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Biomedical subjects

M E Hodson

Publications and source records attributed to M E Hodson.

At least 145 records · Page 8Linked to original sources

Vasculitis complicating cystic fibrosis.

Twelve patients with skin vasculitis complicating cystic fibrosis are described. Seven of these were proven histologically and of these two had systemic vascultitis. Staining of vasculitic tissue by the avidin-biotin immunoperoxidase technique using both monoclonal and polyclonal antisera directed against Haemophilus influenzae, staphylococcus aureus and Pseudomonas aeruginosa did not consistently reveal any bacterial antigens in these tissues. In one patient the vasculitis appeared secondary to ranitidine. There was no evidence of autoimmune disease in any of the patients. Antineutrophil cytoplasmic antibodies were detected in the serum of 40 per cent of the patients with vasculitis complicating cystic fibrosis but in none of 61 controls with cystic fibrosis (but without vasculitis) matched for age and sex and with similar bacteriological flora of sputum.

Adult↗

Treatment of steatorrhoea in cystic fibrosis: a comparison of enteric-coated microspheres of pancreatin versus non-enteric-coated pancreatin and adjuvant cimetidine.

Enteric-coated microspheres of pancreatin were compared with non-enteric-coated pancreatin combined with cimetidine taken 40 min before meals in the treatment of patients with cystic fibrosis. Fourteen adults with steatorrhoea due to cystic fibrosis were investigated in an open, randomized crossover study, over two consecutive 28-day treatment periods. Lipase intake was adjusted to each patient's previous requirements and was the same during both months; they were instructed to continue with their normal diet. Patients collected faeces for 72 h at the end of each month and completed diary cards daily throughout. Bowel actions were less frequent on enteric-coated microspheres of pancreatin than on non-enteric-coated pancreatin/cimetidine (1.7 vs. 2.4/day; P less than 0.001) and stool character was improved (P less than 0.001). Mean daily faecal weight was similar on enteric-coated microspheres of pancreatin to that on the combination (254 g vs. 291 g; N.S.), whereas daily faecal fat excretion tended to be less on enteric-coated microspheres of pancreatin (21 g vs. 27 g; N.S.), and percentage fat absorption tended to be greater (81% vs. 73%; N.S.). Mean body weight increased by 0.3 kg on enteric-coated microspheres of pancreatin and fell by 0.1 kg on the combination (N.S.). These data indicate that enteric-coated microspheres of pancreatin are at least as effective as non-enteric-coated pancreatin with cimetidine in the treatment of steatorrhoea in cystic fibrosis.

Adult↗

The arthropathy of cystic fibrosis.

Musculoskeletal symptoms are frequent in cystic fibrosis (CF). Here the clinical features of 29 patients with CF who had significant arthropathy are described. Twelve had episodic arthritis (EA) characterised by repeated short attacks of severe, incapacitating polyarthritis, which in seven was associated with fever and erythema nodosum. Ten patients had hypertrophic pulmonary osteoarthropathy (HPOA). The onset of symptoms in the group with HPOA was usually later (mean age 20 years v 16 years for EA) and was associated with significantly worse lung function than in patients with CF, either without arthropathy or with EA. Seven patients had arthropathies which could not be classified as EA or HPOA.

Adolescent↗

Characterisation of the normal right ventricular pressure-volume relation by biplane angiography and simultaneous micromanometer pressure measurements.

The normal right ventricular pressure-volume relation was studied by recording biplane right ventriculograms with simultaneous high fidelity pressure recordings in 10 adults found to have normal coronary arteries and haemodynamic function at diagnostic cardiac catheterisation. Right ventricular volume was measured frame by frame from digitised ventriculograms by a modification of Simpson's rule. The accuracy of this method was tested in a study of 22 human and animal right ventricular casts. There was excellent agreement between calculated volumes and those measured by fluid displacement. The derived regression equations were used to correct right ventricular volumes calculated from in vivo studies. The mean (SD) end diastolic volume index for the group was 62 (13) ml/m2, the stroke volume index was 43 (8) ml/m2, and the ejection fraction was 62 (6)%. Right ventricular pressure-volume loops were generated by combining simultaneous volume and pressure curves. The normal right ventricular pressure-volume loop was triangular, departing significantly from the square or rectangle of the normal left ventricular pressure-volume loop. Ejection from the right ventricle began early during the pressure rise and continued as right ventricular pressure fell. As a result phases of isovolumic contraction and relaxation were difficult to define. These observations show that normal right ventricular pressure-volume relations differ considerably from those of the normal left ventricle, presumably reflecting the different loading conditions of the two ventricles.

Adult↗

Vitamin D and parathyroid hormone and bone mineralisation in adults with cystic fibrosis.

