Aggiornamento monografico
Atresia delle vie biliari: quali insidie per il pediatra?
BILIARY ATRESIA
Raffaele Iorio, Anna Maria Salzano, Francesco Cirillo, Gianfranco Vallone*
Dipartimento di Pediatria
*Dipartimento di Scienze Biomorfologiche e Funzionali, Università di Napoli “Federico II”
Aprile 2009 - pagg. 224 -231
Abstract
Biliary atresia (BA) is the most common pediatric cause of cirrhosis, end-stage liver disease and indication for liver transplantation. The clinical presentation is characterized by jaundice with yellow or dark urine and pale stools, which eventually become acholic. BA is difficult to identify during the neonatal period because of the well-nourished appearance of the affected infants. The Kasai portoenterostomy (KP) is the primary treatment for BA and has the greatest likelihood success when performed before the age of 45-60 days. Even with a successful KP, more than 70% of children eventually develop cirrhosis and require liver transplantation before adulthood. So far, no single diagnostic test, such as short of laparotomy, is definitive for diagnosis of BA. The most accurate test for differentiating BA from other forms of cholestasis remains percutanoeus liver biopsy that, however, is pathologist dependent. A number of screening strategies in infants have been studied (measurements of serum conjugated bilirubin and evaluation of stool by stool color card), but so far no convenient means of screening newborns is universally recommended.
Parole chiave
Suggerite dall'AI
Classificazione MeSH
Bibliografia
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4. Bobo L, Ojeh C, Chiu D, Machado A, Colombani P, Schwarz K. Lack of evidence for rotavirus by polymerase chain reaction/ enzyme immunoassay of hepatobiliary samples from children with biliary atresia. Pediatr Res 1997; 41:229-34.
5. Riepenhoff-Talty M, Gouvea V, Evans MJ, et al. Detection of group C rotavirus in infants with extrahepatic biliary atresia. J Infect Dis 1996;174:8-15.
6. Bezerra JA, Tiao G, Ryckman FC, et al. Genetic induction of proinflammatory immunity in children with biliary atresia. Lancet 2002; 360:1653-9.
7. Giannattasio A, Cirillo F, Liccardo D, Russo M, Vallone G, Iorio R. Diagnostic role of US for biliary atresia. Radiology 2008;247: 912-3.
8. Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr 2003;37:4-21.
9. Iinuma Y, Narisawa R, Iwafuchi M, et al. The role of endoscopic retrograde cholangiopancreatography infants with cholestasis. J Pediatr Surg 2000;35:545-9.
10. Hadzic N, Davenport M, Tizzard S, Singer J, Howard ER, Mieli-Vergani G. Longterm survival following Kasai portoenterostomy: is chronic liver disease inevitable? J Pediatr Gastroenterol Nutr 2003;37:430-3.
11. Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health Workshop. Hepatology 2007;46:566-81.
12. Lai MW, Chang MH, Hsu SC, et al. Differential diagnosis of extrahepatic biliary atresia from neonatal hepatitis: a prospective study. J Pediatr Gastroenterol Nutr 1994;18: 121-7.
13. Matsui A, Dodoriki M. Screening for biliary atresia. Lancet 1995;345:1181.
14. Hsiao CH, Chang MH, Chen HL, et al.; Taiwan Infant Stool Color Card Study Group. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 2008;47:1233-40.
15. Desmet V. The cholangiopathies. In: Suchy FJ (Ed). Liver Disease in Children. Philadelphia: Lippincott, Williams & Wilkins, 2001:39-62.
16. Shteyer E, Ramm GA, Xu C, White FV, Shepherd RW. Outcome after portoenterostomy in biliary atresia: pivotal role of degree of liver fibrosis and intensity of stellate cell activation. J Pediatr Gastroenterol Nutr 2006; 42:93-9.
17. Azarow KS, Phillips MJ, Sandler AD, Hagerstrand I, Superina RA. Biliary atresia: should all patients undergo a portoenterostomy? J Pediatr Surg 1997;32:168-72.
