ArticleACG case reports journal2024
Combined Heart and Liver Transplantation in Delayed-Onset Propionic Acidemia.
Article in ACG case reports journal, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- Current status in paediatric liver transplantation for inherited metabolic diseases.Journal of pediatric gastroenterology and nutrition · 2026Review
- Loss of Propionyl-CoA Carboxylase Reprograms Hepatic Metabolism by Suppressing Mitochondrial Pyruvate Carboxylation and Fatty Acid Oxidation.bioRxiv : the preprint server for biology · 2026Article
- An overview on cardiac involvement in Inborn Errors of Metabolism: from clinical clues to nutritional management strategies.Frontiers in cardiovascular medicine · 2025Review
- Propionic Acidemia diagnosed in Amish adults and pregnancy outcomes: A case series.Molecular genetics and metabolism reports · 2024Article
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Authors and funding
4 authors.
Funding
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Abstract
Amish patients with propionic acidemia can have delayed presentations with rapid onset, severe cardiac decompensation despite metabolic control. Medical management is limited to strict low protein diet and correction of underlying catabolic triggers. Liver transplantation is indicated in pediatric patients with recurrent metabolic decompensation. There are limited reports of pediatric combined liver and heart transplantation. We present the first case of combined liver and heart transplantation in an Amish adult with newly diagnosed propionic acidemia and severe nonischemic dilated cardiomyopathy on advanced cardiac life support. Special considerations were necessary for close metabolic status monitoring and prevention of catabolic states.
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