Liver Failure in Advanced Adult-onset Polycystic Kidney Disease.

Polycystic liver disease is the most common extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). Hepatic cysts are typically incidental findings, with occasional complications including cyst haemorrhage, infection and rupture. In contrast to the typically benign course of polycystic liver disease, we present a rare case of fatal decompensated liver failure in a patient with ADPKD. This is a case of a 58-year-old man with end-stage renal disease on haemodialysis presenting with new-onset ascites and decompensated liver failure following bilateral nephrectomy. Cirrhosis in ADPKD is a late manifestation of the disease, but it should be considered in the perioperative risk of patients with ADPKD.

Curious case of calciphylaxis leading to acute mitral regurgitation.

Calciphylaxis is uncommon and typically seen in patients with end-stage renal disease. It has been defined as a vasculopathic disorder characterised by cutaneous ischaemia and necrosis due to calcification, intimal fibroplasia and thrombosis of pannicular arterioles. We present the case of a 74-year-old woman with chronic kidney disease stage III who developed calciphylaxis leading to mitral valve calcification, chordae tendineae rupture and acute mitral regurgitation. Although an alternative explanation can typically be found for non-uraemic calciphylaxis, her evaluation did not reveal any usual non-uraemic causes including elevated calcium-phosphorus product, hyperparathyroidism, or evidence of connective tissue disease. Her wounds improved with sodium thiosulfate, pamidronate, penicillin and hyperbaric oxygen therapies but she ultimately decompensated with the onset of acute mitral regurgitation attributed to rupture of a previously calcified chordae tendineae. This case highlights an unusual case of calciphylaxis without clear precipitant as well as a novel manifestation of the disease.