Introduction
Surgical aspects and immediate post-operative care
Immunoprotection and immunosuppression
Immunoprotection
Immunosuppression
Indications
Group of indications Specific indications | CLKT | SLKT |
---|---|---|
Irreversible liver failure: | Simultaneous liver and kidney failure | Failure of one organ precedes failure of the other one |
Autosomal recessive polycystic kidney disease (ARPKD) | ||
α1-antitripsin deficiency | Extremely rare | Extremely rare |
Primary sclerotic cholangitis Alagille syndrome Boichis syndrome | ||
Lack/low activity of specific enzyme/regulator of immune system: | Recommended in cases of late diagnosis and renal failure (eGFR < 15 ml/min/1.73 m2) | Suggested to consider in cases of early diagnosis and relatively good native renal function (eGFR 15–29 ml/min/1.73 m2) |
Hyperoxaluria type 1 (PH 1) Methylmalonic acidemia (MMA) Type 1a glycogen storage disease (von Gierke disease) | ||
Atypical HUS (aHUS) * | *currently not recommended with availability of eculizumab | *Possible rare indication in case of poor efficacy of eculizumab-based prophylaxis |
Liver transplantation and further failure of native kidneys | Not applicable | eGFR < 15 ml/min/1.73 m2 |
Primary hyperoxaluria type 1 and 2 (PH1, PH2)
Ciliopathies
Autosomal recessive polycystic kidney disease (ARPKD)
Nephronophthisis associated with liver fibrosis (Boichis syndrome)
Methylmalonic acidemia (MMA)
α1-antitrypsin deficiency (α-AT)
Type 1a glycogen storage disease (von Gierke disease)
Atypical hemolytic uremic syndrome (aHUS)
Renal failure after liver transplantation
Indication | Factors | Citation |
---|---|---|
Hyperoxaluria type I (PH1) | High oxalate burden, not responding to pharmacological treatment CKD stage 4: eGFR < 29 ml/min/1.73 m2 (optional; SLKT) CKD stage 5: eGFR < 15 ml/min/1.73 m2 (direct; CLKT) | |
Autosomal recessive polycystic kidney disease (ARPKD) | PELD score > 10 (age, albumin, bilirubin, INR, growth failure) and therapy-resistant portal hypertension/ascending cholangitis/pruritus Complications of portal hypertension Complications of biliary tract (cholangitis) Severe cirrhosis in liver biopsy CKD stage 5: eGFR < 15 ml/min/1.73 m2/dialysis (CLKT) Availability of size-matched deceased donor of liver and kidney | |
CKD after liver transplantation | Hepatorenal syndrome (optional for SLKT), if dialysis after liver transplantation needed for > 6 weeks (renal biopsy needed for final decision) CKD stage 4: eGFR < 30 ml/min/1.73 m2 or CKD stage 5: eGFR < 15 ml/min/1.73 m2 Severe chronic damage in renal biopsy (tubulointerstitial fibrosis > 35%; glomerulosclerosis > 35%) (SLKT) * *data from adult setting |