Vitamin D and parathyroid hormone concentrations were assessed in 31 adults with cystic fibrosis (mean age 24, range 17-52 years), in 28 of whom the bone mineral index in the forearm was also determined. Serum 25-hydroxyvitamin D was subnormal in eight patients, of whom five were receiving vitamin D supplements in standard doses. 1,25-dihydroxyvitamin D and parathyroid hormone concentrations showed no consistent abnormalities. The bone mineral index was lower in patients with cystic fibrosis (p less than 0.02) than in controls. Five patients with unequivocally reduced bone mineral index had a subnormal mean serum 25-hydroxyvitamin D and significantly worse lung function than the other patients. There was a positive correlation between age and bone mineral index (r = 0.68, p less than 0.001). Thus a significant proportion of patients with cystic fibrosis living in a temperate climate are at risk of vitamin D deficiency. Osteopenia is common and is probably related to a combination of hypovitaminosis D, delay in puberty, hypo-oestrogenism in women, and reduced physical activity, rather than to secondary hyperparathyroidism. Since most patients with deficiency of 25-hydroxyvitamin D were receiving oral supplements, parenteral vitamin D supplementation may be appropriate for selected patients who are unable to maintain adequate 25-hydroxyvitamin D concentrations despite oral vitamin D supplements.

Adolescent↗

Surgical management of pneumothorax in cystic fibrosis.

Twenty seven adults with cystic fibrosis who had had either a surgical pleurodesis or pleurectomy for the management of pneumothorax were studied. There were no significant differences in postoperative respiratory function, incidence of recurrent pneumothorax, or incidence of major postoperative complications between the two groups.

Cystic Fibrosis↗

Serum immunoglobulins and immunoglobulin G subclasses in cystic fibrosis related to the clinical state of the patient.

Levels of serum immunoglobulins and immunoglobulin G subclasses were measured in 32 cystic fibrosis (CF) patients, 30 asthmatics and 27 controls. When compared with the asthmatic patients and controls, the CF patients had raised levels of all IgG subclasses as well as total IgG, IgM and IgA, but there was not a statistically significant increase in IgE. The levels of immunoglobulins in the CF patients were examined in relation to the clinical features of the disease. Raised levels of IgG4 were related to levels of IgE, but these raised levels of IgG4 appeared to be part of a general increase in total IgG and not an isolated feature. There was a significant correlation between the total IgG level and its subclasses, IgG1, IgG2, IgG3, IgG4 and IgA. IgG1 was significantly correlated with IgG2 and IgG4; IgG2 with IgG4; and IgG4 with IgE. Total IgG was the immunoglobulin most closely correlated with poor lung function. Serum IgA was higher in patients with positive immediate skin prick reactions to pollens (p less than 0.005) and death within two years of the study was related to high levels of total IgG (p less than 0.01), IgG3 (p less than 0.001), IgA (p less than 0.001), and IgE (p less than 0.005).

Adolescent↗

Antibiotic treatment. Aerosol therapy.

Aerosol antibiotics in control studies have been shown to improve lung function and reduce the number of acute hospital admissions in patients with cystic fibrosis (CF). However, their administration is time-consuming in patients who must already take a large amount of medication daily. Aerosol antibiotics are expensive, but the cost is minimal compared with the cost of a hospital admission or intravenous antibiotics. When this method of administration of antibiotics first began to be widely used in the treatment of CF, there was much concern that hypersensitivity reactions might occur and that drug-resistant organisms would cause clinical problems. Minimal drug hypersensitivity has been reported, and there is no solid evidence that the small increase in resistance to some antibiotics associated with the use of aerosol antibiotics is in any way detrimental to the patients. These minor disadvantages must be weighed against the possible advantage of using aerosol antibiotics in any given clinical situation.

Adolescent↗

Oral ciprofloxacin compared with conventional intravenous treatment for Pseudomonas aeruginosa infection in adults with cystic fibrosis.

40 adult patients with cystic fibrosis (CF) were admitted to hospital with acute exacerbations of infection associated with isolation of Pseudomonas aeruginosa from sputum. The patients were randomly allocated (20 per group) to receive intravenous azlocillin 5 g and gentamicin 80 mg, or oral ciprofloxacin 500 mg. Both treatments were given three times a day for 10 days. The patients were assessed on days 1 and 10, and at 6 weeks. There was a significant improvement in lung function between days 1 and 10 in both groups (p less than 0.001). Significant improvement was maintained at 6 weeks after ciprofloxacin but not in the intravenous group. Improvement after ciprofloxacin was superior at day 10. Sputum weight decreased in both groups (p less than 0.001). Patient-recorded symptoms also improved in both groups. There was no serious toxicity or side-effects. Drug resistant organisms were isolated no more frequently after ciprofloxacin than after intravenous therapy. 17 of the ciprofloxacin-treated patients said they preferred oral treatment to intravenous therapy. Oral ciprofloxacin is a useful short-term treatment for patients with CF who are infected with Ps aeruginosa.

Acute Disease↗

Inhaled ceftazidime compared with gentamicin and carbenicillin in older patients with cystic fibrosis infected with Pseudomonas aeruginosa.

