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Practice guidelines released for evaluating pediatric LT patients
Pediatric liver transplants account for approximately 7.8% of all liver transplants in the US, according to new data. Significant differences exist between pediatric and adult patients and how they should be evaluated as candidates for liver transplantation.
Researchers, including Robert H. Squires, MD, of Children’s Hospital of Pittsburgh, University of Pittsburgh Medical Center, collaborated with the American Association for the Study of Liver Diseases to release the 2014 practice guideline for clinicians to consider when evaluating a pediatric patient for liver transplantation (LT). The American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition also contributed to the development and approval of the guidelines.
Robert H. Squires
“These guidelines are intended to highlight the distinctive features of pediatric liver disease in the context of liver transplant decisions,” Squires told Healio.com/Hepatology.
Key points of the 94 suggested recommendations include: when to refer a pediatric patient for LT; what assessments to include during the LT evaluation; indications for LT; and contraindications.
Referral
Timing for referring a pediatric patient for LT depends on the patient’s circumstances. A referral can be based on whether or not LT is emergent, urgent or anticipatory. Among other considerations, researchers recommend that a clinician make immediate contact with a pediatric LT center for children with acute liver failure or acute decompensation of an established liver disease. Referral for LT evaluation should be anticipated for patients with chronic liver disease and those who have any evidence of deteriorating liver function.
Evaluation
Each patient’s diagnostic evaluation should include documentation of all clinical assessments, laboratory results, nutritional management and other strategies. Physicians and a member of the LT team also should meet to gain a better sense of any clinical, social and psychological factors that may not have been apparent in medical records. Assessments that should be performed, documented and compiled include: hepatology, nutrition, cardiopulmonary, dental, renal, anesthesiology, psychosocial, neurocognitive and neurodevelopmental, and considerations for living-related and living-donor LT.
Indications
Various indications for LT among pediatric patients include: biliary atresia, Alagille syndrome, Wilson’s disease, acute liver failure, hepatoblastoma, hepatocellular carcinoma, hemangioendothelioma, cystic fibrosis-associated liver disease, and urea cycle defects. Each indication is unique, and other factors should be considered before recommending LT.
Contraindications
Donor organs are scarce; therefore it is up to transplant clinicians to determine which pediatric patients are most in need of LT. This also includes considering patients who will most benefit from LT, as various circumstances could result in poor patient outcomes. Contraindications that clinicians should be mindful of are: extrahepatic malignancy, systemic infection, Niemann-Pick disease type C and hemophagocytic lymphohistiocytosis presenting as acute liver failure. Some contraindications may be better treated with another form of medical therapy.
“The cliché that ‘children are not little adults’ is embodied by the new evaluation guidelines for pediatric liver transplantation,” Squires said. “To optimize outcomes, the pediatric liver transplant team must be aware of important social, neurodevelopmental, diagnostic, therapeutic and management issues that are unique to infants, children and adolescents with acute and chronic liver disease.” – by Melinda Stevens
Disclosure: Rene Romero, MD, reports receiving grants from Bristol-Myers Squibb.
Perspective
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This practice guideline is by far one of the better, more complete, and most thorough reviews of the literature regarding liver transplantation in pediatric patients put together by the American Association for the Study of Liver Diseases (AASLD). The guideline would be useful to all involved in pediatric liver transplantation from nurses and coordinators, to residents and fellows, and to the pediatric hepatologists/gastroenterologists and transplant surgeons.
The guideline starts with instructions and recommendations on how to put together a multidisciplinary team to evaluate candidates for liver transplantation, how to place those accepted for transplant on the list and how to follow the listed patients until they are transplanted. It discusses indications and contraindications to liver transplantation in children and then focuses on specific liver diseases from biliary atresia, to metabolic and storage disorders, and the immune diseases and cancers for which liver transplant would be the treatment of choice.
The guideline discusses how to assess the pediatric patients who are in need of a liver transplant, including general and organ system-specific assessment of the potential candidates. It focuses on cardiopulmonary and renal function of the patients. It evaluates the nutritional status of the transplant candidate. It focuses on psychological issues the patient may face and evaluates neurocognitive and neurodevelopmental functioning of the candidates. These are all important parameters in the evaluation of candidates and would be useful guidelines for post-transplant cognitive and developmental follow-up evaluations.
In the end, the guideline addresses organ allocation strategies in pediatric liver transplantation and includes discussion on living donor and split-liver transplantation, as well as potential for future liver cell/hepatocyte transplantation.
The guideline contains close to 100 recommendations that appear at the end of every section. The summaries and discussions are valuable and useful for the readers. This guideline is a much-needed piece of information for all those involved in pediatric liver transplantation.