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Cholestase-assoziierte Lebererkrankungen im Neugeborenen- und Säuglingsalter

Cholestasis-associated hepatopathies in neonates and infants

Der Pathologe volume 29, pages 61–72 (2008)Cite this article

Zusammenfassung

Cholestasen im Neugeborenen- und Säuglingsalter stellen Pädiater und Pathologen nicht selten vor diagnostische Probleme. Eine Besonderheit der Neugeborenenleber besteht in ihrer Eigenschaft, auf unterschiedliche kausalgenetische Faktoren mit einem unspezifischen Reaktionsmuster unter dem Bild der so genannten neonatalen Hepatitis in Erscheinung zu treten. Eine differenzialdiagnostische Abgrenzung der verschiedenen Krankheitsbilder ist histologisch bei Beachtung spezieller morphologischer Teilkomponenten durchaus möglich. So manifestieren sich extrahepatische Gallenabflussstörungen wie Atresien oder Stenosen des Ductus hepaticus bzw. Choledochuszysten zudem mit portalen Gallengangsproliferaten einschließlich Gallenretentionszeichen. Bei einem Alagille-Syndrom (Syn.: arteriohepatische Dysplasie) hingegen ist der Nachweis eines interlobulären Gallengangsmangels ein wichtiges, differenzialdiagnostisches Merkmal. Stoffwechseldefekte, wie die Fruktoseintoleranz und die Galaktosämie, sind zusätzlich mit einer Leberzellverfettung vergesellschaftet. Die Kenntnis des klinischen Verlaufs, der laborchemischen und bildgebenden Daten sind in der Zusammenschau mit dem morphologischen Befund für die definitive Diagnosestellung erforderlich und setzen eine enge Kooperation zwischen Pädiater und Pathologen voraus.

Abstract

Cholestasis in neonates and infants frequently confronts pediatricians and pathologists with diagnostic problems. A specific feature of the liver in neonates is the ability to react to different causative factors with a non-specific hepatitis-like picture, the so-called neonatal hepatitis. A diagnostic discrimination of the various diseases is histologically only possible with close attention to typical morphologic features. Thus, extrahepatic biliary obstructions, such as atresia or stenosis of the hepatic duct or choledochal cysts present with portal bile duct proliferation and signs of bile retention in the neoducts. In Alagille syndrome (arteriohepatic dysplasia), however, paucity of intrahepatic bile ducts is an important diagnostic feature. Metabolic disorders, such as fructosemia and galactosemia are additionally associated with steatosis. Knowledge of the clinical course and laboratory and imaging data are necessary to make the definitive diagnosis in synopsis with the morphologic findings and requires a close co-operation between the pediatrician and the pathologist.

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Danksagung

Für die Herstellung der Schnittpräparate und Färbungen danken wir Frau Susanne Steiner, für die Hilfe bei der Bildbearbeitung danken wir Herrn Gerrit Klemm und seinen Mitarbeiterinnen und Mitarbeitern.

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  1. Institut für Pathologie der Universität Bonn , Sigmund-Freud-Straße 25, 53127, Bonn, Deutschland

    G. Knöpfle, A. Adam & H.-P. Fischer

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  1. G. Knöpfle
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  2. A. Adam
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  3. H.-P. Fischer
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Correspondence to G. Knöpfle.

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Knöpfle, G., Adam, A. & Fischer, HP. Cholestase-assoziierte Lebererkrankungen im Neugeborenen- und Säuglingsalter. Pathologe 29, 61–72 (2008). https://doi.org/10.1007/s00292-007-0955-1

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