Rapid development of diffuse hepatic calcification in a patient with congenital hepatic fibrosis and secondary hyperparathyroidism.

Abstract

Listen

Translate article

A man in his 20s with congenital hepatic fibrosis and polycystic kidney disease (PKD) presented with symptoms of anemia. Upper gastrointestinal endoscopy revealed mild exudative hemorrhage due to gastric antral vascular ectasia, which was treated with argon plasma coagulation. Computed tomography (CT) at admission revealed diffuse hepatic calcification and ectopic calcifications in the left buttock and bilateral inguinal regions. CT performed five months earlier showed no hepatic calcification, indicating rapid progression. The patient had been receiving maintenance hemodialysis for four months because of end-stage chronic kidney disease due to PKD. Laboratory tests revealed hypercalcemia (10.9mg/dL), hyperphosphatemia (8.3mg/dL), markedly elevated intact parathyroid hormone (1,640pg/mL), and low 1,25-dihydroxy vitamin D (17.6pg/mL), consistent with secondary hyperparathyroidism. Treatment with evocalcet and dialysis regimen adjustments led to improvements in laboratory parameters. However, following discharge, the patient experienced recurrent hospitalization due to gastrointestinal bleeding, hepatic encephalopathy, and biliary tract infections. Progressive hepatic calcification ultimately led to a decline in liver function, necessitating simultaneous brain-dead donor liver and kidney transplantation 17months after the initial admission. This case highlights the complex interplay between congenital hepatic fibrosis, mineral metabolism disturbances associated with dialysis, and the rare occurrence of rapidly progressing hepatic calcification.