Pediatr Gastroenterol Hepatol Nutr.  2014 Jun;17(2):121-124.

Omega-3 Polyunsaturated Fatty Acid for Cholestasis due to Bile Duct Paucity

• Bae SH
• Park HS
• Han HS
• Yun IJ

Abstract

Omega (omega)-3 polyunsaturated fatty acids appear to be effective in preventing and treating parenteral nutrition-associated liver disease, and several mechanisms were proposed for this observation. An 8-week-old male infant with cholestasis and acholic stool was diagnosed non-syndromic intrahepatic interlobular bile duct paucity by open-wedge liver biopsy. Initially he was treated with usual supportive medical therapy, including ursodeoxycholic acid. However, the clinical status and laboratory tests did not improve. Omega (omega)-3 polyunsaturated fatty acids (initially intravenous administration and oral administration later), were started and his liver function, including aminotransferase level and bilirubin levels normalized, and the ivory stool color turned green. We report the possible effectiveness of omega-3 polyunsaturated fatty acids as a potent choleretic agent for non-syndromic intrahepatic interlobular bile duct paucity, a very rare structural pediatric hepatic disease.

Keyword

Omega-3 fatty acids; Cholestasis; Bile duct paucity; Infant

MeSH Terms

Administration, Intravenous
Administration, Oral
Bile Ducts*
Bilirubin
Biopsy
Cholestasis*
Fatty Acids, Omega-3
Fatty Acids, Unsaturated
Humans
Infant
Liver
Liver Diseases
Male
Ursodeoxycholic Acid
Bilirubin
Fatty Acids, Omega-3
Fatty Acids, Unsaturated
Ursodeoxycholic Acid

Figure

• 

  Fig. 1 Liver biopsy showed intrahepatic interlobuar bile duct paucity. (A) On pathological examination, the portal tract showed arteries (thick arrows). However, no corresponding bile ducts of similar caliber was found (H&E, ×400). (B) Immunostaining for CK19 showed extensive bile ductular proliferation (×400). The portal tract showed arteries (thick arrow), but no corresponding bile ducts of a similar caliber was found.

• 

  Fig. 2 Schematic of the clinical course of the patient. The patient showed biochemical improvement, and the stool color became green after the treatment with omega-3(ω) polyunsaturated fatty acid. A: acholic stool, G: greenish stool, PUFA: polyunsaturated fatty acid, MRCP: magnetic resonance cholangiopancreatography, DISIDA: diisopropyl iminodiacetic acid scan, TB: total bilirubin, DB: direct bilirubin, ALT: alanine aminotransferase.

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