Biomedical subjects
Publications and source records attributed to E Marzo.
McCune-Albright syndrome is characterized by the triad of polyostotic fibrous dysplasia, precocious puberty, cutaneous pigmentation and cafe-au-lait spots. A 23-year-old woman with McCune-Albright is reported. Findings on CT scan and magnetic resonance imaging of the skull in an affected girl with McCune-Albright syndrome are shown, and also the histopathological study of the right temporal bone biopsy.
The effects of interferon-alpha (IFN-alpha) on clinical and serologic manifestations of mixed cryoglobulinemia (MC) were investigated by randomized, crossover-controlled trial in 26 patients. The trial alternated 6 months with and 6 months without IFN-alpha therapy (2 x 10(6) IU daily for a month, then every other day for 5 months). In 22 patients, pretreatment steroid dosage remained unchanged during the study. Six patients dropped out (three because of side effects), whereas another 20 patients experienced a significant improvement of purpura (P < .02) and serum transaminases (P < .005) during IFN-alpha treatment. The presence of clinical improvement was supported by the outcome measurements of several immunologic parameters. In particular, serum cryoglobulins were significantly reduced (P < .04) during IFN-alpha therapy. A rebound phenomenon of clinical and serologic parameters was observed after IFN-alpha discontinuation. In addition, no variations were recorded during 6 months without therapy. Hepatitis C virus (HCV) RNA was detected in 91% (20/22) of our MC patients; in 2/13 cases HCV RNA was no longer detectable in serum samples after IFN-alpha therapy. Thus, IFN-alpha could be considered as treatment for MC in patients with HCV seropositivity.
To confirm the positive results of a preliminary trial, 26 patients with mixed cryoglobulinaemia were enrolled in a controlled, randomised, crossover trial with interferon alfa-2b. A significant improvement was seen in the purpura score and alanine aminotransferase activities during six months' treatment, and was associated with a significant decrease in cryocrit and a returning to normal of the lymphocyte CD4/CD8 ratio (in eight of nine patients). No significant variations were seen during the six month period without interferon. Only six patients withdrew from treatment, three because of side effects and three because of poor compliance.
The prevalence of antibodies to hepatitis C virus (HCVAb) was investigated in 52 unselected patients with mixed cryoglobulinemia and in 84 patients with other systemic immunologic diseases. HCVAb were detected by an enzyme-linked immunosorbent assay, and their specificity was evaluated by a recombinant-based immunoblot assay. The presence of HBV-related markers was investigated in the same samples. HCVAb were found in 54% of mixed cryoglobulinemia patients, and the finding was confirmed by recombinant-based immunoblot assay in all cases. HCVAb and/or HBV markers were present in 70% of the patients. HCVAb seropositivity was significantly more frequent in mixed cryoglobulinemia patients with biopsy-proven liver involvement (P less than 0.01) and with increased serum transaminase levels (P less than 0.01). HCVAb were virtually absent in control patients with other immunologic diseases. These results support the notion that viral agents, i.e., HCV and possibly HBV, have a role in the pathogenesis of mixed cryoglobulinemia patients.
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The prevalence of antibodies against hepatitis C virus (anti-HCV) in an unselected series of 45 mixed cryoglobulinemia patients was assessed by an enzyme linked immunosorbent assay (Chiron ELISA HCV, Second Generation). The anti-HCV specificity was evaluated by a recombinant based immunoblot assay (Chiron RIBA HCV, Second Generation Assay). HBV-related markers and HIVAb were detected in the same samples. The prevalence of anti-HCV observed in mixed cryoglobulinemia was compared with 80 patients with other immunological systemic diseases. Anti-HCV were found in 91% of mixed cryoglobulinemia patients, and confirmed by RIBA in all cases; on the other hand, anti-HCV were practically absent in other control diseases. HBV markers were recorded in 49% of mixed cryoglobulinemia subjects; while HIVAb were constantly absent. These data give us new insights into the etiopathogenesis of mixed cryoglobulinemia.
The prevalence of hepatitis C virus (HCV) RNA and of antibodies to HCV in an unselected series of 42 mixed cryoglobulinemia patients was investigated in this study. HCV RNA was detected by the polymerase chain reaction technique, and HCV antibodies by two enzyme-linked immunosorbent assays (Chiron ELISA HCV, Second Generation, Emeryville CA, USA; Wellcome Diagnostic, England). HCV RNA was found in 86% of the mixed cryoglobulinemia patients. Using either the Chiron ELISA or the Wellcome ELISA, HCV antibodies were present in 90% of the same samples; anti-HCVAb seropositivity was confirmed in all cases by immunoblot assay (Chiron RIBA HCV, Second Generation Assay). A striking correlation between HCV RNA and anti-HCV antibody seropositivities was recorded. HCV RNA in mixed cryoglobulinemia patients was not correlated with the presence or absence of biopsy-proven liver involvement. These results suggest that the association between HCV and mixed cryoglobulinemia is not fortuitous, and therefore that HCV may have an etiopathogenetic role in this disorder.
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