[Effect of penicillin and D-penicillamine on beta-galactosidase activity in patients with progressive scleroderma].
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Biomedical subjects
Publications and source records attributed to U F Haustein.
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We report on a 10-year-old girl with ichthyosis vulgaris, suffering from pains in her left arm and wrist after 1 1/2 years' treatment with etretinate (Tigason) The activity of the alkaline phosphatase was increased. Scintigraphy with technetium showed increased accumulation of activity as well as beginning transformation processes of the left ulna, which recovered after the reduction of the maintenance dose and are no longer detectable after a total of 3 1/2 years' treatment. The potential bone toxicity must be taken into consideration in long-term treatment with retinoids. Its manifestation should be prevented by thorough follow-up.
In 14 patients with progressive systemic sclerosis (PSS) the activities of acid lysosomal glycosidases (alpha-, beta-galactosidase, beta-glucosidase, beta-glucuronidase, and beta-N-acetyl-glucosaminidase) were determined fluorometrically in serum, leukocytes, and skin tissue. The beta-galactosidase was the only enzyme which exhibited a significantly elevated activity in PSS serum and skin but not leukocytes, as compared to the control. The activity patterns of the studied glycosidases in serum were similar to those found in skin, but differ from the distribution of glycosidase activities in leukocytes. In cultured dermal fibroblasts derived from PSS patients, an elevated intracellular activity of beta-galactosidase was detected. These results suggest that the increased beta-galactosidase activity in the serum originates from the skin fibroblasts.
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In 10 untreated patients suffering from acute bullous pemphigoid the number of peripheral blood T cells (CD 3), T suppressor (CD 8) and T helper cells (CD 4) as well as activation antigens (DR and Tac) bearing lymphocytes was evaluated by monoclonal antibodies. While the pan-T cell population (CD 3) and T suppressor subpopulation (CD 8) were normal, the T helper subpopulation (CD 4) and the number of DR and Tac positive lymphocytes were significantly increased in the acute stage of bullous pemphigoid when compared to the age- and sex-matched controls. Phenotypically those DR positive cells belonged to the pan-T cell population (CD 3) at 66% and to the T helper subpopulation (CD 4) at 53% respectively. Under treatment with immunosuppressants the cell counts returned to normal suggesting a T helper cell activation to be involved in the acute stage of bullous pemphigoid.
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Compared to the linear derivative 8-methoxypsoralen (8-MOP) the angular structured furocoumarin 4,5'-dimethylangelicin (4,5'-DMA) causes smaller photosensitization effects (loss of viability, inhibition of phytohemagglutinin (PHA)-induced transformation rate and E-rosette formation of T-cells, respectively) on cultured human lymphocytes. When 4,5'-DMA or 8-MOP are added to the cultures without additional UV-irradiation, increased or decreased PHA-stimulation rates are observed, respectively. In addition, 4,5'-DMA is able to reduce the binding of sheep erythrocytes to lymphocytes in a higher degree than 8-MOP. These findings suggest different action mechanisms on cytoplasma membranes and intracellular structures of lymphocytes by different furocoumarins.
Positive results of the intracutaneous test with Candida were associated with an increased phagocytosis (p-trend) and an increase of the intracellular killing (p less than or equal to 0.01) of Candida albicans by polymorphonuclear granulocytes.
It is reported on a 42-year-old book-keeper with the granulomatous variant of the chronic mucocutaneous candidiasis which could be followed up for 28 years. The intensive systemic treatment with nystatin, 5-fluorocytosin and miconazol combined with the subcutaneous injection of transfer-factor and the local application of ointments containing nystatin and clotrimazol did not only lead to the complete clearing of the lesions (4 years without any relapse), but also to the normalization of the T-lymphocyte count and the reconstitution of the formerly negative delayed type skin reactivity to candidin.
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