Is Abdominal Paracentesis Drainage Too Risky for Patients With Severe Acute Pancreatitis?

additional support for its pathogenicity. Her sons were referred for genetic testing for HNF1A mutations, given implications for screening and surveillance for HA and DM. On her last follow-up 12 months after surgery she remained completely asymptomatic, with small stable lesions in the left liver lobe on imaging. Biallelic inactivation of the HNF1A gene was identified in 10 of 16 patients with HA, as well as 3 heterozygous germline mutations in 2002, suggesting a link between MODY-3 and HA.3 Shortly after, 2 French families with HNF1A germline mutations were described,4 confirming that biallelic inactivation caused hepatic adenomatosis. Importantly, incomplete penetrance for both MODY-3 and adenomatosis was observed. Our patient did not have abdominal symptoms or complaints, and no significant liver function test abnormalities, despite a large tumor burden. Only 1 previously reported case of MODY-3 diabetes and asymptomatic hepatic adenomatosis has been described in a French family. The consequence of hemorrhage in lesions >5 cm, with consideration for empiric surgical resection, emphasizes the need to recognize the entity of hepatic adenomatosis in families with MODY-3. Although there are no consensus guidelines for hepatic adenomatosis, surveillance for both growth of lesions and for transformation to hepatocellular carcinoma should be considered. We present the first case of hepatic adenomatosis associated with the HNF1A 1340 C>T (p.P447L) variant, leading to haploinsufficiency and hepatic adenomatosis with young-onset diabetes in the proband, and young-onset diabetes in other generations. The discovery of HNF1A mutation in patients with hepatic adenomatosis should prompt clinical evaluation for glucose intolerance and specifically MODY. Conversely, special consideration should be given to counseling and screening for hepatic adenomatosis in patients with young onset DM, particularly without autoantibodies. Specifically, patients with a personal or strong family history of DM at a young age or a family history of hepatic adenomatosis should be examined for germline mutations in the HNF1A gene.