There are differences of opinion about the pathogenesis of Perthes' disease. All are agreed that it is due to ischaemia, but the cause of this and the size and number of infarctions are in dispute. Through the generosity of the contributors six whole femoral heads and core biopsies of five other cases have been studied radiographically and histologically. The findings ranged from an ischaemic arrest of ossification in the capital articular cartilage without infarction to multiple complete infarctions of the epiphysial bone. The ensuing reparative process contributes to the pathology, which is of a range to warrant grading or grouping.
Reliable and accurate assessment of liver histopathology in patients with chronic hepatitis C is important for decision regarding treatment and for evaluation of therapy. However, little data on interobserver variation have been published. In this study, five specialist histopathologists evaluated 46 liver biopsies from 20 patients treated with interferon-alpha. Knodell's and Ishak's scoring systems, De Groote's classification and a four level general necro-inflammatory activity score (GNAS) were applied. Besides kappa statistics, slide by slide analysis was performed. We defined an acceptable slide by slide agreement as eight of ten observer pairs agreed on 80% of the slides. The best agreement was seen for Knodell's and Ishak's fibrosis score, De Groote's classification and GNAS (mean weighted kappa (kappa(w)) = 0.49, 0.51, 0.50 and 0.44, respectively). By condensing data from Knodell's and Ishak's scores to presence or absence of cirrhosis and piecemeal necrosis respectively, concordance was substantial concerning cirrhosis (mean kappa = 0.69 and 0.72, respectively) but only moderate concerning piecemeal necrosis (mean kappa = 0.40 and 0.39, respectively). Slide by slide analysis showed the highest agreement on Knodell's fibrosis score and GNAS; only one point of difference in score was to be accepted to obtain 'eight of ten' agreement. In contrast, five points of difference were necessary to accept in order to reach the same agreement for Knodell's total activity score. Moreover, in serial biopsies the GNAS was sufficient to detect changes in disease activity following treatment. Thus, a simple scoring system with four category scales was reproducible and sufficient for detection of therapy induced changes.
Specimens of femoral heads were studied at necropsy in two cases of Legg-Calve-Perthes' disease. One was that of a boy aged four years ten months who died from appendicitis; the other was from a boy aged six years who died from a malignant glioma. Both had been treated for one and a half years for Legg-Calve-Perthes' disease which was in a stage of repair at the time of death. The diseased femoral heads were moderately flattened but the surface cartilage was intact. Epiphysial bone and bone marrow were partly replaced by cartilage, fibrous tissue and granulation tissue, and new bone was being formed. Inflammatory reaction was inconspicuous. Enchondral bone formation was only slightly decreased, and the structure of the growth plate was undisturbed. There was no sign of systemic bone disease. In the first case the changes indicated that more than one episode of ischaemia had occurred, and an occlusion--probably from an old thrombus--was demonstrated in the posterior inferior retinacular artery of the femoral head. The last episode of ischaemia, furthermore, had caused infarction of part of the metaphysial bone. In both cases, the central area of the metaphysial bone of the affected femur contained fat, but there were few haemopoietic cells and it therefore looked pale. The findings are discussed in relation to previous work on the pathology in Legg-Calve-Perthes' disease, recent information on the vascularisation of the femoral head in children, and experimental and comparative animal studies.
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