Pediatric celiac disease, cryptogenic hypertransaminasemia, and autoimmune hepatitis

J Pediatr Gastroenterol Nutr. 2013 Jun;56(6):663-70. doi: 10.1097/MPG.0b013e31828dc5c5.

Abstract

Objective: The association between celiac disease (CD) and liver disease in pediatrics is widely recognized, but its prevalence is unknown. This study aims to conduct a systematic review and meta-analysis to evaluate the prevalence of CD in children with cryptogenic persistent hypertransaminasemia (HTS) or autoimmune hepatitis (AIH), and vice versa.

Methods: We searched MEDLINE/PubMed, the Cochrane Library, Web of Science, and MD Consult from 1977 to May 2012 for studies reporting either CD and HTS or AIH. Pooled prevalences with 95% confidence intervals (CI) and relative risk (RR) were calculated.

Results: Nine studies (2046 patients) were identified. Pooled prevalences of CD in children with mild, nonspecific cryptogenic persistent HTS and vice versa were 12.0% (95% CI 4.17-29.96) and 36.0% (95% CI 32.15-40.11), respectively. A gluten-free diet normalized transaminase levels in 77% to 100% of patients with CD within 4 to 8 months. Pooled prevalences of CD in children with AIH and vice versa were 6.3% (95% CI 3.87-11.73) and 1.4% (95% CI 0.84-2.15), respectively. The RR of HTS in children with CD versus the general population, and of CD in children with HTS was 6.55 (95% CI 5.65-7.60) and 11.59 (95% CI 3.80-35.33), respectively. The corresponding RR of AIH in children with CD was 188.54 (95% CI 92.23-385.43). The RR of CD in children with AIH was 6.63 (95% CI 3.86-11.40).

Conclusions: CD is associated with elevated transaminase levels in about one-third of newly diagnosed children. Cryptogenic persistent HTS may signal gluten-dependent nonspecific mild hepatitis (12.0% of cases) or more rarely (6.3%) severe CD-related autoimmune hepatopathy. RRs confirm these trends in the considered associations.

Publication types

  • Meta-Analysis
  • Systematic Review

MeSH terms

  • Adolescent
  • Celiac Disease / blood
  • Celiac Disease / diet therapy
  • Celiac Disease / epidemiology*
  • Celiac Disease / physiopathology
  • Child
  • Child, Preschool
  • Comorbidity
  • Diet, Gluten-Free
  • Hepatitis / blood
  • Hepatitis / enzymology
  • Hepatitis / epidemiology*
  • Hepatitis / physiopathology
  • Hepatitis, Autoimmune / blood
  • Hepatitis, Autoimmune / epidemiology*
  • Hepatitis, Autoimmune / immunology
  • Hepatitis, Autoimmune / physiopathology
  • Hepatitis, Chronic / blood
  • Hepatitis, Chronic / enzymology
  • Hepatitis, Chronic / epidemiology
  • Hepatitis, Chronic / physiopathology
  • Humans
  • Infant
  • Liver Cirrhosis / blood
  • Liver Cirrhosis / congenital
  • Liver Cirrhosis / enzymology
  • Liver Cirrhosis / epidemiology
  • Liver Cirrhosis / physiopathology
  • Prevalence
  • Severity of Illness Index
  • Transaminases / blood

Substances

  • Transaminases

Supplementary concepts

  • Cirrhosis, Cryptogenic