Nonsydromic paucity of interlobuler bile ducts: Two children with a benign course

KANSU TANCA A., Doganci T., Erden E., Girgin N.

Turkish Journal of Gastroenterology, cilt.9, sa.2, ss.198-200, 1998 (SCI-Expanded) identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 9 Sayı: 2
  • Basım Tarihi: 1998
  • Dergi Adı: Turkish Journal of Gastroenterology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.198-200
  • Anahtar Kelimeler: Childhood, Paucity of interlobular bile ducts
  • Ankara Üniversitesi Adresli: Evet

Özet

Paucity of interlobular bile ducts, which is defined as a reduction in the number of interlobular bile ducts, is a significant abnormality causing intrahepatic cholestasis in children. The nonsyndromic form which is seen rarely, may be idiopathic or associated with various specific clinical conditions. The syndromic form usually shows clinical improvement, while development of cirrhosis is common in the nonsyndromic form. We describe two children with nonsyndromic paucity of interlobular bile ducts diagnosed in the neonatal period, in whom clinical and biochemical improvement occurred in about six months.