ABsTRAcr Sputum samples from 34 patients with bronchiectasis were assessed subjectively and the results related to objective measurements of elastase activity and albumin content. The results suggest that the macroscopic appearance of the sample is related to the elastase content, 88-7% of the purulent samples but none of the mucoid samples showing elastase activity. The macroscopic appearance was also associated with changes in protein transudation into the secretions. The median sputum:serum albumin concentration ratio was 0-71 x 10-2 (range 0.22-4.7) in the mucoid samples but was greater in purulent samples (p < 0.005), with a median value of 1-52 x 10-2 (range 0*55-12.72), suggesting that purulence in the stable state was associated with low grade pulmonary inflammation or epithelial damage or both. Abnormalities of air flow were found in 24 of the patients (70.6%) but there was a significantly higher ratio of residual volume to total lung capacity (p < 0.025) in patients who regularly produced purulent sputum (mean (SD) RV/TLC 44-4% (9.0%)) than in those with mucoid or mucopurulent secretions (38.0% (9.9%)). A similar difference was found between those who produced elastase positive secretions and those who produced elastase negative ones.
Summary A retrospective histopathological review of 2104 cases of solid tumour was carried out to assess the variability in diagnosis of childhood cancer. Cases were subject to three independent, concurrent opinions from a national panel of specialist pathologists. The conformity between them was analysed using the percentage of agreement and the kappa statistic (ic), a measure of the level of agreement beyond that which could occur by chance alone, and weighted kappa (wic), which demonstrates the degree of variation between opinions. The major groupings of the Birch-Marsden classification were used within which tumours were assigned for kappa analysis according to the clinical significance of the differential diagnoses. The mean agreement for all tumours together was 90%; 1C=0.82, wKi=0.82. Retinoblastoma achieved the highest kappa value (1.0) and lymphoma the lowest (0.66). Of the cases, 16.5% had their original diagnoses amended and the panel confirmed the original diagnosis of paediatric pathologists in 89% of cases compared with 78% for general pathologists. The varying levels of agreement between experts confirm the difficulty of diagnosis in some tumour types, suggesting justification for specialist review in most diagnoses. Specialist training in paediatric pathology is also recommended.
A 2-year-old male with acute lymphocytic leukemia died with histiocytic reticulosis 19 weeks later. A comparison with previously reported cases is made, and the relationship of ALL and HMR is discussed.
SUMMARY In two cases of myelomatosis relapse was accompanied by the development of bulky extramedullary plasma cell tumours, which created serious management problems. In both cases the histology was of a poorly differentiated plasmacytoid tumour with frequent mitoses. In one case paraprotein had completely disappeared from the serum and urine after six months' treatment with melphalan alone but did not correlate with improved survival.It is concluded that extramedullary spread ofmyelomatosis in the form of bulky tumour deposits is a rare and ominous mode of tumour progression or relapse. Rises in serum and urinary paraprotein concentrations are not the inevitable signs of relapse.
Of 160 cases of osteosarcoma seen over an 18-year period, 18 were over the age of 40 at the time of presentation. The clinical presentation and histology of these cases is reviewed. Delay in referral and incorrect histological diagnoses were frequent. Only 2 cases were associated with Paget's disease.
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