38 research outputs found

    Animal Models

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    Maligne pansklerotische zirkumskripte Sklerodermie

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    Electron Microscopic Studies of Harlequin Fetuses

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    Abstract: Four cases of harlequin fetus of various estimated gastational ages (16, 20, 21, 24 weeks) were examined by light and electron microscopy. When the epidermis was keratinized the following features were commonly found: hyperkeratosis with or without granular cells; dilated hair follicles with plugged keratin; a large number of dense or particle‐cored granules In the upper malpighlan layer; absence of cementsomes (lamellar bodies); large vacuotes with peripherally located laminations; large mitochondria with vesicular or membranous cristae; and early formation of the marginal band in keratinocytea and abnormal formotion of the same in luminal cells of the acrosyringlum. A 16‐week specimen had no sign of karatinization, which made It difficult to detect these abnormalities; however, it did have large mitochondria in the keratlnocytes. The mucous membrane of the lip was thickened but not keratinized. Copyright © 1993, Wiley Blackwell. All rights reservedSCOPUS: ar.jinfo:eu-repo/semantics/publishe

    The complement system and systemic sclerosis

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    Serum concentrations of the various complement components including the classical and the alternative pathways were determined in 58 control healthy subjects and 80 patients with systemic sclerosis (SSC). The mean concentrations of C1q, C2, C5, C6, C7, C9, and factor B were significantly increased in the SSC patients in comparison to controls, while the increases were not significant for C3 and C8. C4 was an exception in that the mean levels were found to be decreased with 18 patients having levels <65% of the mean normal value. Properdin was also found to be decreased, but not significantly. We found a similarity between the pattern of serum complement component concentrations in SSC patients and patients with primary biliary cirrhosis, two disorders frequently associated in the same patients. The significance of complement component patterns in these diseases is discusse
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