30 research outputs found

    Ichthyosis, exocrine pancreatic insufficiency, impaired neutrophil chemotaxis, growth retardation, and metaphyseal dysplasia (Shwachman syndrome).

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    The Shwachman syndrome comprises exocrine pancreatic insufficiency, growth retardation, and bone marrow hypoplasia resulting in neutropenia. Clinical, morphological, and ultrastructural studies, as well as hair analysis, were performed in a patient with Shwachman's syndrome and severe ichthyosis. Clinical findings were lamellar ichthyosiform desquamation on the extremities. The hair was scanty and short on the scalp, in the eyelashes, and in the eyebrows. The nails were hyperkeratotic. Morphologic findings were slight, regular acanthosis and severe diffuse hyperkeratosis with variable parakeratosis. The granular layer was thickened. The papillary dermis showed very slight perivascular lymphocyte infiltration. The most prominent ultrastructural finding was the presence of solitary or multiple droplets of varying size in the cytoplasm of the keratinocytes. Hair analysis revealed no abnormalities; the cystine concentration in hair specimens was normal

    Repeated cultured epidermal allografts in the treatment of chronic leg ulcers of various origins

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    Twelve patients with 16 leg ulcers, existing for at least 3 months and not responsive to conventional inpatient therapy of at least 3 weeks, were treated with repeated applications of cultured allogenic keratinocyte sheets. A marked decrease in size was seen in all ulcers but 2. Complete closure of the ulcer was seen in 62% of the ulcers within 8 weeks. Healing was due to enhanced granulation and increased epithelialization, starting from the periphery of the wound. This edge effect suggests that the epidermal allografts act by stimulation of migration and/or multiplication of the acceptor's keratinocytes, rather than by take of the allograft
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