At Jikei University Hospital, 18 living-donor liver transplantations (LDLT) were performed from February 2007 to June 2016. Of these, 10 recipients received LDLT for end-stage liver disease (ESLD) due to primary biliary cholangitis (PBC), and details were as follows; median age of 49 years, female in 9 and two ABO-incompatible transplant. Median postoperative hospital stay of donors and recipients were 10 and 29.5 days, respectively. All donors and recipients made satisfactory recovery, and remain well with a median follow-up of 3.2-year. Initial immuno-suppressants were tacrolimus and steroids, which were switched to cyclosporine A and steroids between 3 and 13 months after LDLT to prevent PBC recurrence. Three patients received liver biopsy after LDLT due to acute cellular rejection, and no histological findings of PBC recurrence were identified in all 3 patients. In conclusion, our treatment strategy for ESLD due to PBC seems to be safe and acceptable.

A female in her 30s had been diagnosed with type I Gaucher disease at 4 years of age based on findings of splenomegaly, thrombocytopenia, and anemia. The glucocerebrosidase (GBA) gene variation was L444P/R433G. Enzyme replacement therapy (ERT) with alglucerase was started and switched to imiglucerase afterwards. Her hepatosplenomegaly improved. Because oral medication with eliglustat was approved, ERT was switched to substrate reduction therapy (SRT) after the patient was checked for genetic polymorphisms of cytochrome p450 (CYP) 2D6. Her quality of life was improved by oral medication. However, we must be alert for drug-drug interactions during SRT. We feel that the findings of this rare case will prove of interest to others.

This study was conducted on 13 patients with chronic hepatitis B who switched from Adefovir dipivoxil (ADV) or Tenofovir disoproxil fumarate (TDF) to Tenofovir alafenamide (TAF). We evaluated e-GFR, s-P, %TRP, L-FABP, FGF23, before switching and after switching at 4, 12 and 20 weeks.

We revealed that L-FABP decreased markedly by switching to TAF, and this result seemed to bring about recovery of renal tubule damage early. In addition, FGF23 value significantly increased at 4 weeks after switching.

Originally, improvement of phosphorus reabsorption rate by recovery of renal tubular damage increases s-P level. However, this result suggested that FGF23 as a regulatory hormone of phosphorus metabolism rises first, before increased s-P. In other words, FGF23 was implied to be an early indicator of the change in phosphorus metabolism.