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Biomedical subjects

D E Stableforth

Publications and source records attributed to D E Stableforth.

At least 37 records · Page 2Linked to original sources

Serum IgG response to Burkholderia cepacia outer membrane antigens in cystic fibrosis: assessment of cross-reactivity with Pseudomonas aeruginosa.

Burkholderia cepacia (Pseudomonas cepacia) is now recognised as an important pathogen in cystic fibrosis patients, and several reports have suggested that sputum-culture-proven colonisation occurs despite the presence of specific antibody. In an attempt to establish the use of antibody studies as diagnostic and prognostic indicators of B. cepacia infection, we have examined the IgG response to B. cepacia outer membrane proteins and lipopolysaccharide in patients also colonised with P. aeruginosa. The B. cepacia strains were grown in a modified iron-depleted chemically defined medium and outer membrane components examined by SDS-PAGE and immunoblotting. IgG antibodies were detected against B. cepacia outer membrane antigens, which were not diminished by extensive preadsorption with P. aeruginosa. The response to B. cepacia O-antigen could be readily removed by adsorption of serum either with B. cepacia whole cells or purified LPS, whereas we were unable to adsorb anti-outer membrane protein antibodies using B. cepacia whole cells. The inability to adsorb anti-outer membrane protein antibodies using B. cepacia whole cells maybe due to non-exposed surface epitopes. Several B. cepacia sputum-culture negative patients colonised with P. aeruginosa had antibodies directed against B. cepacia outer membrane protein. this study suggests that there is a specific anti-B. cepacia LPS IgG response, which is not due to antibodies cross-reactive with P. aeruginosa. Our studies indicate that much of the B. cepacia anti-outer membrane protein response is specific and not attributable to reactivity against co-migrating LPS.

Adolescent↗

Outcome of pregnancy in women with cystic fibrosis.

BACKGROUND: As women with cystic fibrosis are living longer, pregnancy is becoming increasingly common. The combined experience of pregnancies in women with cystic fibrosis from adult centres in the Midlands and North of England has been examined. METHODS: A retrospective study of the case notes of 22 pregnancies in 20 patients with cystic fibrosis examined changes in lung function, body weight, and microbiological status during the course of pregnancy. Duration of pregnancy, birth weight, and maternal survival were amongst other variables studied. The relation between values before pregnancy and important outcome measures were examined. RESULTS: Eighteen of 22 pregnancies were completed producing healthy, non-cystic fibrosis infants (12 female). Mothers lost 13% of FEV1 and 11% of FVC during pregnancy, most of which was regained. Body weight changes were variable, but most mothers gained weight (mean weight gain 5.7 kg). Microbiological status remained unchanged. Six infants were preterm and two were light for dates. Four mothers died up to 3.2 years following delivery. Of the prepregnancy parameters examined, %FEV1 showed the best correlation with maternal weight gain, gestation, birth weight, and maternal survival. CONCLUSIONS: Pregnancy was well tolerated by most mothers with cystic fibrosis although those with moderate to severe lung disease (%FEV1 < 60%) before pregnancy fared worse, producing preterm infants and suffering increased loss of lung function and mortality compared with mildly affected mothers. Prepregnancy %FEV1 appears to be the most useful predictor of important outcome measures in pregnancies in women with cystic fibrosis.

Adolescent↗

Use of a Heimlich flutter valve for pneumothorax in cystic fibrosis.

The use of a Heimlich flutter valve in an adult patient with cystic fibrosis with hypercapnic respiratory failure which allowed resolution of a persisting pneumothorax after failure of conventional tube drainage is reported. The patient was managed at home and avoided surgical pleurodesis which could have jeopardised transplantation at a later date.

Adult↗

The chest radiograph in cystic fibrosis: a new scoring system compared with the Chrispin-Norman and Brasfield scores.

BACKGROUND: Scoring systems for the chest radiograph in cystic fibrosis are used to compare patients and different treatment regimens, and to monitor respiratory disease in individual patients. The Northern chest radiograph score was designed to allow one person to assess the radiological features of lung involvement in cystic fibrosis in as simple, rapid and equally reproducible manner as the established Chrispin and Norman, and Brasfield scoring systems. METHODS: Forty five chest radiographs were scored by 10 physicians with a special interest in cystic fibrosis according to the Brasfield and Northern methods, and by five pairs of physicians according to the Chrispin-Norman criteria. Three individuals and two pairs rescored the radiographs after an interval of 3-5 months. The Northern score was performed with and without a lateral view, using the original posteroanterior radiograph. RESULTS: The Northern score showed a better agreement between observers for the ranking of the radiographs. It was equally well related to respiratory function tests, the Shwachman-Kulczycki score of overall clinical status, and in its discrimination between different radiographs as the other two scoring systems. The Northern score performed equally well with or without a lateral film. CONCLUSIONS: The Northern system fulfils the requirements of a chest radiograph score more successfully than the Chrispin-Norman or Brasfield systems, and does not require a lateral film.

