ArticleHepatobiliary surgery and nutrition2019
Perioperative characteristics and management of liver transplantation for isolated methylmalonic acidemia-the largest experience in China.
Article in Hepatobiliary surgery and nutrition, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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16 citing papers in PubMed, 24 citations in OpenAlex.
- First Single-Center Experience of Minimally Invasive Pediatric Living Donor Liver Transplantation in China.Pediatric transplantation · 2026Article
- Liver transplantation for homozygous familial hypercholesterolemia: a retrospective analysis from Chinese experience.Orphanet journal of rare diseases · 2025Article
- Role of carglumic acid in the long-term management of propionic and methylmalonic acidurias.Orphanet journal of rare diseases · 2024Review
- Clinical Practice Recommendations on Kidney Management in Methylmalonic Acidemia: an Expert Consensus Statement From ERKNet and MetabERN.Kidney international reports · 2024Article
- Improving the second-tier classification of methylmalonic acidemia patients using a machine learning ensemble method.World journal of pediatrics : WJP · 2024Article
- Long-term clinical outcomes and health-related quality of life in patients with isolated methylmalonic acidemia after liver transplantation: experience from the largest cohort study in China.World journal of pediatrics : WJP · 2024Article
- Successful adult domino living donor liver transplantation in methylmalonic acidemia: case report.Translational gastroenterology and hepatology · 2024Article
- Effect of liver transplantation with primary hyperoxaluria type 1: Five case reports and review of literature.World journal of clinical cases · 2023Article
- Domino hepatocyte transplantation using explanted human livers with metabolic defects attenuates D-GalN/LPS-induced acute liver failure.Journal of translational medicine · 2022Article
- Impact of living donor liver with steatosis and idiopathic portal inflammation on clinical outcomes in pediatric liver transplantation: Beijing experience.Hepatobiliary surgery and nutrition · 2022Article
- The effect of liver transplantation for argininemia-the largest experiences in a single center.Translational pediatrics · 2022Article
- Therapeutic potential of living donor liver transplantation from heterozygous carrier donors in children with propionic acidemia.Orphanet journal of rare diseases · 2022Article
- Auxiliary Liver Graft Can Be Protected From HBV Infection in HBsAg Positive Blood Circulation.Frontiers in medicine · 2021Article
- Nutritional Management and Biochemical Outcomes during the Immediate Phase after Liver Transplant for Methylmalonic Acidemia.Nutrients · 2020Article
- Factors influencing in-hospital death for pediatric patients with isolated methylmalonic acidemia: a nationwide inpatient database analysis.Orphanet journal of rare diseases · 2020Article
- The concept of "domino" in liver and hepatocyte transplantation.Therapeutic advances in gastroenterology · 2020Review
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Authors and funding
13 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThere are few detailed consensus and guidelines on perioperative clinical characteristics of liver transplantation (LT) in patients with methylmalonic acidemia (MMA). This retrospective study investigated details of the clinical course and individualized treatment plan of the center with largest experience in China.
methodsA total of 7 MMA patients undergoing LT in Beijing Friendship Hospital from June 2013 to December 2017 were enrolled in the study, whose clinical data (clinical characteristics, laboratory findings, chronological changes in urine MMA levels, treatment, etc.) during perioperative period were analyzed retrospectively. All the patients received strict postoperative management.
resultsAll the 7 cases were confirmed to have isolated MMA, among which, 3 cases received living donor liver transplantation (LDLT), 4 cases received deceased donor liver transplantation (DDLT). A wild fluctuate of metabolic condition was observed within the first few days after surgery and two weeks after LT, the mean base excess of blood value (BE-B) restored to normal whereas plasma bicarbonate (HCO
conclusionsLT is an effective treatment to prevent metabolic crisis, but patients with MMA tend to be metabolically fragile even after surgery. During perioperative period, close monitoring should be given for acidosis episodes so as to implement sodium bicarbonate correction. Metabolism-correcting medications are still needed. Special immunosuppressive regimen is an effective way of maintaining renal function for those with kidney dysfunction.
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