Heme oxygenase-1 (HO-1) is a stress-induced enzyme that catalyses the oxidation of heme to biliverdin. The primary deficiency of this enzyme has been shown in HO-1 knockout mice, and is characterized by intrauterine death and chronic inflammation. The first case of human HO-1 deficiency was reported in 1999. Human HO-1 deficiency has been observed to involve the endothelial cells more severely, resulting in hemolysis and disseminated intravascular coagulation. We report another case of human HO-1 deficiency in a young girl with congenital asplenia, who presented with severe hemolysis, inflammation, nephritis, which was refractory to therapy with corticosteroids, cyclophosphamide, and rituximab.

Background. Median survival in patients with hepatocellular carcinoma (HCC) and portal vein tumor thrombosis (PVTT) is 2–6 months; conventionally liver transplantation is contraindicated. Methods. We studied outcomes following living donor liver transplantation (LDLT) post-PVTT downstaging (DS) with stereotactic body radiotherapy (SBRT), and tumor ablation (with transarterial chemo- or radio-embolization). Results. Of 2348 consecutive LDLTs, 451 were for HCC, including 25 with PVTT (mainly Vp1-3) after successful DS and 20 with Vp1/2 PVTT without previous treatment. DS was attempted in 43, was successful in 27 (63%), and 25 underwent LDLT. Median alpha fetoprotein (AFP) at diagnosis and pre-LDLT were 78.1 ng/mL (3-58 200) and 55 ng/mL (2-7320), respectively. Mean DS to LDLT time was 10.2 weeks (5–16). Excluding 2 postoperative deaths, 1- and 5-year overall survival (OS) and recurrence-free survival (RFS) were 82%, 57%, and 77%, 51%, respectively, comparable to survival in 382 HCC patients without PVTT undergoing upfront LDLT (5-y OS 65%, P = 0.06; RFS 66%, P = 0.33, respectively). There was a trend toward better OS in DS+LDLT versus non-DS LDLT group (5-y OS/RFS—48%/40%). OS was significantly better than in HCC-PVTT patients receiving no intervention or palliative Sorafenib alone (1-y OS of 0%) or Sorafenib with TARE/SBRT (2-y OS of 17%) at our center during the study period. Initial AFP <400 ng/mL and AFP fall (initial minus pre-LDLT) >2000 ng/mL predicted better RFS; Grade III/IV predicted worse OS in DS patients. Conclusions. HCC patients with PVTT can achieve acceptable survival with LDLT after successful DS. Low initial AFP level, a significant drop in AFP with DS and low tumor grade, favorably influence survival in these patients.

Advances in medical and surgical techniques associated with multidisciplinary teams including skilled pediatric liver transplant surgeons, anesthetists, dedicated pediatric hepatologists, pediatric intensivists, interventional radiologists and pathologists resulted in an excellent outcome of living related liver transplants in children. Low age and weight of the baby does not seem to be a contraindication for liver transplantation as outcome were comparable in our experience.

CT volumetry overestimate LL graft and underestimate LLS graft weight. This should be factored in when selecting LL graft by taking higher GRWR.

Conventional selection criteria for liver transplantation (LT) in patients with hepatocellular carcinoma (HCC) are based on tumour size/number only, and do not consider vital surrogates of tumor biology such as alpha‐fetoprotein (AFP) and tumor [18F]fluorodeoxyglucose positron emission tomography ([18F]FDG PET) avidity. We analyzed survival outcomes, and predictors of HCC recurrence in 405 patients with cirrhosis and HCC (HCC‐cirr) who underwent living donor LT (LDLT) using our expanded selection criteria: no extrahepatic disease or major vascular invasion, irrespective of tumor size/number. Fifty‐one percent patients had tumours beyond Milan, and 43% beyond the University of California San Francisco [UCSF] criteria. The 5‐year overall survival (OS) and recurrence‐free survival (RFS) were 64% and 70%, respectively. Three preoperatively available factors predicted recurrence: pre‐LT AFP ≥100 ng/mL (P = 0.005; hazard ratio [HR], 2.190), tumor burden beyond the UCSF criteria (P = 0.001; HR, 2.640), and [18F]FDG PET avidity (P = 0.004; HR, 2.442). A prognostic model based on the number and combination of the aforementioned preoperative risk factors was developed using a competing‐risk RFS model. Three risk groups were identified: low (none or a single risk factor present, 9.3% recurrence), moderate (AFP ≥100 ng/mL and [18F]FDG PET avidity, or beyond UCSF tumor and [18F]FDG PET avidity, 25% recurrence), and high (AFP ≥100 ng/mL and beyond UCSF, or presence of all 3 risk factors, 46% recurrence). Acceptable long‐term outcomes were achieved using our expanded selection criteria. Our prognostic model to predict recurrence based on preoperative biological and morphological factors could guide pretransplant management (downstaging versus upfront LDLT) with the aim of reducing post‐LDLT recurrence.