Biomedical subjects
Publications and source records attributed to K Kozlowski.
Two cases of renal medullary necrosis in young infants demonstrated on excretory urography are reported. 24 cases of renal medullary necrosis in children recognised at autopsy at the Royal Alexandra Hospital for Children between 1953 and 1977 are reviewed. Radiologists should be aware and in search of this entity in all children with history of kidney hypoxia.
Ten patients with congenital bone disease in whom bowing of the long bones was the predominant or only sign are described. The group was composed of three patients with Campomelic dwarfism, one with long bones bowing of Caffey's type, four with femoral bowing (two of them familial), one with bowing of the long bones of the lower limbs and one with bowing of the forearm bones.
Six new patients with anterior intervetebral disc herniations are reported. This is probably one of the most common causes of back pain in children. Excessive sport activity and osteoporosis are probably two main causes of anterior intervertebral disc herniations. Anterior intervertebral disc herniation is a severe complication of prolonged, mild trauma.
Seven cases of rare "Neonatal Death Dwarfism" observed recently in three major Children's Hospitals in Australia are reported. These include Diastrophic Dwarfism, Achondrogenesis II, Lethal Form of Hypophosphatasia, Homozygous Achondroplasia, Minor Form of Asphyxiating Thoracic Dystrophy, Achondrogenesis II (?) and Unclassified Lethal Bone Dysplasia. In all the patients the clinical diagnosis was uncertain or incorrect, whereas a conclusive radiographic diagnosis could be made after X-ray examination in all but one of the cases. Skeletal survey should be performed in each case of neonatal death dwarfism.
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The first two cases, reported in Australia of Oto-Palato-Digital syndrome, in two half brothers and described. They show the most severe X-ray changes ever reported in this syndrome, and were already full-blown in one of the brothers who was radiographed at the age of 1 day. These two cases also broaden our clinical and radiographic spectrum of the disease.
Four boys with anterior lumbar disc herniations probably secondary to chronic trauma are reported. Anterior disc herniations may appear as separate lesions-as in our patients-or coexist with Scheuermann's disease. Although Scheuermann's disease, discitis and tuberculosis are regarded as the most common causes of intervertebral disc lesions in childhood, trauma should be excluded as a possible cause in all children with chronic back pain who show localised radiographic signs of narrowed disc spaces with erosive and sclerotic changes in the adjacent vertebrae. Localised mild platyspondly may be the result of abnormal, prolonged stress and early osteoarthritic changes are to be expected.
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Radiographic analysis of 24 cases of hypophosphatasia (H) from 9 Paediatric Centres was performed. 3 cases were of neonatal (lethal), 18 cases of infantile (severe) and 3 cases of late (benign) type. Some of the patients were in reality borderline cases between these groups. In the authors' material all the patients showed radiographic signs of the disease. These were divided into diagnostic, characteristic and suggestive features. All of the patients had in common generalised (usually irregular) osteoporosis, generalised (usually irregular) metaphyseal changes, craniostenosis (13 of 18 infantile cases) or widened cranial sutures and ofter bowing of the long bones. Besides the well know radiographic features of hypophosphatasia some less well known, rare or 'new' ones such as, 1. spurs of the long bones (Bowdler sign), 2. distal femoral central metaphyseal defects and epiphyseal defects, 3. S-like deformities of the tibiae, 4. abnormal shape of the distal phalanges of the fingers, 5. multiple rib fractures and slender bones, 6. wedging of the lower thoracic and upper lumbar vertebrae, 7. partial premature fusion of the epiphyses, 8. nephrocalcinosis, 9. loss of lamina dura around the teeth, 10. variation in radiographic appearances of a pair of siblings with lethal form, and, 11. rapid changes in roentgen appearances. are discussed. In two of our patients (siblings) phosphoethanolamine was undetectable in the urine. The authors doubt if a normal skeletal survey may be present at any stage in any of the three major types of hypophosphatasia.