About: Post-transplantation Outcomes in Patients with PA or MMA: A Review of the Literature

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type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
has title	Post-transplantation Outcomes in Patients with PA or MMA: A Review of the Literature
Creator	Morais, Vara Roshni, Sufin
Source	PMC
abstract	INTRODUCTION: Liver transplantation is recognised as a treatment option for patients with propionic acidemia (PA) and those with methylmalonic acidemia (MMA) without renal impairment. In patients with MMA and moderate-to-severe renal impairment, combined liver–kidney transplantation is indicated. However, clinical experience of these transplantation options in patients with PA and MMA remains limited and fragmented. We undertook an overview of post-transplantation outcomes in patients with PA and MMA using the current available evidence. METHODS: A literature search identified publications on the use of transplantation in patients with PA and MMA. Publications were considered if they presented adequate demographic and outcome data from patients with PA or MMA. Publications that did not report any specific outcomes for patients or provided insufficient data were excluded. RESULTS: Seventy publications were identified of which 38 were full papers. A total of 373 patients underwent liver/kidney/combined liver–kidney transplantation for PA or MMA. The most typical reason for transplantation was recurrent metabolic decompensation. A total of 27 post-transplant deaths were reported in patients with PA [14.0% (27/194)]. For patients with MMA, 18 post-transplant deaths were reported [11% (18/167)]. A total of 62 complications were reported in 115 patients with PA (54%) with cardiomyopathy (n = 12), hepatic arterial thrombosis (HAT; n = 14) and viral infections (n = 12) being the most commonly reported. A total of 52 complications were reported in 106 patients with MMA (49%) with viral infections (n = 14) and renal failure/impairment (n = 10) being the most commonly reported. CONCLUSIONS: Liver transplantation and combined liver–kidney transplantation appears to benefit some patients with PA or MMA, respectively, but this approach does not provide complete correction of the metabolic defect and some patients remain at risk from disease-related and transplantation-related complications, including death. Thus, all treatment avenues should be exhausted before consideration of organ transplantation and the benefits of this approach must be weighed against the risk of perioperative complications on an individual basis.
has issue date	2020-04-08(xsd:dateTime)
bibo:doi	10.1007/s12325-020-01305-1
bibo:pmid	32270363
has license	cc-by-nc
sha1sum (hex)	ad94ba654adf451dc09582410a44d4031718c069
schema:url	https://doi.org/10.1007/s12325-020-01305-1
resource representing a document's title	Post-transplantation Outcomes in Patients with PA or MMA: A Review of the Literature
has PubMed Central identifier	PMC7141097
has PubMed identifier	32270363
schema:publication	Adv Ther
resource representing a document's body	covid:ad94ba654adf451dc09582410a44d4031718c069#body_text
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