Combined liver-kidney transplantation for rare diseases

Table 1 Rare indications for combined liver-kidney transplantation

Disease	Indication(s) for CLKT
Monogenic diseases with primary hepatic expression without significant parenchymal damage
Atypical hemolytic-uremic syndrome	Renal failure and alternative complement pathway activity
AIP	Renal failure and recurrent medically non-responsive AIP attacks
Primary hyperoxaluria	Renal failure and metabolic control of the disease
Homozygous protein C deficiency	Renal failure and coagulation control
Hereditary complement C3 deficiency	Renal failure and risk reduction of recurrent infections (?)
Monogenic diseases with primary hepatic expression with parenchymal damage
Alpha-1-antitrypsin deficiency	Renal failure and liver failure (cirrhosis)
Glycogen storage disease	Renal failure with hepatocellular adenomatosis/carcinoma and metabolic control of the disease
Monogenic diseases with hepatic and extrahepatic manifestation
Nephronophthisis associated with liver fibrosis	Renal failure and liver failure (cirrhosis)
Lecithin cholesterol acyl transferase deficiency	Renal failure and metabolic control of disease
Methylmalonic acidemia	Renal failure and metabolic decompensation
Other
Antibody mediated rejection of the kidney	Renal failure and in the presence of positive CDC cross-match (?)

CLKT: Combined liver and kidney transplantation; AIP: Acute intermittent porphyria; CDC: Complement-dependent cytotoxicity.