Living-Donor Liver Transplantation for Erythropoietic Protoporphyria: A Case Report and Literature Review.

Erythropoietic protoporphyria (EPP) is a very rare disease with an estimated prevalence of 1 in 200,000 individuals. Decreased ferrochelatase activity causes the accumulation of protoporphyrin in the body, and light exposure results in the generation of active oxygen, causing photosensitivity. Liver damage has the greatest influence on the prognosis, and liver transplantation is the only treatment option for patients with decompensated liver cirrhosis. We report a case of living-donor liver transplantation for decompensated liver cirrhosis associated with EPP. The patient was a 52-year-old male who led a normal life except for mild photosensitivity. When the patient was 37-year-old, hepatic dysfunction was noticed. At 48-year-old, high erythrocyte protoporphyrin levels, skin biopsy, and genetic tests resulted in a diagnosis of EPP. The patient underwent living- donor liver transplantation because of decompensated liver cirrhosis. In the operating room and intensive care unit, a special light-shielding film was applied to all light sources to block light with harmful wavelengths during treatment. Due to the need for special measures, a lecture on patients with EPP was given before surgery to deepen understanding among all medical professionals involved in the treatment. As a result, no adverse events occurred during the perioperative period, and the patient was discharged on the 46th post-operative day. Currently, the transplanted liver is functioning extremely well, and the patient is alive 3 years post-transplant. Herein, we describe a case of living donor liver transplantation for EPP with a brief literature review.

Tumor-to-Tumor Metastasis of Medullary Thyroid Carcinoma to Paraganglioma in a Multiple Endocrine Neoplasia Type 2B Patient: A Case Report and Literature Review.

Tumor-to-tumor metastasis is a rare phenomenon in which primary tumor cells metastasize to other tumors. Herein, we report an extremely rare case of tumor-to-tumor metastasis of medullary thyroid carcinoma to a paraganglioma in a patient with multiple endocrine neoplasia type 2B. Based on genetic examination, a 36-year-old woman was diagnosed with multiple endocrine neoplasia type 2B when she was 24 years old. She had a history of total thyroidectomy for medullary thyroid carcinoma and bilateral adrenalectomy for pheochromocytomas, which were performed when she was 15 years and 29 years old, respectively. Follow-up computed tomography demonstrated a retroperitoneal tumor of 30 mm in diameter beside the left kidney and a liver tumor of 16 mm in diameter located in segment 6. The retroperitoneal and liver tumors were surgically resected and examined by a pathologist. Histological examination revealed the classic Zellballen pattern in the retroperitoneal tumor, rendering the diagnosis of a paraganglioma recurrence. Inside the tumor, a white nodule positive for carcinoembryonic antigen, weakly positive for calcitonin, and negative for tyrosine hydroxylase, was identified and diagnosed as a metastatic medullary thyroid carcinoma with high malignant potential. The liver lesion was diagnosed as a metastasis of the medullary thyroid carcinoma. This is the first report of tumor-to-tumor metastasis of medullary thyroid carcinoma to paraganglioma in a patient with multiple endocrine neoplasia type 2B twenty years after total thyroidectomy.

Bleeding from jejunal varices formed at the Roux-en-Y jejunum site caused by the compression of the left renal vein after living donor liver transplantation with renoportal anastomosis.

BACKGROUND: Renoportal anastomosis is an option for the portal vein reconstruction of a liver transplantation with grade 4 portal vein thrombosis and a splenorenal shunt. Here, we report the case of gastrointestinal bleeding who underwent living donor liver transplantation (LDLT) with renoportal anastomosis. CASE PRESENTATION: Six-year-old female patient who underwent LDLT with renoportal anastomosis at 1 year of age had severe anemia with normal liver function during the follow-up period. The varices at the Roux-en-Y jejunum were considered the source of bleeding, and the compression of the left renal vein, which is known as a cause of Nutcracker syndrome, seemed to induce venous hypertension through the splenorenal shunt, which might induce the formation of the varices. She underwent percutaneous transhepatic sclerotherapy of the varices, and the anemia improved at her last follow-up, 6 months after sclerotherapy. This is the first reported case of Roux-en-Y jejunal varices bleeding related to the compression of the left renal vein after LDLT was performed with renoportal anastomosis. CONCLUSIONS: Although renoportal anastomosis should be cautiously performed when there are no options for severe portal vein thrombosis, the status of the left renal vein and new collateral formation should be observed carefully during the follow-up period in pediatric cases of renoportal anastomosis.

Protection of liver sinusoids by intravenous administration of human Muse cells in a rat extra-small partial liver transplantation model.

