Acute-on-chronic liver failure in children

Table 1 Commonly accepted acute-on-chronic liver failure definitions

	APASL	EASL	NASCELD
Definition	An acute hepatic insult manifesting as jaundice (serum bilirubin ≥ 5 mg/dL) and coagulopathy (INR ≥ 1.5) complicated within 4 wk by clinical ascites and/or encephalopathy in a patient with previously diagnosed or undiagnosed chronic liver disease/cirrhosis, and is associated with a high 28-d mortality	An acute deterioration of pre-existing chronic liver disease usually related to a precipitating event and associated with increased mortality at 3 mo due to multisystem organ failure	A syndrome characterized by acute deterioration of cirrhosis with two or more extrahepatic organ failure
Included patients	Acute liver deterioration in patients with previously diagnosed or undiagnosed chronic liver disease including cirrhosis. Acute hepatic triggering factors	Cirrhosis (compensated or decompensated)	Cirrhosis (compensated or decompensated)
		Renal failure is mandatory	Two extrahepatic organ failure
		Patients with an acute decompensation of cirrhosis	Presentation not necessarily to be liver failure
		Patients with prior decompensation of cirrhosis	Can be repeated episodes of ACLF
Excluded patients	Patients with bacterial infections	HCC	HIV infection
	Patients with cirrhosis who develop acute deterioration of their clinical status are considered to have acute decompensation but not ACLF	HIV infection	Disseminated malignancies
	Prior decompensation.Non-hepatic acute insults (such as sepsis)	Receiving immunosuppressive treatments
Pediatric definition	For children less than 3 years, modified HE assessment scale can be used	None	None
	Clinical and/or radiological ascites can be used for defining ACLF in children

ACLF: Acute-on-chronic liver failure; APASL: The Asian Pacific Association for the Study of the Liver; EASL: The European Association for Liver Studies; HCC: Hepatocellular cancer, HIV: Human immunodeficiency virus; INR: International normalized ratio; NASCELD: The North American Consortium for End-Stage Liver Disease Studies.

Table 2 Chronic liver failure–sequential organ failure assessment score

Organ/systems	0	1	2	3	4
Liver (bilirubin, mg/dL)	< 1.2	≥ 1.2 to < 2.0	≥ 2.0 to < 6.0	≥ 6.0 to < 12.0	≥ 12.0
Kidney (creatinine, mg/dL)	< 1.2	≥ 1.2 to < 2.0	≥ 2.0 to < 3.5	≥ 3.5 to < 5.0(or RRT)	≥ 5.0(or RRT)
Cerebral (HE grade)	No HE	I	II	III	IV
Coagulation (INR)	< 1.1	≥ 1.1 to < 1.25	≥ 1.25 to < 1.5	≥ 1.5 to < 2.5	≥ 2.5 or platelet < 20 × 109 /L
Circulation (mean arterial pressure, mm Hg)	≥ 70	< 70	Dopamine ≤ 5 or dobutamine or terlipressin(µg/kg/min)	Dopamine > 5 or E ≤ 0.1 or NE ≤ 0.1(µg/kg/min)	Dopamine > 15 or E > 0.1 or NE > 0.1(µg/kg/min)
Lungs
PaO/FiO2	> 400	> 300 to ≤ 400	> 200 to ≤ 300	> 100 to ≤ 200	≤ 100
or SpO2/FiO2	> 512	> 357 to ≤ 512	> 214 to ≤3 57	< 89 to ≤ 214	≤ 89

BP: Blood pressure; E: Epinephrine; FiO2: Fraction of inspired oxygen; HE: Hepatic encephalopathy; INR: International normalized ratio; NE: Norepinephrine; PaO2: Partial pressure of arterial oxygen; RRT: Renal replacement therapy; SpO2: Pulse oximetric saturation.

Table 3 Asian Pacific Association for the Study of the Liver Acute-on-Chronic Liver Failure score

Points	Total bilirubin (mg/dL)	HE grade	INR	Lactate (mmol/L)	Creatinine (mg/dL)
1	< 15	0	< 1.8	< 1.5	< 0.7
2	15-25	I-II	1.8-2.5	1.5-2.5	0.7-1.5
3	> 25	III-IV	> 2.5	> 2.5	> 1.5

HE: Hepatic encephalopathy; INR: International normalized ratio.

Table 4 Modified chronic liver failure–sequential organ failure assessment score

Organ/systems	0	1	2	3	4
Liver (bilirubin, mg/dL)	< 1.2	≥ 1.2 to < 2.0	≥ 2.0 to < 6.0	≥ 6.0 to < 12.0	≥ 12.0
Kidney (creatinine, rise from baseline)	< 1.5 ×	1.5 to ≤ 2.0 ×	> 2.0 to ≤ 3 ×	> 3 ×	Need for RRT
Cerebral (HE grade)	0	I	II	III	IV
Coagulation (INR)	< 1.1	≥ 1.1 to ≤ 1.25	> 1.25 to < 1.5	≥ 1.5 to ≤ 2.5	> 2.5
Circulation (systolic BP)	Normal for age	< 5th centile for age	NE < 0.5 µg/kg/min	NE > 0.5 µg/kg/min	NE > 0.5 µg/kg/min and 2nd inotrope
Lungs
PaO/FiO2	> 400	> 300 to ≤ 400	> 200 to ≤ 300	> 100 to ≤ 200	≤ 100

BP: Blood pressure; HE: Hepatic encephalopathy; INR: International normalized ratio; NE: Norepinephrine; PaO2: Partial pressure of arterial oxygen; FiO2: Fraction of inspired oxygen; RRT: Renal replacement therapy.

Table 5 Modified Asian Pacific Association for the Study of the Liver Acute-on-Chronic Liver Failure score

Points	Total bilirubin (mg/dL)	HE grade	INR	Lactate (mmol/L)	Creatinine (rise from baseline)
1	< 15	0	< 1.8	< 1.5	< 1.5 ×
2	15-25	I-II	1.8-2.5	1.5-2.5	1.5 to ≤ 3 ×
3	> 25	III-IV	> 2.5	> 2.5	> 3 × or need RRT

HE: Hepatic encephalopathy; INR: International normalized ratio; RRT: Renal replacement therapy.