Portal Hypertension and Nodular Regenerative Hyperplasia in a Patient With Celiac Disease

Zusammenfassung

Die noduläre regenerative Hyperplasie (NRH) ist eine seltene Ursache des portalen Hochdrucks und oft mit Krankheiten des rheumatischen Formenkreises oder lymphoproliferativen Erkrankungen assoziiert. Wir schildern hier den Fall eines neunzehnjährigen Patienten mit Sprue und portaler Hypertension. In der Leberbiopsie zeigte sich eine NRH als Ursache des portalen Hypertonus. Weder eine Autoimmunkrankheit noch ein Defekt im Gerinnungssystem konnten als mögliche Ursache für die NRH gezeigt werden. Eine Leberbeteiligung im Rahmen der Sprue kommt relativ oft vor, das Auftreten einer NRH bei Zöliakie wurde dagegen bisher nur selten beschrieben. Wir diskutieren die mögliche Beziehung zwischen Zöliakie und NRH.

Abstract

Nodular regenerative hyperplasia (NRH) is an uncommon cause of portal hypertension. The disease is often associated with rheumatological or lymphoproliferative disorders. We report the case of a 19-year old patient with celiac disease and portal hypertension. Biopsy of the liver revealed NRH as the underlying cause. There were no signs of autoimmune diseases or defects in the coagulation system that might cause NRH. Celiac disease is often associated with liver abnormalities, but the association with NRH has rarely been described. We discuss the possible relationship of celiac disease and NRH.

Schlüsselwörter

Portale Hypertension - noduläre regenerative Hyperplasie - Sprue - Zöliakie

Key words

Portal hypertension - nodular regenerative hyperplasia - celiac disease

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