2016, Number 1
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Ann Hepatol 2016; 15 (1)
Intrapulmonary vascular dilation in children with chronic liver diseases: pre- and post-liver transplantation
Khositseth A, Treepongkaruna S, Khemakanok K, Teeraratkul S, Pansrimangkorn V, Thirapattaraphan C, Leelaudomlipi S
Language: English
References: 27
Page: 47-52
PDF size: 196.32 Kb.
ABSTRACT
Background and study aims. Chronic liver disease (CLD) can cause hepatopulmonary syndrome (HPS), defined as triad of liver
disease, hypoxemia, and intrapulmonary vascular dilation (IPVD). The aim of this study was to determine the evidence of IPVD in
a cohort of pediatric patients with CLD pre- and post-liver transplantation (LT).
Material and methods. All pediatric patients with
CLD listed for LT were studied. Pulse oxygen saturation (SpO2), technetium-99m–labeled macroaggregated albumin (99mTc- MAA)
perfusion scan (positive test: uptake of the ≥ 6% in the brain), and echocardiography with saline bubble test (SBT) were performed.
SBT was re-evaluated at 3-6 months after LT. Grading of SBT included grade 0 (no bubble), I (1-9 bubbles), grade II (10-20
bubbles), and grade III (› 20 bubbles).
Results. Eighteen patients, median age 22.5 months (8-108), were enrolled. Most had biliary
atresia (77.8%). Pre-LT, all patients had SpO2 of 100% and none had positive 99mTc- MAA perfusion scan. Two patients (11%)
had negative SBT (grade 0), 1 (5.5%) had grade I, 3 (16.5%) had grade II, and 12 (67%) had grade III, respectively. Post-LT SBT
became negative in all survivors (n = 16), (p = 0.0001).
Conclusions. Most cirrhotic children in this cohort study had evidence of
IPVD by positive SBT. However, none of these met the criteria for diagnosis of HPS. This evidence of IPVD subsided after LT.
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