Cystic Fibrosis↗

Epidemiology of Pseudomonas aeruginosa infection in cystic fibrosis and the use of strain genotyping.

We describe a study of the epidemiology of Pseudomonas aeruginosa (PA) infection in a group of adults with cystic fibrosis who attended a week-long summer camp in the U.K. Sputum samples were collected from 17 patients at the beginning and at the end of the holiday period. Examination of previous sputum samples had identified 11 patients who were chronically colonised with PA. They shared accommodation during the holiday. The sputum samples from these 11 patients were analysed so as to identify the strains of PA by their genotypic characters. All patients were colonised by unique strains before the beginning of the holiday, with the exception of two pairs of patients whose isolates were indistinguishable. After the holiday, eight of the 11 patients harboured strains of the same genotype as was found in their pre-holiday specimens. In three patients, a strain present post-holiday was different from that found in the pre-holiday specimen. In addition, in the case of one patient, two different genotypes were found in the pre-holiday specimen, only one of which was present after the holiday. Evidence of cross-infection of PA during the holiday was not found. Even so, evidence of person-to-person transmission of PA both within the hospital environment and through social contact is presented and discussed.

Adolescent↗

Endurance exercise capacity in adults with cystic fibrosis.

We have compared the maximal and endurance exercise capacities in 22 (15 male) adult cystic fibrosis (CF) patients with a resting oxygen saturation (SaO2) > or = 90%, with age and sex matched controls (CON). The maximum oxygen uptake (VO2max) and heart-rate were lower for the CF group whereas the maximum ventilation reached a higher percentage of predicted. Furthermore, for the CF group there was a relationship between the % predicted VO2max and measures of lung function, confirming a ventilatory limit to maximum exercise in patients with more severe disease and a near normal maximum exercise capacity in those with mild disease. Endurance capacity, defined as the duration of exercise at 80% of each individuals VO2max, was however similar for the two groups [CF: 22.21 (15.82) vs. CON: 24.94 (13.05) min]; despite the CF group exercising under less efficient ventilatory conditions. Endurance capacity was not related to the measurements of lung function. Of the seven CF patients desaturating at maximal exercise (SaO2 < 90%), five desaturated at a work load of 80% VO2max, whereas only one desaturated at 50% VO2max. This study confirms that with increasing severity of disease, maximal exercise capacity may be limited in adult CF patients, whereas endurance capacity (at the same relative work load) is unimpaired. Furthermore, CF patients who desaturate during a progressive maximal exercise test are likely to desaturate during constant work load exercise if the intensity is high but unlikely to do so if the intensity is low. The information from these exercise tests would be of value for prescribing individual training programmes and for evaluating the effects.

Adolescent↗

Epidemic of Pseudomonas cepacia in an adult cystic fibrosis unit: evidence of person-to-person transmission.

An epidemic of Pseudomonas cepacia occurred in an adult cystic fibrosis center in the United Kingdom, despite a policy of segregation of infected and noninfected patients within the hospital. Investigation of the outbreak by ribotyping and pulsed-field gel electrophoresis to characterize P. cepacia strain genomes together with inquiry into social contacts between patients revealed evidence of person-to-person transmission outside the hospital environment. Segregation policies aimed at reducing the spread of this infection in the cystic fibrosis community need to encompass patient contacts outside the hospital environment.

Adolescent↗

A district confidential enquiry into deaths due to asthma.

BACKGROUND: The aim was to establish a continuing district based confidential enquiry into deaths from asthma. METHODS: A confidential enquiry was conducted in an English health district. Subjects comprised 24 residents of the Norwich health district aged between 16 and 65 years who had died between 1988 and 1991 with asthma as the principal cause of death. RESULTS: Twenty one of the patients (88%) died away from hospital. Overall the routine asthma management was appropriate in all respects in only four patients. In five cases the drug treatment was considered inappropriate, in 10 cases (42%) there was no written evidence that the patient had received advice and education, and only six cases had a written management plan. In 17 patients (71%) the fatal attack of asthma developed rapidly (in under three hours). The medical care during the final attack was found to have been inappropriate in six cases. Seventeen cases (71%) had psychological or social factors that were considered to have been of potential importance. CONCLUSIONS: This study has shown the feasibility of organising a confidential enquiry into asthma deaths within a health district. The distinguishing features of such an enquiry are that it is continuing, that the quality of care given to those patients who died is compared against a recognised standard, and that there is a structured system for feeding back the conclusions of the enquiry to the local medical community.

Asthma↗