Some of FH patients cannot survive despite the recent progress in intensive care including artificial liver support (ALS) and such patients need liver transplantations (LTX) as the only effective treatment. Criteria for LTX on FH in our institution contains being subacute type and having the hepatic atrophy added to being fitted the Guideline for LTX in Japanese Acute Hepatic Failure Study Group (JAHFSG). To make the problems concerning LDLTX, which is common LTX in Japan, obvious and to adopt measures to them, we examined the clinical course and outcome of transplanted FH cases and nottransplanted cases in spite of having fulfilled the criteria for LTX. In 22 cases of FH treated in our institution during past five years, nineteen patients fulfilled the criteria for LTX of our institution. In six cases (31.6%) LDLTX had been performed. Though two of the six transplanted patients could not survive because of severe infection, four discharged alive. Our criteria indicated positive predictive value (PPV)=0.79, negative predictive value (NPV)=1.00, sensitivity=1.00, predictive accuracy (PA)=0.81, those were better than those calculated using guideline of JAHFSG, and specificity=0.40 was the same between JAHFSG's guideline and our criteria. Criteria adopted in King's College Hospital indicated sensitivity=0.09, that revealed the difference of pathophysiology, especially of etiology between in Western countries and in Japan. Yoshiba's discrimination's NPV and sensitivity were both 0.56, which meant it should not been applied to these cases which had already developed FH. In 14 patients who had fulfilled the criteria and had not been transplanted, three survived with intensive care and 11 deceased. The reasons why LTX had not been performed consisted of recipients' factors, such as severe infection (4 cases), uncontrollable hemorrhage (4 cases), and of donors' factors, such as absence of compatible donor (3 cases) and families' unwillingness to LTX (2 cases). These results indicate that there are three issues concerning LDLTX for FH. Namely, 1. Indication of LTX, 2. Liver graft donation, 3. Treatment for patients who would not be able to be transplanted. Further consideration on the indication of LTX and establishment of administrative system for LDLTX are badly needed.
View full abstract