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Biomedical subjects

D J Matthew

Publications and source records attributed to D J Matthew.

53 records · Page 3Linked to original sources

Circulating soluble immune complexes containing pseudomonas antigens in cystic fibrosis.

In order to investigate whether circulating immune complexes containing Pseudomonas aeruginosa antigens mediate pulmonary damage in cystic fibrosis, we studied lung function, serum immune complex levels, and immunoglobulin concentrations in relationship to chronic pseudomonas colonisation in 69 affected children. Sixteen of the children with cystic fibrosis had increased levels of immune complexes which contained pseudomonas antigens. There was no significant relationship between lung function corrected for the effect of chronic pseudomonas colonisation and the presence of such complexes or increased levels of complexes detected by Cl1 binding or raised serum immunoglobulin concentrations. Our results suggest that these abnormalities in cystic fibrosis are secondary effects of chronic infection and they do not provide evidence for immune complex mediated lung damage in this disease.

Adolescent↗

Pseudomonas infection, allergy, and cystic fibrosis.

The clinical significance of the high prevalence of positive immediate skin tests in cystic fibrosis is unclear. Using analysis of variance, we have tested the hypothesis that patients with allergic cystic fibrosis have worse lung disease than non-allergic patients. Clinical data, skin prick tests, total or specific IgE antibody levels, chest radiographs, and pulmonary function tests were obtained in 104 cystic fibrosis patients. Patients with positive immediate skin reactions to at least one allergen were more likely to be persistently colonised by Pseudomonas aeruginosa than skin test negative patients. The skin test positive patients were also significantly older (mean difference 2.15 years). Analysis of variance showed that pseudomonas infection was the most significant factor contributing to lung damage and the effect of allergy was not significant. Similar longitudinal analysis of pulmonary function over 5 years and study of the hospital admission rate showed that the only statistically significant factor associated with deterioration was colonisation with P. aeruginosa.

Adolescent↗

The effect of cimetidine on meal-stimulated gastric function and exogenous pancreatic enzymes in cystic fibrosis.

Some patients with cystic fibrosis (CF) have malabsorption of fat and protein in spite of large amounts of supplemental pancreatic enzymes. This is partly due to acid inactivation of exogenous pancreatic enzymes in the stomach. The effect of cimetidine on gastric function and exogenous pancreatic enzymes was assessed by a marker perfusion technique in 4 CF children in a double-blind controlled fashion. Gastric acid secretion was higher in CF patients than in controls (P less than 0.005) and was reduced significantly by oral cimetidine (P less than 0.02). Rapid inactivation of exogenous trypsin and lipase occurred when gastric pH fell to less than 4.5. There was no loss of enzyme activity during treatment with cimetidine when gastric pH remained above 5.5. Activity of lipase and trypsin in the jejunum improved in all subjects. Fat and nitrogen absorption assessed by a balance technique during the study period showed a small improvement in fat absorption while on cimetidine. We conclude that some CF patients have a high meal-stimulated gastric acid output which causes inactivation of trypsin and lipase. Cimetidine was effective in reducing acid secretion in such patients and led to small improvements in fat absorption.

Adolescent↗

Shwachman's syndrome. A review of 21 cases.

21 patients (10 male, 11 female) aged between 11 months and 29 years with Shwachman's syndrome are reviewed. All patients had exocrine pancreatic insufficiency. Haematological features included neutropenia in 19 (95%), anaemia in 10 (50%), and thrombocytopenia in 14 (70%); one patient developed erythroleukaemia. Severe infections occurred in 17 (85%) from which 3 (15%) died. Only one child exceeded the 3rd centile for height, and growth retardation was particularly evident in the older patients. All had skeletal abnormalities or delayed skeletal maturation, or both. Metaphyseal dyschondroplasia affected 13 of the older patients and was associated with skeletal deformities. Eight of 9 children under 2 1/2 years had rib abnormalities. Respiratory function tests in children under 2 years demonstrated reduced thoracic gas volume and chest wall compliance. Older patients had reduced forced expiratory volume and forced vital capacity. Neurological assessment showed developmental retardation or reduced IQ assessments, or both, in 85% of patients studied. Other neurological abnormalities included hypotonia, deafness, and retinitis pigmentosa. Neonatal problems had been present in 16 (80%) of the patients and 5 were of low birthweights. Hepatomegaly with biochemical evidence of liver involvement occurred in the younger patients and resolved with age. Other associated features included dental abnormalities, renal dysfunction, an icthyotic maculopapular rash in 13 (65%), delayed puberty, diabetes mellitus, and various dysmorphic features. These findings stress the diverse manifestations of the syndrome and extend knowledge on a number of aspects. Sibship segregation ratios support an autosomal mode of inheritance and an hypothesis for the pathophysiological basis of this syndrome is advanced.

Adolescent↗

Controlled trial of intermittent aerosol therapy with sodium 2-mercaptoethane sulphonate in cystic fibrosis.

