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- W1966653513 abstract "We have studied a child with severe gingival hyperplasia, hypertrichosis, macroglossia, and abnormal facial features. Hypertrichosis was present at birth and gingival hyperplasia appeared with eruption of the primary teeth. The collagens present in the hyperplastic gingival tissue were normal in type and amount. Fibroblasts obtained from an explant of the hyperplastic tissue and maintained in culture grew at a much slower rate than a cell strain obtained from a normal young adult, but in a manner comparable to an age- and sex-matched control child strain. The patient cells were abnormally fragile, they manifested an abnormal nuclear size, and they had an abnormally short life-span in culture. They did not contain inclusions indicative of the Hurler syndrome, and chromosomal analysis failed to reveal abnormalities. The amount of protein synthesized during a test period was the same as the normal control child cells, but the amount of collagen was only about one-half that produced by the control cells. In contrast, a cell strain obtained from an age-related individual with phenytoin-induced gingival hyperplasia, which is very similar clinically to the spontaneous lesion, exhibited greater rates of protein and collagen synthesis than either the normal control child strain or the patient strain. There were no significant differences among patient cells, control child cells, and phenytoin-induced lesion cells in the pattern of protein synthesis as revealed by one- and two-dimensional fluorography. Thus, fibroblasts obtained from both the spontaneously occurring and the drug-induced gingival hyperplasia appear to be abnormal and the abnormalities persist in culture; however, the defects are not the same." @default.
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- W1966653513 date "1986-07-01" @default.
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- W1966653513 title "A defect in fibroblasts from an unidentified syndrome with gingival hyperplasia as the predominant feature" @default.
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- W1966653513 doi "https://doi.org/10.1111/j.1600-0765.1986.tb01474.x" @default.
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