Search PubMed⌕ Search

Biomedical subjects

J M Kirk

Publications and source records attributed to J M Kirk.

61 records · Page 4Linked to original sources
PubMed1986

Biochemical evidence for an increased and progressive deposition of collagen in lungs of patients with pulmonary fibrosis.

To assess the role of changes in lung collagen in pulmonary fibrosis, the content of this protein was measured in biopsy and autopsy lung from patients with cryptogenic fibrosing alveolitis (CFA), a fibrotic lung disorder of unknown cause. The collagen concentration was measured in lung samples from 21 patients with CFA (14 autopsy and seven open-lung biopsy) and 17 normal subjects; total lung collagen was determined in the right lung of 10 patients who died from CFA and the results were compared with those from 10 normal lungs. There was a wide variation in the collagen concentrations but the mean value (+/- SEM) for patients with CFA (217 +/- 13 mg/g dry weight) was significantly higher (P less than 0.02) than that of the controls (155 +/- 15 mg/g dry weight). The mean collagen concentration of the autopsy samples (243 +/- 20 mg/g dry weight) was significantly higher (P less than 0.05) than that of the biopsy samples (165 +/- 24 mg/g dry weight). The mean total collagen was markedly raised (P less than 0.001) in right lungs of patients with CFA (32.5 +/- 4.3 g) compared with normal lungs (14.0 +/- 1.1 g). When corrected for the predicted lung volume this difference in total lung collagen remained statistically significant (P less than 0.01, mean for patients 4.7 +/- 0.7 mg/ml, controls 2.3 +/- 0.2 mg/ml). These results demonstrate an increased deposition of lung collagen in this form of pulmonary fibrosis. They also suggest that there is a greater collagen concentration in lungs of patients with later disease, indicating a progressive deposition of collagen during the course of the disease.

Adult↗
PubMed1986

Full expression of Hunter's disease in a female with an X-chromosome deletion leading to non-random inactivation.

A 2.5-year-old girl who presented with abdominal distension, hepatomegaly, coarse facies, hirsutism and contraction deformities was investigated for mucopolysaccharidoses. Urinary excretion showed increased total glycosaminoglycans (105 mg/mmol creatinine; normal for age 9-20 mg/mmol) with marked increases of dermatan and heparan sulphates. A number of lysosomal enzyme activities were measured on leucocytes, serum and cultured fibroblasts. Normal or high activities were found for alpha-iduronidase, N-acetylgalactosamine-6-sulphatase, beta-galactosidase, arylsulphatase B and beta-glucuronidase. However a marked deficiency of iduronate sulphate sulphatase activity was observed, consistent with a diagnosis of Hunter's disease. Activities were reduced to less than 2% of mean control values in the patient's leucocytes, serum and cultured fibroblasts. Normal activities were measured in samples from the father and younger sister but a partial deficiency (43% of control serum) was found in the mother. Chromosome studies on the patient revealed a partial deletion of the long arm of one X-chromosome, most probably of band Xq25, which was not inherited from either parent. Studies using BrdU indicated that the deleted X chromosome was consistently late replicating, and as a result the Hunter gene was fully expressed on the other X chromosome.

Child, Preschool↗
PubMed1984

Quantitation of types I and III collagen in biopsy lung samples from patients with cryptogenic fibrosing alveolitis.

The role of changes in the relative amounts of types I and III collagen in cryptogenic fibrosing alveolitis was investigated using a biochemical method adapted for small amounts of human lung tissue. Measurements were performed in 18 biopsy and seven post-mortem lung samples from patients with cryptogenic fibrosing alveolitis and the results were compared with clinical, physiological, radiological and histological data. The mean percentage type III collagen, compared with type I collagen, was significantly lower in post-mortem lung samples from patients with cryptogenic fibrosing alveolitis who died from the disease compared with the biopsy lung samples (p less than 0.01) and with the control lung samples (p less than 0.05). There was a positive correlation between the percentage of type III collagen and response to treatment at six months as shown by the improvement in the percentage predicted Kco (p less than 0.005) and FVC (p less than 0.01) and with the radiological changes (p less than 0.05). These changes remained significant at one year (p less than 0.05 in all cases). These results suggest that patients with a higher proportion of type III collagen have earlier disease and show a better response to treatment than those with a lower proportion of type III collagen. The potential advantages of measuring collagen types for staging cryptogenic fibrosing alveolitis are discussed.

Aged↗
PubMed1984

Serum type III procollagen peptide concentration in cryptogenic fibrosing alveolitis and its clinical relevance.

The serum concentrations of a metabolite produced during synthesis type III collagen, type III procollagen peptide (PCP), were measured in patients with cryptogenic fibrosing alveolitis, and the results were related to the course of the disease and its response to treatment. The mean PCP concentration was significantly higher for patients untreated at the time of sampling than for an age and sex matched control group (p less than 0.001). Seventeen of these patients subsequently started treatment and their response to treatment was assessed by measurement of changes in the transfer coefficient (KCO) and forced vital capacity (FVC) at six months and one year later. Those patients showing a response to treatment, indicated by an increase in the percentage predicted KCO or FVC of more than 10% one year after starting treatment, had a significantly higher mean PCP concentration (p less than 0.01 and p less than 0.001 respectively) than patients with stable or deteriorating physiological indices. When the PCP concentrations of these patients were compared with repeat measurements three to six months after the commencement of treatment 14 out of 17 showed a decrease in PCP levels. This percentage change in PCP concentrations correlated significantly with the change in the percentage predicted KCO and FVC at 12 months after the commencement of treatment (p less than 0.01). These results suggest that measurement of PCP concentrations in patients with cryptogenic fibrosing alveolitis may be useful in the assessment of response to treatment.

Adrenal Cortex Hormones↗
PubMed1983

Measurement of osmolality and sodium concentration in heated-cup sweat collections for the investigation of cystic fibrosis.

A new system (Wescor) for sweat collection and analysis was examined with respect to its suitability for the investigation of children suspected to have cystic fibrosis. The effects of iontophoresis current, sweat collection time, sweat storage and analysis were examined, and as a result the technique was modified to allow collection and storage of sufficient sweat for sodium and potassium as well as osmolality assays in 10-20 minutes. The small electrodes and speed of the procedure make it practical for use with small children, with a reproducibility of 13-24% (coefficient of variation for whole procedure).

Adult↗
PubMedDate not supplied

Pituitary gigantism causing diabetic ketoacidosis.

Although growth hormone excess (acromegaly) in association with glucose intolerance and diabetes mellitus is well documented in adult medicine, it is much less common in the paediatric age group. We report the case of a 13 year-old boy who presented with tall stature secondary to a large growth hormone secreting adenoma of the pituitary gland. Random growth hormone was 630 mIU/l and did not suppress during an oral glucose tolerance test. Following debulking of the tumour, he developed diabetic ketoacidosis requiring insulin treatment, but after further surgery glucose handling returned to normal. He has been started on testosterone to arrest further increase in height.

Adenoma↗