Rationale: Cholestasis in pediatric patients has diverse etiologies and can be broadly classified as intrahepatic or extrahepatic. The common causes of extrahepatic cholestasis are bile duct calculus, inflammation, or pancreatitis. Malignant tumor is a rare cause of bile ducts obstruction in adolescent. Here we report a 14-year-old male patient with cholestasis due to poorly differentiated adenocarcinoma. Patient concerns: A 14-year-old male patient with cholestasis was admitted because of jaundice, weakness, weight loss, and stomach pain for 2 months. The patient had been diagnosed with epilepsy 4 years previously and was being treated with sodium valproate and oxcarbazepine. On admission, laboratory studies showed elevated levels of aspartate aminotransferase (271 IU/L), alanine aminotransferase (224 IU/l), γ -glutamyltransferase (1668.9 IU/L), total bilirubin (66.4 μmol/L), and direct bilirubin (52.6 μmol/L). Additional laboratory tests eliminated common causes of cholestasis such as bacterial/viral infection, autoimmune liver disease, Wilson disease, Alagille syndrome, or progressive familial intrahepatic cholestasis type 3. The results of laboratory investigations showed no improvement after 10 days of treatment with ursodeoxycholic acid and vitamins A, D, and K1. Enhanced magnetic resonance imaging demonstrated a tumor of 22 mm diameter in the duodenal lumen and dilatation of the common bile duct. Endoscopic retrograde cholangiopancreatography detected a tumor in the duodenal lumen. Diagnosis: Considering the clinical features, imaging manifestation, endoscopic findings, and pathologic characteristic, the patient was diagnosed with poorly differentiated adenocarcinoma. Interventions: The patient underwent pancreaticoduodenectomy and chemotherapy. Outcome: The patient recovered well. Elevated levels of tumor biomarkers or abnormal liver function tests have not occurred during the 2-year follow-up. Conclusion: Cholestasis resulting from primary duodenal papillary carcinoma is rare in pediatric patients but should be considered in the differential diagnosis.
Background and objective: Pediatric acute liver failure (PALF) progresses rapidly and has a poor prognosis. Therefore, simple, sensitive and specific clinical indicators are needed. Gamma-glutamyl transpeptidase (GGT) plays a role in predicting the prognosis in infantile cholestatic liver diseases. However, its role in predicting the prognosis in PALF remains unclear. Methods: In present study, children with PALF were divided into a normal GGT group and a high GGT group using the GGT level of 50 U/L as the demarcation line. Age, sex, serum total bilirubin, direct bilirubin, albumin, total bile acid, international normalized ratio (INR) and pediatric end-stage liver disease (PELD) score were compared between the 2 groups. In addition, GGT level was subjected to receiver operating characteristic (ROC) curve analysis, and the area under the curve and the optimal diagnostic cutoff value were calculated. Results: A total of 41 children with PALF were enrolled in the study. INR, PELD score and mortality rate were significantly higher in the normal GGT group in comparison to the high GGT group. GGT level had area under the ROC curve of 0.8194 (95% CI : 0.680-0.959); the optimal diagnostic cutoff values were 60 U/L. At the cutoff value, the sensitivity and specificity of GGT level in predicting the prognosis in PALF were 86.36% and 73.68% respectively. Conclusion: GGT exhibited high sensitivity and specificity in predicting the prognosis in PALF. It can be used as one useful prognostic indicator of PALF.
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