Biomedical subjects

M Mahon

Publications and source records attributed to M Mahon.

40 records · Page 3Linked to original sources

PubMedDate not supplied

An adult case of Andersen's disease--Type IV glycogenosis. A clinical, histochemical, ultrastructural and biochemical study.

A middle-aged man presented with a thirty-year history of progressive, asymmetrical limb-girdle weakness. The muscle biopsy revealed a vacuolar myopathy. The vacuoles which did not disrupt the fibre outline, lay in a subsarcolemmal position. They were PAS-positive and the material was partially resistant to diastase digestion. Electron microscopy showed the vacuoles to contain free unbound glycogen with filamentous material. Leucocyte brancher enzyme activity was normal but the muscle activity was less than half the control value. Histochemical and ultrastructural characteristics of the storage material resemble the amylopectin polysaccharide deposits seen in childhood Type IV glycogenosis.

Glycogen Storage Disease↗

PubMedDate not supplied

Quantitative histological changes in the small intestine of rats artificially reared on different milk substitutes.

Growth of the small intestine was studied in rats reared normally by their mothers (MR) or artificially reared (AR) by intragastric infusion of milk substitutes from postnatal day 5. Two milk substitutes were used: one high in carbohydrate and low in protein as compared with rats' milk (Messer) and the other closely resembling rats' milk in its composition (Auestad). Pups reared on these formulae are termed ARM and ARA, respectively. Pups were killed at 7, 12, and 20 days for quantitative histological measurements on transverse sections of duodenum and ileum. They included cross-sectional areas of muscle and of mucosa and submucosa combined (other tissue), internal and external perimeters, and length of longest villus profile. Artificial rearing affected mucosal and submucosal measures, but did not affect muscle. The effects depended on age, type of milk substitute, and site within the small intestine. There was a tendency for deficient growth of small intestine in the early stages of artificial rearing, especially in the ileum of ARA pups. Enhancement of growth as shown by cross-sectional area of mucosa and submucosa and by longest villus profile occurred later--by 12 days in the duodenum and by 20 days in the ileum of ARM pups. Enhanced growth was less evident in ARA rats, being apparent only in the duodenum, and not until 20 days were the cross-sectional area of mucosa and submucosa and the longest villus profile increased.

Animal Nutritional Physiological Phenomena↗

PubMedDate not supplied

Muscle morphometry in motor neuron disease.

It has previously been suggested that the pathological abnormalities seen in muscle biopsies from patients with motor neuron disease (MND) are of predictive value in relation to the rate of progression of the disease. In this study, quadriceps muscle biopsies from 19 patients with MND and 20 age matched controls were prepared for histochemistry and analysed morphometrically. Pathological features of denervation and reinnervation were observed in all MND patients although considerable variation between patients was noted. Motor neuron disease biopsies also showed increased connective tissue, an increased variation in fibre size, and a random fibre type distribution. Several of these abnormalities were more severe in female patients. Many of these 'abnormalities' were also frequent, albeit to a milder degree, in control biopsies and emphasize the need for age matched controls. The morphometric data was not related to the age of the patient, disease duration, type of MND or muscle strength, thus suggesting that the progression and severity of MND and its prognosis cannot be judged on the basis of quadriceps muscle pathology alone.

Adult↗

PubMedDate not supplied

Sequential studies of a childhood myopathy: a clinical, histochemical and morphometric investigation.

An unusual inherited progressive distal myopathy of early childhood onset is described in two sisters from a consanguineous Asian family. Motor milestones were normal but gait deteriorated slowly thereafter with development of generalized hypotonia and muscle weakness particularly in the wrist extensors and hand muscles. Muscle biopsies obtained at the ages of 6 and 10 years respectively (Case 1) showed significant differences. At 6 years muscle morphology and histochemical appearance were normal although type I fibres predominated (79%) and a substantial pool of 'undifferentiated' fibres (12%) was present. By 10 years there was a significant reduction in type I fibres (-13%) and in 'undifferentiated' fibres (-10%) with a concomitant increase in type II fibres (+23%). Fibre size and shape were normal at the age of 6 years but no further fibre growth was evident 4 years later. The older sister (Case 2, age 13 years) was similarly affected. The possibility of this progressive myopathy being caused by loss of neural control at two separate stages of development is discussed. The importance of performing sequential morphometric studies of muscle biopsies from patients with unusual childhood myopathies is emphasized.

Adolescent↗