We report a case of scleredema adultorum (Buschke's disease) associated with an IgA kappa monoclonal hypergammaglobulinaemia. A significant improvement in the skin was obtained with electron-beam therapy. Scleredema would appear to be linked to monoclonal hypergammaglobulinaemia, but the relationship between the skin disorder and the immunoglobulin abnormality remains to be elucidated.
The monoclonal origin of gonadotropin-secreting pituitary adenomas has been well demonstrated but only few molecular abnormalities have so far been recognized in these tumors. For many years, several authors have suggested a role for GnRH and/or GnRH receptors (GnRH-R) in the development of these pituitary adenomas. To test the hypothesis that mutant genes encoding a constitutively activated GnRH-R might be involved in the pathogenesis of these tumors, the sequence of the GnRH-R gene was analyzed in tumoral pituitary tissue obtained from ten patients (six female, four male). The pituitary gonadotropin-secreting adenoma was associated with in vivo hypersecretion of FSH, LH and/or free a-subunit (n ¼ 7) or was clinically silent (normal plasma levels of gonadotropins or free a-subunit, n ¼ 3). In all cases, immunocytochemical studies of the removed adenoma confirmed their gonadotroph nature by revealing positivity for FSH, LH and/or a-subunit. Genomic DNA was extracted from the pathological tissue obtained at neurosurgery. Eight sequencing primers were used to amplify the three exons of the GnRH-R gene from tumoral DNA. The entire coding sequence of the GnRH-R gene was sequenced in the ten adenomas. No mutation was found in any of the tumor specimens examined.In conclusion, mutations in the GnRH receptor coding sequence occur infrequently if at all in gonadotropin-secreting pituitary adenomas.
This randomized, multicentre prospective study was undertaken to compare the efficacy and tolerance of a one day course of injectable cefotaxime with a five day oral course of co-amoxyclav (amoxycillin plus clavulanic acid) in the treatment of acute otitis media in children. The study was conducted in 116 patients between the ages of three months and 12 years. No significant difference in efficacy was observed between the two treatment groups, but tolerance to the treatments differed significantly. There were no unwanted side-effects in the cefotaxime group whereas 22% of children in the amoxycillin plus clavulanic acid group suffered side-effects (P 0.0007). Short-course antibiotic therapy with cefotaxime appears to be a valuable alternative to conventional treatment regimens for acute otitis media in children.
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