Biomedical subjects
Publications and source records attributed to K Kozlowski.
Four cases of multifocal osteomyelitis of unknown origin in childhood are reported. The variable clinical and radiographic appearances of the disease are illustrated and the diagnostic difficulties in the early stages of the disease are stressed.
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20 cases of primary benign and malignant bone tumours in children were reported. The most common tumours were Ewing's sarcoma, aneurysmal bone cyst, benign osteoblastoma and osteoid osteoma. Some rare primary bone tumours in children (osteochondroma, chondroblastoma?, primary lymphoma of bone and neurofibromatosis with unusual cervical spinal changes) were also reported. The authors believe that radiographic findings together with clinical history and clinical examination may yield a high percentage of accurate diagnoses. Although microscopy is essential in the final diagnosis, the microscopic report should be viewed with caution.
Four cases of idiopathic chondrolysis of the hip in three white girls and one Maori girl are reported. The authors stress the causes why a disease with characteristic clinical and radiographic appearances and normal biochemical findings presents diagnostic difficulties. It is suspected that idiopathic chondrolysis is a metabolic disorder of chondrocytes, triggered by environmental circumstances in susceptible individuals. Idiopathic chondrolysis is probably one of the most common causes of coxarthrosis in women.
Two cases of chronic osteomyelitis with unusual Ewing's like X-ray changes are reported. Inspite of extensive investigations no causative organisms were found. The infection was resistant to different kinds of therapy. The authors suppose that the strange X-ray appearances may be due to an unusual localised immune response of the bone to an unknown, possibly viral agent.
A new type of neonatal death dwarfism is reported. Although it resembles superficially the metatropic dysplasia group of diseases it has some distinctive radiographic features which help to delineate it as a separate entity.
Generalized arterial calcification in infancy is a rare disorder in which death usually occurs in infancy, the diagnosis generally being made at autopsy. Three patients are reported. The diagnosis was made during life in two, enabling new information to be collected. Cardiac catheterization in one provided evidence suggesting stiffness of pulmonary and systemic arterial walls. Another is a long-term survivor with spontaneous regression of calcification. The third case, diagnosed at necropsy, was associated with endocardial fibroelastosis.
Fourteen patients with spondylo-costal dysplasia were analysed. 3 of them presented without obvious associated anomalies "pure" spondylo-costal dysplasias; 2 had several components consistent with Vater (Vacterl)-Association; 2 showed malformations which are often encountered in Vater (Vacterl)-Association; 4 presented with minor malformations; 3 had major associated malformations rarely seen in Vater (Vacterl)-Association. Thoracic spine and costal malsegmentation can be sporadically observed in other "errors in septation complex" (axial mesodermal dysplasia) including severe myelomeningocoele and diastematomyelia.
We have reviewed the radiographic features of 19 patients with spondylo-epimetaphyseal dysplasia with joint laxity (SEMDJL). The salient, diagnostic radiographic manifestations are mild to moderate spondylo-epimetaphyseal dysplasia, progressive infantile kyphoscoliosis, characteristic pelvic, forearm and elbow changes and a highly abnormal bony trabecular pattern. The condition, which is inherited as an autosomal recessive, has only been observed in South Africa among Caucasians, several of whom have German names or German connections. The authors wish to bring the condition to the attention of European radiologists in an attempt to determine the geographic origin of the faulty gene.
Spondylo-epi-metaphyseal dysplasia with joint laxity (SEMDJL) is characterized by severe dwarfism, articular hypermobility and progressive spinal malalignment. The clinical manifestations of 18 affected persons in 13 families of the Afrikans-speaking community of South Africa have been analysed and it has become evident that survival into adulthood is unusual. SEMDJL is inherited as an autosomal recessive trait.
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