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INTRODUCTION

  • Pediatric transplants account for approximately 8% of all liver transplants in the United States.

  • In 2014 the American Association for the Study of Liver Diseases (AASLD); the American Society of Transplantation; and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition published their evidence-based recommendations for pediatric liver transplantation diagnostic, therapeutic, and preventive care.

  • This chapter provides brief summary of these recommendations based on that publication. For further details, please refer to the Further Reading section.

TRANSPLANT EVALUATION TEAM

  • Pediatric transplant candidates should be evaluated by a skilled multidisciplinary team. Risks, benefits, and the process from preoperative evaluation to long-term follow up should be reviewed with the patient (when appropriate) and the family/caregivers in order to determine the candidacy and whether to proceed or not based on an informed decision.

TIMING OF REFERRAL FOR TRANSPLANTATION

  • Immediate referral:

    • Acute liver failure

    • Acute decompensation of preexisting liver disease

  • Urgent referral:

    • Liver-based metabolic crises nonresponsive to medical therapy

    • Hepatoblastoma or hepatocellular carcinoma not amenable to surgical resection

  • Prompt referral:

    • Biliary atresia with total bilirubin >6 mg/dL >3 months after Kasai procedure (hepatoportoenterostomy [HPE])

    • Biliary atresia with total bilirubin 2 to 6 mg/dL

  • Anticipated referral:

    • Deteriorating chronic liver disease

      • Failure to thrive

      • Development of ascites

      • Encephalopathy (may present as low school performance, forgetfulness)

EVALUATION AND PRETRANSPLANT MANAGEMENT

  • Review available records and documents

  • Confirm referring diagnosis, comorbidities, and other factors of relevance to the transplantation process (including potential surgical challenges)

  • Manage and optimize conditions in collaboration with the local medical team

  • Develop a pre- and posttransplant assessment and treat-ment plan

    • Must address potential recurrence of the primary disease (such as autoimmune hepatitis, primary sclerosing cholangitis [PSC], bile salt excretory pump disease), allograft failure, onset of new diagnoses (such as renal dysfunction associated with chronic immunosuppression), and follow-up evaluations (such as irritable bowel syndrome [IBD], cancer screening, infections, compliance)

  • Ascites management:

  • Diuretics

    • Aldosterone agonist

    • Furosemide

  • Paracentesis

  • Transjugular intrahepatic portosystemic shunt (TIPS)/other shunting process

  • Abdominal evaluation for surgery

    • Duplex ultrasound of the liver, spleen, portal vein, and its branches

    • Special consideration for biliary atresia with splenosis symptoms

      • Interrupted vena cava

      • Preduodenal portal vein

      • Aplastic portal vein

      • Splenosis

  • Nutritional assessment and optimization:

    • Children with chronic liver disease require up to 80% more calories for adequate growth

    • Optimized nutrition is associated with improved recipient, graft, and neurodevelopmental outcomes

  • Cardiopulmonary assessment and optimization:

    • Evaluation for hepatopulmonary syndrome and portopulmonary hypertension

    • Transcutaneous oxygen saturation measurement in cases of suspected portosystemic shunting

    • Cardiac Doppler echocardiogram to rule out pulmonary hypertension

      • Right-sided cardiac catheterization if right ventricular systolic pressure >50 mm Hg

    • Pulmonary function tests in instances of cystic fibrosis

  • Renal assessment and optimization:

    • Creatinine

    • Glomerular filtration rate (GFR)

      • Cystatin C

      • Revised Schwartz formula:

        • 0.413 3 [sCr (mg/dL) / height (cm)] = GFR(mL/min/1.73 m2)

    • Acute renal injury

      • Modified risk for renal dysfunction, injury, failure, loss, and end-stage renal disease

    • Determine need ...

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