Childhood cirrhosis, hepatopulmonary syndrome and liver transplantation

Abstract

HPS is a serious and important complication of cirrhotic children that leads to tissue hypoxia and central cyanosis. HPS seems reversible after liver transplantation in all patients.

Pulmonary Evaluation in Pediatric Liver Transplant Candidates

“…The prevalence of HPS in the present patients was 11.4% which is in the range of prevalence in adult patients. It is also nearly similar to the prevalence of HPS in a study by Shneider et al performed in Mount Sinai School of Medicine [18], but less than study by Tumgor et al performed in Turkey [19]. However, the prevalence of HPS was higher in our patients in comparison to the data from an American study conducted by Gupta et al which reported estimated prevalence of HPS to be 3.3% among pediatric patients who are candidates for LT [20].…”

Hepatopulmonary Syndrome in Children with Cirrhotic and Non-Cirrhotic Portal Hypertension: A Single-Center Experience

“…The reported prevalence of HPS in adult cirrhotic patients is between 4 and 19% [14,15]. The reported prevalence of HPS in children with Budd-Chiari syndrome, chronic liver disease, biliary atresia and polysplenia syndrome, and portal vein obstruction is 28,[8][9][10][11][12][13]20, and 0.5%, respectively, and 17-19% in liver transplant recipients [3,5,[16][17][18][19]. The differing prevalence of HPS is primarily due to the heterogeneity of the disease groups and the applied criteria for HPS [14][15][16][17][18].…”

“…According to two retrospective studies, the prevalence of HPS is 9-20% for children with biliary atresia and 0.5% for children with extrahepatic portal hypertension [3,4]. Tumgor et al found that the prevalence of HPS in children with cirrhosis was 19% [5]. There are few clinical studies of HPS in children with portal hypertension.…”

Comparison of Two Diagnostic Criteria for Hepatopulmonary Syndrome–High Prevalence in Biliary Atresia

“…This is the first study to identify the better diagnostic criteria for detecting early HPS in 8 children using the age‐appropriate upper limit of normal value for P (A‐a) O 2 , as used in criteria 2. In the previous studies, the HPS prevalence in children with cirrhosis has varied from 3% to 40% (3–10) with prevalence being lower (3.1%) in a study where pulse oximetry was used to screen for HPS (10). Probably underestimation, tool of screening used in the diagnosis of HPS, etiology, and severity of portal hypertension along with a referral bias to a transplant center could be responsible for the differences in prevalence in various studies.…”