Epidemiology of biliary atresia in France: a national study 1986-96

Background/Aims: The reported incidence of biliary atresia varies from 5 to 32/100 000 live births. The existence of seasonality and/or clustering is controversial. Based on a large population analysis, we examined the incidence of biliary atresia in France, and the space-time distribution of cases. Methods: All patients with biliary atresia living in France and born in the years 1986-96 were recorded. Geographic distribution, seasonality, time clustering and space-time clustering were analysed. Statistical analysis used the Chi square test, the Spearman nonparametric correlation test, the Walter and Elwood test for seasonality of events and Knox analysis for time and space-time clustering. Results: We identified 461 patients: 421 born in metropolitan France (incidence 5.12 [4.63-5.61]/100 000 live births), and 40 born in overseas territories. No significant regional variation in incidence was found in metropolitan France, while the incidence was higher in French Polynesia (incidence 29.4 [15.4-43.3]/100 000 live births) (p<0.001). Seasonality, time-clustering and time-space clustering could not be demonstrated. Conclusions: 1) The incidence of BA was 5.7-fold higher in Polynesia than in metropolitan France. 2) Neither seasonal variation in incidence nor clustering was identified

Maladie de Basedow secondaire à un cancer thyroïdien métastatique.

OBJECTIVES: Metastatic thyroid carcinoma rarely provokes hyperthyroidism. We describe here the main characteristics of this association observed in a 48-year-old woman. CASE REPORT: The patient had presented signs of hyperthyroidism for one year. Clinical examination revealed severe Graves' disease with nodular goiter. Presurgery investigations demonstrated thyroid carcinoma and lung metastasis. Thyroid stimulating immunoglobulins were elevated. After total thyroidectomy, hyperthyroidism persisted, indicating the functional nature of the lung metastases. DISCUSSION: The effect of thyroid stimulating immunoglobulins on the clinical course of thyroid carcinoma and distant metastases remains unknown.Case ReportsEnglish AbstractJournal ArticleReviewSCOPUS: ar.jinfo:eu-repo/semantics/publishe

Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia?

OBJECTIVES: The prognosis of hepatoportoenterostomy (the Kasai operation) for biliary atresia worsens when the age at surgery increases. This study examines whether the Kasai operation remains justified after 3 months of life. STUDY DESIGN: Records for all patients with biliary atresia living in France and born in the years 1986 to 1996 were reviewed, and patients were classified into 3 groups: group 1 (n = 30), no contraindication to the Kasai operation, but orientation to de novo transplantation; group 2 (n = 380), age at Kasai operation or =90 days. Survival with native liver, survival after liver transplantation, and overall survival (Kaplan-Meier method) were compared by using the log-rank test. RESULTS: Five-year (10-year) survival with native liver was 35% (30%) in group 2 and 25% (22%) in group 3 (P =.03). Five-year overall survival was 57%, 74%, and 55% in groups 1, 2, and 3, respectively (P =.003). Poor results in groups 1 and 3 were mainly due to increased pre-transplantation mortality, but survival after transplantation was not significantly different in the 3 groups. CONCLUSIONS: Performance of the Kasai operation after 3 months of age is justified in selected cases, because it may obviate liver transplantation. Preoperative evaluation should exclude patients with advanced liver disease for whom liver transplantation should not be delayed

Early compared with delayed inhaled nitric oxide in moderately hypoxaemic neonates with respiratory failure : a randomised controlled trial

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A Novel 8-Predictors Signature to Predict Complicated Disease Course in Pediatric-onset Crohn’s Disease: A Population-based Study

International audienceBackground The identification of patients at high risk of a disabling disease course would be invaluable in guiding initial therapy in Crohn’s disease (CD). Our objective was to evaluate a combination of clinical, serological, and genetic factors to predict complicated disease course in pediatric-onset CD. Methods Data for pediatric-onset CD patients, diagnosed before 17 years of age between 1988 and 2004 and followed more than 5 years, were extracted from the population-based EPIMAD registry. The main outcome was defined by the occurrence of complicated behavior (stricturing or penetrating) and/or intestinal resection within the 5 years following diagnosis. Lasso logistic regression models were used to build a predictive model based on clinical data at diagnosis, serological data (ASCA, pANCA, anti-OmpC, anti-Cbir1, anti-Fla2, anti-Flax), and 369 candidate single nucleotide polymorphisms. Results In total, 156 children with an inflammatory (B1) disease at diagnosis were included. Among them, 35% (n = 54) progressed to a complicated behavior or an intestinal resection within the 5 years following diagnosis. The best predictive model (PREDICT-EPIMAD) included the location at diagnosis, pANCA, and 6 single nucleotide polymorphisms. This model showed good discrimination and good calibration, with an area under the curve of 0.80 after correction for optimism bias (sensitivity, 79%, specificity, 74%, positive predictive value, 61%, negative predictive value, 87%). Decision curve analysis confirmed the clinical utility of the model. Conclusions A combination of clinical, serotypic, and genotypic variables can predict disease progression in this population-based pediatric-onset CD cohort. Independent validation is needed before it can be used in clinical practice