Cirrhosis in children with celiac disease.
BACKGROUND
Liver involvement represents an extra-intestinal feature of celiac disease (CD) and shows a clinical spectrum varying from nonspecific reactive hepatitis to cirrhosis. Here we report the association of cirrhosis with CD in 5 children.


METHODS
The mean age of the patients was 9.4 +/- 2.8 years. Viral, metabolic, and autoimmune etiology of liver disease was ruled out. Intestinal and liver biopsies were performed to confirm the histologic diagnosis in all subjects.


RESULTS
Three of the patients had chronic diarrhea and hepatosplenomegaly in whom diagnoses of CD and cirrhosis were established at presentation simultaneously. In the other 2 patients, CD was diagnosed following an initial diagnosis of cirrhosis. At diagnosis, alanine aminotransferase (range, 64-271 IU/L) and aspartate aminotransferase (range, 90-225 IU/L) values were elevated. After 1 to 5 years of a gluten-free diet (GFD), normalization of serum aminotransferase levels and clinical improvement were observed in 3 patients with strict GFD. The other 2 patients without improvement of the liver disease had poor dietary compliance.


CONCLUSIONS
CD may be associated with severe hepatic damage in children and strict GFD may have beneficial effect on the course of liver disease. Serologic screening of CD should be included in differential diagnosis of chronic liver disease of unknown origin.
Section of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University, Faculty of Medicine, Ankara, Turkey. hudemir@hacettepe.edu.tr
Hülya
H
Demir
Aysel
A
Yüce
Melda
M
Caglar
Gülsev
G
Kale
Nurten
N
Kocak
Hasan
H
Ozen
Figen
F
Gürakan
Inci Nur
IN
Saltik-Temizel
D000293:Adolescent; D000410:Alanine Transaminase / Q000097:blood*; D000888:Antibodies, Anti-Idiotypic / Q000097:blood*; D001219:Aspartate Aminotransferases / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D002446:Celiac Disease / Q000097:blood / Q000150:complications / Q000175:diagnosis; D002648:Child; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D007074:Immunoglobulin G / Q000276:immunology; D007421:Intestine, Small / Q000473:pathology; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008103:Liver Cirrhosis / Q000097:blood / Q000150:complications / Q000473:pathology; D008297:Male; D012720:Severity of Illness Index; D014463:Ultrasonography
D000888:Antibodies, Anti-Idiotypic; D015415:Biomarkers; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase