title: Coagulopathy and transfusion therapy in pediatric liver transplantation.
abstract: Bleeding and coagulopathy are critical issues complicating pediatric liver transplantation and contributing to morbidity and mortality in the cirrhotic child. The complexity of coagulopathy in the pediatric patient is illustrated by the interaction between three basic models. The first model, "developmental hemostasis", demonstrates how a different balance between pro- and anticoagulation factors leads to a normal hemostatic capacity in the pediatric patient at various ages. The second, the "cell based model of coagulation", takes into account the interaction between plasma proteins and cells. In the last, the concept of "rebalanced coagulation" highlights how the reduction of both pro- and anticoagulation factors leads to a normal, although unstable, coagulation profile. This new concept has led to the development of novel techniques used to analyze the coagulation capacity of whole blood for all patients. For example, viscoelastic methodologies are increasingly used on adult patients to test hemostatic capacity and to guide transfusion protocols. However, results are often confounding or have limited impact on morbidity and mortality. Moreover, data from pediatric patients remain inadequate. In addition, several interventions have been proposed to limit blood loss during transplantation, including the use of antifibrinolytic drugs and surgical techniques, such as the piggyback and lowering the central venous pressure during the hepatic dissection phase. The rationale for the use of these interventions is quite solid and has led to their incorporation into clinical practice; yet few of them have been rigorously tested in adults, let alone in children. Finally, the postoperative period in pediatric cohorts of patients has been characterized by an enhanced risk of hepatic vessel thrombosis. Thrombosis in fact remains the primary cause of early graft failure and re-transplantation within the first 30 d following surgery, and it occurs despite prolongation of standard coagulation assays. Data, however, are currently lacking regarding the use of anti-aggregation/anticoagulation therapies and how to best monitor for thrombosis in the early postoperative period in pediatric patients. Therefore, further studies are necessary to elucidate the interaction between the development of the coagulation system and cirrhosis in children. Moreover, strategies to optimize blood transfusion and anticoagulation must be tested specifically in pediatric patients. In conclusion, data from the adult world can be translated with difficulty into the pediatric field as indication for transplantation, baseline pathologies and levels of pro- and anticoagulation factors are not comparable between the two populations.
authors.affiliation: Mirco Nacoti, Francesco Fazzi, Ezio Bonanomi, Pediatric Intensive Care Unit, Ospedale Papa Giovanni XXIII Piazza OMS, 24127 Bergamo, Italy.
authors.forename: Mirco
authors.initials: M
authors.lastname: Nacoti
authors.affiliation: Mirco Nacoti, Francesco Fazzi, Ezio Bonanomi, Pediatric Intensive Care Unit, Ospedale Papa Giovanni XXIII Piazza OMS, 24127 Bergamo, Italy.
authors.forename: Davide
authors.initials: D
authors.lastname: Corbella
authors.affiliation: Mirco Nacoti, Francesco Fazzi, Ezio Bonanomi, Pediatric Intensive Care Unit, Ospedale Papa Giovanni XXIII Piazza OMS, 24127 Bergamo, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Fazzi
authors.affiliation: Mirco Nacoti, Francesco Fazzi, Ezio Bonanomi, Pediatric Intensive Care Unit, Ospedale Papa Giovanni XXIII Piazza OMS, 24127 Bergamo, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Rapido
authors.affiliation: Mirco Nacoti, Francesco Fazzi, Ezio Bonanomi, Pediatric Intensive Care Unit, Ospedale Papa Giovanni XXIII Piazza OMS, 24127 Bergamo, Italy.
authors.forename: Ezio
authors.initials: E
authors.lastname: Bonanomi
mesh_terms: D000367:Age Factors; D000925:Anticoagulants / Q000009:adverse effects / Q000627:therapeutic use*; D001777:Blood Coagulation / Q000187:drug effects; D001780:Blood Coagulation Tests; D016063:Blood Loss, Surgical / Q000517:prevention & control*; D001803:Blood Transfusion / Q000592:standards; D002648:Child; D002657:Child Development; D002675:Child, Preschool; D058625:End Stage Liver Disease / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016031:Liver Transplantation / Q000009:adverse effects* / Q000592:standards; D000067716:Point-of-Care Testing; D019106:Postoperative Hemorrhage / Q000097:blood / Q000209:etiology / Q000517:prevention & control*; D017410:Practice Guidelines as Topic; D011237:Predictive Value of Tests; D012307:Risk Factors; D013927:Thrombosis / Q000097:blood / Q000209:etiology / Q000517:prevention & control*; D065227:Transfusion Reaction; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants
keywords: Children; Coagulation; Liver disease; Point of care coagulation; Thrombosis; Transfusion; Transplantation