Examination of abortuses.
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Biomedical subjects
Publications and source records attributed to A J Barson.
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Two cases of hydrocolpos are described presenting soon after birth. One infant who died on the first day had an intrauterine peritonitis caused by compression of the caecum on the pelvic brim. The other surviving infant developed a caecal perforation secondary to Hirschsprung's disease. Neither infant had rectal atresia, which is the commonest cause of intestinal obstruction in these infants. The association of hydrocolpos with polydactyly in one of these cases may represent an autosomal recessive trait.
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Postmortem radiographs from 112 infants with thoracolumbosacral or lumbosacral myelomeningoceles were surveyed for associated vertebral and rib malformations. Sixty-four exhibited a variety of anomalies including hemivertebrae, fused vertebral bodies, fused vertebral arches, fused ribs, absence of ribs and absence of vertebral bodies. Anomalies were commonly associated with the cranial end of a dysraphic spina bifida region; in addition hemivertebrae and rib fusion exhibited peak incidences centred around the seventh cervical and fourth thoracic vertebra respectively. Fusion of vertebral arch elements increased in frequency throughout the thoracic region towards T10. The twelfth pair of ribs were absent in 20% of cases. Apparent absence of vertebral bodies from the caudal end of the vertebral column was seen in 24% of the radiographs.
Four cases of a locally invasive sarcoma of the chest wall are described in children aged between 8 and 14 years. Although its morphology resembles a Ewing's sarcoma of bone there are light and electron microscopical features that are distinctive. The sarcoma is thought to represent an entity which has not previously been defined. Its clinical importance lies in the fact that the prognosis with appropriate treatment appears to be good. One child died after 2 1/2 years, but the others are alive 6, 8, and 16 years after presentation.
Four cases of fat embolism are described in infants receiving prolonged intravenous infusion of fat (Intralipid 20%). This therapeutic complication has been termed 'fat overloading syndrome' but bears a clinical similarity to post-traumatic fat embolism. These 4 cases are the first to be recorded in infancy, and with histopathological proof of fat embolism. These 4 cases are the first to be recorded in infancy, and with histopathological proof of fat embolism. Transient high rates of infusion of Intralipid appears to be a factor in the aetiology of the condition.
A technique is described for the construction of life-size three-dimensional models of infants with external evidence of disease. The use of these for teaching and research purposes is outlined.
Anterior pituitary glands from 200 fetuses and infants ranging in age from 22 weeks' gestation to one year of life were examined histologically for the presence of concretions. Characteristically these are round, lamellated bodies, often strongly basophilic, containing phosphates and measuring between 10 micrometer and 70 micrometer in diameter. They decreased in incidence postnatally and were rarely seen 6 months after birth. It is suggested that these concretions are a degenerative change in cells subject to the vigorous secretory activity that is part of the normal physiology of the anterior lobe of the pituitary from mid-gestation.
Ten human adult spinal cords were assessed for weight, length, thickness, cholesterol, water and DNA-P content on both a regional and a mean segmental regional basis. The mean segmental DNA-P content, unlike the other parameters, was exceptional in being constant in the cervical, thoracic and lumbar regions. It is concluded that the cellularity of the cord between C1 and L5 is determined segmentally, but is reduced in the segments of the sacrococcygeal cord because of embryonic atrophy.
A case of idiopathic arterial calcification is described in a dysmature infant dying of massive pulmonary haemorrhage on the fourth day after a gestation of 36 weeks. The mother had disseminated lupus erythematosis and lupus nephritis treated with large amounts of prednisolone. Unlike most of the previously recorded cases of idiopathic arterial calcification of infancy subintimal proliferation of fibrous tissue and involvement of the coronary arteries did not occur. It is suggested that this non-occlusive form of arterial calcification may be a distinct entity.
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The spinal cords were removed from 29 infants between 17 and 46 weeks gestation, all with various forms of spinal dysraphia. The spinal cords were assessed for weight, length, thickness, size and number of cells, and myelination, and then were compared with 86 control spinal cords. The strongest characteristic of the dysraphic cords was a reduced degree of myelination. Cell size and number generally were appropriate for the weight of the cords. There was a tendency for cord weight, length and thickness to be reduced, though in part this was a reflection of poor over-all bodily growth. In a small group of infants these measurements were increased in comparison with the controls.
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Twenty-six cases of endocardial fibroelastosis were collected from three hospitals in Manchester over a ten-year period. Nine cases occurred in 4 families and these are discussed in detail. X-linked recessive inheritance seems likely in one family in which two probable female carriers had subarachnoid haemorrhages. In a second family an apparently normal man produced two children with endocardial fibroelastosis by different mothers suggesting autosomal dominant inheritance with incomplete penetrance. Autosomal recessive inheritance may be involved in the remaining two families but this was not associated with consanguinity. Genetic heterogeneity is evident in endocardial fibroelastosis and the majority of cases occur sporadically. An accurate family history is therefore necessary but it is difficult to give precise recurrence risks in sporadic cases.
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