Evaluation of the Pediatric Patient for Liver Transplantation

Squires, Robert H.; Ng, Vicky L.; Romero, René; Ekong, Udeme D.; Hardikar, Winita; Emre, Sükrü; Mazariegos, George

doi:10.1097/mpg.0000000000000431

Cited by 73 publications

(27 citation statements)

References 52 publications

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“…There are likely multiple contributing factors associated with this finding. Improvements in directed therapies and supportive care, (21,22) improved PALFSG-associated health care provider experience in managing the complexity of PALF, recommendations for ageappropriate evaluations within the PALFSG, (23) and published diagnostic strategies and practice guidelines (24) collectively have enhanced the care afforded to these critically ill patients. While difficult to quantitate, these factors have assuredly impacted the multifaceted decisions surrounding the pursuit of LT as a therapeutic option.…”

Section: Discussionmentioning

confidence: 99%

Liver Transplant Listing in Pediatric Acute Liver Failure: Practices and Participant Characteristics

et al. 2018

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Liver transplant (LT) decisions in pediatric acute liver failure (PALF) are complex. Three phases of the PALF registry, containing data on 1,144 participants over 15 years, were interrogated to characterize clinical features associated with listing status. A decrease in the cumulative incidence of listing (P < 0.005) and receiving (P < 0.05) LT occurred without an increase in the cumulative incidence of death (P = 0.67). Time to listing was constant and early (1 day; quartiles 1-3 = 0-2; P = 0.88). The most frequent reasons for not listing were "not sick enough" and "medically unsuitable." Participants listed for LT were more likely male, with coma grade scores >0; had higher international normalized ratio, bilirubin, lactate, and venous ammonia; and had lower peripheral lymphocytes and transaminase levels compared to those deemed "not sick enough." Participants listed versus those deemed "medically unsuitable" were older; had higher serum aminotransferase levels, bilirubin, platelets, and albumin; and had lower lactate, venous ammonia, and lymphocyte count. An indeterminate diagnosis was more prevalent in listed participants. Ventilator (23.8%) and vasopressor (9.2%) support occurred in a significant portion of listed participants but less frequently than in those who were not "medically suitable." Removal from the LT list was a rare event. Conclusion: The cumulative incidence of listing for and receiving LT decreased throughout the PALF study without an increase in the cumulative incidence of death. While all participants fulfilled entry criteria for PALF, significant differences were noted between participants listed for LT and those deemed "not sick enough" as well as those who were "medically unsuitable." Having an indeterminate diagnosis and a requirement for cardiopulmonary support appeared to influence decisions toward listing; optimizing listing decisions in PALF may reduce the frequency of LT without increasing the frequency of death.

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Section: Discussionmentioning

confidence: 99%

Liver Transplant Listing in Pediatric Acute Liver Failure: Practices and Participant Characteristics

et al. 2018

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“…Metabolic disorders comprise the second largest category of indications for pediatric liver transplantation [41]. As liver transplantation outcomes continue to improve at centers of excellence, the strategy has been successfully applied to a growing number of conditions such as maple syrup urine disease (MSUD), for which efficacy is now established [37,42].…”

Section: Liver Transplantation For the Treatment Of Metabolic Disordersmentioning

confidence: 99%

“…As survival after pediatric liver transplantation has improved over the past decade [41], attention has turned to preventing posttransplant medical complications and optimizing long-term quality of life. Despite progress on this front, there remains a finite potential for abrogating all morbidity and mortality after transplant.…”

Section: Liver Transplantation For the Treatment Of Metabolic Disordersmentioning

confidence: 99%

Liver transplantation for treatment of severe S-adenosylhomocysteine hydrolase deficiency

Strauss

Ferreira

Bottiglieri³

et al. 2015

Molecular Genetics and Metabolism

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“…through May 31, 2013, ALF accounted for 11% of pediatric liver transplants. (1) In a large number of PALF cases, etiology has eluded discovery.…”

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confidence: 99%

Pediatric acute liver failure of undetermined cause: A research workshop

et al. 2017

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Pediatric Acute liver failure (PALF) is a potentially devastating condition which occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of transplantation and death than other diagnostic groups. A single day workshop sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases brought together clinicians and basic scientists to integrate aligned research findings and develop a foundation for new mechanistic studies and future treatment trials. The clinical phenotype of indeterminate PALF shares important similarities to the hyperinflammatory state characteristic of Hemophagocytic lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS). A failure of cytotoxic T cells to limit or contract inflammatory responses may propagate injury and lead to a local and systemic milieu that does not support normal hepatic regeneration. Evidence was presented that bone marrow (BM) derived Sinusoidal endothelial cell PROgenitor Cells (sprocs) play a vital role in hepatic regeneration. Overwhelming systemic inflammatory responses may suppress mobilization of BM sprocs and dampen hepatic recovery. Conclusion: Thus, experience gained through treatment trials of HLH and MAS in childhood may inform study design for therapy of PALF. Successful approaches to limiting neuro-inflammation through reduction of systemic inflammation and standardized neuroprotection protocols that limit glial injury could significantly improve intact survival. Finally, since PALF is a rare disease, investigative efforts must include broad multi-center collaboration and careful stewardship of biorepository specimens.

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Evaluation of the Pediatric Patient for Liver Transplantation

Cited by 73 publications

References 52 publications

Liver Transplant Listing in Pediatric Acute Liver Failure: Practices and Participant Characteristics

Liver Transplant Listing in Pediatric Acute Liver Failure: Practices and Participant Characteristics

Liver transplantation for treatment of severe S-adenosylhomocysteine hydrolase deficiency

Pediatric acute liver failure of undetermined cause: A research workshop

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