Long-Term Medical Management of the Pediatric Patient After Liver Transplantation
Publication Date: February 1, 2014
Recommendations
ROUTINE MONITORING AND MANAGEMENT
Growth and Nutritional Rehabilitation
Optimize the nutritional status before and after LT. (1 – StrongB)
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To encourage growth, routine immunosuppression protocols should minimize steroid exposure during the first 6 to 12 months after transplantation. (1 – StrongA)
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Measure the height and weight to identify patients with growth impairment who may benefit from reduced steroid exposure. (1 – StrongB)
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Monitor the body mass index and consider obesity management. (2 – WeakC)
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Endocrine and Bone Metabolism
Monitor patients for persistent hepatic osteodystrophy, risk factors for fractures, and scoliosis. (1 – StrongB)
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Continue mineral and fat-soluble vitamin supplementation (especially D2, or D3) until vitamin D levels are normal. (1 – StrongB)
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Psychosocial Development
The follow-up of school-aged LT recipients should include an assessment of school functioning and school absence. ( 1 – Strong , A)
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Be aware of posttraumatic stress disorder or other mental health issues and refer a patient for a formal psychiatric evaluation if significant symptoms are present. (1 – StrongB)
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Neurocognitive Function
Screen neurocognitive function before transplantation for LT candidates older than 5 years and at key junctures afterward to determine special education needs. (1 – StrongB)
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Assess recipients for hearing loss in the first postoperative year and periodically thereafter as indicated. (1 – StrongB)
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Provide rehabilitation immediately after transplantation: physical therapy for infants with delayed motor development and speech and occupational therapy for older children with deficits. (1 – StrongB)
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Adherence
The transplant team assesses and treats nonadherence with a multidisciplinary approach. (2 – WeakB)
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Screen for nonadherence with objective methods such as the monitoring of immunosuppressant levels. (1 – StrongB)
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Screening and Detection of Late Surgical Complications
Surgical complications are optimally investigated and treated at a transplant center. (2 – WeakB)
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Protocol Liver Biopsy
Protocol liver biopsy 1 year after transplantation is not required. ( 1 – Strong , B)
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Screening for Skin Cancer
Encourage protective clothing, regular screening for skin lesions, and sunscreen. (1 – StrongB)
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Safe Living
Minimize infection risks related to hygiene, food, water, animals/pets, and travel. (2 – WeakC)
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Recipients can travel abroad 6 months after transplantation with normal precautions and the advice of their transplant center. (2 – WeakC)
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Combat childhood infections with recombinant or killed vaccines. (1 – StrongA)
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Immunize household contacts. Recipients and relatives should receive the annual influenza immunization. (1 – StrongB)
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IMMUNOSUPPRESSION
Acute Rejection
Serial measurements of bilirubin, ALT, AST, GGT, and immunosuppressant blood levels are the main means of detecting graft dysfunction and AR. (1 – StrongB)
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A histological assessment of a liver biopsy sample remains the best means of diagnosing AR. (1 – StrongA)
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Chronic Rejection
CR is a major cause of late graft loss and should be considered in the setting of poorly responsive AR with biopsy findings supportive of CR. (1 – StrongA)
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Treat CR with one of a variety of choices: use a higher serum level of the immunosuppressive (eg, tacrolimus), switch to different immunosuppressives (eg, from tacrolimus to mTOR inhibitors), and/or add other immunosuppressives (eg, mycophenolate). (1 – StrongB)
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Adverse Effects of Immunosuppression
Renal Function
Regularly screen renal function with the eGFR and practice calcineurin minimization. Consider renal-sparing drugs when the calculated GFR is <70 mL/minute/1.73 m2 . (1 – StrongB)
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Diabetes Mellitus
Screen LT recipients older than 5 years annually with fasting glucose in the early post-LT period and during long-term follow-up. Diagnose and treat posttransplant diabetes mellitus with the current standardized criteria. (1 – StrongA)
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Cardiovascular Disease
Screen recipients annually for cardiovascular risks (body mass index, blood pressure, and fasting lipids) and treat them according to age-specific guidelines. Consider modifying immunosuppression regimens. (1 – StrongB)
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Withdrawal of Immunosuppression
Corticosteroids may be withdrawn within 6 months of transplantation for patients who receive tacrolimus as their primary immunosuppression. (1 – StrongB)
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For patients more than 1 year after transplantation with normal liver blood tests, maintain tacrolimus therapy with target immunosuppression levels <6 ng/mL. (1 – StrongC)
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More than 5 years after transplantation, immunosuppression minimization (defined as a CNI once daily) may be considered if there is no history of CR, liver tests are normal, and a biopsy sample shows minimal or no portal inflammation and less than stage 3 fibrosis. (2 – WeakC)
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Complete immunosuppression withdrawal may be indicated if there are significant immune-related complications, but this should occur only within clinical trials. (2 – WeakC)
