Publications by authors named "Seisuke Sakamoto"
Article Synopsis
- * A 13-year-old girl with carbamoyl phosphate synthetase I (CPS1) deficiency underwent liver transplantation from her mother, facing significant challenges due to her severe scoliosis which led to portal vein complications necessitating multiple surgical interventions.
- * The case highlights the importance of meticulous preoperative assessments and tailored surgical strategies for patients with structural abnormalities to prevent complications and ensure successful outcomes.
Article Synopsis
- Liver transplantation (LDLT) can effectively treat children with propionic acidemia (PA), showing a 100% survival rate in a study of 12 patients.
- The study compared outcomes based on the timing of LDLT, dividing patients into groups based on whether the surgery occurred within a year of their first metabolic decompensation or later.
- Early LDLT may prevent complications like cardiomyopathy and neurological issues, despite later patients showing some pre-existing conditions and post-surgery developmental challenges.
Aim: To assess the preoperative disease characteristics and indications for living donor liver transplantation (LDLT), complications, patient survival, and prognosis after LDLT for fibropolycystic liver disease (FLD) in children.
Methods: We undertook a cross-sectional survey of patients who underwent LDLT for FLD between January 2002 and December 2020.
Results: A total of 35 patients (22 male and 13 female individuals) with FLD were included in this study, of whom 19 (54.
Article Synopsis
- Natural killer (NK) cells show potential as therapy for hard-to-treat cancers like hepatocellular carcinoma (HCC), but are often dysfunctional in HCC patients.
- This study introduces LMNC-NK cells, derived from liver graft perfusate, which are more plentiful and exhibit enhanced cytotoxic properties compared to traditional NK cells from peripheral blood.
- Through extensive gene analysis, the research highlights significant differences in gene expression that contribute to LMNC-NK cells’ superior effectiveness against HCC, suggesting their use as a promising treatment option.
Article Synopsis
- The text discusses the limited information on the use of daptomycin for treating persistent bacteremia in pediatric patients, especially those who have undergone solid organ transplantation.
- It presents a case study of an eight-year-old girl whose persistent bacteremia, caused by Staphylococcus epidermidis, was successfully treated with daptomycin after adjusting the dosage due to her renal dysfunction.
- A literature review revealed very few cases of daptomycin use in children with persistent bacteremia, emphasizing the need for more research on its clinical efficacy and pharmacokinetics in this special patient group.
Article Synopsis
- Collaboration among international pediatric oncology groups has led to improved survival rates for children undergoing liver transplantation (LT) for unresectable liver tumors, establishing LT as the standard care method.
- Hepatoblastoma and hepatocellular carcinoma (HCC) are the primary types of liver cancers treated with LT, showing over 80% long-term survival rates for hepatoblastoma and better outcomes for HCC when compared to liver resection.
- The development of living donor liver transplantation has enhanced waitlist outcomes, graft quality, and timing with chemotherapy, while ongoing research and a multidisciplinary approach are essential for advancing treatments for these rare pediatric malignancies.
Article Synopsis
- - A nationwide study in Japan analyzed pediatric deceased donor liver transplantation (pDDLT) outcomes, focusing on the pediatric prioritization system implemented in 2018 to improve organ allocation.
- - Data from 1999 to 2021 revealed a 1-year graft survival rate of 86.6% and identified four key risk factors affecting survival, including donor characteristics and recipient conditions.
- - The new prioritization system led to better allocation of pediatric donor livers to pediatric recipients, increasing the number of pDDLTs and significantly enhancing survival rates and overall transplant conditions.
Article Synopsis
- Scientists studied patients with a rare disease called methylmalonic acidemia (MMA) who had liver transplants to see how well they did over time.
- They found that after 10 years, most of the patients were still alive and doing well, especially if they got their transplant within two years of getting sick.
- However, some patients still had health issues like learning difficulties and kidney problems, but their MMA levels went down a lot after the transplant.
Background: Methylmalonic acidemia (MMA) is an autosomal recessive disorder caused by defects in propionyl-CoA (P-CoA) catabolism; of note, liver neoplasms rarely occur as a long-term complication of the disorder. Herein, we report the case of a patient with MMA and hepatocellular carcinoma (HCC) who was successfully treated with a living-donor liver transplant (LDLT) following prior kidney transplantation.
Case Report: A 25-year-old male patient with MMA underwent LDLT with a left lobe graft because of metabolic instability and liver neoplasms.
Article Synopsis
- Liver transplantation (LT) is essential for treating children with severe liver disease, particularly using left lateral segment (LLS) grafts in split and living donor procedures.
- Small infants face challenges with LLS grafts due to size mismatches, prompting investigations into reducing graft thickness to improve suitability.
- Successful outcomes in a study from Tokyo show a 15-year graft survival rate of 89.9% for small infants receiving reduced-size LLS grafts, highlighting the importance of tailoring grafts to recipient size for better survival rates.
Article Synopsis
- Choline is vital for health, and its deficiency can lead to a liver condition called steatohepatitis, primarily due to issues with how phosphatidylcholine is processed in the intestines.
- Researchers studied mice lacking the Atp8b1 gene, which is important for lipid transport in intestinal cells, and found that these mice developed steatohepatitis in just four weeks.
- Supplementing choline in the diet of these mice helped reverse their liver condition, suggesting that similar choline supplementation might benefit patients with ATP8B1-related liver issues.
