Sclerosing cholangitis in children,☆☆,

https://doi.org/10.1016/S0022-3476(94)70253-5Get rights and content

Abstract

We report on 56 children with sclerosing cholangitis (SC) seen between 1972 and 1992. The first symptoms occurred at a mean age of 3.7 years; 15 infants had neonatal cholestatic jaundice. At diagnosis, cholestatic jaundice was present in 25 children, hepatomegaly in 54, splenomegaly in 41, and ascites in 12. Serum alkaline phosphatase activity was increased in 49 patients and γ-glutamyltransferase activity in all patients tested. Most often the histopathologic findings were extensive portal fibrosis and neoductular proliferation. Cholangiography showed abnormal intrahepatic bile ducts in all children and abnormal extrahepatic bile ducts in 35 (63%). The children were separated into three groups: (1) those with SC of neonatal onset (27%); (2) those with SC of postneonatal onset associated with another disease (55%)—histiocytosis X in 14 children, immunodeficiency syndromes in 8, chronic inflammatory bowel disease or autoimmune hepatitis in 8, and congenital psoriasis in 1; and (3) those with SC of postneonatal onset without an associated disease (18%). Biliary cirrhosis was present in all but three children after 6 months to 19.3 years of follow-up. Eleven children died of portal hypertension or liver failure, and six died of a complication related to the associated disease. Fifteen children had liver transplantation; 11 of these are alive 6 months to 61/2 years later without recurrence of SC. The overall estimated median survival time of children with SC was 10 years from clinical onset. These results indicate that SC should be suspected in all children with a chronic cholestatic disease and increased serum γ-glutamyl transferase activity, especially when diseases known to be associated with SC are present. The prognosis is poor, but liver transplantation should be considered except in those with severe immunodeficiency syndromes. (J PEDIATR 1994;124:49-56)

Section snippets

METHODS

Between 1972 and 1992, we investigated 56 children for cholestatic liver disease and found that they had SC, as shown on cholangiograms displaying typical bile duct abnormalities.12 The diagnosis of SC was established by operative cholangiography in 12 children, percutaneous transhepatic cholangiography or cholecystography in 40 children, and endoscopic retrograde cholangiography in 4. The clinical or radiologic features of some of these children have been previously reported in part.13, 14, 15

Clinical and biochemical findings

(Table I). There were 32 boys and 24 girls. The parents were related in 13 instances. Fifteen children had neonatal cholestatic jaundice starting within the first 2 weeks of life. Among the 41 with later onset, initial signs occurred at a mean age of 5 ± 4 years (range, 3 months to 14 years) and consisted of cholestatic jaundice in 9, hepatomegaly in 28, splenomegaly in 1, and gastric variceal hemorrhage in 1. Abnormalities were found on liver function tests during a routine examination in two

DISCUSSION

Sclerosing cholangitis is being recognized more frequently since the development of percutaneous transhepatic cholangiography and cholecystography. In 1987 a review of 78 pediatric cases showed that SC in children was most often associated with an underlying disease (75%),5 but only 55% of the children reported on here had an associated disease and IBD was relatively uncommon. This difference may reflect referral patterns. Children who had SC without an apparent associated extrabiliary disease

References (31)

  • RW Chapman et al.

    Primary sclerosing cholangitis: a review of its clinical features, cholangiography and hepatic histology

    Gut

    (1980)
  • E Lebovics et al.

    Outcome of primary sclerosing cholangitis: analysis of long-term observation of 38 patients

    Arch Intern Med

    (1987)
  • A Sisto et al.

    Primary sclerosing cholangitis in children: study of five cases and review of the literature

    Pediatrics

    (1987)
  • JJ Davis et al.

    Sclerosing cholangitis associated with chronic cryptosporidiosis in a child with a congenital immunodeficiency disorder

    Am J Gastroenterol

    (1987)
  • AD Jorge et al.

    Family incidence of primary sclerosing cholangitis associated with immunological diseases

    Endoscopy

    (1987)
  • Cited by (171)

    • Bile Duct Diseases

      2023, MacSween's Pathology of the Liver, Eighth Edition
    • Jaundice

      2018, Nelson Pediatric Symptom-Based Diagnosis
    • Liver diseases of childhood

      2018, Practical Hepatic Pathology: A Diagnostic Approach: Second Edition
    View all citing articles on Scopus

    From the Service d' Hépatologie Pédiatrique, Service de Radiologie Pédiatrique, and Unité de Recherche INSERM U 347, Hôpital de Bicêtre, 94275 Le Kremlin-Bicêtre, France

    ☆☆

    Reprint requests: Dominique Debray, MD, Service d'Hépatologie Pédiatrique, Hôpital de Bicêtre, 78 rue du Général Leclerc, 94275 Bicêtre, France

    0022-3476/94/$1.00 + .10 9/20/50368

    View full text