Andrew Grieve

Aspartate Aminotransferase-to-Platelet ratio index (APRi) in infants with biliary atresia: prognostic value at presentation.

BACKGROUND Biliary atresia (BA) is a progressive obliterative cholangiopathy leading to liver fibrosis and cirrhosis. The aspartate aminotransferase-to-platelet ratio index (APRi) has been used in other liver diseases and in older children with BA as a surrogate marker of liver fibrosis. The aim of this study was to calculate APRi at time of presentation and relate this to operative findings and early outcome. METHODS Prospective single surgeon cohort study of infants with BA (January 1999-December 2010). Initial APRi values were related to other biochemical indices and liver appearance at the time of Kasai portoenterostomy. Data are expressed as median (interquartile range). Non-parametric comparison was performed and a P-value of≤0.05 was regarded as significant. RESULTS Overall 260 infants were included in the study. Median APRi was 0.67 (0.43-1.12) at a median age of surgery of 58 (range 14-209) days. APRi correlated with age (rs=0.44; P<0.0001), spleen size (rs=0.48; P<0.0001) and bilirubin (rs=0.45; P<0.0001). Liver assessment at operation was divided into cirrhosis [n=28 (10.8%)] or non-cirrhosis. Using a cut-off value of 1.22 [AUC 0.83 (95% CI 0.73-0.90)] showed a sensitivity of 75% and a specificity of 84% for macroscopic cirrhosis. Native liver survival was significantly different but improved only for those in the lowest APRi quartile (<0.43; P<0.009). APRi values at presentation had no significant association with later development of significant oesophageal varices. CONCLUSION APRi at the time of KP is a useful adjunct in evaluating severity of liver disease in BA at presentation.

Pediatric multifocal myofibroblastic tumors with involvement of the gallbladder: HIV- and Epstein-Barr virus–associated smooth muscle cell tumors

Epstein-Barr virus associated smooth muscle cell tumors are commonly found in immunocompromised patients. These tumors occur most commonly in patients with AIDS and with greater incidence in children. The incidence of gallbladder tumors in these patients is rare, however. We report the case of a 10-year-old female patient who presented to our unit. She is HIV positive and on antiretroviral treatment. She required an emergency cholecystectomy to relieve external compression of the common bile duct where an empyematous gallbladder was found. Histopathology confirmed the presence of an Epstein-Barr virus-associated smooth muscle tumor. She is noted to have other asymptomatic lesions. Surgical intervention is reserved for symptomatic lesions and improves the immunocompromised state, although there is a propensity for local recurrence of the tumor.

Gastric duplication cysts as a rare cause of haematemesis: diagnostic challenges in two children

Gastric duplication cysts are rare congenital alimentary tract anomalies. We describe the importance of imaging in two children with haematemesis due to gastric duplication cysts. We emphasize the necessity for a high clinical index of suspicion for early diagnosis.