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Biomedical subjects

P Sprague

Publications and source records attributed to P Sprague.

At least 19 recordsLinked to original sources

Metaphyseal chondrodysplasia, type Jansen.

The Jansen type of metaphyseal dysplasia is a rare disorder with significant clinical and radiographic variability. Two cases of classical Jansen disease and one with some distinctive features suggestive of the Jansen variant are reported.

Child↗

Dominant craniometaphyseal dysplasia--a family study over five generations.

A two month old male infant being investigated for nasal obstruction was noted to have the typical facies and radiological changes of craniometaphyseal dysplasia. Investigation of the family detected 9 individuals in 4 generations with radiological evidence of craniometaphyseal dysplasia. Their ages ranged from 2 months to 70 years. Three presented with nasal obstruction, one with facial nerve dysfunction and three had developed deafness in or before their third decade. Two were asymptomatic. One family member, now dead, was known to have had early onset of deafness but had not been examined or X-rayed. All of the affected individuals were noted to have had the typical facies in childhood. Characteristic radiological findings in the cranium and long bones were present to a varying degree and were most prominent in those presenting in infancy.

Adolescent↗

Primary rib tumours in children (report of 27 cases with short literature review).

27 cases of primary malignant and benign rib tumours are reported. The most common malignant rib tumour in childhood is Ewing sarcoma (20 cases). Some other rare bone tumours and tumorous conditions (reticulosarcoma, aneurysmal bone cyst, monostotic rib eosinophilic granuloma, osteoid osteoma and lymphangioma) are also described. In the authors' opinion Ewing sarcoma presents with characteristic clinico-radiographic findings in most of the cases. Other monostotic, primary rib tumours and tumorous conditions in childhood--with the exception of exchondroma and enchondroma--rarely show diagnostic radiographic features.

Adolescent↗

Primary bone tumours of the pelvis in childhood--Ewing's sarcoma of the ilium, pubis and ischium (report of 30 cases). (Part I).

30 children with Ewing's sarcoma, the most common malignant pelvic tumour in childhood, were analysed. The diagnosis of Ewing's sarcoma is relatively easy and can be established in most of the cases on plain radiography. The diagnostic radiographic features of the tumour are discussed. The two most important conditions in differential diagnosis are eosinophilic granuloma and the rare primary bone lymphoma. Osteomyelitis should rarely cause confusion unless the clinico-radiographic findings are not properly evaluated.

Bone Neoplasms↗

Primary bone tumours of the hand. Report of 21 cases.

Twenty-one primary bone tumours of the hand in children from 8 paediatric hospitals are reported. Osteochondromas and enchondromas were not included. Our material consisted of 16 patients with common tumours (3 Ewing's sarcoma, 5 aneurysmal bone cyst, 6 osteoid osteoma and 2 epidermoid cyst) and 5 patients with uncommon tumours (osteoma, simple bone cyst, haemangiopericytoma, capillary angiomatous tumour and benign ossifying fibroma or osteoblastoma). The X-ray diagnosis of the common tumours should have high concordance with histology, whereas that of uncommon tumours is much more difficult and uncertain. The characteristic features of Ewing's sarcoma are stressed as all our children with this tumour had a delayed diagnosis and a fatal outcome. Differential diagnosis with other short tubular bone lesions of the hand - specifically osteomyelitis - is discussed and the possibilities of microscopic diagnosis are stressed.

Adolescent↗