Nuclear magnetic resonance--its application to diagnostic radiology.
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Biomedical subjects
Publications and source records attributed to B J Cremin.
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A prospective study of 100 consecutive stillbirths/perinatal deaths was performed by routine radiography and subsequent autopsy. The object of the study was to correlate the clinical and the autopsy causes of death with any abnormal radiographic findings. A prime objective was to study the incidence of major skeletal abnormalities in the series and to ascertain if routine radiography could be used to detect abnormalities that might require genetic counselling. No skeletal abnormalities or dysplasias were detected that did not have obvious external stigmata. The use of routine radiography on undeformed stillbirths is not recommended.
The fetal alcohol syndrome has recently been clinically recognised and has a characteristic facial phenotype. The condition is the result of chronic maternal alcohol ingestion affecting the first trimester. A survey of our cases has shown that skeletal maldevelopment is a part of the syndrome and can help in its early recognition.
Both childhood and adult polycystic disease is seen in our series of eleven children, demonstrating the broad spectrum of both renal and hepatic disease clinically and radiographically. The relationship of radiographic patterns and survival is assessed. Confusion in the definition and classification of these disease entities is discussed.
Takayasu arteritis with renovascular hypertension in an 8-year-old girl is described. At the onset both renal arteries were affected, but 2 years later one renal artery had been recanalized and the uncontrolled hypertension was cured by contralateral nephrectomy.
A ten year old South African girl of mixed ancestry presented with gross facial abnormalities and dwarfism consequent upon a bizarre skeletal dysplasia. The main radiographic features were craniostenosis, fibrous dysplasia, metaphyseal lucencies and platyspondyl. Intelligence was normal and there were no systemic ramifications. The term "osteoglophonic dwarfism" is a succinct and apt designation for this remarkable disorder.
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Craniotubular dysplasias and craniotubular hyperostoses are a group of confusing disorders in which the cranial involvement may cause compression of facial and auditory nerves. Two of the hyperostotic conditions that regularly produce cranial nerve compression are sclerosteosis and the recessive form of endosteal hyperostosis (van Buchem's disease). Both have identical radiological features with cranial involvement presenting in childhood. The Dutch and South African cases have come from relatively close-knit communities, those in the latter country being Afrikaners who had their origins in Holland. The radiological features of three such cases of sclerosteosis are briefly presented.
Osteopetrosis is an unusual bone disorder in which the skeleton is radiographically dense. The condition is conventionally subclassified into a benign autosomal dominant adult form and a malignant autosomal recessive variety. Among 14 affected individuals whom we have studied, 4 adults had an intermediate type of osteopetrosis in which serious complications included osteomyelitis, pathological fractures and dyshaemopoiesis. The fundamental biochemical relationship of this disorder with the classic forms of osteopetrosis is uncertain. The osteopetroses must be distinguished from other sclerosing bone conditions which have a different course and prognosis. Sclerosteosis and craniometaphyseal dysplasia, both of which occur in South Africa, are of practical importance in this context.
Six cases of infantile cortical hyperostosis or Caffey's disease are presented, with opinions as to its prevalence, incidence and origin.
Objectives of this study were to evaluate some of the factors influencing vesico-ureteric reflux and intrarenal reflux. Reflux occurs in about one-third to a half of Caucasian children with urinary tract infection and although not greatly influenced by examination technique or sex it is affected by age, diuretics and race. Intrarenal reflux occurs in about 10% of cases with total reflux and does not appear to cause scars on its own. Autopsy studies can provide valuable information on papillary morphology and reaction to pressure, but information is of doubtful reliability in the first months of life and in fixed specimens. The literature pertaining to vesico-ureteral reflux, intrarenal reflux and related research projects is briefly reviewed.
Hypochrondroplasia is an inherited skeletal dysplasia that resembles achondroplasia in mild degree. Radiographic manifestations encountered in 12 affected individuals in South Africa include slight shortening of all segments of the tubular bones, moderate caudal diminution of the lumbar interpedicular distances, increased lumbar lordosis with sacral tilt and distal prolongation of the fibula. Hypochondroplasia can be distinguished from other osteochondrodystrophies such as achondroplasia, pseudoachondroplasia and metaphyseal chondroplasia by the recognition of its clinical and radiographic manifestations.20
Non-function of a kidney invaded by nephroblastoma has not been extensively documented and may be featured only when a relatively large series is reviewed. It occurred in nine (15.5%) of 58 cases seen over an eight year period. Two of these nine cases had bilateral involvement and one showed curvilinear calcification. The mechanism of non-function appears to be due to replacement of most of the kidney substance by tumour, and this may be augmented by invasion of the renal pelvis causing malignant hydronephrosis. Ultrasonography has become increasingly utilised in confirming the diagnosis of renal tumours, but arteriography may still be the critical examination in the 'unusual' case.
Pyle disease is a rare genetic skeletal disorder which is conventionally classified with craniotubular dysplasias. The radiographic manifestations in three affected adults included widening of the metaphyseal portions of the long bones which extended through a major portion of the diaphyses, with cortical thinning and mild cranial sclerosis. The femora presented the characteristic Erlenmeyer flask configuration. Pyle disease is clinically, radiographically and genetically distinct from craniometaphyseal dysplasia, a relatively common condition with which it has been confused.
Intravenous cholangiography (IVC) was performed on 102 children aged between one and 13 years. Eighty of the patients who were non-icteric were divided into four groups and received contrast medium containing either 53, 75, 106 or 150 mg iodine/kg body weight by intravenous injection over ten minutes. Complete demonstration of the common bile duct (CBD) was achieved in 85% of patients and in a similar proportion of all age groups. There was some delay in opacification of the CBD in young children but this was not of practical significance. The two higher dosage regimen gave significantly better results than the two lower dosages (p less than 0.001) and this improvement was evident in the young and older age groups. Nausea and vomiting occurred as side effects in 6%. In eight patients in whom there was inadequate demonstration by the standard IVC, an infusion technique over 3--8 hours was carried out. This improved the demonstration in three children with diagnostic results in two.
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