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Renal outcome and plasma methylmalonic acid levels after isolated or combined liver or kidney transplantation in patients with methylmalonic acidemia: a multicenter analysis

Background - Methylmalonic acidemia (MMAemia) is characterized by accumulation of methylmalonic acid (MMA) in all body tissues. To minimize disease-related complications, isolated kidney (KTx), liver (LTx) or combined liver-kidney transplantation (LKTx) have been suggested. However, the impact of th...

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Main Authors: Dello Strologo, Luca (Author) , Spada, Marco (Author) , Vici, Carlo Dionisi (Author) , Atti, Marta Ciofi Degli (Author) , Rheault, Michelle (Author) , Bjerre, Anna Kristina (Author) , Boyer, Olivia (Author) , Calvo, Pier Luigi (Author) , D'Antiga, Lorenzo (Author) , Harshman, Lyndsay A. (Author) , Hörster, Friederike (Author) , Kölker, Stefan (Author) , Jahnukainen, Timo (Author) , Knops, Noël (Author) , Krug, Pauline (Author) , Krupka, Kai (Author) , Lee, Angela (Author) , Levtchenko, Elena (Author) , Marks, Stephen D. (Author) , Stojanovic, Jelena (Author) , Martelli, Laura (Author) , Mazariegos, George (Author) , Montini, Giovanni (Author) , Shenoy, Mohan (Author) , Sidhu, Sangeet (Author) , Tangeras, Trine (Author) , Testa, Sara (Author) , Vijay, Suresh (Author) , Wac, Katarzyna (Author) , Wennberg, Lars (Author) , Concepcion, Waldo (Author) , Garbade, Sven (Author) , Tönshoff, Burkhard (Author)
Format: Article (Journal)
Language:English
Published: 11 October 2022
In: Molecular genetics and metabolism
Year: 2022, Volume: 137, Issue: 3, Pages: 265-272
ISSN:1096-7206
DOI:10.1016/j.ymgme.2022.09.010
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.ymgme.2022.09.010
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S1096719222004061
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Author Notes:Luca Dello Strologo, Marco Spada, Carlo Dionisi Vici, Marta Ciofi Degli Atti, Michelle Rheault, Anna Kristina Bjerre, Olivia Boyer, Pier Luigi Calvo, Lorenzo D'Antiga, Lyndsay A. Harshman, Friederike Hörster, Stefan Kölker, Timo Jahnukainen, Noël Knops, Pauline Krug, Kai Krupka, Angela Lee, Elena Levtchenko, Stephen D. Marks, Jelena Stojanovic, Laura Martelli, George Mazariegos, Giovanni Montini, Mohan Shenoy, Sangeet Sidhu, Marco Spada, Trine Tangeras, Sara Testa, Suresh Vijay, Katarzyna Wac, Lars Wennberg, Waldo Concepcion, Sven F. Garbade, Burkhard Tönshoff

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520 |a Background - Methylmalonic acidemia (MMAemia) is characterized by accumulation of methylmalonic acid (MMA) in all body tissues. To minimize disease-related complications, isolated kidney (KTx), liver (LTx) or combined liver-kidney transplantation (LKTx) have been suggested. However, the impact of these different transplant strategies on outcome are unclear. - Methods - In this multicenter retrospective observational study, we compared plasma MMA levels and estimated glomerular filtration rate (eGFR) data of 83 patients. Sixty-eight patients (82%) had a mut0-type MMAemia, one patient had a mut−-type MMAemia, and seven (7.3%) had an inherited defect in cobalamin metabolism (cblA- or cblB-type MMAemia). Median observation period was 3.7 years (0-15.1 years). - Results - Twenty-six (31%) patients underwent KTx, 24 (29%) LTx and 33 (40%) LKTx. Posttransplant, mean plasma MMA concentration significantly decreased in all three cohorts; but at month 12, plasma MMA in KTx (1372 ± 1101 μmol/L) was 7.8-fold higher than in LTx (176 ± 103 μmol/L; P < 0.001) and 6.4-fold higher than in LKTx (215 ± 110 μmol/L; P < 0.001). Comparable data were observed at month 24. At time of transplantation, mean eGFR in KTx was 18.1 ± 24.3 mL/min/1.73 m2, in LTx 99.8 ± 29.9 mL/min/1.73 m2, and in LKTx 31.5 ± 21.2 mL/min/1.73 m2. At month 12 posttransplant, mean eGFR in KTx (62.3 ± 30.3 mL/min/1.73 m2) was 33.4% lower than in LTx (93.5 ± 18.3 mL/min/1.73 m2; P = 0.0053) and 25.4% lower than in LKTx (83.5 ± 26.9 mL/min/1.73 m2; P = 0.0403). - Conclusions - In patients with isolated MMAemia, LTx and LKTx lead to markedly lower plasma MMA levels during the first 2 years posttransplant than KTx and are associated with a better preservation of kidney function. LTx should therefore be part of the transplant strategy in MMAemia. 
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650 4 |a Estimated glomerular filtration rate 
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650 4 |a Liver transplantation 
650 4 |a Methylmalonic acid 
650 4 |a Methylmalonic acidemia 
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