Severity of Ascites Is Associated with Increased Mortality in Patients with Cirrhosis Secondary to Biliary Atresia

Abstract

Background Very few prior studies have investigated the presence of ascites as a prognostic factor in children with cirrhosis. To the best of our knowledge, there are no prior studies evaluating the relationship between severity of ascites and patient survival in children with biliary atresia and cirrhosis. Aims To evaluate the association between severity of ascites and survival of children with cirrhosis and biliary atresia. Methods All children with cirrhosis secondary to biliary atresia evaluated at our institution from 2000 to 2014 were included in this study. Patients were classified into four groups: NA = no ascites; A1 = grade 1 ascites; A2 = grade 2 ascites; and A3 = grade 3 ascites. The primary endpoint of the study was mortality within the first year after patient inclusion. Ninetyday mortality was also evaluated. Prognostic factors related to both endpoints also were studied. Results One-year patient survival for NA was 97.1%, versus 80.8% for A1, versus 52% for A2, versus 13.6 for A3 (p < 0.001). The presence of ascites increased mortality by 17 times. In the multivariate analysis, clinically detectable ascites (HR 3.14,

Beyond Varices: Complications of Cirrhotic Portal Hypertension in Pediatrics

“…• Sodium restriction to 1-2 g/d to avoid excess salt intake to achieve negative sodium balance and fluid loss 5,7 • No specific recommendations for medical management for AKI-HRS • Single-center studies found use of terlipressin (as available) as continuous infusion with albumin showed improvement or stabilization in renal function 87,88 • Prioritization for urgent liver transplantation presence of ascites at the time of transplant listing is associated with enhanced waitlist mortality. 49,50,52 Children with BA and grade 3 ascites had 1-year survival of only 13.6% (HR: 17.3, p < 0.01) as compared with 97.1% in children without any ascites (p < 0.001). 50 This is even higher than previously reported HR of 2.59 (95% CI: 1.44-4.64, p ¼ 0.001) of ascites leading to increased mortality risk at 90 days after LT listing.…”

“…49,50,52 Children with BA and grade 3 ascites had 1-year survival of only 13.6% (HR: 17.3, p < 0.01) as compared with 97.1% in children without any ascites (p < 0.001). 50 This is even higher than previously reported HR of 2.59 (95% CI: 1.44-4.64, p ¼ 0.001) of ascites leading to increased mortality risk at 90 days after LT listing. 49 These findings support timely LT referral and evaluation in children who develop ascites.…”

“…8,9,11 Thus, variceal hemorrhage in certain patients may not necessitate urgent LT. 8,9 The presence of chronic and significant ascites is associated with decreased 1-year survival in children and thus becomes a strong indication for LT, especially when there is associated significant hyponatremia. 49,50 Progression to AKI-HRS in the setting of ascites, given its reversibility with LT, necessitates prioritization for urgent LT. 147 Finally, as SBP has been associated with high mortality in children and poor survival with native-liver, proceeding with LT in any child after a first episode of SBP is warranted. 63,67 As there are limited data in children on the role of LT in HPS, POPH, CCM, and HE, appropriate timing of LT is complicated.…”

HEP-Net opinion on the management of ascites and its complications in the setting of decompensated cirrhosis in the resource constrained environment of Pakistan

“…It is a poor prognostic indicator, with only 50% surviving beyond two years. 2 , 3 Mortality worsens significantly to 20% to 50% at one year if the ascites becomes refractory to medical therapy. 4 Pakistan has one of the highest prevalence of viral hepatitis in the world and patients with ascites secondary to liver cirrhosis make a major percentage of both inpatient and outpatient burden.…”

Cardiac and renal effects of liver cirrhosis in a growing animal model

“…Liver cirrhosis results from progressive replacement of normal parenchyma by fibrosis, nodule formation and the development of signs and symptoms of portal hypertension and organ failure. In children, the leading cause of cirrhosis requiring liver transplantation is biliary atresia 1 , 2 . Cirrhosis may also lead to manifestations in other organs, like heart 3 , lungs 4 , brain 5 , and kidneys 6 .…”