Acute liver failure in children: The role of defects in fatty acid oxidation

Hepatology

Volumn 41, Issue 4, 2005, Pages 696-699

  Alonso, Estella M ^a,b  

^a CHILDREN'S MEMORIAL HOSPITAL   (United States)

^b NORTHWESTERN UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYL COENZYME A; BUTYRYL COENZYME A DEHYDROGENASE; CARNITINE; DICARBOXYLIC ACID; FLAVINE ADENINE NUCLEOTIDE; LIPID EMULSION; LONG CHAIN ACYL COENZYME A DEHYDROGENASE; LONG CHAIN FATTY ACID; MEDIUM CHAIN ACYL COENZYME A DEHYDROGENASE; NONSTEROID ANTIINFLAMMATORY AGENT; SALICYLIC ACID; VALPROIC ACID;

APLASTIC ANEMIA; CELL FUNCTION; CHILD; DIET RESTRICTION; DRUG CONTRAINDICATION; EDITORIAL; ENZYME ACTIVATION; ENZYME ACTIVITY; ENZYME ASSAY; FATTY ACID OXIDATION DEFECT; FATTY ACID TRANSPORT; GENE MUTATION; GLUCONEOGENESIS; GLUCOSE INFUSION; GLYCOLYSIS; HEPATITIS; HUMAN; HYPOGLYCEMIA; KETOGENESIS; KETONURIA; LIVER BIOPSY; LIVER CELL; LIVER DYSFUNCTION; LIVER FAILURE; LIVER TRANSPLANTATION; METABOLIC DISORDER; MUSCLE BIOPSY; PRIORITY JOURNAL; SCREENING TEST; URINALYSIS;

CHILD; HUMANS; LIPID METABOLISM, INBORN ERRORS; LIVER FAILURE, ACUTE;

EID: 16244371012     PISSN: 02709139     EISSN: None     Source Type: Journal    
DOI: 10.1002/hep.20680     Document Type: Editorial

References (25)

1. Psacharopoulos HT, Mowat AP, Davies M, Portmann B, Silk DBA, Williams R. Fulminant hepatic failure in childhood: an analysis of 31 cases. Arch Dis Child 1980;55:252-258.
2. Devictor D, Tahiri C, Rousset A, Massenavette B, Russo M, Huault G. Management of fulminant hepatic failure in children-an analysis of 56 cases. Crit Care Med 1993;21:S348-S349.
3. Whitington PF, Soriano HE, Alonso EM. Fulminant hepatic failure in children. In: Suchy FJ, Sokol RJ, Balistreri WF, eds. Liver Disease in Children. Philadelphia: Lippincott Williams & Wilkins, 2001:63-88.
4. Ostapowicz G, Fontana RJ, Schiodt FV, Larson A, Davern TJ, Han SH, et al. Results of a prospective study of acute liver failure at 17 tertiary care centers in the United States. Ann Intern Med 2002;137:947-954.
5. Alonso EM, Sokol RJ, Hart J, Tyson RW, Narkewicz MR, Whitington PF. Fulminant hepatitis associated with centrilobular hepatic necrosis in young children. J Pediatr 1995;127:888-894.
6. Shneider BL, Rinaldo P, Emre S, Bucuvalas J, Squires R, Narkewicz M, et al. Abnormal concentrations of esterified carnitine in bile: a feature of pediatric acute liver failure with poor prognosis. HEPATOLOGY 2005;41:717-721.
7. Treem W. Inborn defects in mitochondrial fatty acid oxidation. In: Suchy FJ, Sokol RJ, Balistreri WF, eds. Liver Disease in Children. Philadelphia: Lippincott Williams & Wilkins, 2001:735-785.
8. Rinaldo P. Fatty acid transport and mitochondrial oxidation disorders. Semin Liver Dis 2001;21:489-500.
9. Stanley C, Hale D. Genetic disorders of mitochondrial fatty acid oxidation. CurrOp Pediatr 1994;6:476-481.
10. Treem W, Wilzleben CA, Piccoli DA, Stanley CA, Hale DE, Coates PH, et al. Medium-chain and long-chain acyl-CoA dehydrogenase deficiency: clinical, pathologic and ultrastructural differentiation from Reye's Syndrome. HEPATOLOGY 1986;6:1270-1278.
11. Roe C, Millington D, Maltby D, Kinnebrew R, Recognition of medium chain acyl CoA dehydrogenase deficiency in asymptomatic siblings of children dying of sudden infant death o Reye-like syndromes. J Pediatr 1986;108:13-18.
12. Rowe P, Valle D, Brusilow S. Inborn errors of metabolism in children referred with Reye's syndrome a changing pattern. JAMA 1988;260:3167-70.
13. Orlowski JP. Whatever happened to Reye's syndrome? Did it ever really exist? Crit Care Med 1999;27:1582-7.
14. Belay ED, Bresee JS, Holman RC, Khan AS, Shahriari A, Schonberger LB. Reye's syndrome in the United States from 1981 through 1997. N Engl J Med 1999;340:1377-82.
15. Casteels-Van Daele M, Van Geet C, Wouters C, Eggermont E. Reye syndrome revisited: a descriptive term covering a group of heterogeneous disorders. Eur J Pediatr 2000;159:641-8.
16. Glasgow JF, Middleton B. Reye syndrome-insights on causation and prognosis. Arch Dis Child 2001;85:351-353.
17. Boles RG, Buck EA, Blitzer MG, Platt MS, Cowan TM, Martin SK, et al. Retrospective biochemical screening of fatty acid oxidation disorders in postmortem livers of 418 cases of sudden death in the first year of life. J Pediatr 1998;132:924-933.
18. Rashed MS, Ozand PT, Bennett MJ, Barnard JJ, Govindaraju DR, Rinaldo P. Inborn errors of metabolism diagnosed in sudden death cases by acylcarnitine analysis of postmortem bile. Clin Chem 1995;41:1109-14.
19. Ibdah JA, Bennett MJ, Rinaldo P, Zhao Y, Gibson B, Sims HF, et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med 1999;340:1723-1731.
20. Hagenfeldt L, Venizelos N, von Dobeln U. Clinical and biochemical presentation of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency. J Inherit Metab Dis 1995;18:245-248.
21. Tyni T, Palotie A, Vinikka L, Valanne L, Salo MK, von Dobeln U, et al. Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency with the G1528C mutation: Clinical presentation of thirteen patients. J Pediatr 1997;130:67-76.
22. AJ Odaib AA, Shneider BL, Bennett MJ, Pober BR, Reyes-Mugica M, Friedman AL, et al. A defect in the transport of long-chain fatty acids associated with acute liver failure. N Engl J Med 1998;339:1752-1757.
23. Bennett MJ, Spotswood SD, Ross KF, Comfort S, Koonce R, Boriack RL, et al. Fatal hepatic short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency: clinical, biochemical, and pathological studies on three subjects with this recently identified disorder of mitochondrial beta-oxidation. Pediatr Dev Pathol 1999;2:337-345.
24. Rinaldo P, AJ Odaib AA, Bennett MJ. Complementation analysis of six patients with a defect of long-chain fatty acid transport in fibroblasts [Abstract]. J Inherit Metab Dis 1999;22(Suppl):15.
25. O'Brien L, Rinaldo P, Sims HF, et al. Fulminant hepatic failure associated with mutations in the medium L-3-hydroxyacyl-CoA dehydrogenase gene [Abstract]. J Inherit Metab Dis 2000;23(Suppl):A127.