Table, Supplemental Digital Content 2.
Proposed criteria of pediatric ACLF (PACLF):
PACLF criteria is met by the presence of at least one of the following extra-hepatic organ failures in a child hospitalized with decompensated cirrhosis.
Respiratory Failure Need for mechanical ventilation Circulatory Failure Need for vasoactive medications Renal Failure Need for renal replacement therapy Neurologic Failure Grade III/IV hepatic encephalopathy
Table, Supplemental Digital Content 2. Proposed pediatric acute-on-chronic liver failure (PACLF) criteria.
decompensated cirrhosis.
All (n=66)
No PACLF (n=46)
PACLF (n=20)
Biliary Atresia* 43 (65%) 32 (70%) 11 (55%)
Genetic causes of cholestasis (PFIC, Alagille#) 4 (6%) 1 (2%) 3 (15%)
Autoimmune Hepatitis (AIH) 1 (2%) 0 (0%) 1 (5%)
Biliary Cirrhosis 3 (5%) 2 (4%) 1 (5%)
Primary Sclerosing Cholangitis (PSC) 3 (5%) 2 (4%) 1 (5%)
PSC/AIH Overlap Syndrome 3 (5%) 3 (7%) 0 (0%)
Alpha 1 Anti-trypsin Deficiency 2 (3%) 2 (4%) 0 (0%)
Cryptogenic Cirrhosis 6 (9%) 3 (7%) 3 (15%)
Congenital Hepatic Fibrosis 1 (2%) 1 (2%) 0 (0%)
*A total of 4 patients had syndromic biliary atresia with situs inversus, polysplenia. Two patients had dextrocardia with no other cardiac anomalies, one patient had levocardia with interrupted IVC with azygous continuation, and one patient had complex cardiac disease including coarctation of aorta repaired pre-transplant and VSD, fibrous subaortic stenosis both repaired post-transplant.
#A total of 2 patients with Alagille syndrome were included. Both patients had a diagnosis of mild pulmonary artery stenosis with normal cardiac function.
Table, Supplemental Digital Content 3. Underlying diagnosis of all patients hospitalized for decompensated cirrhosis
Footnote: Pediatric Acute-on-chronic liver failure (PACLF)
Table, Supplemental Digital Content 4.
Characteristics of Organ Failures in PACLF Hospitalizations.
PACLF during Hospitalization (n=23)
PACLF on Admission (n=9)
Number of organ failures
1 organ failure 10 (43%) 6 (67%)
2 organ failures 7 (30%) 2 (22%)
3 organ failures 3 (13%) 1 (11%)
4 organ failures 3 (13%) 0
Type of organ failure
Respiratory Failure 17 (74%) 4 (44%)
Cardiovascular Failure 12 (52%) 6 (67%)
Renal Failure 7 (30%) 0 (0%)
Hepatic Encephalopathy 9 (39%) 3 (33%)
Pediatric acute-on-chronic liver failure (PACLF).
Table, Supplemental Digital Content 4. Characteristics of organ failures during PACLF hospitalizations.
Footnote: Pediatric Acute-on-chronic liver failure (PACLF)
All (n=186) No PACLF (n=163)
PACLF (n=23) p-value*
Sodium 130.2 ± 6.1 130.3 ± 0.7 126.3 ± 1.2 0.001
Creatinine 0.43 ± 0.52 0.38 ± 0.06 0.94 ± 0.09 <0.001 White Blood Cell
Count
15.3 ± 10.0 13.9 ± 1.0 27.6 ± 1.7 <0.001
Hemoglobin 8.7 ± 1.9 8.8 ± 0.2 7.1 ± 0.4 <0.001
Platelet Count 136.0 ± 103.5 143.3 ± 10.8 47.2 ± 20.1 <0.001
Albumin 2.2 ± 0.6 2.2 ± 0.1 2.0 ± 0.1 NS
Total Bilirubin 12.2 ± 12.1 12.7 ± 1.5 23.0 ± 2.2 <0.001
ALT 188.0 ± 414.2 141.0 ± 35.5 542.0 ± 82.6 <0.001
AST 389.1 ± 1281.5 228.5 ± 102.2 1493.7 ± 253.8 <0.001
GGT 345.5 ± 405.8 340.8 ± 45.7 229.6 ± 86.8 NS
INR 1.8 ± 1.0 1.8 ± 0.1 2.9 ± 0.2 <0.001
Ammonia 114.5 ± 81.8 104.2 ± 16.2 133.7 ± 18.3 NS
Linear mixed models performed for multiple hospitalizations per patient. Least square means ± standard error (SE) are reported.
Alanine aminotransferase (ALT), aspartate aminotransferase (AST), gamma-glutamyl transferase (GGT), International Normalized Ratio (INR), Pediatric acute-on-chronic liver failure (PACLF). Not significant (NS).
* Comparison between PACLF vs. No PACLF.
Table, Supplemental Digital Content 5. Peak laboratory values during hospitalization.
Footnote: Linear mixed models performed for multiple hospitalizations per patient. Least square means ± standard error (SE) are reported.
Alanine aminotransferase (ALT), aspartate aminotransferase (AST), gamma-glutamyl
transferase (GGT), International Normalized Ratio (INR), Pediatric acute-on-chronic liver failure (PACLF). Not significant (NS). * Comparison between PACLF vs. No PACLF.
Table, Supplemental Digital Content 6. Comparison of medications and procedures performed during PACLF and non-PACLF hospitalizations
Footnote: * Comparison between PACLF vs. Non-ACLF, Pediatric Acute-on-chronic liver failure (PACLF)
Table, Supplemental Digital Content 6. Comparison of medications and procedures performed during PACLF and non-PACLF hospitalizations.
All (n=186)
No PACLF (n=163)
PACLF
(n=23) p-value* Medications
Octreotide 41 (22%) 27 (17%) 14 (61%) <0.0001
Vasoactive medications 11 (6%) 0 (0%) 11 (48%) <0.0001
Antibiotics 147 (80%) 124 (76%) 23 (100%) 0.005
Diuretics 107 (58%) 86 (53%) 21 (91%) <0.001
25% Albumin Infusion 98 (53%) 78 (48%) 20 (87%) <0.001 Packed Red Blood Cells
Transfusion
74 (40%) 55 (34%) 19 (83%) <0.0001
Procedures
Esophagogastroduodenoscopy 43 (23%) 33 (20%) 10 (44%) 0.01
Paracentesis 51 (24%) 33 (18%) 18 (58%) <0.0001
