A NEW SYNDROME COMBINING PTERYGIUM COLLI WITH DEVELOPMENTAL ANOMALIES OF THE EYELIDS AND LYMPHATICS OF THE LOWER EXTREMITIES.
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Fluorescence microlymphography was performed near the medial ankle in 12 healthy controls, 12 patients with congenital and 12 with sporadic lymphedema of the lower extremities. Diameters of lymphatic capillaries were determined on the videoscreen by playback of the tape recordings using a morphometric technique. In the patients with congenital disease (Nonne-Milroy) aplasia of microlymphatics was diagnosed in 8 instances, ectasia in 4. Patients with sporadic lymphedema and manifestation after puberty exhibited initial lymphatics of normal caliber. In controls mean capillary diameter was 56.3 +/- 9.0 microns, in congenital disease with ectasia 99.3 +/- 9.0 microns (difference significant at the p less than 0.005 level) and in the sporadic form 49.7 +/- 7.7 microns. Congenital lymphedema may be subdivided into a form with aplastic and ectatic microvessels which possibly show different responses to therapy.
Disorders of haemostasis and altered platelet activity have been documented in patients with malignant disease but their relation to response to treatment and prognosis are not known. Thrombin activity (fibrinopeptide A (FpA), plasmin mediated fibrinolysis (B beta 15-42) antigen), and platelet alpha granule release (beta thromboglobulin) were studied in 37 patients with small cell lung cancer to find out whether these indices show a relationship to chemoresponse. There was evidence of considerably increased thrombin activity, with a median fibrinopeptide A concentration of 13.2 (normal less than 4) pmol/ml, but only modestly increased fibrinolysis, with a median B beta 14-42 antigen concentration of 5.6 (normal less than 3) pmol/ml. Thus the ratio of fibrinopeptide A to B beta 15-42 concentration (FpA:B beta) was raised, with a median value of 2.2 (normal less than 1.33). In addition, 57% of patients had increased platelet alpha granule release, the median beta thromboglobulin concentration being 50 (normal less than 50) ng/ml. There was a significant association between increased thrombin generation and lack of response to chemotherapy. Furthermore, non-responders had higher FpA:B beta ratios. The same haemostatic markers were studied in nine patients who have been in complete remission for at least two years after chemotherapy for small cell lung cancer. There was a significant difference in thrombin activity and also in the ratio of thrombin activity to lysis between the pretreatment group and the group of two year survivors. Lack of response to chemotherapy appears to be related to increased thrombin activity. Such an association has not previously been reported in patients with malignant disease.
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A patient is described who presented with sudden collapse due to acute respiratory failure caused by tracheal compression from a thyroid cyst. He made a complete recovery and the cyst was electively excised two weeks later.
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The otic capsule is unique in retaining calcified cartilage, known as globuli interossei, throughout life and shows changes consequently, which are peculiar to it. In Paget's disease of bone, the otic capsule appears resistant to involvement and this occurs with extensive disease at a late stage. In contrast, otosclerosis is a new bone formation of unknown cause that is limited to the otic capsule. In osteogenesis imperfecta, the poor formation of collagen leads to abnormally thin bony trabeculae with a poorly formed otic capsule. In osteopetrosis, the otic capsule is greatly expanded by increased globuli interossei, as a result of defective osteoclast function. When fractured the middle layer of the otic capsule does not form callus, but heals by fibrosis.
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