Improved survival in children with HPS: Experience from two high volume liver transplant centers across continents.

BACKGROUND: Severe HPS increases morbidity and mortality after LT in children. We reviewed the combined experience of LT for HPS in children from two LT centers in Europe and Asia. METHODS: All children with "proven" HPS as per ERS Task Force criteria (detailed in manuscript) who underwent LT were categorized into M (PaO2 ≥80 mmHg), Mo (PaO2  = 60-79 mmHg), S (50-59 mmHg), and VS (PaO2 <50 mmHg) HPS, based on room air PaO2 . RESULTS: Twenty-four children with HPS underwent 25 LT (one re-transplantation) at a median age of 8 years (IQR, 5-12), after a median duration of 8 (4-12) months following HPS diagnosis. Mechanical ventilation was required for a median of 3 (1.5-27) days after LT. Ten children had "S" post-operative hypoxemia, requiring iNO for a median of 5 (6-27) days. "VS" category patients had significantly prolonged invasive ventilation (median 35 vs. 3 and 1.5 days; p = .008), ICU stay (median 39 vs. 8 and 8 days; p = .007), and hospital stay (64 vs. 26.5 and 23 days; p < .001) when compared to "S" and "M/Mo" groups, respectively. The need for pre-transplant home oxygen therapy was the only factor predicting need for re-intubation. Patient and graft survival at 32 (17-98) months were 100% and 95.8%. All children ultimately had complete resolution of HPS. CONCLUSIONS: VS HPS is associated with longer duration of mechanical ventilation and hospital stay, which emphasizes the need for early LT in these children.

The utility of ultrasound guidance in transjugular liver biopsy: our experience.

PURPOSE: To demonstrate the utility of ultrasound (US) guidance in improving the safety and efficacy of transjugular liver biopsy (TJLB) by analyzing all the TJLBs performed by us in the last 4 years. MATERIALS AND METHODS: Forty-seven patients who underwent TJLB in the last 4 years in our two centers were retrospectively analyzed. US guidance was used for all but for one patient during the two crucial steps of the procedure-for internal jugular vein (IJV) access and during the parenchymal biopsy. Biopsies were obtained under real-time sonographic guidance from the right lobe after confirming an adequate room for needle throw without breaching the liver capsule or any major hilar structures. Post-procedure complications, tissue yield, and histopathological adequacy of samples were analyzed. RESULTS: Overall technical success rate was 100%. No major complications were found where ultrasound-assisted TJLB (uTJLB) was performed. One patient who underwent non-US guided TJLB had an event of a capsular breach and intra-peritoneal hemorrhage requiring coil embolization. Samples were of adequate dimension. Histopathological positivity was 100%. The overall complication rate for uTJLB was 4.7% which was due to minor complications in two patients. CONCLUSIONS: Addition of US unit to the angiographic suite is effortless and Interventional Radiologists being already skilled in US can easily implement this simple yet valuable modification to conventional TJLB procedures. Our experience on uTJLB further emphasizes the role of US guidance in improving the procedural success rate, safety profile, and efficacy in the histopathological outcome of TJLB in all patients irrespective of age and disease burden.