Table 3.

Outcome of Children with MPV17-Related Encephalohepatopathy

Liver Transplant?OutcomeFrequency
Yes (17/96; 18%) Death 110/17 (59%)
Survival7/17 (41%)
No (79/96; 82%) Death from liver failure 265/79 (82%)
Survival 314/79 (18%)
1.

Death most commonly occurred in the post-transplantation period due to sepsis, respiratory failure, or multiorgan failure.

2.

The majority died during infancy (52/65; 80%); some died during early childhood (1-5 years) (10/65; 15%), adolescence (2/65; 3%), or early adulthood (1/65; 2%).

3.

The oldest reported affected individual is 25 years old [El-Hattab et al 2018]. Note: This does NOT mean that survival past 25 years is not possible.

From: MPV17-Related Mitochondrial DNA Maintenance Defect

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