Biliary atresia (BA) is a neonatal liver disease characterized by progressive obstruction and fibrosis of the extrahepatic biliary tree as well as fibrosis and inflammation of the liver parenchyma. Recent studies found that infants who will go on to develop BA have elevated direct bilirubin levels in the first few days of life, suggesting that the disease starts in utero. The etiology and pathogenesis of BA, however, remain unknown. Here, we discuss recent studies examining potential pathogenetic mechanisms of BA, including genetic susceptibility, involvement of the immune system, and environmental insults such as viruses and toxins, although it is possible that there is not a single etiological agent but rather a large group of injurious insults that result in a final common pathway of extrahepatic bile duct obstruction and liver fibrosis. The management and diagnosis of BA have not advanced significantly in the past decade, but given recent advances in understanding the timing and potential pathogenesis of BA, we are hopeful that the next decade will bring early diagnostics and novel therapeutics.

Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic disease. The hepatic manifestations of ARPKD can range from asymptomatic to portal hypertension and massively dilated biliary system that results in liver transplantation. Hepatic complications of ARPKD typically present with signs of portal hypertension (splenomegaly and thrombocytopenia) or cholangitis. Liver disease in ARPKD does not always correlate with severity of renal disease. Management of ARPKD-related liver disease is largely treating specific symptoms, such as antibiotics for cholangitis or endoscopic treatment for variceal bleeding. If complications cannot be managed medically, liver transplantation may be indicated. This mini-review will discuss the clinical manifestations and management of children with ARPKD liver disease.

Gastrogastric fistula (GGF) is a known complication of gastric bypass surgery. Revisional surgery for GGF repair can be technically challenging. We describe our experience with endoscopic repair of small GGFs. A retrospective review was performed to identify patients in whom symptomatic GGF was repaired endoscopically at our institution between September 2004 and September 2008. At endoscopy, the fistulous margins were debrided using cold biopsy forceps or ablated using Argon Plasma Coagulation (APC). The fistula was then repaired with endoclips. Status of GGF repair was assessed intra-operatively, at 2 weeks by upper gastrointestinal (UGI) series, and at regular follow-up thereafter. GGF repair was attempted in eight female patients (mean age = 47 years). The average time interval between gastric bypass surgery and GGF presentation was 81 months. The presenting symptoms included nausea, vomiting, abdominal pain, and weight regain. The average duration of endoscopic procedure was 55 min. All GGFs were small (<20 mm). Endoscopic repair of GGF was successful intra-operatively in all patients. Two patients had failure of GGF repair at 2 weeks. Other two patients experienced recurrent symptoms after several weeks and had a delayed failure of GGF repair diagnosed by UGI series. Endoscopic repair has remained successful in four patients at 8-46 months follow-up. Endoscopic repair of small GGFs using endoclips is feasible. It must be considered as an option for management of small GGFs, given its safety, and ease of performance compared to revisional surgery.

Autoimmune liver diseases (ALDs), including autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cholangitis (PBC), are an indication for liver transplantation (LT) in 3%-5% of transplants in the United States. 1,2 Not all patients with ALD go on to need LT, but indications for LT can include acute liver failure, decompensated cirrhosis, a Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease score ≥ 15, malignancy, or refractory pruritus (PBC). Transplantation for ALD presents unique considerations given the possibility of recurrent disease and increased incidence of rejection. 3,4 Despite the potential for recurrent disease, outcomes after LT for ALD are similar to other indications. In adult LT recipients, long-term survival for AIH was similar to alcoholic cirrhosis, although worse than PBC and PSC, possibly related to immunosuppression and serious infections. 5 Patients with AIH post-LT were also at an increased risk for graft loss compared with PBC, PSC, and alcoholism, with a graft survival rate of 73.2% at 5 years and 50.9% at 15 years. 5 According to a United Network for Organ Sharing database analysis, patient survival for children transplanted for both AIH and PSC was >90% at 5 years. 6 RECURRENT DISE ASE AFTER TR ANSPL ANTBiochemical, imaging, histological features, and treatment of recurrent ALD are summarized in Table 1.

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