Liver adenomatosis in patients with hepatocyte nuclear factor-1 alpha maturity onset diabetes of the young (HNF1A-MODY): Clinical, radiological and pathological characteristics in a French series.

doi:10.1111/1753-0407.12959

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Liver adenomatosis in patients with hepatocyte nuclear factor-1 alpha maturity onset diabetes of the young (HNF1A-MODY): Clinical, radiological and pathological characteristics in a French series.

Journal of diabetes - 1 Jan 2020

Haddouche Aini, Bellanne-Chantelot Christine, Rod Anne, Fournier Luc, Chiche Laurence, Gautier Jean-Francois, Timsit Jose, Laboureau Sandrine, Chaillous Lucy, Valero Rene, Larger Etienne, Jeandidier Nathalie, Wilhelm Jean-Marie, Popelier Marc, Guillausseau Pierre-Jean, Thivolet Charles, Lecomte Pierre, Benhamou Pierre-Yves, Reznik Yves

Abstract excerpt

BACKGROUND: Liver adenomatosis (LA) is a rare disease resulting from biallelic inactivation of the hepatocyte nuclear factor-1 alpha (HNF1A) gene, which induces the proliferation of adenoma cells in liver parenchyma. Liver adenomatosis has only been documented in case reports from patients carrying a HNF1A germline mutation. We have evaluated the frequency of LA among a large cohort of patients with...

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PMID: 31166087
DOI: 10.1111/1753-0407.12959

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HNF1A

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