questionnaire in a British sample of adolescents and adults with cystic fibrosis (CF). METHODS: Two hundred and twenty-three adolescents and adults with CF completed the SF-36 with a further 185 approached and not responding by non-completion of the questionnaire. The structure and internal reliability of the instrument was assessed by principal components analysis, Cronbach alpha coefficients and item to domain correlations. Differences between disease severity groups were assessed by analysis of variance. RESULTS: Factor analysis of the SF-36 scores broadly confirmed domain structures for the SF-36. Cronbach alpha coefficients were high (range 0.82-0.91) and item-to-same domain correlations were stronger than item-to-unrelated domain correlations. Examination of differences between mild, moderate and severe disease states revealed four significant main effects for: physical functioning, role limitation due to physical functioning, general health perceptions and energy and vitality. The analysis also revealed the presence of numerous ceiling effects across domains. CONCLUSIONS: The domain structure of the SF-36 was demonstrated to be robust. However, the discriminatory ability of the measure was disappointing. The presence of ceiling effects and the low frequency of differences between intermediate disease severity groups indicated that the SF-36 was not discriminatory with respect to mild disease states or progression of illness.

Adolescent↗