Aggiornamento
Approccio clinico al bambino con enzimi epatici elevati, “quasi” vent’anni dopo...
A CLINICAL APPROACH TO A CHILD WITH ELEVATED LIVER ENZYMES, TWENTY YEARS LATER...
Giuseppe Maggiore
Unitŕ di Gastroenterologia ed Epatologia, Dipartimento di Pediatria, Azienda Ospedaliera-Universitaria Pisana
Gennaio 2010 - pagg. 21 -32
Abstract
Isolated alteration of liver enzyme particularly in an apparently healthy child can be a diagnostic
challenge for the clinician. In this review the Authors provide a guide to alteration
of liver enzyme activity based on four main clinical-biochemical syndromes. “Acute pure
cytolytic syndrome” reflects acute hepatocellular damage as can be observed in infectious,
autoimmune toxic or ischemic hepatitis or in case of acute severe muscular damage. Some
acute hepatocellular damage can be cryptogenic in origin. “Acute mixed cytolytic
syndrome” reflects acute biliary and hepatocellular damage as observed in acute biliary
obstruction of normal or damaged bile tree, viral infections with cholangiotropic viruses,
vasculitic damage of the biliary tree as observed in Kawasaki disease and drug induced
liver injury with cholestatic features. “Chronic pure cytolytic syndrome” can reflect either
chronic muscle or liver damage of different etiology and is generally asymptomatic. Inborn
error of metabolism may account for a large number of cases. Isolated increase in aspartate
aminotransferase suggests macrotransaminasemia. “Chronic mixed cytolytic syndrome”
can be observed in case of persistent bile duct damage of various causes.
Classificazione MeSH
Bibliografia
1. Maggiore G, De Giacomo C, Giancola A.
Aumento delle transaminasi sieriche nel bambino.
Medico e Bambino 1990;9:14.
2. Massei F, Palla G, Ughi C, Macchia P, Maggiore G. Cholestasis as a presenting feature of acute Epstein-Barr virus infection. Pediatr Infect Dis J 2001;20:721-2.
3. Sokal EM, Melchior M, Cornu C, et al. Acute parvovirus B19 infection associated with fulminant hepatitis of favourable prognosis in young children. Lancet 1998;352:1739-41.
4. Bernard O, Hadchouel M, Scotto J, Odičvre M, Alagille D. Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood. J Pediatr 1981;99:704-11.
5. Squires RH, Shneider BL, Bucuvalas J, et al. Acute liver failure in children, the first 348 patients in the pediatric acute liver failure study group. J Pediatr 2006;148:652-8.
6. Murray KF, Hadzic N, Wirth S, Basset M, Kelly D. Drug-related hepatotoxicity and acute liver failure. J Pediatr Gastroenterol Nutr 2008;47:395-405.
7. Whitehead MW, Hawkes ND, Hainsworth I, Kingham JG. A prospective study of the causes of notably raised aspartate aminotransferase of liver origin. Gut 1999;45:129-33.
8. Kamath BM, Dhawan A, Mieli-Vergani G. Raised serum transaminases: not always liver disease. Arch Dis Child 2000;82:270-1.
9. Caropreso M, Fortunato G, Lenta S, et al. Prevalence and long-term course of macroaspartate aminotransferase in children. J Pediatr 2009;154:744-8.
10. Sciveres M, Riva S, Campani D, et al. Visceral leishmaniasis mimicking autoimmune hepatitis. J Pediatr Gastroenterol Nutr 2009; 48:639-42.
11. Maggiore G, Sciveres M. Il fegato grasso del bambino: malattia o disturbo cosmetico? Medico e Bambino 2007;26:105-10.
12. Maggiore G, Caprai S. Liver involvement in celiac disease. Indian J Pediatr 2006;73:809-12.
13. Caprai S, Vajro P, Ventura A, Sciveres M, Maggiore G; SIGENP Study Group for Autoimmune Liver Disorders in Celiac Disease. Autoimmune liver disease associated with celiac disease in childhood: a multicenter study. Clin Gastroenterol Hepatol 2008;6:803-6.
14. Caprai S, Loudianos G, Massei F, Gori L, Lovicu M, Maggiore G. Direct diagnosis of Wilson disease by molecular genetics. J Pediatr 2006;148:138-40.
15. Mandato C, Brive L, Miura Y, et al. Cryptogenic liver disease in four children: a novel congenital disorder of glycosylation. Pediatr Res 2006;59:293-8.
16. Iorio R, Sepe A, Giannattasio A, Cirillo F, Vegnente A. Hypertransaminasemia in childhood as a marker of genetic liver disorders. J Gastroenterol 2005;40:820-6.
17. Maggiore G, Larizza D, Lorini R, De Giacomo C, Scotta MS, Severi F. Propylthiouracil hepatotoxicity mimicking autoimmune chronic active hepatitis in a girl. J Pediatr Gastroenterol Nutr 1989;8:547-8.
18. Bugeac N, Pacht A, Mandel H, et al. The significance of isolated elevation of serum aminotransferases in infants and young children. Arch Dis Child 2007;92:1109-12.