A randomized cross-over study was undertaken to compare nebulized (1) ceftazidime with (2) a combination of gentamicin and carbenicillin, and (3) saline, each given for 4 months, in patients with cystic fibrosis infected with Pseudomonas aeruginosa. Mean peak expiratory flow on ceftazidime, 299 litres/min, and on gentamicin and carbenicillin, 297 litres/min, were greater than on saline, 278 litres/min (P less than 0.02 and P less than 0.05 respectively). Similarly mean forced expiratory volume in 1 second on ceftazidime, 1.70 litres, and on gentamicin and carbenicillin, 1.70 litres, were greater than on saline, 1.48 litres (P less than 0.02 and P less than 0.01 respectively). Mean forced vital capacity on gentamicin and carbenicillin, 2.93 litres, was also greater than on saline (P less than 0.05). We were unable to demonstrate any difference in efficacy between the antibiotic regimens. The patients were admitted to hospital less frequently during the study year compared with the previous year (P less than 0.05). Sixty-nine per cent of patients had a clinically significant (20%) increase in forced expiratory volume in 1 second on an antibiotic regimen compared with that on entry to study, but a minority of patients appear not to respond to this form of treatment.

Adolescent↗

Platelet hyperaggregability in cystic fibrosis.

Platelet function was investigated in 15 patients with cystic fibrosis (CF) and in ten age-matched controls. Marked hyperaggregability of platelets to adrenaline, collagen and arachidonic acid was observed in platelet rich plasma (PRP) prepared from patients with cystic fibrosis. Thromboxane A2 (TXA2) release from these platelets was also markedly enhanced. Hyperaggregability and increased TXA2 release observed in patients with CF was not due to the higher platelet counts in these patients since hyperaggregability was observed even in those patients whose platelet counts were similar to those in controls. Platelet hyperaggregability and increased thromboxane release in these patients were also independent of their body weight and occurred despite supplementation with vitamin E. Hyperaggregability of platelets in CF may be clinically relevant since it may contribute to the pathogenesis of bronchoconstriction through the release of TXA2 and other bronchoconstrictor platelet products such as serotonin.

Adolescent↗

Amenorrhoea in cystic fibrosis.

Menstrual history in relation to nutritional status and lung function was assessed in 45 adolescent and adult patients with cystic fibrosis (CF). Twenty patients had regular menstrual cycles (of whom six had been pregnant), 12 had primary or secondary amenorrhoea, 11 had irregular cycles and two were premenarcheal. Patients with regular menstruation had a significantly higher body mass index and percentage body fat (estimated from skinfold thickness measurements) than those with amenorrhoea (P less than 0.001), and also better lung function (P less than 0.01). A linear discriminant analysis indicated that of the four indices studied percentage body fat was the most important predictor of menstrual function. Ovarian and uterine morphology were studied by ultrasonography in 28 patients and 17 healthy controls. In 10 patients multicystic ovaries were noted. Even in patients with regular cycles uterine size was reduced (P less than 0.01) and in those with amenorrhoea appeared to be related to thinness. Amenorrhoea in CF is an appropriate adaptive response in preventing pregnancy, but the resultant oestrogen deficiency and psychological effects must be considered.

Adolescent↗

A monocyte function test (complement receptor enhancement) to assess the extent of bronchial carcinoma.

A monocyte function test (complement receptor enhancement) was undertaken on 45 patients with suspected bronchial carcinoma in whom this diagnosis was subsequently substantiated. In 38 (84%) complement receptor enhancement was below a range of values previously established for monocytes from 48 healthy control subjects and from 12 patients with non-malignant respiratory diseases. It was found that all 38 patients with reduced complement receptor enhancement had either local or widespread metastases, as determined by the chest radiograph and a variable combination of chest tomography, liver, bone and brain scanning, computed tomography, fibreoptic bronchoscopy, bone marrow aspiration, liver function tests, mediastinoscopy, and surgical assessment, where appropriate. By contrast, at the time of presentation there was no clear relationship between abnormal values in several conventional screening haematological or biochemical tests and the stage of the tumour. These results suggest that complement receptor enhancement might serve as a useful pointer to the extent of the disease, since low values were associated with extension within the chest and very low values were found with widespread metastases.

Adult↗

Pharmacokinetics of contraceptive steroids in patients with cystic fibrosis.

The pharmacokinetics of the commonly used contraceptive steroids ethinyloestradiol and levonorgestrel were investigated after oral and intravenous administration in six women with cystic fibrosis. The results were compared with data obtained from healthy women of similar age. The total body clearance of ethinyloestradiol was significantly higher in the patients with cystic fibrosis (0.61 (SD 0.19) l/h/kg) than in control women (0.32 (0.16) l/h/kg; p less than 0.02). In addition, the oral bioavailability of ethinyloestradiol was greater in women with cystic fibrosis than in controls (76.9% (11.7%) compared with 47.3% (7.5%); p less than 0.001). As a result of these two changes, the area under the plasma concentration--time curve after an oral dose of ethinyloestradiol was similar in patients and controls. The pharmacokinetics of levonorgestrel did not differ significantly between patients with cystic fibrosis and healthy women. The data suggest that women with cystic fibrosis will receive similar contraceptive protection from these steroids as do healthy women.

Adult↗