Table 4.
Twenty-Four Literature Cases of OTCD with Acute Liver Failure, Liver Dysfunction or Hepatocellular Injury
| Sex Liver Injury |
Age | Presentation | AST IU/L | ALT IU/L | PT | INR | PTT | Bili | NH3 μmol/L | V | Author | Year |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| F HCI |
4 y | Recurrent emesis, lethargy, hemiparesis stupor (Case 10 in literature) | 2,232 | 2,610 | NR | NR | NR | NR | 658 | NR | Sunshine38 | 1972 |
| M HCI |
10 m | Emesis, hypotonia, brother died at 7 months | 1,590 | 740 | NR | NR | NR | NR | NR | NR | Van der Heiden53 | 1978 |
| F HCI |
14 m | Episodic emesis, loss of milestones | 600 | NR | NR | NR | NR | NR | Unknown (later 315 and 407) | NR | La Brecque13 | 1979 |
| M HCI |
15 m | Recurrent “Reye syndrome” | 542 | 481 | NR | NR | NR | NR | 29 | NR | Yokoi41 | 1981 |
| M ALF HCI |
9m | Emesis, seizures, encephalopathy | NR | 1,200 | 28% | NR | NR | NR | 406 | NR | Landrieu15 | 1982 |
| M HCI |
21 y | Emesis, nausea, progressive encephalopathy, death, brother died at 10 years | 720 | 561 | NR | NR | NR | Nl | 1,012 | NR | Tallan16 | 1983 |
| F HCI |
2 y | Emesis, lethargy, “Reye syndrome” | 679 | 976 | NR | NR | NR | NR | 113 | NR | Hayasaka54 | 1987 |
| M HCI |
6 m | Emesis, episodic lethargy, Coagulation “severely disturbed” | 900 | 600 | NR | NR | NR | NR | NR | NR | Wendel55 | 1989 |
| M LD |
12.5 years | Emesis, lethargy, altered mental status – combative | 90 | 86 | 17.2 | NR | NR | 6.4 | 282 | NR | Capistrano-Estrada56 | 1994 |
| F HCI |
23 m | Recurrent emesis, lethargy, | 777 | 1,213 | NR | NR | NR | NR | 1026 | NR | Pridmore46 | 1994 |
| extensor plantar reflexes | ||||||||||||
| F HCI |
22 m | Recurrent emesis, lethargy, delay, acute cerebellar ataxia with increased AST/ALT, “atypical Reye syndrome” | 1,052 | 275 | NR | NR | NR | NR | 218 | NR | Pridmore46 | 1994 |
| M HCI LD |
13 y | Emesis, ataxia, altered mental status, clonus | 68 | 347 | 16.5 | NR | NR | 2.4 (0.1) | 408 | NR | Myers47 | 1995 |
| F HCI |
3 y | Emesis | 1,206 | 1,663 | 42% | NR | 43 | NR | 294 | NR | Zammarchi48 | 1996 |
| F ALF HCI |
5 m | Emesis | 1,692 | 2,328 | 18% | NR | 48 | NR | 150 | NR | Zammarchi48 | 1996 |
| F LD |
3 y | Coma in illness | 187 | 114 | 17.6 | NR | NR | NR | 343 | NR | Inui57 | 1996 |
| F ALF |
3 d | Hypoglycemia, lactic acidemia, seizures, hypotonia | NR | NR | NR | NR | NR | NR | 2301 | 10% | Klowsowski58 | 1998 |
| F HCI |
3 y | Aggression, confusion, abnormal movements | NR | 299 | NR | NR | NR | NR | NR | NR | Schultz59 | 2000 |
| F HCI |
28 y | GI bleed | 196 | 466 | 15 | 1.3 | NR | 1.1 | 247 | NR | Trivedi7 | 2001 |
| M HCI |
15 m | Recurrent emesis, elevated AST/ALT, FTT, normal ammonia, Gln, orotic, possible HFI | 300 | 1,727 | NR | NR | NR | NR | 40 | NR | Burlina60 | 2006 |
| F ALF HCI |
14 m | Emesis | 2,212 | 3,609 | NR | 5.1 | 67 s | Nl | 74 | NR | Mustafa8 | 2006 |
| M HCI |
36 y | Lethargy post steroid treatment | 295 | 317 | NR | 1.1 | NR | 3.1/0.9 | 494 | NR | Atiq9 | 2008 |
| F ALF HCI |
3 y | Emesis, lethargy, aggression | 7,900 | 5,000 | NR | 3.1 | NR | NR | 161 | 25% | Teufel10 | 2009 |
| M ALF |
24 y | Emesis, hallucinations, h/o ADHD, growth retardation | 114 | NR | NR | 2.7 | NR | Nl | 348 | Low | Thurlow11 | 2010 |
| F ALF |
1.5 y | Cyclic vomiting, lethargy | NR | 3,500 | 55 | NR | NR | NR | 207 | NR | Mira61 | 2011 |
Legend: ALF: Acute liver failure. LD: Liver dysfunction. HCI: Hepatocellular injury. V: Factor V. RR: Respiratory rate. Rx: Treatment. Tx: Liver transplant. ND: Not done. AMS: Altered mental status. HFI: Hereditary fructose intolerance. ADHD: Attention deficit hyperactivity disorder. FTT: Failure to thrive. NR: Not recorded. Gln: Glutamine F: Female. M: Male, m: Months old. y: Years old. Nl: Normal