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Modifications hétérozygotes homozygotes et composites du gène HFE ont été rapportés dans une famille avec une pancréatite héréditaire et distincts avec l'hémochromatose disease.6Hereditary (HH) (OMIM 235200) est une maladie métabolique caractérisée par l'accumulation de l'excès de fer dans une variété d'organes, conduisant de leur échec et le développement de maladies graves, telles que la cirrhose, le diabète sucré, la cardiomyopathie et l'arthrite. En réponse au fer, les cellules étoilées du foie produisent un excès de collagène et d'autres protéines de la matrice conduisant à fibrosis.11 Bien que des mécanismes similaires sont cruciales pour le développement de la fibrose du pancréas, l'effet du fer sur la cellules étoilées du pancréas n'a pas été bien étudiée. Dans un modèle murin de l'hémochromatose, la surcharge en fer dans le pancréas a entraîné une fibrose de la glande de l'infiltration des macrophages massive et la perte de epithelium.12 sécrétoires Chez les patients souffrant d'hémochromatose, le développement du diabète est une étape irrévocable à la progression de la maladie qui conduit à la mort prématurée , même si l'excès de fer est ensuite removed.It est connu que la consommation chronique excessive d'alcool accentue l'expression clinique de hemochromatosis.Top de pageMATERIALS et l'approbation du comité d'éthique METHODSPatientsAfter, le consentement éclairé a été obtenu et le sang total ont été recueillies auprès de 958 patients consécutifs atteints de pancréatite chronique, pancréatite aiguë, et l'adénocarcinome pancréatique. Les patients ont été recrutés dans tous les établissements participants entre 1997 et 2003 et originaires d'Allemagne (n 884), Autriche (n 11), et la Finlande (n 63). Le diagnostic de pancréatite chronique était basée sur criteria.2 déjà publié, 21 patients ont été classés en fonction de leur diagnostic: 2,22,23 pancréatite alcoolique (349 patients: 290 hommes, 59 femmes; âge moyen de 50 ans); pancréatite idiopathique (231 les patients adultes: 134 hommes, 97 femmes; âge moyen 49 années et 112 enfants [50 mâles, 62 femelles; signifient l'âge de 8 ans]) et pancréatite familiale (64 patients, 25 hommes, 39 femmes; âge moyen 8 ans). En outre, 34 patients atteints de pancréatite aiguë (18 hommes, 16 femmes, âge moyen 52 années) et 168 patients atteints de histologiquement confirmé adénocarcinome pancréatique (101 hommes, 67 femmes; âge moyen 62 années) ont été inclus. Un sous-ensemble de nos patients (n 157) étaient des enfants. Pour 36 enfants, le sang pour l'analyse génétique était disponible des deux parents; pour neuf enfants, le sang était disponible d'un parent. Chaque établissement participant a également recueilli le sang de donneurs de sang choisis au hasard afin de s'assurer que les contrôles proviennent de la même population (588 de l'allemand et 93 d'origine finlandaise, recueillies entre 1997 et 2003). Sang ont également été recueillies à partir de 100 personnes avec l'abus d'alcool chronique d'origine allemande sans antécédents ou des symptômes de pancréatite aiguë ou chronique (recruté à l'Institut central de santé mentale, Mannheim pour les études d'association génétique). Dans un sous-ensemble de patients (n 135), les dossiers hospitaliers ont été examinés et les données concernant la présence de diabète sucré ont été enregistrées. les produits de restriction ont été séparés sur un NuSieve (Teknova, Hollister, CA) gel d'agarose à 3% (Rsa I) et un gel d'agarose à 3% (MboI), et visualisés par coloration au bromure d'éthidium. Un sous-ensemble d'échantillons ont été analysés par amplification PCR suivie d'une analyse de courbe de fusion en utilisant des sondes de transfert d'énergie par résonance de fluorescence et les LightCycler (Roche Diagnostics, Bâle, Suisse), tel que publié antérieurement avec de légères analysisDifferences modifications.26Statistical entre les groupes ont été comparés à la et Fisher tests exacts. Correction pour les tests multiples selon Bonferroni a été appliquée le cas échéant. association de l'allèle de la maladie chez les trios parent a été testée par la test.27 valeur AP de déséquilibre de transmission inférieure à 0,05 a été considérée significant.Top de pageRESULTSThe caractéristiques démographiques de la population de l'étude sont résumés dans le tableau 1. Le génotype et la distribution des allèles des altérations