Article Synopsis
- COACH syndrome is a rare genetic disorder causing liver fibrosis and related complications, prompting the need for liver transplantation (LT) in affected patients.
- This study reports the outcomes of 4 female children who underwent LT for COACH syndrome, all experiencing severe portal hypertension and various degrees of renal impairment.
- The findings suggest that LT is an effective treatment for severe portal hypertension in COACH syndrome, although ongoing monitoring of renal function post-transplant is essential.
Article Synopsis
- Immediate extubation (IE) after liver transplantation (LT) is now common in pediatric patients, but no established selection criteria exist for its use, prompting the development of new protocols.
- A retrospective study analyzed LT cases from 2016-2020 to compare outcomes between immediate extubation (IE) and non-immediate extubation (NIE) patients, noting a total of 81 IE and 185 NIE cases.
- The results showed that all patients in the IE group were successfully extubated without re-intubation, while a significant number of delayed NIE patients required tracheostomies, indicating that earlier extubation is safer.
Article Synopsis
- Intestinal transplantation (ITx) is a key treatment for intestinal failure (IF) in Japan, primarily affecting children with various intestinal disorders.
- A study using a web-based survey found that 42 ITx procedures were performed on 38 patients by the end of 2022, with most donors being cadavers and a few being living donors.
- The survival rates after ITx were high at 1 year (92%), 5 years (73%), and 10 years (59%), and about 90% of patients no longer needed parenteral nutrition, highlighting significant improvements in quality of life post-transplantation.
Article Synopsis
- - The study examined the safety of using penicillin derivatives in pediatric liver transplant recipients, specifically focusing on those with Epstein-Barr virus (EBV) infection, as there's a concern about the potential for ampicillin rash.
- - Conducted at a major pediatric transplantation center in Japan, the research included 286 liver transplant patients, dividing them into EBV-positive and EBV-negative groups to compare rash occurrences linked to antibiotics.
- - Results showed that neither group experienced ampicillin rash, and the rate of antibiotic-associated rash was similar, indicating that penicillin derivatives can be safely administered to patients even with EBV infections post-transplant.
Prevalence, management and efficacy of treatment in portal vein obstruction after paediatric liver transplantation: protocol of the retrospective international multicentre PORTAL registry.
Bader A Alfares Hubert P J van der Doef Barbara E Wildhaber Thomas Casswall Greg Nowak Seisuke Sakamoto
BMJ Open
July 2023
Article Synopsis
- - The study investigates portal vein obstruction (PVO) in pediatric liver transplant patients, focusing on its prevalence, treatment practices, and outcomes through a global collaboration of 25 medical centers.
- - It utilizes a registry to gather data from patients diagnosed with PVO between 2001 and 2021, aiming to measure factors like treatment success and complications following interventions.
- - Approved by an ethics board, the study plans to share findings in academic journals and conferences, contributing to the understanding of PVO management post-liver transplantation.
Article Synopsis
- The study investigates the genetic causes of non-syndromic biliary atresia (BA) by analyzing the exome data of 15 Japanese patients and 509 control individuals using advanced statistical methods.
- The analysis found that damaging variants in the MFHAS1 gene were significantly more common in patients with BA, suggesting it may be a risk factor for the disease.
- Other known genetic variations associated with BA were less prominent, highlighting that most genetic contributions to non-syndromic BA are limited to rare variants like those in MFHAS1.
Portal vein pressure and flow modulation in pediatric liver transplantation.
Pediatr Transplant
November 2023
Article Synopsis
- Liver transplantation is increasingly performed in smaller, more severely affected pediatric patients, specifically those with biliary atresia, which complicates portal vein reconstruction.
- Preventing intraoperative portal vein thrombosis is crucial, necessitating detailed pre-surgical imaging to assess portal vein structure and flow, although specific objective measures for assessing sufficient flow are lacking.
- Recent research suggests that a portal vein pressure of 25 mmHg can be a reliable indicator for achieving adequate portal vein flow prior to graft implantation, but further studies are needed for optimal portal vein reconstruction strategies in pediatric cases.
Article Synopsis
- Progressive familial intrahepatic cholestasis type 1 (PFIC1) is a rare liver disorder caused by mutations in the ATP8B1 gene, often requiring liver transplantation (LT) due to severe liver disease.
- In a case study of three patients, outcomes varied significantly after LT, including one patient who improved but faced severe complications, and another who experienced fatal complications like pancreatitis and sepsis.
- The findings suggest that individual treatment plans are essential for managing post-transplant complications in PFIC1 patients.
Background: Malignant rhabdoid tumors (MRTs) are rare, aggressive tumors that mainly affect children and currently lack effective chemotherapeutic regimens. Liver MRTs are particularly challenging to manage due to the difficulty of performing one-stage liver resection, and preemptive liver transplantation is associated with high recurrence rates. However, the associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) technique offers a promising surgical approach for advanced-stage liver tumors where conventional liver resection is not feasible.
View Article and Find Full Text PDFBackground: Pediatric liver transplantation is an established treatment for end-stage liver disease in children. However, it is still posing relevant challenges, such as optimizing the graft selection according to the recipient size. Unlike adults, small children tolerate large-for-size grafts and insufficient graft volume might represent an issue in adolescents when graft size is disproportionate.
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