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DISEASE-SPECIFIC ISSUES AND RECURRENT DISEASE
Be aware of the risk of recurrence of primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) in children after transplantation and the need to continue steroids. (1 – StrongB)
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Periodic screening for colon cancer after transplantation for PSC with colitis may be beneficial; the optimal intervals are unknown. (2 – WeakB)
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A multidisciplinary approach to hepatoblastoma care involving oncology, radiology, hepatology, and surgery can improve posttransplant survival. (2 – WeakB)
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Patients with cystic fibrosis require close multispecialist care after isolated LT, with particular attention paid to nutrition, lung function, and infectious risks. (1 – StrongA)
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INFECTIONS
Late Viral Infections
Cytomegalovirus
Diagnose with quantitative nucleic acid–based or CMV pp65 antigenemia viral load assays in patients with a compatible clinical syndrome. (1 – StrongA)
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No specific prophylactic strategy is routinely indicated for CMV donor-negative/recipient-negative children, but the use of intravenous ganciclovir for all CMV donor-positive/recipient-negative recipients is recommended. (1 – StrongA)
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The primary transplant center should coordinate the management of symptomatic or asymptomatic patients with detectable CMV polymerase chain reaction and/or rising titer CMV viral loads. (1 – StrongB)
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Intravenous ganciclovir is recommended as the initial antiviral therapy; continue this until the CMV load becomes undetectable. (2 – WeakC)
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Consider ganciclovir resistance in patients with refractory clinical symptoms or children with persistent/rising CMV loads despite at least 14 days of antiviral therapy. Consider genotypic testing for resistance mutations and second-line therapies (foscarnet and cidofovir). (1 – StrongB)
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Epstein-Barr Virus and Posttransplant Lymphoproliferative Disorder
Determine the EBV serostatus of recipients and donors to identify patients at high risk for PTLD. (1 – StrongB)
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Seronegative patients before transplantation should be screened with EBV viral loads annually afterward to determine their susceptibility to a primary infection. Screen recipients at increased risk for EBV disease (donor-positive/recipientnegative) and PTLD weekly or biweekly during the first year after transplantation. (1 – StrongB)
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Patients presenting with typical symptoms such as persistent fever and lymphadenopathy should be clinically evaluated for PTLD with histopathology and EBV viral loads. Those with rising EBV viral loads should be discussed with their transplant center; management might include reduced immunosuppression and/or specific therapy. (1 – StrongB)
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Community-Acquired Respiratory Viruses
Immunize recipients against community-acquired viruses (influenza A, B) annually. No guidance exists for respiratory syncytial virus prophylaxis. (1 – StrongB)
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Pneumocystis jirovecii
Give at least 6 months’ prophylaxis with trimethoprim/sulfamethoxazole. (1 – StrongB)
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ADOLESCENT ISSUES
All adolescent girls should receive advice about fertility, contraception, and safe immunosuppression during pregnancy and should avoid mycophenolate. (2 – WeakB)
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All girls with menstrual problems should be reviewed by a gynecologist for advice and management. (2 – WeakC)
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Transfer adolescents who become pregnant to adult care to manage their immunosuppression. (2 – WeakB)
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Inquire about prospective health insurance at the age of 17 to 19 years (depending on the locale). (2 – WeakB)
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Discuss the avoidance of substance abuse and smoking, advise minimal alcohol intake, and review risky behaviors annually. (2 – WeakC)
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Transition to Adult Care
The transition process is multidisciplinary and should begin around the age of 10 to 11 years according to developmental maturity. (2 – WeakB)
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Prepare a standard transition protocol involving pediatric and adult providers. (2 – WeakB)
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Before the transfer, achieve readiness by building the patient’s understanding of the illness, selfmanagement skills, and ability to assume responsibility over his or her care. (2 – WeakB)
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Identify an adult care group to work closely with the transferring pediatrician and the patient for at least 1 year before the transfer. (2 – WeakC)
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Recommendation Grading
Disclaimer
Overview
Title
Long-Term Medical Management of the Pediatric Patient After Liver Transplantation
Authoring Organization
American Association for the Study of Liver Diseases
Publication Month/Year
February 1, 2014
Supplemental Implementation Tools
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Document Objectives
These recommendations suggest preferred approaches to the diagnostic, therapeutic, and preventive aspects of care for Long-Term Medical Management of the Pediatric Patient After Liver Transplantation
Target Patient Population
Post liver transplant patients
Inclusion Criteria
Female, Male, Adolescent, Child
Health Care Settings
Ambulatory, Childcare center, Hospital, Operating and recovery room, Outpatient
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Assessment and screening, Prevention, Management, Treatment
Diseases/Conditions (MeSH)
D010372 - Pediatrics, D014180 - Transplantation, D016031 - Liver Transplantation
Keywords
management, transplantation, Transplantation
Source Citation
LIVER TRANSPLANTATION 19:798–825, 2013