I want to congratulate the authors of a recent AASLD/NASPGHAN report (Hepatology 2014; 60: 362-98 & JPGN 2014; 59: 112-31) and in particular Rene Romero who has been a terrific colleague in Atlanta. I’m grateful for his timely advice to me and my colleagues along with the excellent care that he has provided to children referred for liver transplantation (LT).
The link to full article, Evaluation of Pediatric Liver Transplantation, and other AASLD practice guidelines can be accessed from this website: AASLD guidelines.
Some of the useful recommendations include the following:
- For biliary atresia, “patients who are post hepatoportoenterostomy (HPE) should be promptly referred for LT evaluation if the total bilirubin is greater than 6 mg/dL beyond 3 months from HPE” and should be considered if total bilirubin is ≥2 mg/dL. According to the authors in the Hepatology article, ‘84% of those with a total bilirubin <2 mg/dL will survive with native liver beyond 2 years of age whereas only 16% will if total bilirubin is >6 mg/dL.’ [Interestingly, this information is stated differently in the JPGN article where the authors state “up to 70% of patients with BA may have prolonged transplant-free survival if the total serum bilirubin falls below 2 mg/dL” w/in 3 months following HPE.]
- For biliary atresia: HPE is recommended as 1st line treatment except in infants with “evidence of decompensated liver disease.”
- Ascites management: can be managed with an aldosterone antagonist. Reserve more aggressive measures (paracentesis, TIPS, surgical shunt) for those with compromised respiratory effort or severely impaired quality of life.
- Recurrent disease: families should be informed that autoimmune hepatitis, PSC, and bile salt excretory pump disease can recur post-LT.
- Recommends screening with use of pulse oxygen with the patient in upright position in all patients with possible portosystemic shunting.
- Discusses immunizations for child and for family members (all family members need to fully immunized).
- The review provides recommendations specific for virtually each liver condition, including Alagille, Wilson’s, Tyrosinemia, PFIC, hemangioendothelioma, Cystic fibrosis, urea cycle defects, autoimmune hepatitis, PSC, Hepatoblastoma, Alpha-1 Antitrypsin deficiency, Bile Acid Synthesis Disorders, Glycogen Storage Disease, Fatty Acid Oxidation defects, Parenteral Nutrition-Associated Liver Disease (PNALD), and many others.
- Contraindicated for LT: Alper’s syndrome/valproate-associated liver failure, mitochondrial disease with severe extrahepatic disease, Hepatocellular carcinoma with extrahepatic disease and rapid progression, uncontrolled systemic infection, Niemann-Pick Disease Type C, and severe medically-refractory portopulmonary hypertension
Take-home message: This practice guideline is an excellent resource in the evaluation of pediatric liver transplantation and pre-transplant management. The ease of accessing the entire report online is a big plus too.