Bifocal nummular headache: the first three cases

Nummular headache (NH) has been defined as a focal head pain that is exclusively felt in a small area of the head surface. Here we describe three patients who presented with focal head pain in two separate areas. This finding seems to be consistent with bifocal NH and further enlarges the clinical diversity of this headache disorder. The pathogenic mechanisms of NH may be active in multiple cranial areas in some particular patients.     

Comprehensive Cancer Centre Amsterdam Barrett Advisory Committee: first results

BACKGROUND: Treatment and follow-up policy for Barrett's oesophagus are dependent on the grade of dysplasia. However, sampling error of random biopsy protocols and subjectivity of pathological grading may hamper endoscopic surveillance. METHODS: The Amsterdam Comprehensive Cancer Center Barrett Advisory Committee (BAC) is a regional multidisciplinary consultative working-group, offering revision of pathology, revision of pathology plus additional endoscopic diagnostics, or referral for treatment. RESULTS: Between July 1998 and July 1999 30 patients were referred to the B.A.C for advice; eighteen patients were referred because of suspicion of high grade dysplasia. Reassessment of biopsies, including additional quantitative pathology, with or without additional endoscopic diagnostics, led to adjustment of the grading of dysplasia in 15 patients (50%). A suspicion of low grade dysplasia was rejected in nine out of ten cases. Adjustment of the original diagnosis often influenced further therapy or follow-up. CONCLUSIONS: reassessment of conventional pathology, quantitative pathology, and additional diagnostic procedures might improve the accuracy of diagnosis and staging of malignant degeneration of Barrett's oesophagus, although experience is still limited. The complexity of the management of these patients demands a specialised multidisciplinary approach. A Barrett Advisory Committee can offer valuable contributions to the treatment of patients with Barrett's oesophagus.     

Age-specific regulation of clotting factor IX gene expression in normal and transgenic mice

Factor IX (FIX), a circulating serine protease that serves as an essential component of the blood coagulation pathway, has been shown to increase with age in humans. We show here that murine FIX mRNA and activity levels also increase with age. Furthermore, one form of hemophilia B, hemophilia B Leyden, which is caused by mutations within the promoter region of the FIX gene, has a distinct age-dependent phenotype. To determine the source of the age-related increases in FIX gene expression, we have analyzed the regulation of the normal FIX gene promoter and FIX Leyden gene promoter with the +13 mutation during aging by generating transgenic mice that contain the -189 to +21 bp promoter segment ligated to a chloramphenicol acetyltransferase reporter gene. We have established that the normal FIX promoter and the Leyden promoter transgenes are expressed in a tissue-specific manner in vivo. The normal FIX promoter transgene does not show any differences in the pattern of expression with age or sex of the organism, whereas the Leyden promoter transgene showed age-dependent male-specific expression. This is the first demonstration of the FIX Leyden phenotype in a transgenic mouse model.