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Udgivelsesdato: 2008/3

Udgivelsesdato: 2008/6

Udgivelsesdato: 2008/7

In a collaborative multicenter case-control study, we investigated the effect of factor V Leiden mutation, prothrombin gene mutation, and inherited deficiencies of protein C, protein S, and antithrombin on the risk of Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT). We compared 43 BCS patients and 92 PVT patients with 474 population-based controls. The relative risk of BCS was 11.3 (95% CI 4.8-26.5) for individuals with factor V Leiden mutation, 2.1(95% CI 0.4-9.6) for those with prothrombin gene mutation, and 6.8 (95% CI 1.9-24.4) for those with protein C deficiency. The relative risk of PVT was 2.7 (95% CI 1.1-6.9) for individuals with factor V Leiden mutation, 1.4 (95% CI 0.4-5.2) for

__Background__ The incidence of coeliac disease varies internationally. __Aims__ To assess the incidence of childhood coeliac disease in The Netherlands and to study the clinical features and the presence of associated disorders. __Subjects__ Identified cases of childhood coeliac disease in The Netherlands in 1993-4 by means of the Dutch Paediatric Surveillance Unit. __Methods__ Inclusion criteria were born in The Netherlands, diagnosed with at least one biopsy of the small bowel in 1993-4 and age at diagnosis 0-14 years. The data were cross checked by the Dutch Network and National Database of Pathology and compared with data from a previous study on childhood coeliac disease, 1975-90. __Results__ A total of 193 coeliac patients were identified by means of the Surveillance Unit, another 20 through the National Database of Pathology. The mean crude incidence rate of diagnosed childhood coeliac disease was 0-54/1000 live births, which is in the range of rates found in other western European countries and significantly higher than the mean crude incidence rate of0 18/1000 live births found in The Netherlands in 1975-90. The clinical presentation was classic: chronic diarrhoea, abdominal distension, and growth failure. Associated disorders were present in 11.7% of the cases. __Conclusions__ The incidence of diagnosed childhood coeliac disease in The Netherlands seems to have increased significantly during the past few years. In a period of 20 years no significant changes could be found in the clinical picture at presentation of coeliac disease in Dutch children.

In the histopathology of malignant mesotheliomas three different types (epithelial, connective tissue and mixed type) are distinguished. Some authors believe all tumours to be of mixed type, but consider that due to inadequate sampling or small biopsies this may be missed frequently. In this study the relationship between the histopathological diagnosis and the amount of tissue examined was investigated. In a series of 124 cases of malignant pleural mesothelioma a high percentage of mixed type tumours was found (55%). In cases where the decisive diagnostic procedure had been an Abrams biopsy (the "small-specimen" technique) mixed-type histology was found in 36%. If thoracoscopy, thoracotomy or autopsy (the "large-specimen" techniques) had delivered a definite diagnosis, mixed-type histology was found in 63%. Apparently diagnosing the mixed-type variety depends on the amount of tumour tissue obtained. However, the assumption that all mesotheliomas are of mixed type cannot be confirmed.