du gène HFE étudiées chez les patients et les contrôles d'origine allemande et finlandaise sont présentés dans le tableau 2. Dans la population de l'étude d'origine allemande, aucune différence statistiquement significative n'a été observée dans le génotype et fréquences alléliques entre les patients atteints de pancréatite alcoolique, la pancréatite idiopathique, la pancréatite chronique familiale, pancréatite aiguë, et le carcinome canalaire pancréatique par rapport aux témoins sains et alcoolisées. Dans la population à l'étude d'origine finlandaise, la distribution des génotypes et des alleles a été similaire entre les patients atteints de pancréatite chronique alcoolique et des témoins sains. En outre, les génotypes et allèles fréquences étaient similaires et pas statistiquement différente entre les patients et les contrôles d'origine allemande et finlandaise. Aucune différence significative n'a été observée entre les patients atteints de pancréatite chronique de cause différente. Analyse des 19 informatif trios mère n'a pas révélé une augmentation de la transmission de l'allèle soit. La présence d'un diabète secondaire chez les patients atteints de pancréatite chronique n'est pas associée à tout génotype ou allèle spécifique (P 0,05, tableau 3). Toutefois, seul un sous-ensemble de patients atteints de la consommation d'alcool de développer une pancréatite. La cause de la pancréatite chronique idiopathique reste largement inconnu. Certains patients ayant des antécédents familiaux de pancréatite ont des mutations dans PRSS1 ou SPINK1, tandis que d'autres ne le font pas. Par conséquent, on suppose que de nouveaux facteurs de risque, non encore identifiés sont importants dans le développement de la maladie chez ces individus. La découverte de prédispositions génétiques à la pancréatite chronique suggère que plus peuvent rester à découvert. Dans la présente étude des altérations du gène HFE n'ont pas été associés à une pancréatite chronique alcoolique, idiopathique ou familiale. De plus, aucune association n'a été observée chez les patients atteints d'un adénocarcinome pancréatique. En plus des contrôles sains, nous avons utilisé les patients avec l'abus d'alcool chronique sévère sans pancréatite chronique en tant que groupe de contrôle pour les patients atteints de pancréatite alcoolique. Néanmoins, la comparaison des patients atteints de pancréatite alcoolique, les patients alcooliques sans pancréatite, et des contrôles sains n'a pas révélé de différences significatives. Nos résultats concordent avec ceux de Frenzer et al., 15 qui, en se concentrant sur la cirrhose alcoolique, a utilisé un petit nombre de patients atteints de pancréatite chronique en tant que témoins. Dans notre étude, les génotypes observés étaient comparables à la signalés précédemment, les données des études de population en Allemagne et Finland.28, 29We hypothèse que HFE variantes agissant par l'intermédiaire du métabolisme du fer modifié peut prédisposer les patients à la fibrose ou la carcinogenèse. Plusieurs facteurs possibles peuvent expliquer l'absence d'association entre les génotypes HFE et diverses maladies pancréatiques. Tout d'abord, le fer lié gènes autres que HFE peut être important. Récemment, d'autres gènes qui jouent un rôle important dans le maintien de l'homéostasie du fer étaient identified.30 mutations dans l'hepcidine (HAMP), fer protéine de transfert importateur récepteur 2 (TFR2), et hémojuvéline (HJV) ont été identifiées chez des patients avec metabolism.7 de fer fortement altéré, 30 modifications génétiques de ces gènes n'ont pas été explorées dans notre étude. Deuxièmement, parce que le seul HFE variantes n'ont pas présenté un facteur prédisposant à nos patients, une relation entre HFE et d'autres gènes qui jouent un rôle dans le métabolisme du fer ou de défense antioxydant cellulaire peut être important. Les complexes de produit génique HFE de type sauvage avec le récepteur de la transferrine (TFRC). On a montré que les deux variants HFE étudiées pour augmenter l'affinité du récepteur pour la transferrine et d'augmenter l'absorption du fer cellulaire. Troisièmement, parce que nous n'avons pas déterminé des marqueurs du métabolisme du fer dans le sang ou les tissus, nous ne pouvons pas exclure totalement le rôle du fer dans la prédisposition des patients à une pancréatite ou un cancer du pancréas. 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