Table III. Outcome of LT in mitochondrial hepatopathies secondary to RC disorders.

Authors	Number of patients (age)	Presentation	Diagnosis	Survival	Follow-up	Comments
Sokal et al (1999; Europe and US)52	11 (1-7 mo)	ALF	RC enzyme assay and clinical	5 of 11	5 mo to 8 y	All 3 with diarrhea and vomiting died
Durand et al (2001; France)19	5 (<1 y)	ALF	RC enzyme assay and clinical	2 of 5	3.5 years	No extrahepatic involvement pre-LT
Dubern et al (2001; France)53	5 (<1 y)	ALF	RC enzyme assay and clinical	2 of 5	Not specified	No extrahepatic involvement pre-LT
Rabinowitz et al (2004)54	1 (neonate)	Liver failure, neurologic symptoms	DGUOK genotyping	Died		Died a few months after LT
Dimmock et al (2008; literature)41	10 (<10 mo; one 3 y)	Liver failure, neurologic symptoms	DGUOK genotyping	2 of 10		No benefit of LT if neurologic features present
El-Hattab etal (2010; literature and US)30	10 (infancy)	Liver failure, neurologic symptoms	MPV17 genotyping	5 of 10; 2 of 3 NNH, 3 of 7 other presentations	4-21 y	Patients with NNH had progression of neurologic features post-LT
Iwama etal (2010; literature)55	12 (<10 mo; one 3 y)	Liver failure	RC enzyme assay and clinical	0 of 7	4 mo to 5 y	No benefit of LT if neurologic features present
Total	54			16 (30%)