scholarly journals Hemostatic Balance in Pediatric Acute Liver Failure: Epidemiology of Bleeding and Thrombosis, Physiology, and Current Strategies

2020 ◽  
Vol 8 ◽  
Author(s):  
Yonca Bulut ◽  
Anil Sapru ◽  
Gavin D. Roach

Pediatric Acute Liver Failure (PALF) is a rapidly progressive clinical syndrome encountered in the pediatric ICU which may rapidly progress to multi-organ dysfunction, and on occasion to life threatening cerebral edema and hemorrhage. Pediatric Acute Liver Failure is defined as severe acute hepatic dysfunction accompanied by encephalopathy and liver-based coagulopathy defined as prolongation of International Normalized Ratio (INR) >1.5. However, coagulopathy in PALF is complex and warrants a deeper understanding of the hemostatic balance in acute liver failure. Although an INR value of >1.5 is accepted as the evidence of coagulopathy and has historically been viewed as a prognostic factor of PALF, it may not accurately reflect the bleeding risk in PALF since it only measures procoagulant factors. Paradoxically, despite the prolongation of INR, bleeding risk is lower than expected (around 5%). This is due to “rebalanced hemostasis” due to concurrent changes in procoagulant, anticoagulant and fibrinolytic systems. Since the liver is involved in both procoagulant (Factors II, V, IX, XI, and fibrinogen) and anticoagulant (Protein C, Protein S, and antithrombin) protein synthesis, PALF results in “rebalanced hemostasis” or even may shift toward a hypercoagulable state. In addition to rebalanced coagulation there is altered platelet production due to decreased thrombopoietin production by liver, increased von Willebrand factor from low grade endothelial cell activation, and hyperfibrinolysis and dysfibrinogenemia from altered synthetic liver dysfunction. All these alterations contribute to the multifactorial nature of coagulopathy in PALF. Over exuberant use of prophylactic blood products in patients with PALF may contribute to morbidities such as fluid overload, transfusion-associated lung injury, and increased thrombosis risk. It is essential to use caution when using INR values for plasma and factor administration. In this review we will summarize the complexity of coagulation in PALF, explore “rebalanced hemostasis,” and discuss the limitations of current coagulation tests. We will also review strategies to accurately diagnose the coagulopathy of PALF and targeted therapies.

2014 ◽  
Vol 164 (2) ◽  
pp. 407-409 ◽  
Author(s):  
Frank DiPaola ◽  
Michael Grimley ◽  
John Bucuvalas

2018 ◽  
Vol 67 (4) ◽  
pp. 441-445 ◽  
Author(s):  
Catherine A. Chapin ◽  
Saeed Mohammad ◽  
Lee M. Bass ◽  
Sarah A. Taylor ◽  
Susan Kelly ◽  
...  

2020 ◽  
Vol 23 (6) ◽  
pp. 539
Author(s):  
Songpon Getsuwan ◽  
Chatmanee Lertudomphonwanit ◽  
Pornthep Tanpowpong ◽  
Chollasak Thirapattaraphan ◽  
Thipwimol Tim-Aroon ◽  
...  

2019 ◽  
Author(s):  
Haiyan Fu ◽  
Ruiqin Zhao ◽  
Gelan Bai ◽  
Hui Zhou

Abstract Background and objective: Pediatric acute liver failure (PALF) progresses rapidly and has a poor prognosis. Therefore, simple, sensitive and specific clinical indicators are needed. Gamma-glutamyl transpeptidase (GGT) plays a role in predicting the prognosis in infantile cholestatic liver diseases. However, its role in predicting the prognosis in PALF remains unclear. Methods: In present study, children with PALF were divided into a normal GGT group and a high GGT group using the GGT level of 50 U/L as the demarcation line. Age, sex, serum total bilirubin, direct bilirubin, albumin, total bile acid, international normalized ratio (INR) and pediatric end-stage liver disease (PELD) score were compared between the 2 groups. In addition, GGT level was subjected to receiver operating characteristic (ROC) curve analysis, and the area under the curve and the optimal diagnostic cutoff value were calculated. Results: A total of 41 children with PALF were enrolled in the study. INR, PELD score and mortality rate were significantly higher in the normal GGT group in comparison to the high GGT group. GGT level had area under the ROC curve of 0.8194 (95% CI : 0.680-0.959); the optimal diagnostic cutoff values were 60 U/L. At the cutoff value, the sensitivity and specificity of GGT level in predicting the prognosis in PALF were 86.36% and 73.68% respectively. Conclusion: GGT exhibited high sensitivity and specificity in predicting the prognosis in PALF. It can be used as one useful prognostic indicator of PALF.


2019 ◽  
Vol 13 (2) ◽  
pp. 121-123
Author(s):  
Martina Finocchi ◽  
Ombretta Para ◽  
Giacomo Zaccagnini ◽  
Lorenzo Corbo ◽  
Lucia Maddaluni ◽  
...  

It is known that a wild spectrum of hepatic manifestations can be common presentations of metastatic breast cancer. Pseudocirrhosis pattern has been often described as almost always secondary to systemic chemotherapy and it is defined by morphological liver changes that mimic cirrhosis including capsular retraction, nodularity, parenchyma atrophy and caudate lobe, radiologically identifiable. Acute liver injury is an occasional complication in oncologic patients, and it outlines an organ failure when there is evidence of encephalopathy and coagulopathy (international normalized ratio >1.5) in the absence of pre-existing liver disease, with an illness of <26 weeks duration. The two most common etiologies are leukemia/lymphoma followed by breast cancer but also in this case, liver is involved almost always after chemotherapy, hormonotherapy or radiotherapy. Here we present a case of rapid evolving acute liver failure presented as cryptogenic pseudocirrhosis without any evidence of primitive breast cancer but an incidental demonstration.


2020 ◽  
Vol 34 (4) ◽  
Author(s):  
Angela Lee ◽  
Julianne Mendoza ◽  
Aleah L. Brubaker ◽  
Daniel J. Stoltz ◽  
Rebecca McKenzie ◽  
...  

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