Pediatr Gastroenterol Hepatol Nutr.  2013 Dec;16(4):273-278.

Urosodeoxycholic Acid Therapy in a Child with Trimethoprim-Sulfamethoxazole-induced Vanishing Bile Duct Syndrome

• Cho HJ
• Jwa HJ
• Kim KS
• Gang DY
• Kim JY

Abstract

We present a case of a 7-year-old boy who had cholestasis after trimethoprim-sulfamethoxazole combination therapy. Liver biopsy was performed 36 days after the onset of jaundice because of no evidence of improving cholestasis. Liver histology revealed portal inflammation, bile plug, and biliary stasis around the central vein with the loss of the interlobular bile ducts. Immunohistochemical stains for cytokeratin 7 and 19 were negative. These findings were consistent with those of vanishing bile duct syndrome (VBDS). Chlestasis was progressively improved with dose increment of urosodeoxycholic acid from conventional to high dose. This is the first case report of trimethoprime-sulfamethoxazole associated VBDS in Korean children. The case suggests that differential diagnosis of VBDS should be considered in case of progressive cholestatic hepatitis with elevation of alkaline phosphatase and gamma-glutamyl transpeptidase after or during taking medicine to treat nonhepatobiliary diseases illness.

Keyword

Cholestasis; Trimethoprim-sulfamethoxazole combination

MeSH Terms

Alkaline Phosphatase
Bile Ducts*
Bile*
Biopsy
Child*
Cholestasis
Coloring Agents
Diagnosis, Differential
gamma-Glutamyltransferase
Hepatitis
Humans
Inflammation
Jaundice
Keratin-7
Liver
Male
Trimethoprim, Sulfamethoxazole Drug Combination
Veins
Alkaline Phosphatase
Coloring Agents
Keratin-7
gamma-Glutamyltransferase

Figure

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  Fig. 1 Hepatobilliary scan shows no excretion of radioisotope to the gallbladder and duodenum.

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  Fig. 2 Histologic findings. (A, B) Bile stasis and bile plugs around the central veins, a widened portal area with loss of interlobular bile ducts, and an increased number of the canal of Hering (H&E, ×200). (C) Negative immunohistochemical staining for cytokeratin 19 (×200).

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  Fig. 3 Biochemical and clinical coursein a child with vanishing bile duct syndrome related to trimethoprim-sulfamethoxazole use. AST: aspartate transaminase, ALT: alanine transaminase, ALP: alkaline phosphatase, GGT: gamma-glutamyltranspeptidase, TB: total bilirubin, DB: direct bilirubin.

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