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 HNF1A‐maturity onset diabetes of the young (MODY), previously termed “MODY3,” and herein describe its clinical, radiological, and pathological characteristics.

中文翻译：

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年轻肝细胞核因子 1 α 成熟型糖尿病 (HNF1A-MODY) 患者的肝腺瘤病：法国系列的临床、放射学和病理学特征。

肝腺瘤病 (LA) 是一种罕见疾病，由肝细胞核因子 1 α ( HNF1A ) 基因双等位基因失活引起，可诱导肝实质中腺瘤细胞增殖。仅在携带HNF1A种系突变的患者的病例报告中记录了肝腺瘤病。我们评估了一大群HNF1A青少年发病型糖尿病 (MODY)（以前称为“MODY3”）患者中 LA 的频率，并在本文中描述了其临床、放射学和病理学特征。