Introduction: The big variety of clinical presentations of celiac disease makes of the diagnosis a challenge: mild hypertransaminasemia (1) is frequent but rare cases of liver failure related to untreated celiac disease were reported(2)(3).
Methods: We report an unusual presentation of celiac disease in a 16 month old girl who was hospitalized twice for a transient liver failure. The context of a febrile gastroenteritis, during the first hospitalization, misled the diagnosis of celiac disease towards a viral infection or a metabolic disease. Low prothrombine activity (9%) and altered state of consciousness on the initial presentation became normal after one week of close observation, perfusion of fresh frozen plasma and the administration of intravenous vitamin K. One month later, the child was readmitted for frequent episodes of watery diarrhea, cachexia, abdominal distension and pitting edema. Blood tests showed elevated liver enzymes (SGPT/SGOT = 1540/1410 IU/l), hypoproteinemia (4.9g/dl), hypoalbuminemia (2.6 g/dl) and hypoprothrombinemia (27% activity), along with failure to thrive.
Results: Paraclinical workup showed: negative serologies for viral hepatitis A, B, C, cytomegalovirus and epstein barr virus. Anti nuclear antibodies, anti DNA, anti liver-kidney microsomes, anti smooth muscles, anti liver cytosol 1, Anti thyroglobulines and anti thyroperoxidase antibodies were negative; abdominal ultrasound was normal; percutaneous liver biopsy identified macro and a micro cellular steatosis without any inflammatory infiltrates, and without any fibrosis. Anti transglutaminases IgA were positive (superior to 200 RU /ml ). Upper endoscopy with small bowel biopsy confirmed the diagnosis of celiac disease. After 6 months of a gluten free diet, the clinical condition, and the growth parameters improved considerably, with normalization of the liver function tests.
Conclusion: This case shows the importance of considering celiac disease as a potential cause of acute liver failure. Hepatic steatosis can be one of the histological features encountered in celiac disease. Gluten free diet may reverse hepatic failure
1-Hypertransaminasemia in Pediatric Celiac Disease Patients and Its Prevalence as a Diagnostic Clue. Farre C, Esteve M, Curcoy A and al. Am J Gastroentero 2002;97:3176–3181
2-Celiac disease in patients with severe liver disease: gluten-free diet may reverse hepatic failure. Kaukinen K, Halme L, Collin P and al. . Gastroenterology 2002;122:881–8
3-Celiac Disease and hypoprothrombinemia. Hussaini S H, Ahmed S, Heatley R, Nutrition 1999;15:389–391