Biomedical subjects
Publications and source records attributed to A Giannetti.
A case of dyskeratosis congenita with depressed responsiveness to policlonal mitogens (PHA, Con-A, PKM) and negative skin tests (PPD, SK-SD, Candidin, Trichophytin) is reported. Chromatid breaks and reduction of the capacity of DNA repair were also present. The findings are discussed in relationship to the prognosis of dyskeratosis congenita.
Two separate functional defects of the alternate pathway of complement (defective yeast opsonisation and defective rabbit erythrocyte lysis) are very frequent (of the order of 55% for the former and 35% for the latter) among atopic children and their unaffected relatives. The defects are independent of each other and of sex, age, season of sampling, type of atopic syndrome and IgE level. The alternative pathway initiation mechanism is probably involved in both defects.
Wegener's Granulomatosis was suspected in a 27-month-old female with a nodular, necrotizing lesion of the nose, diffuse subcutaneous nodules, and erythematous desquamation of the entire body. From 20 months of age on she had a purulent nasal discharge, recurrent infections of the upper and lower respiratory tract, a Coombs positive anemia, and enlargement of the spleen and liver. Treatment with azathioprine and corticosteroids produced transient improvement but three months later a dramatic relapse occurred. Cyclophosphamide was substituted for azathioprine but 10 days later the patient died and the autopsy confirmed the diagnosis of Wegener's Granulomatosis. The early age of onset of the disease may explain the unfavorable outcome, despite treatment with cytotoxic agents.
A case of chronic bullous dermatosis of childhood in a 3-year-old boy is described. Immunoflourescence tests were negative and biopsy of the jejunal mucosa showed marked villous atrophy. The dermatosis was brought under control by a combination of diaminodiphenylsulphone and systemic steroids. The relationship with other bullous eruptions of childhood such as dermatitis herpetiformis and bullous pemphigoid is discussed.
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Immunofluorescence investigations in patients with mixed cryoglobulinaemia, Schönlein-Henoch purpura and purpuric allergic reaction with disseminated intravascular coagulation have been performed. In the lesional skin from 8 patients with mixed cryoglobulinaemia, granular vascular deposits of immunoglobulins of the same classes as those of the circulating cryoglobulins were detectable in all cases; complement and fibrinogen were concomitantly present. In the unaffected skin, IgM and complement were detected in the walls of the capillaries. In the early purpuric lesions from 6 patients with Schönlein-Henoch syndrome and from a patient with diseeminated vascular coagulation from acute allergic reaction to phenylbutazone, deposits of fibrinogen occurred mainly in the vessel walls.
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