18. Lang T, Kappler M, Dietz H, Harms HK, Bertele-Harms R. Biliary atresia: which factors predict the success of a Kasai operation? An analysis of 36 patients. Eur J Med Res 2000;5:110-4.
19. Ohi R. Biliary atresia. A surgical perspective. Clin Liver Dis 2000;4:779-804.
20. Shneider BL, Brown MB, Haber B, et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr 2006;148:467-74.
21. Hoofnagle JH, Sokol RJ. Neonatal screening for biliary atresia. Hepatology 2006;43: 646.
22. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000;355:25-9.
2. Petersen C. Pathogenesis and treatment opportunities for biliary atresia. Clin Liver Dis 2006;10:73-88.
3. Bouyahia O, Khelifi I, Mazigh SM, et al. Cholestasis in infants: a study of the Children’s Hospital of Tunisia. Tunis Med 2008; 86:128-35.
4. Bobo L, Ojeh C, Chiu D, Machado A, Colombani P, Schwarz K. Lack of evidence for rotavirus by polymerase chain reaction/ enzyme immunoassay of hepatobiliary samples from children with biliary atresia. Pediatr Res 1997; 41:229-34.
5. Riepenhoff-Talty M, Gouvea V, Evans MJ, et al. Detection of group C rotavirus in infants with extrahepatic biliary atresia. J Infect Dis 1996;174:8-15.
6. Bezerra JA, Tiao G, Ryckman FC, et al. Genetic induction of proinflammatory immunity in children with biliary atresia. Lancet 2002; 360:1653-9.
7. Giannattasio A, Cirillo F, Liccardo D, Russo M, Vallone G, Iorio R. Diagnostic role of US for biliary atresia. Radiology 2008;247: 912-3.
8. Sokol RJ, Mack C, Narkewicz MR, Karrer FM. Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr 2003;37:4-21.
9. Iinuma Y, Narisawa R, Iwafuchi M, et al. The role of endoscopic retrograde cholangiopancreatography infants with cholestasis. J Pediatr Surg 2000;35:545-9.
10. Hadzic N, Davenport M, Tizzard S, Singer J, Howard ER, Mieli-Vergani G. Longterm survival following Kasai portoenterostomy: is chronic liver disease inevitable? J Pediatr Gastroenterol Nutr 2003;37:430-3.
11. Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health Workshop. Hepatology 2007;46:566-81.
12. Lai MW, Chang MH, Hsu SC, et al. Differential diagnosis of extrahepatic biliary atresia from neonatal hepatitis: a prospective study. J Pediatr Gastroenterol Nutr 1994;18: 121-7.
13. Matsui A, Dodoriki M. Screening for biliary atresia. Lancet 1995;345:1181.
14. Hsiao CH, Chang MH, Chen HL, et al.; Taiwan Infant Stool Color Card Study Group. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 2008;47:1233-40.
15. Desmet V. The cholangiopathies. In: Suchy FJ (Ed). Liver Disease in Children. Philadelphia: Lippincott, Williams & Wilkins, 2001:39-62.
16. Shteyer E, Ramm GA, Xu C, White FV, Shepherd RW. Outcome after portoenterostomy in biliary atresia: pivotal role of degree of liver fibrosis and intensity of stellate cell activation. J Pediatr Gastroenterol Nutr 2006; 42:93-9.
17. Azarow KS, Phillips MJ, Sandler AD, Hagerstrand I, Superina RA. Biliary atresia: should all patients undergo a portoenterostomy? J Pediatr Surg 1997;32:168-72.
18. Lang T, Kappler M, Dietz H, Harms HK, Bertele-Harms R. Biliary atresia: which factors predict the success of a Kasai operation? An analysis of 36 patients. Eur J Med Res 2000;5:110-4.
19. Ohi R. Biliary atresia. A surgical perspective. Clin Liver Dis 2000;4:779-804.
20. Shneider BL, Brown MB, Haber B, et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr 2006;148:467-74.
21. Hoofnagle JH, Sokol RJ. Neonatal screening for biliary atresia. Hepatology 2006;43: 646.
22. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet 2000;355:25-9.
Corrispondenza: riorio@unina.it