19. Zancan L, Bettiol T, Rini A, et al. Chronic idiopathic hypertransaminasemia. Minerva Pediatr 1996;48:209-16.
20. Craxě A, Almasio P. Diagnostic approach to liver enzyme elevation. J Hepatol 1996;25 (suppl 1):47-51.
21. Giannini EG, Testa R, Savarino V. Liver enzyme alteration: a guide for clinitians. CMAJ 2005;172:367-79.
22. Maggiore G. Approccio diagnostico al bambino con aminotransferasi elevate. Area Pediatrica 2002;3:4-15.
23. Maggiore G. L’esplorazione bioumorale del fegato: il bilancio epatico. Medico e Bambino 2009;28:649-53.
24. Salueńa I, Ortega L, Devesa MJ, et al. Utility of liver biopsy in the etiologic diagnosis of biochemical liver abnormalities of unknown cause. Gastroenterol Hepatol 2007;30:325-30.
2. Massei F, Palla G, Ughi C, Macchia P, Maggiore G. Cholestasis as a presenting feature of acute Epstein-Barr virus infection. Pediatr Infect Dis J 2001;20:721-2.
3. Sokal EM, Melchior M, Cornu C, et al. Acute parvovirus B19 infection associated with fulminant hepatitis of favourable prognosis in young children. Lancet 1998;352:1739-41.
4. Bernard O, Hadchouel M, Scotto J, Odičvre M, Alagille D. Severe giant cell hepatitis with autoimmune hemolytic anemia in early childhood. J Pediatr 1981;99:704-11.
5. Squires RH, Shneider BL, Bucuvalas J, et al. Acute liver failure in children, the first 348 patients in the pediatric acute liver failure study group. J Pediatr 2006;148:652-8.
6. Murray KF, Hadzic N, Wirth S, Basset M, Kelly D. Drug-related hepatotoxicity and acute liver failure. J Pediatr Gastroenterol Nutr 2008;47:395-405.
7. Whitehead MW, Hawkes ND, Hainsworth I, Kingham JG. A prospective study of the causes of notably raised aspartate aminotransferase of liver origin. Gut 1999;45:129-33.
8. Kamath BM, Dhawan A, Mieli-Vergani G. Raised serum transaminases: not always liver disease. Arch Dis Child 2000;82:270-1.
9. Caropreso M, Fortunato G, Lenta S, et al. Prevalence and long-term course of macroaspartate aminotransferase in children. J Pediatr 2009;154:744-8.
10. Sciveres M, Riva S, Campani D, et al. Visceral leishmaniasis mimicking autoimmune hepatitis. J Pediatr Gastroenterol Nutr 2009; 48:639-42.
11. Maggiore G, Sciveres M. Il fegato grasso del bambino: malattia o disturbo cosmetico? Medico e Bambino 2007;26:105-10.
12. Maggiore G, Caprai S. Liver involvement in celiac disease. Indian J Pediatr 2006;73:809-12.
13. Caprai S, Vajro P, Ventura A, Sciveres M, Maggiore G; SIGENP Study Group for Autoimmune Liver Disorders in Celiac Disease. Autoimmune liver disease associated with celiac disease in childhood: a multicenter study. Clin Gastroenterol Hepatol 2008;6:803-6.
14. Caprai S, Loudianos G, Massei F, Gori L, Lovicu M, Maggiore G. Direct diagnosis of Wilson disease by molecular genetics. J Pediatr 2006;148:138-40.
15. Mandato C, Brive L, Miura Y, et al. Cryptogenic liver disease in four children: a novel congenital disorder of glycosylation. Pediatr Res 2006;59:293-8.
16. Iorio R, Sepe A, Giannattasio A, Cirillo F, Vegnente A. Hypertransaminasemia in childhood as a marker of genetic liver disorders. J Gastroenterol 2005;40:820-6.
17. Maggiore G, Larizza D, Lorini R, De Giacomo C, Scotta MS, Severi F. Propylthiouracil hepatotoxicity mimicking autoimmune chronic active hepatitis in a girl. J Pediatr Gastroenterol Nutr 1989;8:547-8.
18. Bugeac N, Pacht A, Mandel H, et al. The significance of isolated elevation of serum aminotransferases in infants and young children. Arch Dis Child 2007;92:1109-12.
19. Zancan L, Bettiol T, Rini A, et al. Chronic idiopathic hypertransaminasemia. Minerva Pediatr 1996;48:209-16.
20. Craxě A, Almasio P. Diagnostic approach to liver enzyme elevation. J Hepatol 1996;25 (suppl 1):47-51.
21. Giannini EG, Testa R, Savarino V. Liver enzyme alteration: a guide for clinitians. CMAJ 2005;172:367-79.
22. Maggiore G. Approccio diagnostico al bambino con aminotransferasi elevate. Area Pediatrica 2002;3:4-15.
23. Maggiore G. L’esplorazione bioumorale del fegato: il bilancio epatico. Medico e Bambino 2009;28:649-53.
24. Salueńa I, Ortega L, Devesa MJ, et al. Utility of liver biopsy in the etiologic diagnosis of biochemical liver abnormalities of unknown cause. Gastroenterol Hepatol 2007;30:325-30.
Corrispondenza: g.maggiore@clp.med.unipi.it