Small-for-size syndrome (SFSS) has a poor prognosis due to excessive shear stress and sinusoidal microcirculatory disturbances in the acute phase after living-donor liver transplantation (LDLT). Multilineage-differentiating stress enduring (Muse) cells are reparative stem cells found in various tissues and currently under clinical trials. These cells selectively home to damaged sites via the sphingosine-1-phosphate (S1P)-S1P receptor 2 system and repair damaged tissue by pleiotropic effects, including tissue protection and damaged/apoptotic cell replacement by differentiating into tissue-constituent cells. The effects of intravenously administered human bone marrow-Muse cells and -mesenchymal stem cells (MSCs) (4 × 105 ) on liver sinusoidal endothelial cells (LSECs) were examined in a rat SFSS model without immunosuppression. Compared with MSCs, Muse cells intensively homed to the grafted liver, distributed to the sinusoids and vessels, and delivered improved blood chemistry and Ki-67(+) proliferative hepatocytes and -LSECs within 3 days. Tissue clearing and three-dimensional imaging by multiphoton laser confocal microscopy revealed maintenance of the sinusoid continuity, organization, and surface area, as well as decreased sinusoid interruption in the Muse group. Small-interfering RNA-induced knockdown of hepatocyte growth factor and vascular endothelial growth factor-A impaired the protective effect of Muse cells on LSECs. Intravenous injection of Muse cells might be a feasible approach for LDLT with less recipient burden.

Effect of enhanced recovery after surgery protocol on recovery after open hepatectomy: a randomized clinical trial.

PURPOSE: Enhanced recovery after surgery (ERAS) is beneficial to patients undergoing digestive surgery. However, its efficacy in patients undergoing open hepatectomy remains unclear. METHODS: Consecutive patients scheduled for open hepatectomy were randomly assigned to undergo either ERAS or conventional postoperative management. The primary endpoint was the amount of time that elapsed before patients were considered medically fit for discharge (MFD) and length of hospital stay (LOHS). Secondary endpoints included morbidity, mortality, the time to first flatus, defecation, first walk, and freedom from infusion. Perioperative serum nutritional markers, insulin resistance, respiratory quotient (RQ), and resting energy expenditure (REE) were also assessed. RESULTS: Between August 2014 and March 2017, 57 patients were randomized into 2 groups; ERAS group (n = 29) and conventional management (n = 28). The median MFD was not significantly different between the ERAS and conventional management groups (6.5 vs. 7 days; P = 0.381). Recovery from gastrointestinal paresis was significantly quicker in the ERAS group (1.8 vs. 2.4 days; P = 0.004). There were no significant differences in serum markers, insulin resistance, RQ, and REE. CONCLUSION: This trial did not demonstrate greater efficacy of the ERAS protocol following open hepatectomy in terms of the MFD and LOHS. However, the ERAS protocol was associated with better recovery from postoperative gastrointestinal paresis, suggesting that it is useful for patients undergoing open hepatectomy.

Risks of Living Donor Liver Transplantation Using Small-For-Size Grafts.

BACKGROUND: In living donor liver transplantation (LDLT), a graft-to-recipient weight ratio (GRWR) of under 0.8 is recognized as the critical graft size. Our aim was to compare the survival rates of recipients with small-for-size grafts (SFSG: GRWR <0.8), normal-sized grafts (NSG), and large-for-size grafts (LFSG: GRWR ≥ 3.5) and to investigate the mortality risk with SFSG. METHODS: Between 1991 and April 2019, we performed 188 LDLT surgeries. Recently, we added splenectomy when portal vein pressure is high (>17 mm Hg) to interrupt the splenic bloodstream. We divided all LDLT cases retrospectively into 3 groups: an SFSG group (n = 22), NSG group (n = 154), and LFSG group (n = 12). We investigated the survival rates in these groups. Furthermore, we divided the SFSG group into 2 subgroups: an SFSG with splenectomy (SFSG+S) group (n = 7) and an SFSG without splenectomy group. We investigated the occurrence rates of lethal complications such as portal vein thrombosis, hepatic artery thrombosis, and hepatic vein thrombosis. RESULTS: The 5-year survival rate in the SFSG group was significantly lower (52.8%) than in the other groups (NSG: 84.5%; LFSG: 83.3%), but that of the SFSG+S group was similar (80.0%) to that of other groups. There was no difference in the occurrence of postoperative complications such as portal vein thrombosis, hepatic artery thrombosis, or hepatic vein thrombosis between the SFSG+S group and other groups. CONCLUSIONS: Graft survival of LDLT using SFSG+S was as good as that of normal-sized grafts. Reducing portal vein pressure was important for SFSG.