Twenty-seven patients with cystic fibrosis completed a controlled trial comparing the effects of an inhaled mucolytic drug, sodium-2-mercaptoethane sulphonate (Mistabron, UCB Pharmaceutical Division, Brussels, Belgium), with inhaled iso-osmolar hypertonic saline. As a group the 22 patients with chronic sputum production showed small but statistically significant improvement in pulmonary function tests after Mistabron therapy, both when compared with a control period, and with iso-osmolar saline results. Subjective measurements by diary card failed to show any changes. No significant changes were found in five patients with no measurable sputum production. The inhalations were given after physiotherapy and were well tolerated. There were no significant side effects. The results suggest that Mistabron has a beneficial therapeutic effect unrelated to its high osmolality, and the intermittent inhalation of Mistabron may have a role in the treatment of selected patients with cystic fibrosis.

Adolescent↗

Short-term effects of chest physiotherapy on pulmonary function in children with cystic fibrosis.

In a short-term controlled study of 20 patients with cystic fibrosis and chronic sputum production, chest physiotherapy resulted in a significantly improved peak flow over a whole day, with no significant difference in other forced expiratory flows, when compared to a control day. This suggests that physiotherapy may be effective in enhancing clearance of sputum from central large airways, but it has little obvious effect on more peripheral airways. A bronchodilator given before physiotherapy had no added effects. It is important that we examine ways of improving physiotherapy to aid clearance of secretions from the more peripheral airways.

Adolescent↗

Response to bronchial provocation and exercise in children with cystic fibrosis.

Ten of fifteen cystic fibrosis children with positive skin prick tests to common antigens gave an immediate bronchial reaction to the antigen inhaled, five of them also gave a late reaction; however only one gave a history of asthma. The antigen most commonly eliciting a positive skin reaction in cystic fibrosis patients is Aspergillus fumigatus. In six children tested to this antigen the bronchial response varied, two were negative, one gave an immediate reaction and three gave a dual (immediate and late) reaction. None of the children showed the characteristic pattern of response to exercise seen in asthmatic patients, an initial rise in Peak Expiratory Flow Rate followed by a fall of greater than 14% below the resting level. Two patients showed an abnormal rise in Peak Expiratory Flow Rate during exercise, a pattern described previously in cystic fibrosis. The results suggest that bronchial allergy, immediate or late does completely explain susceptibility to asthma, and that other factors including perhaps the type of bronchial reactivity shown by bronchoconstriction after exercise may be required. However the majority of the children tested had bronchial allergy and anti-allergy therapy such as inhaled sodium cromoglycate may have a place in the management of selected patients with cystic fibrosis.

Adolescent↗

Prevention of eczema.

In a prospective study of the development of reaginic allergy, infants of allergic parents were either subjected to an allergen-avoidance regimen from birth for six months or managed conventionally. The group on the allergen-avoidance regimen had less eczema at six months and one year than did the control group at six months. They also had a lower mean serum-total-IgE level at six weeks.

Allergens↗

The relationship between chest radiographic scores and respiratory function tests in children with cystic fibrosis.

Chest radiographic scores and respiratory function on 80 sets of results from 50 patients with Cystic Fibrosis were analyzed. Chest radiographic scores were assessed independently using the method of Chrispin and Norman. Respiratory function tests were found to correlate well with the chest radiographic score, the best correlation being with the forced expiratory volume in 0.75 sec. to forced vital capacity ratio F.E.V. 0.75/ F.V.C. (r = -0.674 n = 80 p less than 0.001).

Adolescent↗

Virological findings and blood gas tensions in acute lower respiratory tract infections in children.

Sequential blood gas tensions and pH have been measured in 84 children selected from 486 admitted to hospital during a 15-month period with acute lower respiratory tract infections. Of those selected 73 were treated conservatively and 11 by intermittent positive-pressure ventilation; one infant in the latter group died. Respiratory syncytial virus (R.S.V.) was isolated from 24 out of 62 patients studied and the main pathogen in the most severely affected infants. Statistical analysis showed that age and R.S.V. infection were independent determinants of severity, as reflected by a peak Pco(2) measurement at the height of the illness (age, P < 0.01; R.S.V. 0.05 > P > 0.01).

Age Factors↗

Acute respiratory failure in bronchiolitis and pneumonia in infancy. Modes of presentation and treatment.

The modes of presentation and the management of acute respiratory failure in 11 infants with severe lower respiratory tract infections (due to respiratory syncytial virus in eight) are described. Progressive respiratory difficulties leading to exhaustion, peripheral circulatory collapse, recurrent apnoeic attacks, or generalized convulsions were the main clinical presentations resulting in severe ventilatory failure. In nine infants preventilation carbon dioxide tensions exceeded 65 mm Hg. It seems likely that the use of intermittent positive-pressure ventilation in these patients contributed to the low mortality rate, less than 0.5%, from such illnesses during the 15-month study period.

Acid-Base Equilibrium↗