A bone dysplasia for diagnosis.
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Biomedical subjects
Publications and source records attributed to R Astley.
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Forty-one children with osteogenesis imperfecta have been reviewed. A minority (7/41) showed small metaphyseal fractures, resembling those seen in non-accidental injury, but in all of these there was obvious generalized bone disease so that confusion with non-accidental injury did not occur.
Pulmonary venous injection of contrast medium in the near-wedge position produced a flow into the pulmonary arterial tree in 15 out of 21 children with cyanotic congenital heart disease. In 5 there was good opacification of both sides from a single injection. The technique provides an additional method of demonstrating the anatomy of the pulmonary arteries where surgical treatment is contemplated.
A case of pulmonary atresia with intact ventricular septum is reported in which total surgical correction was carried out successfully at 32 hours of age. Cardiac catheterisation at 17 months has revealed virtually normal haemodynamic and angiographic findings. Surgical correction of this condition in the neonatal period is discussed.
A long-term prospective follow-up of 113 children with vomiting due to a small hiatal hernia is described. When reviewed by the same clinical and radiological observers 20 or more years later, over 90% of unoperated non-stricture patients were asymptomatic whereas only 44% of the stricture and/or surgically treated group were without symptoms. Half or possibly more of the asymptomatic patients still had a hernia and it is possible that these may suffer a recurrence of symptoms later in adult life. The loculus of thoracic stomach tended to retain the same shape; there was a slightly better prognosis for the locular type of hernia compared with the tubular type. Complicating oesophageal strictures can decrease or disappear without surgery other than dilatation; the results of treatment by radical surgery were disappointing. There is need for an even more prolonged follow-up into later adult life.
Two brothers are described with identical features of short-limbed dwarfism, normal face, hands and feet, respiratory difficulties and mild scoliosis. It proved impossible to offer a definitive diagnosis, though radiological features as a whole bore similarities to those of metatropic dwarfism, with the most notable feature the narrowing of the ossified components of the vertebral bodies.
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Of 38 children investigated between 1966 and 1971 who had a blood lead concentration greater than 37 mug/100 ml eight had encephalopathy and one died; all these eight had a blood lead concentration of 99 mug/100 ml or above. Blood lead levels are related to haemoglobin concentrations and anaemia is common in children with blood lead concentrations of 37-60 mug/100 ml, levels previously accepted as harmless.Children with blood lead concentrations greater than 60 mug/100 ml show radiological evidence of lead intoxication, and treatment for this should be considered when blood lead concentration exceeds 37 mug/100 ml. Children presenting with unexplained encephalopathy should be radiographed for evidence of lead intoxication.
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