2006, Number 2
<< Back Next >>
Ann Hepatol 2006; 5 (2)
Vanishing bile duct syndrome in a child with toxic epidermal necrolysis: An interplay of unbalanced immune regulatory mechanisms
Karnsakul W, Arkachaisri T, Atisook K, Wisuthsarewong W, Sattawatthamrong Y, Aanpreung P
Language: English
References: 18
Page: 116-119
PDF size: 108.74 Kb.
Text Extraction
Vanishing bile duct syndrome (VBDS) is a rare disorder and requires a liver biopsy for a diagnosis. The condition has not been reported in children with toxic epidermal necrolysis (TEN). The etiology of VBDS in our patient with TEN is most likely from drug hypersensitivity. A high index of suspicion will prompt clinicians to start more specific investigations and treatments. The use of immunosuppressive agents, intravenous immunoglobulin and ursodeoxycholic acid has not been consistently successful in these patients. A
new approach with biologic agents such as anti-tumor
necrosis factor-α may be a promising therapy and reduce
severe adverse outcomes.
REFERENCES
Srivastava M, Perez-Atayde A, Jonas MM. Drug-associated acuteonset vanishing bile duct and Stevens-Johnson syndromes in a child. Gastroenterology 1998; 115: 743-6.
Garcia M, Mhanna MJ, Chung-Park MJ, Davis PH, Srivastava MD. Efficacy of early immunosuppressive therapy in a child with carbamazepine-associated vanishing bile duct and Stevens-Johnson syndromes. Dig Dis Sci 2002; 47: 177-82.
Sherlock S. The syndrome of disappearing intrahepatic bile ducts. Lancet 1987; 2: 493-6.
Chamuleau RA, Diekman MJ, Bos PJ, Smitt JH, Bosma A, Schellekens PT. Reappearance of vanished bile ducts. Hepatogastroenterology 1992; 39: 523-4.
Bruguera M, Llach J, Rodes J. Nonsyndromic paucity of intrahepatic bile ducts in infancy and idiopathic ductopenia in adulthood: the same syndrome? Hepatology 1992; 15: 830-4.
O’Brien CB, Shields DS, Saul SH, Reddy KR. Drug-induced vanishing bile duct syndrome: response to ursodiol. Am J Gastroenterol 1996; 91: 1456-7.
Moradpour D, Altorfer J, Flury R, Greminger P, Meyenberger C, Jost R, Schmid M. Chlorpromazine-induced vanishing bile duct syndrome leading to biliary cirrhosis. Hepatology 1994; 20: 1437-41.
Becker DS. Toxic epidermal necrolysis. Lancet 1998; 351: 1417-20.
Halebian PH, Corder VJ, Madden MR, Finklestein JL, Shires GT. Improved burn center survival of patients with toxic epidermal necrolysis managed without corticosteroids. Ann Surg 1986; 204: 503-12.
Revuz JE, Roujeau JC. Advances in toxic epidermal necrolysis. Semin Cutan Med Surg 1996; 15: 258-66.
Stutts JT, Washington K, Bernard JA. Cholestatic jaundice with skin desquamation in a 12- year-old girl. J Pediatr 1999; 134: 649-53.
Paquet P, Pierard GE, Quatresooz P. Novel treatments for drug-induced toxic epidermal necrolysis (Lyell’s syndrome). Int Arch Allergy Immunol 2005; 136: 205-16.
Tangkanakul W, Smits HL, Jatanasen S, Ashford DA. Leptospirosis: an emerging health problem in Thailand. Southeast Asian J Trop Med Public Health 2005; 36: 281-8.
Doudier B, Garcia S, Quennee V, Jarno P, Brouqui P. Prognostic factors associated with severe leptospirosis. Clin Microbiol Infect 2006; 12: 299-300.
Nally JE, Chantranuwat C, Wu XY, Fishbein MC, Pereira MM, Da Silva JJ, Blanco DR, et al. Alveolar septal deposition of immunoglobulin and complement parallels pulmonary hemorrhage in a guinea pig model of severe pulmonary leptospirosis. Am J Pathol 2004; 164(3): 1115-27.
Yao F, Behling CA, Saab S, Li S, Hart M, Lyche KD. Trimethoprimsulfamethoxazole-induced vanishing bile duct syndrome. Am J Gastroenterol 1997; 92: 167-9.
Degott C, Feldmann G, Larrey D, Durand-Schneider AM, Grange D, Machayekhi JP, Moreau A, et al. Drug-induced prolonged cholestasis in adults: a histological semiquantitative study demonstrating progressive ductopenia. Hepatology 1992; 15: 244-51.
Smith LA, Ignacio JR, Winesett MP, Kaiser GC, Lacson AG, Gilbert-Barness E, Gonzalez-Peralta RP, et al. Vanishing bile duct syndrome: amoxicillin-clavulanic acid associated intra-hepatic cholestasis responsive to ursodeoxycholic acid. J Pediatr Gastroenterol Nutr 2005; 41: 469-73.